Paul Wales

693 timestamped statements across 7 collections — auto-found in recorded discussions, each timestamp jumps to the exact moment.

Abdominal Wall Defects · guest expert Colorectal / ARM & Hirschsprung · guest expert Etiologies (Gastroschisis/NEC/Atresia/Volvulus) · guest expert Intestinal Failure · guest expert Intestinal Rehab · guest expert Motility / Pseudo-obstruction · guest expert Short Bowel Syndrome · guest expert

Featured diaries

Ep 22 · 8:40
One thing I just want to say about oral feeding is that even in the scenario where it's really non-nutritive. I think it's important for people to sort of look at that oral feeding, not so much as an internal nutrition perspective, but more of a skill development perspective that they never learn how to eat by mouth and suck and swallow and process, then they won't eat solids later when you want them to, and you're stuck basically with tube feeding on an older child.
Ep 79 · 8:40
One thing I just want to say about oral feeding is that even in the scenario where it's really non-nutritive. I think it's important for people to sort of look at that oral feeding, not so much as an internal nutrition perspective, but more of a skill development perspective that they never learn how to eat by mouth and suck and swallow and process, then they won't eat solids later when you want them to, and you're stuck basically with tube feeding on an older child.
Ep 23 · 3:03
an intestinal rehabilitation program, as we defined also in these Aspen guidelines that just came out, is a multidisciplinary or interdisciplinary collaborative patient care paradigm that that brings coordinated care for children with intestinal failure through comprehensive management of Their specialized nutrition and other associated needs that they have.
Ep 112 · 3:03
an intestinal rehabilitation program, as we defined also in these Aspen guidelines that just came out, is a multidisciplinary or interdisciplinary collaborative patient care paradigm that that brings coordinated care for children with intestinal failure through comprehensive management of Their specialized nutrition and other associated needs that they have.
Ep 34 · 3:03
an intestinal rehabilitation program, as we defined also in these Aspen guidelines that just came out, is a multidisciplinary or interdisciplinary collaborative patient care paradigm that that brings coordinated care for children with intestinal failure through comprehensive management of Their specialized nutrition and other associated needs that they have.
Ep 8 · 3:03
an intestinal rehabilitation program, as we defined also in these Aspen guidelines that just came out, is a multidisciplinary or interdisciplinary collaborative patient care paradigm that that brings coordinated care for children with intestinal failure through comprehensive management of Their specialized nutrition and other associated needs that they have.

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Intestinal rehabilitation: What is intestinal rehab? - Episode 1

Ep 23 · 1:46
quote let's go back one step before that and sort of define what is intestinal failure because that sort of leads into who's going to benefit from an intestinal rehab program.
Ep 23 · 2:03
quote It it's, it's really a functional problem. Essentially, when someone's gut for whatever the diagnosis, whatever the reason, their gut function is insufficient to absorb enough nutrients, fluids, calories to support survival and in the in the case of children, growth, which is a big distinction between adults and children.
Ep 23 · 2:05
clinical Intestinal failure is defined as insufficient gut function to absorb enough nutrients, fluids, and calories to support survival and, in children, growth
Ep 23 · 3:03
clinical An intestinal rehabilitation program is a multidisciplinary collaborative patient care paradigm providing coordinated care for children with intestinal failure through comprehensive management of specialized nutrition and associated needs
Ep 23 · 3:03
quote an intestinal rehabilitation program, as we defined also in these Aspen guidelines that just came out, is a multidisciplinary or interdisciplinary collaborative patient care paradigm that that brings coordinated care for children with intestinal failure through comprehensive management of Their specialized nutrition and other associated needs that they have.
Ep 23 · 3:44
quote It's an amalgamation of experts that come from different aspects of care, providing a holistic, comprehensive, coordinated approach to patient management.
Ep 23 · 4:39
quote Patients that are gonna come to intestinal rehab program have intestinal failure, and we can divide the causes of intestinal failure into three categories.
Ep 23 · 4:50
epidemiological Short bowel syndrome is by far the most common category of intestinal failure in pediatric patients
Ep 23 · 4:50
quote There's short bowel syndrome, which by far is the most common category in pediatric patients with intestinal failure
Ep 23 · 5:06
clinical Causes of short bowel syndrome include congenital anomalies such as intestinal atresia, malrotation, volvulus, gastroschisis, and long segment Hirschsprung's disease
Ep 23 · 5:18
clinical Acquired diseases of the newborn such as necrotizing enterocolitis cause short bowel syndrome
Ep 23 · 5:45
clinical Motility disorders occur when abnormalities of the intestinal muscle itself or the nerves that control that muscle prevent coordinated movement of food and stool
Ep 23 · 5:45
quote So if we think of the intestine like a pipe, and that pipe is made of muscle that contracts in a coordinated way to push food and stool from one end to the other, you can have abnormalities of the muscle itself, or of the nerves that control that muscle.
Ep 23 · 6:10
clinical Children with motility disorders are dependent on intravenous support
Ep 23 · 6:17
clinical Enteropathies or congenital diarrheas are conditions where the patient has all of their bowel but the mucosal lining that digests and absorbs does not work
Ep 23 · 6:17
quote these are conditions where the patient has all of their bowel, but the mucosa, the inside lining that, that absorbs, uh, digests and absorbs doesn't work.
Ep 23 · 7:11
quote The reason it's important to at least identify these three categories is that some patients will have elements of 12, or 3 of the categories, the way they present.
Ep 23 · 7:11
clinical Some patients will have elements of one, two, or all three categories of intestinal failure
Ep 23 · 7:45
epidemiological Most intestinal failure patients are infants or babies, but some older kids develop intestinal failure
Ep 23 · 7:56
clinical Inflammatory bowel disease or Crohn's disease can lead to gut loss and intestinal failure in older patients
Ep 23 · 8:04
epidemiological Trauma, malignancy, and vascular thrombosis leading to gut loss are diagnoses seen more in older patients or adult series and less in pediatrics
Ep 23 · 12:46
quote But now what we're seeing is we have kids that have developed chronic comorbidities, that comorbidities that never, we didn't really see to the same extent because they didn't live long enough.
Ep 23 · 12:46
clinical Long-term survivors now develop chronic comorbidities including renal dysfunction, metabolic bone disease, neurocognitive issues, and quality of life issues

Intestinal Rehabilitation, Episode 1: What is intestinal rehabilitation?

Ep 24 · 1:46
quote let's go back one step before that and sort of define what is intestinal failure because that sort of leads into who's going to benefit from an intestinal rehab program
Ep 24 · 2:05
clinical Intestinal failure is defined as insufficient gut function to absorb enough nutrients, fluids, and calories to support survival and, in children, growth
Ep 24 · 3:03
clinical An intestinal rehabilitation program is a multidisciplinary collaborative patient care paradigm that brings coordinated care for children with intestinal failure through comprehensive management of their specialized nutrition and associated needs
Ep 24 · 3:44
quote It's an amalgamation of experts that come from different aspects of care, providing a holistic, comprehensive, coordinated approach to patient management
Ep 24 · 3:55
opinion Intestinal rehabilitation streamlines care and improves communication with families and between care providers and team members
Ep 24 · 4:50
epidemiological Short bowel syndrome is by far the most common category of intestinal failure in pediatric patients
Ep 24 · 5:06
clinical Causes of short bowel syndrome include congenital anomalies such as intestinal atresia, malrotation, volvulus, gastroschisis, and long segment Hirschsprung's disease
Ep 24 · 5:18
clinical Acquired diseases of the newborn such as necrotizing enterocolitis can cause short bowel syndrome
Ep 24 · 5:45
quote So if we think of the intestine like a pipe, and that pipe is made of muscle that contracts in a coordinated way to push food and stool from one end to the other, you can have abnormalities of the muscle itself, or of the nerves that control that muscle
Ep 24 · 5:45
clinical Motility disorders occur when the bowel is unable to push contents through in a coordinated way due to abnormalities of the muscle itself or the nerves that control that muscle
Ep 24 · 6:17
clinical Congenital enteropathies are conditions where the patient has all of their bowel but the mucosa does not work, leading to hypersecretion and profuse fluid losses
Ep 24 · 7:11
clinical Some patients will have elements of two or three categories of intestinal failure in the way they present
Ep 24 · 7:56
clinical Older pediatric patients can develop intestinal failure from inflammatory bowel disease or Crohn's disease where they have lost gut as a result of complications
Ep 24 · 8:04
epidemiological Trauma, malignancy, and vascular thrombosis leading to gut loss are diagnoses seen more in older patients or adult series and less in pediatrics
Ep 24 · 12:46
clinical Children with intestinal failure who survive long-term now develop chronic comorbidities including renal dysfunction, metabolic bone disease, neurocognitive issues, and quality of life issues

Intestinal rehabilitation: What is intestinal rehab? - Episode 1

Ep 112 · 1:46
quote let's go back one step before that and sort of define what is intestinal failure because that sort of leads into who's going to benefit from an intestinal rehab program.
Ep 112 · 2:03
quote It it's, it's really a functional problem. Essentially, when someone's gut for whatever the diagnosis, whatever the reason, their gut function is insufficient to absorb enough nutrients, fluids, calories to support survival and in the in the case of children, growth, which is a big distinction between adults and children.
Ep 112 · 2:05
clinical Intestinal failure is defined as insufficient gut function to absorb enough nutrients, fluids, and calories to support survival and, in children, growth
Ep 112 · 3:03
clinical An intestinal rehabilitation program is a multidisciplinary collaborative patient care paradigm providing coordinated care for children with intestinal failure through comprehensive management of specialized nutrition and associated needs
Ep 112 · 3:03
quote an intestinal rehabilitation program, as we defined also in these Aspen guidelines that just came out, is a multidisciplinary or interdisciplinary collaborative patient care paradigm that that brings coordinated care for children with intestinal failure through comprehensive management of Their specialized nutrition and other associated needs that they have.
Ep 112 · 3:44
quote It's an amalgamation of experts that come from different aspects of care, providing a holistic, comprehensive, coordinated approach to patient management.
Ep 112 · 4:39
quote Patients that are gonna come to intestinal rehab program have intestinal failure, and we can divide the causes of intestinal failure into three categories.
Ep 112 · 4:50
quote There's short bowel syndrome, which by far is the most common category in pediatric patients with intestinal failure
Ep 112 · 4:50
epidemiological Short bowel syndrome is by far the most common category of intestinal failure in pediatric patients
Ep 112 · 5:06
clinical Causes of short bowel syndrome include congenital anomalies such as intestinal atresia, malrotation, volvulus, gastroschisis, and long segment Hirschsprung's disease
Ep 112 · 5:18
clinical Acquired diseases of the newborn such as necrotizing enterocolitis cause short bowel syndrome
Ep 112 · 5:45
clinical Motility disorders occur when abnormalities of the intestinal muscle itself or the nerves that control that muscle prevent coordinated movement of food and stool
Ep 112 · 5:45
quote So if we think of the intestine like a pipe, and that pipe is made of muscle that contracts in a coordinated way to push food and stool from one end to the other, you can have abnormalities of the muscle itself, or of the nerves that control that muscle.
Ep 112 · 6:10
clinical Children with motility disorders are dependent on intravenous support
Ep 112 · 6:17
clinical Enteropathies or congenital diarrheas are conditions where the patient has all of their bowel but the mucosal lining that digests and absorbs does not work
Ep 112 · 6:17
quote these are conditions where the patient has all of their bowel, but the mucosa, the inside lining that, that absorbs, uh, digests and absorbs doesn't work.
Ep 112 · 7:11
quote The reason it's important to at least identify these three categories is that some patients will have elements of 12, or 3 of the categories, the way they present.
Ep 112 · 7:11
clinical Some patients will have elements of one, two, or all three categories of intestinal failure
Ep 112 · 7:45
epidemiological Most intestinal failure patients are infants or babies, but some older kids develop intestinal failure
Ep 112 · 7:56
clinical Inflammatory bowel disease or Crohn's disease can lead to gut loss and intestinal failure in older patients
Ep 112 · 8:04
epidemiological Trauma, malignancy, and vascular thrombosis leading to gut loss are diagnoses seen more in older patients or adult series and less in pediatrics
Ep 112 · 12:46
clinical Long-term survivors now develop chronic comorbidities including renal dysfunction, metabolic bone disease, neurocognitive issues, and quality of life issues
Ep 112 · 12:46
quote But now what we're seeing is we have kids that have developed chronic comorbidities, that comorbidities that never, we didn't really see to the same extent because they didn't live long enough.

Intestinal Rehabilitation, Episode 1: What is intestinal rehabilitation?

