From
StayCurrentMD
Intestinal Rehabilitation, Episode 6: Cholestasis
With Dr. Michael Helmrath & Dr. Paul Wales · hosted by Dr. Ellen Encisco & Dr. Cecilia Gigena
Chapter 1 of 7 · Fundamentals
Cholestasis definition
Introduction and Definition of Cholestasis
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Educational content from recorded physician discussions — not medical advice. Talk to your (or your child's) care team about your situation.
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What the experts said
Cincinnati Children's Hospital institutionally defined cholestasis as conjugated bilirubin greater than 3 mg/dL or 50 micromoles/L sustained for 2 weeks and not associated with a septic event.
A 2021 JPN publication defined cholestasis as conjugated bilirubin around 2 mg/dL or 34 micromoles/L for 2 weeks, not associated with a septic event.
As treatment has improved with different lipid emulsions and nutrition approaches, cholestasis has become more an indicator of underlying diseases to address rather than a primary morbidity/mortality factor.
In young children, intestinal failure-associated liver disease presents as cholestatic liver disease, whereas in adolescents and adults it tends to be steatosis (fatty deposition).
Prematurity is not modifiable by the clinical team, but lack of enteral feeding, sepsis, and TPN components are modifiable risk factors.
Prevention of cholestasis requires aggressive introduction of enteral feeding to establish enterohepatic circulation and surgical procedures to optimize anatomy for feed delivery.
Limiting intravenous fat to 1 g/kg/day can help prevent cholestasis.
New lipid emulsions including Omegaven (used first in the US) and SMOF (used in Europe and Canada, now prevalent in the US for 3-4 years) can reverse or prevent cholestasis.
A major advantage of SMOF is the ability to provide more calories from fat (as much as 2-2.5 g/kg) while supporting healthy growth.
Two strategies for reversing cholestasis are dose restriction and change of lipid composition.
Conventional intralipid (soybean-based) produces prostaglandins and eicosanoids that are pro-inflammatory when metabolized.
SMOF lipid promotes bile flow, is hepatoprotective, and can be delivered at conventional dose to achieve somatic growth and provide neurologic nutrients while protecting the liver.
SMOF lipid does not contain enough arachidonic acid, so dose restriction of SMOF can lead to essential fatty acid deficiency.
When SMOF is delivered at conventional dosing, no patients develop essential fatty acid deficiency.
Conventional dosing for lipids is settling around 2.5 g/kg/day, but nutrition guidelines for preterm infants and babies state 3-4 g/kg/day.
A bilirubin of 2 mg/dL is not advanced liver disease, is not dangerous, and is usually transient; Dr. Wales would not change lipid emulsion at this threshold.
When refeeding a cholestatic liver after proximal jejunostomy takedown, direct bilirubin typically rises in the first week as bile acid pool is reintroduced and the liver becomes more active in bile salt production.
GGT, AST, and ALT will go up in the first week or two after anastomosis takedown surgery, then slowly come down over several weeks.
When direct bilirubin rises after refeeding, the main differential to rule out is urinary tract infection or gram-negative infection; extensive imaging such as ultrasounds is not needed.
It is important to provide proximal drainage of the duodenum in high-risk intestinal failure patients; Dr. Helmrath places a lake drain for this purpose.
Ongoing cholestasis with proximal blockage puts pressure in the biliary system at a much higher level and speeds up the cholestatic process.
G-tubes do not decompress the duodenum.
During secondary surgical or autologous reconstruction procedures, a liver biopsy is commonly taken to provide an up-to-date microscopic snapshot.
Liver function and biochemistry are followed routinely as inpatient and at outpatient clinic visits.
Elastography (FibroScan) is available for monitoring but is good for mild/no fibrosis or very advanced fibrosis; it is not as sensitive for patients with intermediate fibrosis.
Follow-up visit frequency for children on TPN at home ranges from every 1-4 months depending on patient stability; actively adapting patients may be seen more frequently due to rapid changes being made.
Advanced liver disease is defined as conjugated bilirubin above 5 or 6 mg/dL.
Historically, 25-50% of intestinal failure patients died because of associated liver disease; now it is less than 2%.
Risk factors for intestinal failure-associated liver disease include prematurity, lack of enteral feeding, sepsis, and TPN components.
SMOF lipid emulsion is composed of soybean oil, medium chain triglycerides, olive oil, and fish oil.
Patients on SMOF lipid emulsions can still develop cholestasis due to other factors beyond lipid composition.
A lake drain helps identify proximal bowel and leaves the bowel at appropriate size to make future anastomosis easier without large size mismatch.
Prophylactic cholecystectomy is not recommended because the gallbladder helps with enterohepatic circulation and many patients will not need it.