Journal of Pediatric Surgery Article Review: February 2022 - BAPS Issue

Adrian Wolf's research focuses on creating renal organoids with glomerular differentiation from pluripotent stem cells to understand why babies are born without kidneys or with abnormal kidneys or lower urinary tract.

Renal organoids could potentially be used as replacement therapy instead of formal kidney transplant and could one day be functionally useful in living patients.

Stem cells can be made from blood samples of children born with abnormal kidneys to grow organoids in culture that reproduce the disease in a dish, allowing researchers to look for druggable targets.

Growing a ureter from stem cells is difficult, and without a ureter, even a functional kidney organoid would have nowhere for urine to go.

Kidney organoids are only a few millimeters across, so using them as renal replacement therapy would require implanting hundreds of them into a person.

Kidney organoids cannot be connected to large arteries, and their glomeruli could not make much urine without high pressure, high flow blood supply.

In the UK multicenter ELBT study, 16 out of 22 patients (73%) had successful esophageal anastomosis after lengthening procedure.

In ELBT cases where the procedure did not work well, all babies managed to have cervical esophagostomy and survive, with reconstruction typically using gastric transposition.

There were three deaths after anastomosis in the UK ELBT series.

Mark Davenport states that ELBT results reflect what is happening in the real world, it is not universally successful, and its role in long gap esophageal atresia management will remain controversial.

The Netherlands has 17 million people with approximately 30 to 40 cases of esophageal atresia per year across 6 pediatric surgical centers.

In the Netherlands, centralization began in 2014 with oncology cases limited to one hospital, expanded in 2015 to include abdominal, thoracic and neck cases, and in 2018 added neurosurgical and orthopedic oncology.

In the Netherlands, biliary atresia and choledochal cysts are now done in only 1 of the 6 pediatric surgical centers.

Esophageal atresia cases in the Netherlands are now done in only 3 hospitals.

Anorectal malformations and Hirschsprung disease are done in 4 out of 6 hospitals in the Netherlands.

Mark Rinan's center performs about 30 neuroblastomas per year and about 35 renal tumors per year, and results improved dramatically with centralization.