Ep 113 · 1:46
quote let's go back one step before that and sort of define what is intestinal failure because that sort of leads into who's going to benefit from an intestinal rehab program
Ep 113 · 2:05
clinical Intestinal failure is defined as insufficient gut function to absorb enough nutrients, fluids, and calories to support survival and, in children, growth
Ep 113 · 3:03
clinical An intestinal rehabilitation program is a multidisciplinary collaborative patient care paradigm that brings coordinated care for children with intestinal failure through comprehensive management of their specialized nutrition and associated needs
Ep 113 · 3:44
quote It's an amalgamation of experts that come from different aspects of care, providing a holistic, comprehensive, coordinated approach to patient management
Ep 113 · 3:55
opinion Intestinal rehabilitation streamlines care and improves communication with families and between care providers and team members
Ep 113 · 4:50
epidemiological Short bowel syndrome is by far the most common category of intestinal failure in pediatric patients
Ep 113 · 5:06
clinical Causes of short bowel syndrome include congenital anomalies such as intestinal atresia, malrotation, volvulus, gastroschisis, and long segment Hirschsprung's disease
Ep 113 · 5:18
clinical Acquired diseases of the newborn such as necrotizing enterocolitis can cause short bowel syndrome
Ep 113 · 5:45
clinical Motility disorders occur when the bowel is unable to push contents through in a coordinated way due to abnormalities of the muscle itself or the nerves that control that muscle
Ep 113 · 5:45
quote So if we think of the intestine like a pipe, and that pipe is made of muscle that contracts in a coordinated way to push food and stool from one end to the other, you can have abnormalities of the muscle itself, or of the nerves that control that muscle
Ep 113 · 6:17
clinical Congenital enteropathies are conditions where the patient has all of their bowel but the mucosa does not work, leading to hypersecretion and profuse fluid losses
Ep 113 · 7:11
clinical Some patients will have elements of two or three categories of intestinal failure in the way they present
Ep 113 · 7:56
clinical Older pediatric patients can develop intestinal failure from inflammatory bowel disease or Crohn's disease where they have lost gut as a result of complications
Ep 113 · 8:04
epidemiological Trauma, malignancy, and vascular thrombosis leading to gut loss are diagnoses seen more in older patients or adult series and less in pediatrics
Ep 113 · 12:46
clinical Children with intestinal failure who survive long-term now develop chronic comorbidities including renal dysfunction, metabolic bone disease, neurocognitive issues, and quality of life issues

Intestinal rehabilitation: What is intestinal rehab? - Episode 1

Ep 34 · 1:46
quote let's go back one step before that and sort of define what is intestinal failure because that sort of leads into who's going to benefit from an intestinal rehab program.
Ep 34 · 2:03
quote It it's, it's really a functional problem. Essentially, when someone's gut for whatever the diagnosis, whatever the reason, their gut function is insufficient to absorb enough nutrients, fluids, calories to support survival and in the in the case of children, growth, which is a big distinction between adults and children.
Ep 34 · 2:05
clinical Intestinal failure is defined as insufficient gut function to absorb enough nutrients, fluids, and calories to support survival and, in children, growth
Ep 34 · 3:03
clinical An intestinal rehabilitation program is a multidisciplinary collaborative patient care paradigm providing coordinated care for children with intestinal failure through comprehensive management of specialized nutrition and associated needs
Ep 34 · 3:03
quote an intestinal rehabilitation program, as we defined also in these Aspen guidelines that just came out, is a multidisciplinary or interdisciplinary collaborative patient care paradigm that that brings coordinated care for children with intestinal failure through comprehensive management of Their specialized nutrition and other associated needs that they have.
Ep 34 · 3:44
quote It's an amalgamation of experts that come from different aspects of care, providing a holistic, comprehensive, coordinated approach to patient management.
Ep 34 · 4:39
quote Patients that are gonna come to intestinal rehab program have intestinal failure, and we can divide the causes of intestinal failure into three categories.
Ep 34 · 4:50
quote There's short bowel syndrome, which by far is the most common category in pediatric patients with intestinal failure
Ep 34 · 4:50
epidemiological Short bowel syndrome is by far the most common category of intestinal failure in pediatric patients
Ep 34 · 5:06
clinical Causes of short bowel syndrome include congenital anomalies such as intestinal atresia, malrotation, volvulus, gastroschisis, and long segment Hirschsprung's disease
Ep 34 · 5:18
clinical Acquired diseases of the newborn such as necrotizing enterocolitis cause short bowel syndrome
Ep 34 · 5:45
clinical Motility disorders occur when abnormalities of the intestinal muscle itself or the nerves that control that muscle prevent coordinated movement of food and stool
Ep 34 · 5:45
quote So if we think of the intestine like a pipe, and that pipe is made of muscle that contracts in a coordinated way to push food and stool from one end to the other, you can have abnormalities of the muscle itself, or of the nerves that control that muscle.
Ep 34 · 6:10
clinical Children with motility disorders are dependent on intravenous support
Ep 34 · 6:17
clinical Enteropathies or congenital diarrheas are conditions where the patient has all of their bowel but the mucosal lining that digests and absorbs does not work
Ep 34 · 6:17
quote these are conditions where the patient has all of their bowel, but the mucosa, the inside lining that, that absorbs, uh, digests and absorbs doesn't work.
Ep 34 · 7:11
quote The reason it's important to at least identify these three categories is that some patients will have elements of 12, or 3 of the categories, the way they present.
Ep 34 · 7:11
clinical Some patients will have elements of one, two, or all three categories of intestinal failure
Ep 34 · 7:45
epidemiological Most intestinal failure patients are infants or babies, but some older kids develop intestinal failure
Ep 34 · 7:56
clinical Inflammatory bowel disease or Crohn's disease can lead to gut loss and intestinal failure in older patients
Ep 34 · 8:04
epidemiological Trauma, malignancy, and vascular thrombosis leading to gut loss are diagnoses seen more in older patients or adult series and less in pediatrics
Ep 34 · 12:46
clinical Long-term survivors now develop chronic comorbidities including renal dysfunction, metabolic bone disease, neurocognitive issues, and quality of life issues
Ep 34 · 12:46
quote But now what we're seeing is we have kids that have developed chronic comorbidities, that comorbidities that never, we didn't really see to the same extent because they didn't live long enough.

Intestinal Rehabilitation, Episode 1: What is intestinal rehabilitation?

Ep 35 · 1:46
quote let's go back one step before that and sort of define what is intestinal failure because that sort of leads into who's going to benefit from an intestinal rehab program
Ep 35 · 2:05
clinical Intestinal failure is defined as insufficient gut function to absorb enough nutrients, fluids, and calories to support survival and, in children, growth
Ep 35 · 3:03
clinical An intestinal rehabilitation program is a multidisciplinary collaborative patient care paradigm that brings coordinated care for children with intestinal failure through comprehensive management of their specialized nutrition and associated needs
Ep 35 · 3:44
quote It's an amalgamation of experts that come from different aspects of care, providing a holistic, comprehensive, coordinated approach to patient management
Ep 35 · 3:55
opinion Intestinal rehabilitation streamlines care and improves communication with families and between care providers and team members
Ep 35 · 4:50
epidemiological Short bowel syndrome is by far the most common category of intestinal failure in pediatric patients
Ep 35 · 5:06
clinical Causes of short bowel syndrome include congenital anomalies such as intestinal atresia, malrotation, volvulus, gastroschisis, and long segment Hirschsprung's disease
Ep 35 · 5:18
clinical Acquired diseases of the newborn such as necrotizing enterocolitis can cause short bowel syndrome
Ep 35 · 5:45
quote So if we think of the intestine like a pipe, and that pipe is made of muscle that contracts in a coordinated way to push food and stool from one end to the other, you can have abnormalities of the muscle itself, or of the nerves that control that muscle
Ep 35 · 5:45
clinical Motility disorders occur when the bowel is unable to push contents through in a coordinated way due to abnormalities of the muscle itself or the nerves that control that muscle
Ep 35 · 6:17
clinical Congenital enteropathies are conditions where the patient has all of their bowel but the mucosa does not work, leading to hypersecretion and profuse fluid losses
Ep 35 · 7:11
clinical Some patients will have elements of two or three categories of intestinal failure in the way they present
Ep 35 · 7:56
clinical Older pediatric patients can develop intestinal failure from inflammatory bowel disease or Crohn's disease where they have lost gut as a result of complications
Ep 35 · 8:04
epidemiological Trauma, malignancy, and vascular thrombosis leading to gut loss are diagnoses seen more in older patients or adult series and less in pediatrics
Ep 35 · 12:46
clinical Children with intestinal failure who survive long-term now develop chronic comorbidities including renal dysfunction, metabolic bone disease, neurocognitive issues, and quality of life issues

Intestinal Rehabilitation, Episode 2: Overwhelming intestinal damage, Part 1

Ep 36 · 1:17
opinion The outlook has changed and what's gone hand in hand with that is a bit more of an aggressive approach to surgical resection
Ep 36 · 1:39
clinical Comorbidities such as neurologic status, pulmonary function, cardiac function, or significant genetic or chromosomal anomaly factor into decision making about how to proceed
Ep 36 · 2:38
guideline Intestinal transplant is not experimental therapy but part of the continuum of therapy for a child with short bowel syndrome
Ep 36 · 2:52
quote Is it ethically appropriate to open and close a baby when you know that you could remove all of their intestines?
Ep 36 · 4:31
clinical In the very acute time after bowel infarction, the risk is dying of sepsis and multi-organ failure
Ep 36 · 4:44
clinical Intermediate and longer term complications include intestinal failure associated liver disease, recurrent sepsis, or line problems
Ep 36 · 5:02
epidemiological Success in the last two decades is because we're way better at preventing sepsis and liver disease
Ep 36 · 6:14
clinical Babies born with duodenal atresia or very proximal jejunal atresia are born with elevated direct or conjugated bilirubins
Ep 36 · 6:42
clinical Factors affecting liver function include prematurity, sepsis, choice of TPN, and presence or absence of enteral nutrition
Ep 36 · 12:15
quote Let the baby decide, you know, some may continue to spiral because of just overwhelming sepsis and may not make it, but many, many will.
Intestinal Failure 236 entries

Intestinal rehabilitation: What is intestinal rehab? - Episode 1

Ep 8 · 1:46
quote let's go back one step before that and sort of define what is intestinal failure because that sort of leads into who's going to benefit from an intestinal rehab program.
Ep 8 · 2:03
quote It it's, it's really a functional problem. Essentially, when someone's gut for whatever the diagnosis, whatever the reason, their gut function is insufficient to absorb enough nutrients, fluids, calories to support survival and in the in the case of children, growth, which is a big distinction between adults and children.
Ep 8 · 2:05
clinical Intestinal failure is defined as insufficient gut function to absorb enough nutrients, fluids, and calories to support survival and, in children, growth
Ep 8 · 3:03
clinical An intestinal rehabilitation program is a multidisciplinary collaborative patient care paradigm providing coordinated care for children with intestinal failure through comprehensive management of specialized nutrition and associated needs
Ep 8 · 3:03
quote an intestinal rehabilitation program, as we defined also in these Aspen guidelines that just came out, is a multidisciplinary or interdisciplinary collaborative patient care paradigm that that brings coordinated care for children with intestinal failure through comprehensive management of Their specialized nutrition and other associated needs that they have.
Ep 8 · 3:44
quote It's an amalgamation of experts that come from different aspects of care, providing a holistic, comprehensive, coordinated approach to patient management.
Ep 8 · 4:39
quote Patients that are gonna come to intestinal rehab program have intestinal failure, and we can divide the causes of intestinal failure into three categories.
Ep 8 · 4:50
quote There's short bowel syndrome, which by far is the most common category in pediatric patients with intestinal failure
Ep 8 · 4:50
epidemiological Short bowel syndrome is by far the most common category of intestinal failure in pediatric patients
Ep 8 · 5:06
clinical Causes of short bowel syndrome include congenital anomalies such as intestinal atresia, malrotation, volvulus, gastroschisis, and long segment Hirschsprung's disease
Ep 8 · 5:18
clinical Acquired diseases of the newborn such as necrotizing enterocolitis cause short bowel syndrome
Ep 8 · 5:45
clinical Motility disorders occur when abnormalities of the intestinal muscle itself or the nerves that control that muscle prevent coordinated movement of food and stool
Ep 8 · 5:45
quote So if we think of the intestine like a pipe, and that pipe is made of muscle that contracts in a coordinated way to push food and stool from one end to the other, you can have abnormalities of the muscle itself, or of the nerves that control that muscle.
Ep 8 · 6:10
clinical Children with motility disorders are dependent on intravenous support
Ep 8 · 6:17
clinical Enteropathies or congenital diarrheas are conditions where the patient has all of their bowel but the mucosal lining that digests and absorbs does not work
Ep 8 · 6:17
quote these are conditions where the patient has all of their bowel, but the mucosa, the inside lining that, that absorbs, uh, digests and absorbs doesn't work.
Ep 8 · 7:11
quote The reason it's important to at least identify these three categories is that some patients will have elements of 12, or 3 of the categories, the way they present.
Ep 8 · 7:11
clinical Some patients will have elements of one, two, or all three categories of intestinal failure
Ep 8 · 7:45
epidemiological Most intestinal failure patients are infants or babies, but some older kids develop intestinal failure
Ep 8 · 7:56
clinical Inflammatory bowel disease or Crohn's disease can lead to gut loss and intestinal failure in older patients
Ep 8 · 8:04
epidemiological Trauma, malignancy, and vascular thrombosis leading to gut loss are diagnoses seen more in older patients or adult series and less in pediatrics
Ep 8 · 12:46
clinical Long-term survivors now develop chronic comorbidities including renal dysfunction, metabolic bone disease, neurocognitive issues, and quality of life issues
Ep 8 · 12:46
quote But now what we're seeing is we have kids that have developed chronic comorbidities, that comorbidities that never, we didn't really see to the same extent because they didn't live long enough.

Intestinal Rehabilitation, Episode 1: What is intestinal rehabilitation?

Ep 9 · 1:46
quote let's go back one step before that and sort of define what is intestinal failure because that sort of leads into who's going to benefit from an intestinal rehab program
Ep 9 · 2:05
clinical Intestinal failure is defined as insufficient gut function to absorb enough nutrients, fluids, and calories to support survival and, in children, growth
Ep 9 · 3:03
clinical An intestinal rehabilitation program is a multidisciplinary collaborative patient care paradigm that brings coordinated care for children with intestinal failure through comprehensive management of their specialized nutrition and associated needs
Ep 9 · 3:44
quote It's an amalgamation of experts that come from different aspects of care, providing a holistic, comprehensive, coordinated approach to patient management
Ep 9 · 3:55
opinion Intestinal rehabilitation streamlines care and improves communication with families and between care providers and team members
Ep 9 · 4:50
epidemiological Short bowel syndrome is by far the most common category of intestinal failure in pediatric patients
Ep 9 · 5:06
clinical Causes of short bowel syndrome include congenital anomalies such as intestinal atresia, malrotation, volvulus, gastroschisis, and long segment Hirschsprung's disease
Ep 9 · 5:18
clinical Acquired diseases of the newborn such as necrotizing enterocolitis can cause short bowel syndrome
Ep 9 · 5:45
clinical Motility disorders occur when the bowel is unable to push contents through in a coordinated way due to abnormalities of the muscle itself or the nerves that control that muscle
Ep 9 · 5:45
quote So if we think of the intestine like a pipe, and that pipe is made of muscle that contracts in a coordinated way to push food and stool from one end to the other, you can have abnormalities of the muscle itself, or of the nerves that control that muscle
Ep 9 · 6:17
clinical Congenital enteropathies are conditions where the patient has all of their bowel but the mucosa does not work, leading to hypersecretion and profuse fluid losses
Ep 9 · 7:11
clinical Some patients will have elements of two or three categories of intestinal failure in the way they present
Ep 9 · 7:56
clinical Older pediatric patients can develop intestinal failure from inflammatory bowel disease or Crohn's disease where they have lost gut as a result of complications
Ep 9 · 8:04
epidemiological Trauma, malignancy, and vascular thrombosis leading to gut loss are diagnoses seen more in older patients or adult series and less in pediatrics
Ep 9 · 12:46
clinical Children with intestinal failure who survive long-term now develop chronic comorbidities including renal dysfunction, metabolic bone disease, neurocognitive issues, and quality of life issues

Intestinal Rehabilitation, Episode 2: Overwhelming intestinal damage, Part 1

Ep 10 · 1:17
opinion The outlook has changed and what's gone hand in hand with that is a bit more of an aggressive approach to surgical resection
Ep 10 · 1:39
clinical Comorbidities such as neurologic status, pulmonary function, cardiac function, or significant genetic or chromosomal anomaly factor into decision making about how to proceed
Ep 10 · 2:38
guideline Intestinal transplant is not experimental therapy but part of the continuum of therapy for a child with short bowel syndrome
Ep 10 · 2:52
quote Is it ethically appropriate to open and close a baby when you know that you could remove all of their intestines?
Ep 10 · 4:31
clinical In the very acute time after bowel infarction, the risk is dying of sepsis and multi-organ failure
Ep 10 · 4:44
clinical Intermediate and longer term complications include intestinal failure associated liver disease, recurrent sepsis, or line problems
Ep 10 · 5:02
epidemiological Success in the last two decades is because we're way better at preventing sepsis and liver disease
Ep 10 · 6:14
clinical Babies born with duodenal atresia or very proximal jejunal atresia are born with elevated direct or conjugated bilirubins
Ep 10 · 6:42
clinical Factors affecting liver function include prematurity, sepsis, choice of TPN, and presence or absence of enteral nutrition
Ep 10 · 12:15
quote Let the baby decide, you know, some may continue to spiral because of just overwhelming sepsis and may not make it, but many, many will.

Intestinal Rehabilitation, Episode 2: Overwhelming intestinal damage, Part 2

Ep 11 · 1:23
quote The goalposts have moved over the last two decades.
Ep 11 · 1:37
clinical In Toronto, ultra-short gut is classified as less than 20% of expected bowel length for age
Ep 11 · 1:42
clinical In a term baby, ultra-short gut corresponds to 20 to 30 centimeters of bowel
Ep 11 · 1:52
clinical Overall survival in ultra-short gut patients in the current era is over 90%
Ep 11 · 2:10
clinical Ultra-short gut patients who reached autonomy required multiple nutritional supplements
Ep 11 · 2:26
clinical Ultra-short gut patients with remnant ileum as part of residual bowel tended to do better
Ep 11 · 2:50
clinical Ultra-short gut patients with longer colonic remnants tended to adapt and get off TPN
Ep 11 · 6:10
quote I don't subscribe to the notion that Every kid needs to die with a laparotomy indecision.
Ep 11 · 7:40
opinion Lipid restriction was never a practice subscribed to by the Toronto program
Ep 11 · 11:53
quote The surgeon's role in the patient with short bowel syndrome is, is obviously significant. Partly for what we do and partly for what we don't do, right? Those intraoperative decisions have a lifelong impact.
Ep 11 · 12:00
opinion Intraoperative decisions in short bowel syndrome have a lifelong impact
Ep 11 · 12:08
opinion Decisions made at the time of intraabdominal catastrophe can make the difference between staying on or getting off TPN or whether the child survives
Ep 11 · 14:21
clinical Kids may look worse over the first 24-36 hours postoperatively, then stop getting worse before they start improving
Ep 11 · 14:21
quote Often, some of these kids do get, they kind of look worse over the next 24, 36 hours, and then they start to, you know, they may not even be getting better, they just stop getting worse.

Intestinal Rehabilitation, Episode 3: Enteral Autonomy, Part 1

Ep 12 · 2:02
quote That remaining bowel, that residual intestine goes through this process of adaptation and where the bowel is trying to compensate to reestablish function, to absorb enough nutrients and fluids to maintain survival.
Ep 12 · 2:16
clinical Adaptation is driven by intraluminal nutrients and their interaction with pancreatic biliary secretions and trophic gut peptides
Ep 12 · 2:42
clinical Structural changes during adaptation include mucosal hypertrophy with increased villous length, increased blood supply through angiogenesis, bowel dilation, and gut lengthening in younger children, all increasing surface area for absorption
Ep 12 · 3:04
clinical Functional changes during adaptation include slowed motility to allow more contact time and upregulation of enterocyte transporters
Ep 12 · 3:18
quote So, each, each cell is working harder, if you will, to try to, to, Function better and our job as a team is to try to promote that the best we can.
Ep 12 · 5:35
clinical Until recently there was no standardized definition for enteral autonomy
Ep 12 · 6:04
clinical TPN complications include line infections, liver disease, and vascular thrombosis
Ep 12 · 6:20
clinical In the past, patients were lost to complications such as liver disease before they could reach their adaptive potential; management of these complications has improved significantly
Ep 12 · 6:39
guideline Current ASPEN guidelines define enteral autonomy as independence of parenteral support for 12 weeks with maintenance of adequate growth and hydration
Ep 12 · 6:53
quote It's one thing to turn off parenteral support, but, you know, you're not really off TPN or off parental support unless you can actually grow off TPN.
Ep 12 · 7:50
epidemiological A 2012 PIFCO paper by Squires showed 50% of patients achieved enteral autonomy over 5-6 years, 25% died, and 25% got transplanted
Ep 12 · 8:25
epidemiological Recent papers in the last 5-6 years show 60-80% of patients achieve enteral autonomy, with a higher proportion surviving to reach autonomy
Ep 12 · 9:55
clinical Small bowel length is an independently significant variable for adaptation capacity
Ep 12 · 10:18
clinical The ileum has a much greater capacity to adapt than the jejunum
Ep 12 · 10:40
clinical At 5 years old, a child has about 425 centimeters of small bowel, with the steepest growth rate between 35 weeks gestation to about 6 months postnatal
Ep 12 · 11:25
opinion The ileocecal valve itself is not the important factor; most people who lose their ileocecal valve also lose their terminal ileum, which is the bigger factor for adaptive potential
Ep 12 · 11:53
clinical If a patient has the majority of their small bowel, it almost does not matter how much colon they have; probability of enteral autonomy is 85-100%

CAPS - The use of urine sodium to creatinine ratio as a marker of total body sodium in infants with intestinal failure - Sara Choi

Ep 14 · 5:58
quote I encourage the audience to, to highly consider voting for this paper because it's, it has a massive clinical importance.
Ep 14 · 6:05
quote This is often an underappreciated problem.
Ep 14 · 6:08
clinical Patients with stomas, even those without intestinal failure, run the risk of sodium depletion which has a significant impact on their growth
Ep 14 · 6:19
clinical Stomas and high stool losses are a large source of sodium bicarb as well as magnesium loss
Ep 14 · 6:38
clinical When we replace sodium, it does help restore growth, but it doesn't allow catch up growth
Ep 14 · 6:38
quote it's important to remember that when we replace sodium, it does help restore growth, but it doesn't allow catch up growth.
Ep 14 · 6:45
quote it's important that we track these patients serially, so that we can detect a trend evolving, so that we can avoid sodium depletion happening in the first place.
Ep 14 · 7:30
quote I would argue that, that having potassium chloride and an osmolaity added to the urine lytes in addition to just the sodium gives you a better picture of, of whether someone is truly sodium depleted.
Ep 14 · 7:41
clinical If potassium level is higher than sodium level in urine, that often will tell you that the aldosterone pathway is turned on

Pediatric Vascular Access in Brief: Preoperative, Operative, and Postoperative Considerations

Ep 15 · 10:35
clinical Antibiotic locking solutions have been around for a long time but predominantly develop resistance
Ep 15 · 10:47
quote And frankly, the problem with them is that you develop resistance.
Ep 15 · 10:56
clinical Ethanol locks are antimicrobial without resistance and kill both planktonic bacteria (floating in lumen) and sessile bacteria (embedded in biofilm)
Ep 15 · 10:56
quote It's antimicrobial, it doesn't have any resistance, and it'll kill bacteria that are both planktonic, meaning floating around in the, in the lumen, or sessile, meaning that they're embedded in a biofilm along the wall of the catheter.
Ep 15 · 11:24
clinical Kite lock is a 4% tetrasodium EDTA chemical found in Canada
Ep 15 · 11:36
clinical Kite lock is antimicrobial without resistance, antifibrinolytic, and antithrombotic
Ep 15 · 11:59
clinical Kite lock has been licensed in Canada for pediatric use, and is now licensed in Europe and Australia

Intestinal Rehabilitation, Episode 4: Surgical Management, Part 1

Ep 18 · 4:18
epidemiological The point of intake for the majority of short bowel syndrome patients is at birth because etiologies are usually neonatal causes
Ep 18 · 4:36
epidemiological Congenital anomalies of the GI tract or acquired conditions like necrotizing enterocolitis are the main causes
Ep 18 · 4:50
quote Making rational decisions at that initial surgery will set that baby up for success or failure.
Ep 18 · 5:42
clinical If reestablishing bowel continuity, you must deal with size discrepancy between the two ends
Ep 18 · 5:42
quote If you are thinking of reestablishing continuity, I feel very strongly that you have to deal with the size discrepancy.
Ep 18 · 7:08
quote In general, gastroschisis, they don't tend to do as well regardless.
Ep 18 · 7:08
clinical Gastroschisis patients don't tend to do as well regardless of intervention
Ep 18 · 7:12
clinical STEP procedures tend to not work as well in gastroschisis patients
Ep 18 · 9:09
clinical The STEP procedure requires bowel diameter of about 5 centimeters to be worthwhile
Ep 18 · 11:29
clinical There is a risk of bowel twisting in longitudinal orientation if alignment is not maintained during STEP
Ep 18 · 13:23
guideline Target caliber for STEP in a baby is about 1.5 centimeters, and for older infant or child is 2 to 2.5 centimeters
Ep 18 · 13:36
clinical Making the bowel too narrow during STEP can obstruct the patient, especially in someone with borderline motility
Ep 18 · 14:19
clinical A proposed advantage of STEP is that the surgeon can maintain caliber all the way from top to bottom by careful measurements
Ep 18 · 14:35
clinical There is potential for leak in the crotch of the staple line if not reinforced
Ep 18 · 15:08
clinical Dog ears at the top and bottom of the STEP segment can dilate over time and form blind loops
Ep 18 · 16:56
quote I don't step the duodenum. I start where the bowel starts to dilate.

Intestinal Rehabilitation, Episode 4: Surgical Management, Part 1

Ep 19 · 4:18
epidemiological The majority of pediatric short bowel syndrome cases present at birth due to neonatal causes: congenital GI anomalies or acquired conditions like necrotizing enterocolitis
Ep 19 · 5:42
quote If you are thinking of reestablishing continuity, I feel very strongly that you have to deal with the size discrepancy.
Ep 19 · 5:42
clinical When reestablishing bowel continuity, size discrepancy between bowel ends must be addressed or a functional obstruction will result even if the anastomosis is patent
Ep 19 · 6:02
clinical If adequate bowel length exists, size discrepancy can be managed by resection back to appropriate caliber or tapering enteroplasty
Ep 19 · 6:28
clinical In short bowel cases, serial transverse enteroplasty (STEP) can address size discrepancy without resecting mucosa
Ep 19 · 7:08
clinical Gastroschisis patients don't tend to do as well regardless of intervention; STEP procedures in gastroschisis patients consistently underperform
Ep 19 · 7:08
quote In general, gastroschisis, they don't tend to do as well regardless. If, and if I look at the step procedures that I've done over the years, the ones that tend to not do as well or tend to always be the gastroschisis.
Ep 19 · 9:09
clinical STEP procedure requires bowel diameter of at least 5 centimeters to be worthwhile
Ep 19 · 9:09
quote I don't usually do that operation anyway unless the bowel is an adequate diameter to make it worthwhile, and in my head over the years, it's always been about 5 centimeters anyway.
Ep 19 · 9:50
clinical In scenarios where immediate anastomosis is not prudent, distal bowel can be accessed with a feeding tube to provide distal feeding and grow the bowel in caliber before a subsequent operation
Ep 19 · 10:45
clinical The STEP procedure was originally described by HP Kim and Tom Jackson
Ep 19 · 11:29
clinical Maintaining bowel alignment during STEP is critical; there is risk of longitudinal twisting if alignment is not maintained
Ep 19 · 12:26
clinical An endo GIA stapler with vascular (white) load cartridge (2.5mm crimping to 1mm) is preferred over open GIA and prevents leaks better than the classic blue load
Ep 19 · 12:34
clinical STEP staple lines should be oriented perpendicular to the mesentery at 90 and 270 degrees (3 o'clock and 9 o'clock positions)
Ep 19 · 13:23
clinical Target bowel caliber after STEP is 1.5 cm in babies and 2 to 2.5 cm in older infants or children
Ep 19 · 13:36
clinical Making the bowel too narrow during STEP can cause obstruction, especially in patients with borderline motility
Ep 19 · 14:35
clinical A U-stitch with 4-0 PDS should be placed in the crotch of each staple line to prevent potential leaks
Ep 19 · 15:08
clinical Dog ears (blind loops) can form at the top and bottom of the STEP segment where bowel transitions in and out, and these can dilate over time
Ep 19 · 16:51
clinical The duodenum should not be stepped; STEP should start where the bowel begins to dilate, usually distal to the duodenum
Ep 19 · 16:56
quote I don't step the duodenum. I start where the bowel starts to dilate.
Ep 19 · 17:20
clinical If duodenal narrowing is needed, a stapler should be used on the lateral side away from the bile duct and ampulla; plication sutures usually fail

Intestinal Rehabilitation, Episode 4: Surgical Management, Part 2

Ep 20 · 1:52
quote I think we have to remember that the biggest benefit of the step, for instance, is the fact that you're tapering the bowel and reestablishing a more normal caliber to improve motility.
Ep 20 · 1:52
clinical The biggest benefit of the STEP procedure is tapering the bowel and reestablishing a more normal caliber to improve motility
Ep 20 · 3:04
clinical It can take up to 6 months before significant improvement in absorptive capacity is seen after STEP
Ep 20 · 3:04
quote It can take up to 6 months before you actually start to see a significant improvement in absorptive capacity.
Ep 20 · 3:12
clinical Absorptive capacity improvement is measured with fecal fat, alpha-1 antitrypsin clearance, xylose absorption, and citrulline levels
Ep 20 · 3:24
clinical The delay in improvement is due to inflamed, sick, leaky mucosa in the setting of bacterial overgrowth needing time to heal
Ep 20 · 6:24
clinical Staple-line ulcer bleeding is an underreported complication that is difficult to manage
Ep 20 · 6:49
quote My hypothesis is that this is a microbiome problem. I think you have a pro-inflammatory environment.
Ep 20 · 6:52
clinical Staple-line ulcers tend to occur in type 2 anatomy (small bowel to colonic remnant in the absence of an intact colon ileocecal valve)
Ep 20 · 7:12
clinical Pathology of staple-line ulcers shows non-specific inflammation with no vasculitis, viral elements, or obvious ischemia
Ep 20 · 7:12
quote It's non-specific inflammation. There's no vasculitis, there's no viral elements, there's no obvious ischemia, and the management can be super difficult.
Ep 20 · 10:05
clinical Most referrals for intestinal failure have had multiple operations before coming to a specialized center
Ep 20 · 10:09
opinion Surgical planning may not be conducive to just one operation and may require setting up for the next case

Intestinal Rehabilitation, Episode 6: Cholestasis

Ep 21 · 1:09
guideline Cholestasis is institutionally defined as conjugated bilirubin greater than 3 mg/dL or 50 micromoles/L sustained for 2 weeks and not associated with a septic event
Ep 21 · 1:09
quote Institutionally defined it as a conjugated bilirubin greater than 3 mg per deciliter or 50 micromoles per liter sustained for 2 weeks and not associated with a septic event.
Ep 21 · 1:20
guideline A 2021 JPN publication defines cholestasis as conjugated bilirubin around 2 mg/dL or 34 micromoles/L for 2 weeks, not associated with a septic event
Ep 21 · 2:57
clinical In young children, intestinal failure causes cholestatic liver disease, whereas in adolescents and adults it tends to cause steatosis (fatty deposition)
Ep 21 · 2:57
quote In the setting of young children, it's more of a cholestatic liver disease, whereas in older people, adolescents and adults, they tend to have Steatosis, there's a fatty deposition within the liver, and the term we use now is intestinal failure associated liver disease because that sort of indicates that it's a multifactorial process.
Ep 21 · 3:30
clinical Prematurity is not modifiable by the clinical team, but other risk factors (enteral feeding, sepsis, TPN components) are modifiable
Ep 21 · 3:30
quote Now, prematurity, we as clinicians, as a team, we don't have much control over that. But the other factors are modifiable, and we can have an impact on that.
Ep 21 · 4:09
clinical Prevention requires aggressive introduction of enteral feeding and surgical procedures to optimize anatomy for feed delivery
Ep 21 · 7:08
clinical Conventional intralipid (soybean-based) when metabolized leads to production of prostaglandins and eicosanoids that are pro-inflammatory
Ep 21 · 7:19
clinical SMOF lipid promotes bile flow, is hepatoprotective, and can be delivered at conventional dose while supporting somatic growth and neurologic development
Ep 21 · 7:45
clinical SMOF lipid does not have enough arachidonic acid, so dose restriction can lead to essential fatty acid deficiency
Ep 21 · 7:57
clinical When SMOF is delivered at conventional dosing, nobody develops essential fatty acid deficiency
Ep 21 · 8:06
guideline Recommended lipid dosing is settling around 2.5 g/kg, but nutrition guidelines for preterms and babies state 3-4 g/kg/day
Ep 21 · 8:29
opinion A bilirubin of 2 mg/dL is not advanced liver disease, is not dangerous, and is usually transient; approximately 90% of patients will improve with observation alone
Ep 21 · 8:52
quote My opinion is that a billy of 2 is not by any stretch advanced liver disease, and it's not dangerous, and it's usually transient. So I personally would not change the lipid emulsion out of Billy to.
Ep 21 · 12:11
clinical Liver biopsy is commonly performed during secondary surgical or autologous reconstruction procedures to provide an up-to-date microscopic snapshot
Ep 21 · 13:00
clinical Elastography or fibroscan is available for monitoring but is good for mild/no fibrosis or very advanced fibrosis; it is less sensitive for patients in the middle range
Ep 21 · 13:31
clinical Outpatient follow-up frequency for children on TPN at home ranges from every 1-4 months depending on patient stability

Intestinal Rehabilitation, Episode 7: Refeeding in a neonatal patient

Ep 22 · 0:57
clinical Refeeding goals include establishing normal feeding habits to promote gut function and optimize quality of life and social aspects of eating
Ep 22 · 0:57
quote Even if a kid has a complicated disease process, one of our goals as a team is to try to establish the most normal feeding habits we can that not only promote gut function and everything else, but also to optimize quality of life and the importance of the social aspects of eating that we all experience with our families and friends.
Ep 22 · 4:34
clinical In short bowel syndrome, protein absorption is fairly well preserved, so the benefit of hydrolyzed or amino acid formulas is primarily from an allergy perspective
Ep 22 · 5:04
clinical Long-chain fat is a stronger driver for intestinal adaptation than MCT
Ep 22 · 6:06
clinical One milliliter of parenteral nutrition is not isocaloric with one milliliter of formula
Ep 22 · 6:06
quote You're right, and they're not isocaloric, and so 1 mL of PN is not 1 mL of formula.
Ep 22 · 6:11
clinical Advancing enteral feeds beyond 100-120 mL/kg can cause problems with sodium and calcium in addition to calorie and protein deficits, because milk composition differs from parenteral solution
Ep 22 · 8:05
guideline Continuous feeds can be used as a supplemental approach, with bolus feeds during the day and continuous feeds overnight
Ep 22 · 8:05
guideline Bolus feeds are preferred as the default feeding method; patients should fail bolus feeds before transitioning to continuous feeds as the sole delivery mode
Ep 22 · 8:40
quote One thing I just want to say about oral feeding is that even in the scenario where it's really non-nutritive. I think it's important for people to sort of look at that oral feeding, not so much as an internal nutrition perspective, but more of a skill development perspective that they never learn how to eat by mouth and suck and swallow and process, then they won't eat solids later when you want them to, and you're stuck basically with tube feeding on an older child.
Ep 22 · 8:40
clinical Oral feeding is important for skill development even when non-nutritive; children who never learn to suck, swallow, and process food by mouth will not eat solids later and will remain tube-dependent
Ep 22 · 12:17
clinical Distally fed bowel has improved size discrepancy at the time of anastomosis because the bowel has been used
Ep 22 · 13:51
clinical A gastric tube provides versatility for nutrition supplementation, medication delivery, and venting for gassy or bloated patients to improve enteral tolerance

Intestinal Rehabilitation Episode 8: Refeeding of an Older Patient

Ep 23 · 1:24
quote The benefit in the curse in a patient like that is that they do know how to eat.
Ep 23 · 1:24
opinion Older patients who have established eating behavior before bowel loss have both a benefit and curse in that they know how to eat
Ep 23 · 1:50
quote So even if you can't cure this patient and get them off TPN. It's important to make compromises to optimize quality of life the best you can.
Ep 23 · 1:50
opinion Even if a patient cannot be cured and weaned off TPN, compromises should be made to optimize quality of life
Ep 23 · 2:14
clinical The general strategy is to push macronutrient modules of protein and fat which are well tolerated
Ep 23 · 2:21
quote Most of these kids don't tolerate simple sugars very well.
Ep 23 · 2:21
clinical Most short bowel patients don't tolerate simple sugars very well
Ep 23 · 2:25
clinical Pushing solids and minimizing fluid intake helps reduce dumping
Ep 23 · 2:31
clinical Smaller meals more frequently of solids separated from liquids and minimization of simple sugars is the recommended approach
Ep 23 · 2:52
clinical Patients who have transitioned off TPN often come back with problems when diet history reveals they have gotten loose with diet choices
Ep 23 · 3:31
clinical Minimizing sugars reduces symptoms related to bacterial overgrowth such as bloating and gas
Ep 23 · 3:48
clinical Fluid losses whether by stoma or other source must be replaced
Ep 23 · 3:56
clinical If patients are on IV support, some fluid can be replaced intravenously
Ep 23 · 4:01
clinical When trying to wean off IV support, keeping patients hydrated through enteral replacements is important
Ep 23 · 4:11
clinical Fluid transport requires sodium and glucose, so replacement solution must contain both
Ep 23 · 4:11
quote For transport requires sodium and glucose, and so your solution that you're replacing with has to contain some glucose and some salt.
Ep 23 · 4:48
clinical Gatorade doesn't work well for rehydration because it has too much sugar
Ep 23 · 4:53
quote It's actually too much sugar in it.
Ep 23 · 6:28
clinical Some kids end up back on parenteral support to get through puberty
Ep 23 · 6:33
clinical When not growing anymore as an adult, borderline or marginal gut function is often enough to sustain them
Ep 23 · 7:22
clinical After getting off TPN, patients often get into trouble with micronutrient deficiencies
Ep 23 · 8:51
clinical Weight must be balanced with height as a growth metric
Ep 23 · 8:57
clinical This population commonly shows round babies where weight for height is elevated
Ep 23 · 9:01
clinical There is increasing data looking at quality of weight - how much is fat weight versus lean body mass

Advancements in Pediatric Intestinal Failure: Innovative Therapies and Improved Outcomes

Ep 28 · 5:23
clinical Liver disease in intestinal failure ranges from mild cholestasis to profound steatosis in older children or adolescents and adults, with ongoing inflammation, fibrosis, and ultimately cirrhosis
Ep 28 · 5:41
clinical Risk factors for intestinal failure-associated liver disease include prematurity, lack of enteral feeding, inability to feed due to anatomy, recurrent infections, sepsis, and components of TPN
Ep 28 · 6:12
clinical Intestinal rehab programs improve outcomes through integration of care, improved communication, and better continuity
Ep 28 · 6:54
quote What we ask our Families to do at home is significant.
Ep 28 · 7:11
quote We educate and empower our parents because we've all seen. High-quality families that over time demonstrate evidence of burnout.
Ep 28 · 7:25
epidemiological Literature shows improvement of outcomes with intestinal rehab programs including improved survival related to liver dysfunction, decreased septic episodes, reduction in central line complications, reduced ICU admissions, and patients coming off transplant lists
Ep 28 · 8:02
clinical Lipids are a source of essential fatty acids, a non-protein energy source important for growth, and fatty acids have a major role in cellular pathways
Ep 28 · 8:15
clinical Soybean-based lipid emulsions have high phytosterol content, high omega-6 long chain polyunsaturated fatty acid content, and low antioxidant content
Ep 28 · 8:31
clinical Prolonged soybean-based lipid exposure is associated with deterioration of liver dysfunction, specifically cholestasis
Ep 28 · 8:51
clinical For every day an infant is exposed to soybean lipid dosed at greater than 2.5 g per kilo per day, there is a 3% increase in the odds ratio of developing advanced liver disease
Ep 28 · 8:51
quote For every day that an infant is exposed to soybean lipid dosed at greater than 2.5 g per kilo per day, which is more conventional dosing. That is associated with a 3% increase in the odds ratio of developing advanced liver disease.
Ep 28 · 9:51
clinical Omega-6 lipids are metabolized to arachidonic acid, which produces leukotrienes and prostaglandins with a more pro-inflammatory profile
Ep 28 · 10:01
clinical Omega-3 lipids are metabolized through EPA and DHA and lead to production of cytokines with a less inflammatory profile
Ep 28 · 10:15
clinical Omega-3 lipids improve bile flow by decreasing lithogenicity of bile, decreasing steatosis, stimulating improved beta oxidation and clearance, lowering oxidative stress, and supporting immune function
Ep 28 · 10:42
clinical Restricting soybean lipid exposure improves cholestasis when dosed at 1 g per kilo per day or less
Ep 28 · 11:00
clinical In preterm babies, fat is important for growth, especially for neurocognitive development, and there is risk of essential fatty acid deficiency if lipid dosing is restricted too much
Ep 28 · 11:22
clinical DHA and arachidonic acid are important for retinal and brain development
Ep 28 · 11:26
quote None of the lipid emulsions today were designed for premature babies. They were all designed for adults in a critical care setting.
Ep 28 · 11:26
clinical None of the current lipid emulsions were designed for premature babies; they were all designed for adults in a critical care setting
Ep 28 · 12:03
quote So our patients with central lines, it's literally their lifeline. And without venous access, we can't support our families.
Ep 28 · 12:24
epidemiological There are approximately 240,000 central line-associated bloodstream infections in the United States annually, with each costing about $30,000, totaling about $2 billion per year nationwide
Ep 28 · 12:48
clinical 4% tetrasodium EDTA (kite lock) is an antithrombotic, anti-fibrinolytic, and antimicrobial that satisfies all three criteria important for a good lock solution
Ep 28 · 13:23
clinical Kite lock is licensed in Europe and Australia but not available in the United States
Ep 28 · 13:29
clinical A multi-center randomized trial of kite lock versus heparin went live 4 weeks ago and is actively recruiting patients
Ep 28 · 13:58
clinical In short bowel syndrome, there are 3 anatomical subtypes: Type 1 is high jejunostomy, Type 2 has lost distal small bowel and ileum with small bowel-colonic anastomosis, and Type 3 has lost mid-small bowel but retains some ileum and intact colon
Ep 28 · 14:26
epidemiological The most common anatomical subtype of short bowel syndrome in pediatrics is Type 2
Ep 28 · 14:33
clinical Type 1 and Type 2 short bowel syndrome patients struggle because they lack an ileum, which is where GLP-2 is naturally produced
Ep 28 · 14:53
clinical Native GLP-2 hormone has a half-life of 7 minutes, making it impractical for clinical use
Ep 28 · 14:58
clinical Teduglutide is a GLP-2 analog with one amino acid alteration that has a half-life of 2 hours and is given once daily by subcutaneous injection
Ep 28 · 15:23
clinical Patients receiving teduglutide show a 40% reduction in TPN fluid and calorie requirements over a six-month period
Ep 28 · 15:32
clinical 70% of patients receiving teduglutide achieved the study endpoint of a 20% reduction in TPN requirements
Ep 28 · 15:48
clinical Teduglutide is licensed for children greater than 1 year of age
Ep 28 · 16:02
clinical Apraglutide is another GLP-2 analog with a longer half-life that can be given once a week and works even better than teduglutide, producing more bowel lengthening
Ep 28 · 16:36
epidemiological These interventions have led to a decrease in transplantation since 2008, largely because of the successes of intestinal rehabilitation
Ep 28 · 16:41
epidemiological Outcomes of intestinal transplant at 5 years are about 65%
Ep 28 · 16:49
quote Whatever we can do to avoid transplant and lifelong immunosuppression, that is our goal.
Intestinal Rehab 236 entries

Intestinal rehabilitation: What is intestinal rehab? - Episode 1

Ep 46 · 1:46
quote let's go back one step before that and sort of define what is intestinal failure because that sort of leads into who's going to benefit from an intestinal rehab program.
Ep 46 · 2:03
quote It it's, it's really a functional problem. Essentially, when someone's gut for whatever the diagnosis, whatever the reason, their gut function is insufficient to absorb enough nutrients, fluids, calories to support survival and in the in the case of children, growth, which is a big distinction between adults and children.
Ep 46 · 2:05
clinical Intestinal failure is defined as insufficient gut function to absorb enough nutrients, fluids, and calories to support survival and, in children, growth
Ep 46 · 3:03
quote an intestinal rehabilitation program, as we defined also in these Aspen guidelines that just came out, is a multidisciplinary or interdisciplinary collaborative patient care paradigm that that brings coordinated care for children with intestinal failure through comprehensive management of Their specialized nutrition and other associated needs that they have.
Ep 46 · 3:03
clinical An intestinal rehabilitation program is a multidisciplinary collaborative patient care paradigm providing coordinated care for children with intestinal failure through comprehensive management of specialized nutrition and associated needs
Ep 46 · 3:44
quote It's an amalgamation of experts that come from different aspects of care, providing a holistic, comprehensive, coordinated approach to patient management.
Ep 46 · 4:39
quote Patients that are gonna come to intestinal rehab program have intestinal failure, and we can divide the causes of intestinal failure into three categories.
Ep 46 · 4:50
quote There's short bowel syndrome, which by far is the most common category in pediatric patients with intestinal failure
Ep 46 · 4:50
epidemiological Short bowel syndrome is by far the most common category of intestinal failure in pediatric patients
Ep 46 · 5:06
clinical Causes of short bowel syndrome include congenital anomalies such as intestinal atresia, malrotation, volvulus, gastroschisis, and long segment Hirschsprung's disease
Ep 46 · 5:18
clinical Acquired diseases of the newborn such as necrotizing enterocolitis cause short bowel syndrome
Ep 46 · 5:45
quote So if we think of the intestine like a pipe, and that pipe is made of muscle that contracts in a coordinated way to push food and stool from one end to the other, you can have abnormalities of the muscle itself, or of the nerves that control that muscle.
Ep 46 · 5:45
clinical Motility disorders occur when abnormalities of the intestinal muscle itself or the nerves that control that muscle prevent coordinated movement of food and stool
Ep 46 · 6:10
clinical Children with motility disorders are dependent on intravenous support
Ep 46 · 6:17
quote these are conditions where the patient has all of their bowel, but the mucosa, the inside lining that, that absorbs, uh, digests and absorbs doesn't work.
Ep 46 · 6:17
clinical Enteropathies or congenital diarrheas are conditions where the patient has all of their bowel but the mucosal lining that digests and absorbs does not work
Ep 46 · 7:11
clinical Some patients will have elements of one, two, or all three categories of intestinal failure
Ep 46 · 7:11
quote The reason it's important to at least identify these three categories is that some patients will have elements of 12, or 3 of the categories, the way they present.
Ep 46 · 7:45
epidemiological Most intestinal failure patients are infants or babies, but some older kids develop intestinal failure
Ep 46 · 7:56
clinical Inflammatory bowel disease or Crohn's disease can lead to gut loss and intestinal failure in older patients
Ep 46 · 8:04
epidemiological Trauma, malignancy, and vascular thrombosis leading to gut loss are diagnoses seen more in older patients or adult series and less in pediatrics
Ep 46 · 12:46
clinical Long-term survivors now develop chronic comorbidities including renal dysfunction, metabolic bone disease, neurocognitive issues, and quality of life issues
Ep 46 · 12:46
quote But now what we're seeing is we have kids that have developed chronic comorbidities, that comorbidities that never, we didn't really see to the same extent because they didn't live long enough.

Intestinal Rehabilitation, Episode 1: What is intestinal rehabilitation?

Ep 47 · 1:46
quote let's go back one step before that and sort of define what is intestinal failure because that sort of leads into who's going to benefit from an intestinal rehab program
Ep 47 · 2:05
clinical Intestinal failure is defined as insufficient gut function to absorb enough nutrients, fluids, and calories to support survival and, in children, growth
Ep 47 · 3:03
clinical An intestinal rehabilitation program is a multidisciplinary collaborative patient care paradigm that brings coordinated care for children with intestinal failure through comprehensive management of their specialized nutrition and associated needs
Ep 47 · 3:44
quote It's an amalgamation of experts that come from different aspects of care, providing a holistic, comprehensive, coordinated approach to patient management
Ep 47 · 3:55
opinion Intestinal rehabilitation streamlines care and improves communication with families and between care providers and team members
Ep 47 · 4:50
epidemiological Short bowel syndrome is by far the most common category of intestinal failure in pediatric patients
Ep 47 · 5:06
clinical Causes of short bowel syndrome include congenital anomalies such as intestinal atresia, malrotation, volvulus, gastroschisis, and long segment Hirschsprung's disease
Ep 47 · 5:18
clinical Acquired diseases of the newborn such as necrotizing enterocolitis can cause short bowel syndrome
Ep 47 · 5:45
clinical Motility disorders occur when the bowel is unable to push contents through in a coordinated way due to abnormalities of the muscle itself or the nerves that control that muscle
Ep 47 · 5:45
quote So if we think of the intestine like a pipe, and that pipe is made of muscle that contracts in a coordinated way to push food and stool from one end to the other, you can have abnormalities of the muscle itself, or of the nerves that control that muscle
Ep 47 · 6:17
clinical Congenital enteropathies are conditions where the patient has all of their bowel but the mucosa does not work, leading to hypersecretion and profuse fluid losses
Ep 47 · 7:11
clinical Some patients will have elements of two or three categories of intestinal failure in the way they present
Ep 47 · 7:56
clinical Older pediatric patients can develop intestinal failure from inflammatory bowel disease or Crohn's disease where they have lost gut as a result of complications
Ep 47 · 8:04
epidemiological Trauma, malignancy, and vascular thrombosis leading to gut loss are diagnoses seen more in older patients or adult series and less in pediatrics
Ep 47 · 12:46
clinical Children with intestinal failure who survive long-term now develop chronic comorbidities including renal dysfunction, metabolic bone disease, neurocognitive issues, and quality of life issues

Intestinal Rehabilitation, Episode 2: Overwhelming intestinal damage, Part 1

Ep 48 · 1:17
opinion The outlook has changed and what's gone hand in hand with that is a bit more of an aggressive approach to surgical resection
Ep 48 · 1:39
clinical Comorbidities such as neurologic status, pulmonary function, cardiac function, or significant genetic or chromosomal anomaly factor into decision making about how to proceed
Ep 48 · 2:38
guideline Intestinal transplant is not experimental therapy but part of the continuum of therapy for a child with short bowel syndrome
Ep 48 · 2:52
quote Is it ethically appropriate to open and close a baby when you know that you could remove all of their intestines?
Ep 48 · 4:31
clinical In the very acute time after bowel infarction, the risk is dying of sepsis and multi-organ failure
Ep 48 · 4:44
clinical Intermediate and longer term complications include intestinal failure associated liver disease, recurrent sepsis, or line problems
Ep 48 · 5:02
epidemiological Success in the last two decades is because we're way better at preventing sepsis and liver disease
Ep 48 · 6:14
clinical Babies born with duodenal atresia or very proximal jejunal atresia are born with elevated direct or conjugated bilirubins
Ep 48 · 6:42
clinical Factors affecting liver function include prematurity, sepsis, choice of TPN, and presence or absence of enteral nutrition
Ep 48 · 12:15
quote Let the baby decide, you know, some may continue to spiral because of just overwhelming sepsis and may not make it, but many, many will.

Intestinal Rehabilitation, Episode 2: Overwhelming intestinal damage, Part 2

Ep 49 · 1:23
quote The goalposts have moved over the last two decades.
Ep 49 · 1:37
clinical In Toronto, ultra-short gut is classified as less than 20% of expected bowel length for age
Ep 49 · 1:42
clinical In a term baby, ultra-short gut corresponds to 20 to 30 centimeters of bowel
Ep 49 · 1:52
clinical Overall survival in ultra-short gut patients in the current era is over 90%
Ep 49 · 2:10
clinical Ultra-short gut patients who reached autonomy required multiple nutritional supplements
Ep 49 · 2:26
clinical Ultra-short gut patients with remnant ileum as part of residual bowel tended to do better
Ep 49 · 2:50
clinical Ultra-short gut patients with longer colonic remnants tended to adapt and get off TPN
Ep 49 · 6:10
quote I don't subscribe to the notion that Every kid needs to die with a laparotomy indecision.
Ep 49 · 7:40
opinion Lipid restriction was never a practice subscribed to by the Toronto program
Ep 49 · 11:53
quote The surgeon's role in the patient with short bowel syndrome is, is obviously significant. Partly for what we do and partly for what we don't do, right? Those intraoperative decisions have a lifelong impact.
Ep 49 · 12:00
opinion Intraoperative decisions in short bowel syndrome have a lifelong impact
Ep 49 · 12:08
opinion Decisions made at the time of intraabdominal catastrophe can make the difference between staying on or getting off TPN or whether the child survives
Ep 49 · 14:21
clinical Kids may look worse over the first 24-36 hours postoperatively, then stop getting worse before they start improving
Ep 49 · 14:21
quote Often, some of these kids do get, they kind of look worse over the next 24, 36 hours, and then they start to, you know, they may not even be getting better, they just stop getting worse.

Intestinal Rehabilitation, Episode 3: Enteral Autonomy, Part 1

Ep 52 · 2:02
quote That remaining bowel, that residual intestine goes through this process of adaptation and where the bowel is trying to compensate to reestablish function, to absorb enough nutrients and fluids to maintain survival.
Ep 52 · 2:16
clinical Adaptation is driven by intraluminal nutrients and their interaction with pancreatic biliary secretions and trophic gut peptides
Ep 52 · 2:42
clinical Structural changes during adaptation include mucosal hypertrophy with increased villous length, increased blood supply through angiogenesis, bowel dilation, and gut lengthening in younger children, all increasing surface area for absorption
Ep 52 · 3:04
clinical Functional changes during adaptation include slowed motility to allow more contact time and upregulation of enterocyte transporters
Ep 52 · 3:18
quote So, each, each cell is working harder, if you will, to try to, to, Function better and our job as a team is to try to promote that the best we can.
Ep 52 · 5:35
clinical Until recently there was no standardized definition for enteral autonomy
Ep 52 · 6:04
clinical TPN complications include line infections, liver disease, and vascular thrombosis
Ep 52 · 6:20
clinical In the past, patients were lost to complications such as liver disease before they could reach their adaptive potential; management of these complications has improved significantly
Ep 52 · 6:39
guideline Current ASPEN guidelines define enteral autonomy as independence of parenteral support for 12 weeks with maintenance of adequate growth and hydration
Ep 52 · 6:53
quote It's one thing to turn off parenteral support, but, you know, you're not really off TPN or off parental support unless you can actually grow off TPN.
Ep 52 · 7:50
epidemiological A 2012 PIFCO paper by Squires showed 50% of patients achieved enteral autonomy over 5-6 years, 25% died, and 25% got transplanted
Ep 52 · 8:25
epidemiological Recent papers in the last 5-6 years show 60-80% of patients achieve enteral autonomy, with a higher proportion surviving to reach autonomy
Ep 52 · 9:55
clinical Small bowel length is an independently significant variable for adaptation capacity
Ep 52 · 10:18
clinical The ileum has a much greater capacity to adapt than the jejunum
Ep 52 · 10:40
clinical At 5 years old, a child has about 425 centimeters of small bowel, with the steepest growth rate between 35 weeks gestation to about 6 months postnatal
Ep 52 · 11:25
opinion The ileocecal valve itself is not the important factor; most people who lose their ileocecal valve also lose their terminal ileum, which is the bigger factor for adaptive potential
Ep 52 · 11:53
clinical If a patient has the majority of their small bowel, it almost does not matter how much colon they have; probability of enteral autonomy is 85-100%

CAPS - The use of urine sodium to creatinine ratio as a marker of total body sodium in infants with intestinal failure - Sara Choi

Ep 58 · 5:58
quote I encourage the audience to, to highly consider voting for this paper because it's, it has a massive clinical importance.
Ep 58 · 6:05
quote This is often an underappreciated problem.
Ep 58 · 6:08
clinical Patients with stomas, even those without intestinal failure, run the risk of sodium depletion which has a significant impact on their growth
Ep 58 · 6:19
clinical Stomas and high stool losses are a large source of sodium bicarb as well as magnesium loss
Ep 58 · 6:38
clinical When we replace sodium, it does help restore growth, but it doesn't allow catch up growth
Ep 58 · 6:38
quote it's important to remember that when we replace sodium, it does help restore growth, but it doesn't allow catch up growth.
Ep 58 · 6:45
quote it's important that we track these patients serially, so that we can detect a trend evolving, so that we can avoid sodium depletion happening in the first place.
Ep 58 · 7:30
quote I would argue that, that having potassium chloride and an osmolaity added to the urine lytes in addition to just the sodium gives you a better picture of, of whether someone is truly sodium depleted.
Ep 58 · 7:41
clinical If potassium level is higher than sodium level in urine, that often will tell you that the aldosterone pathway is turned on

Pediatric Vascular Access in Brief: Preoperative, Operative, and Postoperative Considerations

Ep 63 · 10:35
clinical Antibiotic locking solutions have been around for a long time but predominantly develop resistance
Ep 63 · 10:47
quote And frankly, the problem with them is that you develop resistance.
Ep 63 · 10:56
clinical Ethanol locks are antimicrobial without resistance and kill both planktonic bacteria (floating in lumen) and sessile bacteria (embedded in biofilm)
Ep 63 · 10:56
quote It's antimicrobial, it doesn't have any resistance, and it'll kill bacteria that are both planktonic, meaning floating around in the, in the lumen, or sessile, meaning that they're embedded in a biofilm along the wall of the catheter.
Ep 63 · 11:24
clinical Kite lock is a 4% tetrasodium EDTA chemical found in Canada
Ep 63 · 11:36
clinical Kite lock is antimicrobial without resistance, antifibrinolytic, and antithrombotic
Ep 63 · 11:59
clinical Kite lock has been licensed in Canada for pediatric use, and is now licensed in Europe and Australia

Intestinal Rehabilitation, Episode 4: Surgical Management, Part 1

Ep 66 · 4:18
epidemiological The point of intake for the majority of short bowel syndrome patients is at birth because etiologies are usually neonatal causes
Ep 66 · 4:36
epidemiological Congenital anomalies of the GI tract or acquired conditions like necrotizing enterocolitis are the main causes
Ep 66 · 4:50
quote Making rational decisions at that initial surgery will set that baby up for success or failure.
Ep 66 · 5:42
clinical If reestablishing bowel continuity, you must deal with size discrepancy between the two ends
Ep 66 · 5:42
quote If you are thinking of reestablishing continuity, I feel very strongly that you have to deal with the size discrepancy.
Ep 66 · 7:08
quote In general, gastroschisis, they don't tend to do as well regardless.
Ep 66 · 7:08
clinical Gastroschisis patients don't tend to do as well regardless of intervention
Ep 66 · 7:12
clinical STEP procedures tend to not work as well in gastroschisis patients
Ep 66 · 9:09
clinical The STEP procedure requires bowel diameter of about 5 centimeters to be worthwhile
Ep 66 · 11:29
clinical There is a risk of bowel twisting in longitudinal orientation if alignment is not maintained during STEP
Ep 66 · 13:23
guideline Target caliber for STEP in a baby is about 1.5 centimeters, and for older infant or child is 2 to 2.5 centimeters
Ep 66 · 13:36
clinical Making the bowel too narrow during STEP can obstruct the patient, especially in someone with borderline motility
Ep 66 · 14:19
clinical A proposed advantage of STEP is that the surgeon can maintain caliber all the way from top to bottom by careful measurements
Ep 66 · 14:35
clinical There is potential for leak in the crotch of the staple line if not reinforced
Ep 66 · 15:08
clinical Dog ears at the top and bottom of the STEP segment can dilate over time and form blind loops
Ep 66 · 16:56
quote I don't step the duodenum. I start where the bowel starts to dilate.

Intestinal Rehabilitation, Episode 4: Surgical Management, Part 1

Ep 67 · 4:18
epidemiological The majority of pediatric short bowel syndrome cases present at birth due to neonatal causes: congenital GI anomalies or acquired conditions like necrotizing enterocolitis
Ep 67 · 5:42
clinical When reestablishing bowel continuity, size discrepancy between bowel ends must be addressed or a functional obstruction will result even if the anastomosis is patent
Ep 67 · 5:42
quote If you are thinking of reestablishing continuity, I feel very strongly that you have to deal with the size discrepancy.
Ep 67 · 6:02
clinical If adequate bowel length exists, size discrepancy can be managed by resection back to appropriate caliber or tapering enteroplasty
Ep 67 · 6:28
clinical In short bowel cases, serial transverse enteroplasty (STEP) can address size discrepancy without resecting mucosa
Ep 67 · 7:08
clinical Gastroschisis patients don't tend to do as well regardless of intervention; STEP procedures in gastroschisis patients consistently underperform
Ep 67 · 7:08
quote In general, gastroschisis, they don't tend to do as well regardless. If, and if I look at the step procedures that I've done over the years, the ones that tend to not do as well or tend to always be the gastroschisis.
Ep 67 · 9:09
quote I don't usually do that operation anyway unless the bowel is an adequate diameter to make it worthwhile, and in my head over the years, it's always been about 5 centimeters anyway.
Ep 67 · 9:09
clinical STEP procedure requires bowel diameter of at least 5 centimeters to be worthwhile
Ep 67 · 9:50
clinical In scenarios where immediate anastomosis is not prudent, distal bowel can be accessed with a feeding tube to provide distal feeding and grow the bowel in caliber before a subsequent operation
Ep 67 · 10:45
clinical The STEP procedure was originally described by HP Kim and Tom Jackson
Ep 67 · 11:29
clinical Maintaining bowel alignment during STEP is critical; there is risk of longitudinal twisting if alignment is not maintained
Ep 67 · 12:26
clinical An endo GIA stapler with vascular (white) load cartridge (2.5mm crimping to 1mm) is preferred over open GIA and prevents leaks better than the classic blue load
Ep 67 · 12:34
clinical STEP staple lines should be oriented perpendicular to the mesentery at 90 and 270 degrees (3 o'clock and 9 o'clock positions)
Ep 67 · 13:23
clinical Target bowel caliber after STEP is 1.5 cm in babies and 2 to 2.5 cm in older infants or children
Ep 67 · 13:36
clinical Making the bowel too narrow during STEP can cause obstruction, especially in patients with borderline motility
Ep 67 · 14:35
clinical A U-stitch with 4-0 PDS should be placed in the crotch of each staple line to prevent potential leaks
Ep 67 · 15:08
clinical Dog ears (blind loops) can form at the top and bottom of the STEP segment where bowel transitions in and out, and these can dilate over time
Ep 67 · 16:51
clinical The duodenum should not be stepped; STEP should start where the bowel begins to dilate, usually distal to the duodenum
Ep 67 · 16:56
quote I don't step the duodenum. I start where the bowel starts to dilate.
Ep 67 · 17:20
clinical If duodenal narrowing is needed, a stapler should be used on the lateral side away from the bile duct and ampulla; plication sutures usually fail

Intestinal Rehabilitation, Episode 4: Surgical Management, Part 2

Ep 68 · 1:52
quote I think we have to remember that the biggest benefit of the step, for instance, is the fact that you're tapering the bowel and reestablishing a more normal caliber to improve motility.
Ep 68 · 1:52
clinical The biggest benefit of the STEP procedure is tapering the bowel and reestablishing a more normal caliber to improve motility
Ep 68 · 3:04
clinical It can take up to 6 months before significant improvement in absorptive capacity is seen after STEP
Ep 68 · 3:04
quote It can take up to 6 months before you actually start to see a significant improvement in absorptive capacity.
Ep 68 · 3:12
clinical Absorptive capacity improvement is measured with fecal fat, alpha-1 antitrypsin clearance, xylose absorption, and citrulline levels
Ep 68 · 3:24
clinical The delay in improvement is due to inflamed, sick, leaky mucosa in the setting of bacterial overgrowth needing time to heal
Ep 68 · 6:24
clinical Staple-line ulcer bleeding is an underreported complication that is difficult to manage
Ep 68 · 6:49
quote My hypothesis is that this is a microbiome problem. I think you have a pro-inflammatory environment.
Ep 68 · 6:52
clinical Staple-line ulcers tend to occur in type 2 anatomy (small bowel to colonic remnant in the absence of an intact colon ileocecal valve)
Ep 68 · 7:12
clinical Pathology of staple-line ulcers shows non-specific inflammation with no vasculitis, viral elements, or obvious ischemia
Ep 68 · 7:12
quote It's non-specific inflammation. There's no vasculitis, there's no viral elements, there's no obvious ischemia, and the management can be super difficult.
Ep 68 · 10:05
clinical Most referrals for intestinal failure have had multiple operations before coming to a specialized center
Ep 68 · 10:09
opinion Surgical planning may not be conducive to just one operation and may require setting up for the next case

Intestinal Rehabilitation, Episode 6: Cholestasis

Ep 73 · 1:09
quote Institutionally defined it as a conjugated bilirubin greater than 3 mg per deciliter or 50 micromoles per liter sustained for 2 weeks and not associated with a septic event.
Ep 73 · 1:09
guideline Cholestasis is institutionally defined as conjugated bilirubin greater than 3 mg/dL or 50 micromoles/L sustained for 2 weeks and not associated with a septic event
Ep 73 · 1:20
guideline A 2021 JPN publication defines cholestasis as conjugated bilirubin around 2 mg/dL or 34 micromoles/L for 2 weeks, not associated with a septic event
Ep 73 · 2:57
quote In the setting of young children, it's more of a cholestatic liver disease, whereas in older people, adolescents and adults, they tend to have Steatosis, there's a fatty deposition within the liver, and the term we use now is intestinal failure associated liver disease because that sort of indicates that it's a multifactorial process.
Ep 73 · 2:57
clinical In young children, intestinal failure causes cholestatic liver disease, whereas in adolescents and adults it tends to cause steatosis (fatty deposition)
Ep 73 · 3:30
clinical Prematurity is not modifiable by the clinical team, but other risk factors (enteral feeding, sepsis, TPN components) are modifiable
Ep 73 · 3:30
quote Now, prematurity, we as clinicians, as a team, we don't have much control over that. But the other factors are modifiable, and we can have an impact on that.
Ep 73 · 4:09
clinical Prevention requires aggressive introduction of enteral feeding and surgical procedures to optimize anatomy for feed delivery
Ep 73 · 7:08
clinical Conventional intralipid (soybean-based) when metabolized leads to production of prostaglandins and eicosanoids that are pro-inflammatory
Ep 73 · 7:19
clinical SMOF lipid promotes bile flow, is hepatoprotective, and can be delivered at conventional dose while supporting somatic growth and neurologic development
Ep 73 · 7:45
clinical SMOF lipid does not have enough arachidonic acid, so dose restriction can lead to essential fatty acid deficiency
Ep 73 · 7:57
clinical When SMOF is delivered at conventional dosing, nobody develops essential fatty acid deficiency
Ep 73 · 8:06
guideline Recommended lipid dosing is settling around 2.5 g/kg, but nutrition guidelines for preterms and babies state 3-4 g/kg/day
Ep 73 · 8:29
opinion A bilirubin of 2 mg/dL is not advanced liver disease, is not dangerous, and is usually transient; approximately 90% of patients will improve with observation alone
Ep 73 · 8:52
quote My opinion is that a billy of 2 is not by any stretch advanced liver disease, and it's not dangerous, and it's usually transient. So I personally would not change the lipid emulsion out of Billy to.
Ep 73 · 12:11
clinical Liver biopsy is commonly performed during secondary surgical or autologous reconstruction procedures to provide an up-to-date microscopic snapshot
Ep 73 · 13:00
clinical Elastography or fibroscan is available for monitoring but is good for mild/no fibrosis or very advanced fibrosis; it is less sensitive for patients in the middle range
Ep 73 · 13:31
clinical Outpatient follow-up frequency for children on TPN at home ranges from every 1-4 months depending on patient stability

Intestinal Rehabilitation, Episode 7: Refeeding in a neonatal patient

Ep 79 · 0:57
quote Even if a kid has a complicated disease process, one of our goals as a team is to try to establish the most normal feeding habits we can that not only promote gut function and everything else, but also to optimize quality of life and the importance of the social aspects of eating that we all experience with our families and friends.
Ep 79 · 0:57
clinical Refeeding goals include establishing normal feeding habits to promote gut function and optimize quality of life and social aspects of eating
Ep 79 · 4:34
clinical In short bowel syndrome, protein absorption is fairly well preserved, so the benefit of hydrolyzed or amino acid formulas is primarily from an allergy perspective
Ep 79 · 5:04
clinical Long-chain fat is a stronger driver for intestinal adaptation than MCT
Ep 79 · 6:06
clinical One milliliter of parenteral nutrition is not isocaloric with one milliliter of formula
Ep 79 · 6:06
quote You're right, and they're not isocaloric, and so 1 mL of PN is not 1 mL of formula.
Ep 79 · 6:11
clinical Advancing enteral feeds beyond 100-120 mL/kg can cause problems with sodium and calcium in addition to calorie and protein deficits, because milk composition differs from parenteral solution
Ep 79 · 8:05
guideline Bolus feeds are preferred as the default feeding method; patients should fail bolus feeds before transitioning to continuous feeds as the sole delivery mode
Ep 79 · 8:05
guideline Continuous feeds can be used as a supplemental approach, with bolus feeds during the day and continuous feeds overnight
Ep 79 · 8:40
quote One thing I just want to say about oral feeding is that even in the scenario where it's really non-nutritive. I think it's important for people to sort of look at that oral feeding, not so much as an internal nutrition perspective, but more of a skill development perspective that they never learn how to eat by mouth and suck and swallow and process, then they won't eat solids later when you want them to, and you're stuck basically with tube feeding on an older child.
Ep 79 · 8:40
clinical Oral feeding is important for skill development even when non-nutritive; children who never learn to suck, swallow, and process food by mouth will not eat solids later and will remain tube-dependent
Ep 79 · 12:17
clinical Distally fed bowel has improved size discrepancy at the time of anastomosis because the bowel has been used
Ep 79 · 13:51
clinical A gastric tube provides versatility for nutrition supplementation, medication delivery, and venting for gassy or bloated patients to improve enteral tolerance

Intestinal Rehabilitation Episode 8: Refeeding of an Older Patient

Ep 80 · 1:24
quote The benefit in the curse in a patient like that is that they do know how to eat.
Ep 80 · 1:24
opinion Older patients who have established eating behavior before bowel loss have both a benefit and curse in that they know how to eat
Ep 80 · 1:50
opinion Even if a patient cannot be cured and weaned off TPN, compromises should be made to optimize quality of life
Ep 80 · 1:50
quote So even if you can't cure this patient and get them off TPN. It's important to make compromises to optimize quality of life the best you can.
Ep 80 · 2:14
clinical The general strategy is to push macronutrient modules of protein and fat which are well tolerated
Ep 80 · 2:21
clinical Most short bowel patients don't tolerate simple sugars very well
Ep 80 · 2:21
quote Most of these kids don't tolerate simple sugars very well.
Ep 80 · 2:25
clinical Pushing solids and minimizing fluid intake helps reduce dumping
Ep 80 · 2:31
clinical Smaller meals more frequently of solids separated from liquids and minimization of simple sugars is the recommended approach
Ep 80 · 2:52
clinical Patients who have transitioned off TPN often come back with problems when diet history reveals they have gotten loose with diet choices
Ep 80 · 3:31
clinical Minimizing sugars reduces symptoms related to bacterial overgrowth such as bloating and gas
Ep 80 · 3:48
clinical Fluid losses whether by stoma or other source must be replaced
Ep 80 · 3:56
clinical If patients are on IV support, some fluid can be replaced intravenously
Ep 80 · 4:01
clinical When trying to wean off IV support, keeping patients hydrated through enteral replacements is important
Ep 80 · 4:11
clinical Fluid transport requires sodium and glucose, so replacement solution must contain both
Ep 80 · 4:11
quote For transport requires sodium and glucose, and so your solution that you're replacing with has to contain some glucose and some salt.
Ep 80 · 4:48
clinical Gatorade doesn't work well for rehydration because it has too much sugar
Ep 80 · 4:53
quote It's actually too much sugar in it.
Ep 80 · 6:28
clinical Some kids end up back on parenteral support to get through puberty
Ep 80 · 6:33
clinical When not growing anymore as an adult, borderline or marginal gut function is often enough to sustain them
Ep 80 · 7:22
clinical After getting off TPN, patients often get into trouble with micronutrient deficiencies
Ep 80 · 8:51
clinical Weight must be balanced with height as a growth metric
Ep 80 · 8:57
clinical This population commonly shows round babies where weight for height is elevated
Ep 80 · 9:01
clinical There is increasing data looking at quality of weight - how much is fat weight versus lean body mass

Advancements in Pediatric Intestinal Failure: Innovative Therapies and Improved Outcomes

Ep 108 · 5:23
clinical Liver disease in intestinal failure ranges from mild cholestasis to profound steatosis in older children or adolescents and adults, with ongoing inflammation, fibrosis, and ultimately cirrhosis
Ep 108 · 5:41
clinical Risk factors for intestinal failure-associated liver disease include prematurity, lack of enteral feeding, inability to feed due to anatomy, recurrent infections, sepsis, and components of TPN
Ep 108 · 6:12
clinical Intestinal rehab programs improve outcomes through integration of care, improved communication, and better continuity
Ep 108 · 6:54
quote What we ask our Families to do at home is significant.
Ep 108 · 7:11
quote We educate and empower our parents because we've all seen. High-quality families that over time demonstrate evidence of burnout.
Ep 108 · 7:25
epidemiological Literature shows improvement of outcomes with intestinal rehab programs including improved survival related to liver dysfunction, decreased septic episodes, reduction in central line complications, reduced ICU admissions, and patients coming off transplant lists
Ep 108 · 8:02
clinical Lipids are a source of essential fatty acids, a non-protein energy source important for growth, and fatty acids have a major role in cellular pathways
Ep 108 · 8:15
clinical Soybean-based lipid emulsions have high phytosterol content, high omega-6 long chain polyunsaturated fatty acid content, and low antioxidant content
Ep 108 · 8:31
clinical Prolonged soybean-based lipid exposure is associated with deterioration of liver dysfunction, specifically cholestasis
Ep 108 · 8:51
clinical For every day an infant is exposed to soybean lipid dosed at greater than 2.5 g per kilo per day, there is a 3% increase in the odds ratio of developing advanced liver disease
Ep 108 · 8:51
quote For every day that an infant is exposed to soybean lipid dosed at greater than 2.5 g per kilo per day, which is more conventional dosing. That is associated with a 3% increase in the odds ratio of developing advanced liver disease.
Ep 108 · 9:51
clinical Omega-6 lipids are metabolized to arachidonic acid, which produces leukotrienes and prostaglandins with a more pro-inflammatory profile
Ep 108 · 10:01
clinical Omega-3 lipids are metabolized through EPA and DHA and lead to production of cytokines with a less inflammatory profile
Ep 108 · 10:15
clinical Omega-3 lipids improve bile flow by decreasing lithogenicity of bile, decreasing steatosis, stimulating improved beta oxidation and clearance, lowering oxidative stress, and supporting immune function
Ep 108 · 10:42
clinical Restricting soybean lipid exposure improves cholestasis when dosed at 1 g per kilo per day or less
Ep 108 · 11:00
clinical In preterm babies, fat is important for growth, especially for neurocognitive development, and there is risk of essential fatty acid deficiency if lipid dosing is restricted too much
Ep 108 · 11:22
clinical DHA and arachidonic acid are important for retinal and brain development
Ep 108 · 11:26
quote None of the lipid emulsions today were designed for premature babies. They were all designed for adults in a critical care setting.
Ep 108 · 11:26
clinical None of the current lipid emulsions were designed for premature babies; they were all designed for adults in a critical care setting
Ep 108 · 12:03
quote So our patients with central lines, it's literally their lifeline. And without venous access, we can't support our families.
Ep 108 · 12:24
epidemiological There are approximately 240,000 central line-associated bloodstream infections in the United States annually, with each costing about $30,000, totaling about $2 billion per year nationwide
Ep 108 · 12:48
clinical 4% tetrasodium EDTA (kite lock) is an antithrombotic, anti-fibrinolytic, and antimicrobial that satisfies all three criteria important for a good lock solution
Ep 108 · 13:23
clinical Kite lock is licensed in Europe and Australia but not available in the United States
Ep 108 · 13:29
clinical A multi-center randomized trial of kite lock versus heparin went live 4 weeks ago and is actively recruiting patients
Ep 108 · 13:58
clinical In short bowel syndrome, there are 3 anatomical subtypes: Type 1 is high jejunostomy, Type 2 has lost distal small bowel and ileum with small bowel-colonic anastomosis, and Type 3 has lost mid-small bowel but retains some ileum and intact colon
Ep 108 · 14:26
epidemiological The most common anatomical subtype of short bowel syndrome in pediatrics is Type 2
Ep 108 · 14:33
clinical Type 1 and Type 2 short bowel syndrome patients struggle because they lack an ileum, which is where GLP-2 is naturally produced
Ep 108 · 14:53
clinical Native GLP-2 hormone has a half-life of 7 minutes, making it impractical for clinical use
Ep 108 · 14:58
clinical Teduglutide is a GLP-2 analog with one amino acid alteration that has a half-life of 2 hours and is given once daily by subcutaneous injection
Ep 108 · 15:23
clinical Patients receiving teduglutide show a 40% reduction in TPN fluid and calorie requirements over a six-month period
Ep 108 · 15:32
clinical 70% of patients receiving teduglutide achieved the study endpoint of a 20% reduction in TPN requirements
Ep 108 · 15:48
clinical Teduglutide is licensed for children greater than 1 year of age
Ep 108 · 16:02
clinical Apraglutide is another GLP-2 analog with a longer half-life that can be given once a week and works even better than teduglutide, producing more bowel lengthening
Ep 108 · 16:36
epidemiological These interventions have led to a decrease in transplantation since 2008, largely because of the successes of intestinal rehabilitation
Ep 108 · 16:41
epidemiological Outcomes of intestinal transplant at 5 years are about 65%
Ep 108 · 16:49
quote Whatever we can do to avoid transplant and lifelong immunosuppression, that is our goal.

Intestinal rehabilitation: What is intestinal rehab? - Episode 1

Ep 5 · 1:46
quote let's go back one step before that and sort of define what is intestinal failure because that sort of leads into who's going to benefit from an intestinal rehab program.
Ep 5 · 2:03
quote It it's, it's really a functional problem. Essentially, when someone's gut for whatever the diagnosis, whatever the reason, their gut function is insufficient to absorb enough nutrients, fluids, calories to support survival and in the in the case of children, growth, which is a big distinction between adults and children.
Ep 5 · 2:05
clinical Intestinal failure is defined as insufficient gut function to absorb enough nutrients, fluids, and calories to support survival and, in children, growth
Ep 5 · 3:03
quote an intestinal rehabilitation program, as we defined also in these Aspen guidelines that just came out, is a multidisciplinary or interdisciplinary collaborative patient care paradigm that that brings coordinated care for children with intestinal failure through comprehensive management of Their specialized nutrition and other associated needs that they have.
Ep 5 · 3:03
clinical An intestinal rehabilitation program is a multidisciplinary collaborative patient care paradigm providing coordinated care for children with intestinal failure through comprehensive management of specialized nutrition and associated needs
Ep 5 · 3:44
quote It's an amalgamation of experts that come from different aspects of care, providing a holistic, comprehensive, coordinated approach to patient management.
Ep 5 · 4:39
quote Patients that are gonna come to intestinal rehab program have intestinal failure, and we can divide the causes of intestinal failure into three categories.
Ep 5 · 4:50
quote There's short bowel syndrome, which by far is the most common category in pediatric patients with intestinal failure
Ep 5 · 4:50
epidemiological Short bowel syndrome is by far the most common category of intestinal failure in pediatric patients
Ep 5 · 5:06
clinical Causes of short bowel syndrome include congenital anomalies such as intestinal atresia, malrotation, volvulus, gastroschisis, and long segment Hirschsprung's disease
Ep 5 · 5:18
clinical Acquired diseases of the newborn such as necrotizing enterocolitis cause short bowel syndrome
Ep 5 · 5:45
clinical Motility disorders occur when abnormalities of the intestinal muscle itself or the nerves that control that muscle prevent coordinated movement of food and stool
Ep 5 · 5:45
quote So if we think of the intestine like a pipe, and that pipe is made of muscle that contracts in a coordinated way to push food and stool from one end to the other, you can have abnormalities of the muscle itself, or of the nerves that control that muscle.
Ep 5 · 6:10
clinical Children with motility disorders are dependent on intravenous support
Ep 5 · 6:17
quote these are conditions where the patient has all of their bowel, but the mucosa, the inside lining that, that absorbs, uh, digests and absorbs doesn't work.
Ep 5 · 6:17
clinical Enteropathies or congenital diarrheas are conditions where the patient has all of their bowel but the mucosal lining that digests and absorbs does not work
Ep 5 · 7:11
quote The reason it's important to at least identify these three categories is that some patients will have elements of 12, or 3 of the categories, the way they present.
Ep 5 · 7:11
clinical Some patients will have elements of one, two, or all three categories of intestinal failure
Ep 5 · 7:45
epidemiological Most intestinal failure patients are infants or babies, but some older kids develop intestinal failure
Ep 5 · 7:56
clinical Inflammatory bowel disease or Crohn's disease can lead to gut loss and intestinal failure in older patients
Ep 5 · 8:04
epidemiological Trauma, malignancy, and vascular thrombosis leading to gut loss are diagnoses seen more in older patients or adult series and less in pediatrics
Ep 5 · 12:46
quote But now what we're seeing is we have kids that have developed chronic comorbidities, that comorbidities that never, we didn't really see to the same extent because they didn't live long enough.
Ep 5 · 12:46
clinical Long-term survivors now develop chronic comorbidities including renal dysfunction, metabolic bone disease, neurocognitive issues, and quality of life issues

Intestinal Rehabilitation, Episode 1: What is intestinal rehabilitation?

Ep 6 · 1:46
quote let's go back one step before that and sort of define what is intestinal failure because that sort of leads into who's going to benefit from an intestinal rehab program
Ep 6 · 2:05
clinical Intestinal failure is defined as insufficient gut function to absorb enough nutrients, fluids, and calories to support survival and, in children, growth
Ep 6 · 3:03
clinical An intestinal rehabilitation program is a multidisciplinary collaborative patient care paradigm that brings coordinated care for children with intestinal failure through comprehensive management of their specialized nutrition and associated needs
Ep 6 · 3:44
quote It's an amalgamation of experts that come from different aspects of care, providing a holistic, comprehensive, coordinated approach to patient management
Ep 6 · 3:55
opinion Intestinal rehabilitation streamlines care and improves communication with families and between care providers and team members
Ep 6 · 4:50
epidemiological Short bowel syndrome is by far the most common category of intestinal failure in pediatric patients
Ep 6 · 5:06
clinical Causes of short bowel syndrome include congenital anomalies such as intestinal atresia, malrotation, volvulus, gastroschisis, and long segment Hirschsprung's disease
Ep 6 · 5:18
clinical Acquired diseases of the newborn such as necrotizing enterocolitis can cause short bowel syndrome
Ep 6 · 5:45
quote So if we think of the intestine like a pipe, and that pipe is made of muscle that contracts in a coordinated way to push food and stool from one end to the other, you can have abnormalities of the muscle itself, or of the nerves that control that muscle
Ep 6 · 5:45
clinical Motility disorders occur when the bowel is unable to push contents through in a coordinated way due to abnormalities of the muscle itself or the nerves that control that muscle
Ep 6 · 6:17
clinical Congenital enteropathies are conditions where the patient has all of their bowel but the mucosa does not work, leading to hypersecretion and profuse fluid losses
Ep 6 · 7:11
clinical Some patients will have elements of two or three categories of intestinal failure in the way they present
Ep 6 · 7:56
clinical Older pediatric patients can develop intestinal failure from inflammatory bowel disease or Crohn's disease where they have lost gut as a result of complications
Ep 6 · 8:04
epidemiological Trauma, malignancy, and vascular thrombosis leading to gut loss are diagnoses seen more in older patients or adult series and less in pediatrics
Ep 6 · 12:46
clinical Children with intestinal failure who survive long-term now develop chronic comorbidities including renal dysfunction, metabolic bone disease, neurocognitive issues, and quality of life issues

Intestinal rehabilitation: What is intestinal rehab? - Episode 1

Ep 3 · 1:46
quote let's go back one step before that and sort of define what is intestinal failure because that sort of leads into who's going to benefit from an intestinal rehab program.
Ep 3 · 2:03
quote It it's, it's really a functional problem. Essentially, when someone's gut for whatever the diagnosis, whatever the reason, their gut function is insufficient to absorb enough nutrients, fluids, calories to support survival and in the in the case of children, growth, which is a big distinction between adults and children.
Ep 3 · 2:05
clinical Intestinal failure is defined as insufficient gut function to absorb enough nutrients, fluids, and calories to support survival and, in children, growth
Ep 3 · 3:03
quote an intestinal rehabilitation program, as we defined also in these Aspen guidelines that just came out, is a multidisciplinary or interdisciplinary collaborative patient care paradigm that that brings coordinated care for children with intestinal failure through comprehensive management of Their specialized nutrition and other associated needs that they have.
Ep 3 · 3:03
clinical An intestinal rehabilitation program is a multidisciplinary collaborative patient care paradigm providing coordinated care for children with intestinal failure through comprehensive management of specialized nutrition and associated needs
Ep 3 · 3:44
quote It's an amalgamation of experts that come from different aspects of care, providing a holistic, comprehensive, coordinated approach to patient management.
Ep 3 · 4:39
quote Patients that are gonna come to intestinal rehab program have intestinal failure, and we can divide the causes of intestinal failure into three categories.
Ep 3 · 4:50
epidemiological Short bowel syndrome is by far the most common category of intestinal failure in pediatric patients
Ep 3 · 4:50
quote There's short bowel syndrome, which by far is the most common category in pediatric patients with intestinal failure
Ep 3 · 5:06
clinical Causes of short bowel syndrome include congenital anomalies such as intestinal atresia, malrotation, volvulus, gastroschisis, and long segment Hirschsprung's disease
Ep 3 · 5:18
clinical Acquired diseases of the newborn such as necrotizing enterocolitis cause short bowel syndrome
Ep 3 · 5:45
quote So if we think of the intestine like a pipe, and that pipe is made of muscle that contracts in a coordinated way to push food and stool from one end to the other, you can have abnormalities of the muscle itself, or of the nerves that control that muscle.
Ep 3 · 5:45
clinical Motility disorders occur when abnormalities of the intestinal muscle itself or the nerves that control that muscle prevent coordinated movement of food and stool
Ep 3 · 6:10
clinical Children with motility disorders are dependent on intravenous support
Ep 3 · 6:17
clinical Enteropathies or congenital diarrheas are conditions where the patient has all of their bowel but the mucosal lining that digests and absorbs does not work
Ep 3 · 6:17
quote these are conditions where the patient has all of their bowel, but the mucosa, the inside lining that, that absorbs, uh, digests and absorbs doesn't work.
Ep 3 · 7:11
clinical Some patients will have elements of one, two, or all three categories of intestinal failure
Ep 3 · 7:11
quote The reason it's important to at least identify these three categories is that some patients will have elements of 12, or 3 of the categories, the way they present.
Ep 3 · 7:45
epidemiological Most intestinal failure patients are infants or babies, but some older kids develop intestinal failure
Ep 3 · 7:56
clinical Inflammatory bowel disease or Crohn's disease can lead to gut loss and intestinal failure in older patients
Ep 3 · 8:04
epidemiological Trauma, malignancy, and vascular thrombosis leading to gut loss are diagnoses seen more in older patients or adult series and less in pediatrics
Ep 3 · 12:46
clinical Long-term survivors now develop chronic comorbidities including renal dysfunction, metabolic bone disease, neurocognitive issues, and quality of life issues
Ep 3 · 12:46
quote But now what we're seeing is we have kids that have developed chronic comorbidities, that comorbidities that never, we didn't really see to the same extent because they didn't live long enough.

Intestinal Rehabilitation, Episode 1: What is intestinal rehabilitation?

Ep 4 · 1:46
quote let's go back one step before that and sort of define what is intestinal failure because that sort of leads into who's going to benefit from an intestinal rehab program
Ep 4 · 2:05
clinical Intestinal failure is defined as insufficient gut function to absorb enough nutrients, fluids, and calories to support survival and, in children, growth
Ep 4 · 3:03
clinical An intestinal rehabilitation program is a multidisciplinary collaborative patient care paradigm that brings coordinated care for children with intestinal failure through comprehensive management of their specialized nutrition and associated needs
Ep 4 · 3:44
quote It's an amalgamation of experts that come from different aspects of care, providing a holistic, comprehensive, coordinated approach to patient management
Ep 4 · 3:55
opinion Intestinal rehabilitation streamlines care and improves communication with families and between care providers and team members
Ep 4 · 4:50
epidemiological Short bowel syndrome is by far the most common category of intestinal failure in pediatric patients
Ep 4 · 5:06
clinical Causes of short bowel syndrome include congenital anomalies such as intestinal atresia, malrotation, volvulus, gastroschisis, and long segment Hirschsprung's disease
Ep 4 · 5:18
clinical Acquired diseases of the newborn such as necrotizing enterocolitis can cause short bowel syndrome
Ep 4 · 5:45
quote So if we think of the intestine like a pipe, and that pipe is made of muscle that contracts in a coordinated way to push food and stool from one end to the other, you can have abnormalities of the muscle itself, or of the nerves that control that muscle
Ep 4 · 5:45
clinical Motility disorders occur when the bowel is unable to push contents through in a coordinated way due to abnormalities of the muscle itself or the nerves that control that muscle
Ep 4 · 6:17
clinical Congenital enteropathies are conditions where the patient has all of their bowel but the mucosa does not work, leading to hypersecretion and profuse fluid losses
Ep 4 · 7:11
clinical Some patients will have elements of two or three categories of intestinal failure in the way they present
Ep 4 · 7:56
clinical Older pediatric patients can develop intestinal failure from inflammatory bowel disease or Crohn's disease where they have lost gut as a result of complications
Ep 4 · 8:04
epidemiological Trauma, malignancy, and vascular thrombosis leading to gut loss are diagnoses seen more in older patients or adult series and less in pediatrics
Ep 4 · 12:46
clinical Children with intestinal failure who survive long-term now develop chronic comorbidities including renal dysfunction, metabolic bone disease, neurocognitive issues, and quality of life issues

Intestinal Rehabilitation, Episode 4: Surgical Management, Part 1

Ep 5 · 4:18
epidemiological The majority of pediatric short bowel syndrome cases present at birth due to neonatal causes: congenital GI anomalies or acquired conditions like necrotizing enterocolitis
Ep 5 · 5:42
quote If you are thinking of reestablishing continuity, I feel very strongly that you have to deal with the size discrepancy.
Ep 5 · 5:42
clinical When reestablishing bowel continuity, size discrepancy between bowel ends must be addressed or a functional obstruction will result even if the anastomosis is patent
Ep 5 · 6:02
clinical If adequate bowel length exists, size discrepancy can be managed by resection back to appropriate caliber or tapering enteroplasty
Ep 5 · 6:28
clinical In short bowel cases, serial transverse enteroplasty (STEP) can address size discrepancy without resecting mucosa
Ep 5 · 7:08
clinical Gastroschisis patients don't tend to do as well regardless of intervention; STEP procedures in gastroschisis patients consistently underperform
Ep 5 · 7:08
quote In general, gastroschisis, they don't tend to do as well regardless. If, and if I look at the step procedures that I've done over the years, the ones that tend to not do as well or tend to always be the gastroschisis.
Ep 5 · 9:09
clinical STEP procedure requires bowel diameter of at least 5 centimeters to be worthwhile
Ep 5 · 9:09
quote I don't usually do that operation anyway unless the bowel is an adequate diameter to make it worthwhile, and in my head over the years, it's always been about 5 centimeters anyway.
Ep 5 · 9:50
clinical In scenarios where immediate anastomosis is not prudent, distal bowel can be accessed with a feeding tube to provide distal feeding and grow the bowel in caliber before a subsequent operation
Ep 5 · 10:45
clinical The STEP procedure was originally described by HP Kim and Tom Jackson
Ep 5 · 11:29
clinical Maintaining bowel alignment during STEP is critical; there is risk of longitudinal twisting if alignment is not maintained
Ep 5 · 12:26
clinical An endo GIA stapler with vascular (white) load cartridge (2.5mm crimping to 1mm) is preferred over open GIA and prevents leaks better than the classic blue load
Ep 5 · 12:34
clinical STEP staple lines should be oriented perpendicular to the mesentery at 90 and 270 degrees (3 o'clock and 9 o'clock positions)
Ep 5 · 13:23
clinical Target bowel caliber after STEP is 1.5 cm in babies and 2 to 2.5 cm in older infants or children
Ep 5 · 13:36
clinical Making the bowel too narrow during STEP can cause obstruction, especially in patients with borderline motility
Ep 5 · 14:35
clinical A U-stitch with 4-0 PDS should be placed in the crotch of each staple line to prevent potential leaks
Ep 5 · 15:08
clinical Dog ears (blind loops) can form at the top and bottom of the STEP segment where bowel transitions in and out, and these can dilate over time
Ep 5 · 16:51
clinical The duodenum should not be stepped; STEP should start where the bowel begins to dilate, usually distal to the duodenum
Ep 5 · 16:56
quote I don't step the duodenum. I start where the bowel starts to dilate.
Ep 5 · 17:20
clinical If duodenal narrowing is needed, a stapler should be used on the lateral side away from the bile duct and ampulla; plication sutures usually fail