StayCurrentMD · Surgical treatment of congenital hyperinsulinism: Results from 500 pancreatectomies in neonates and children
Article1 min read·Published Oct 2018Older

Surgical treatment of congenital hyperinsulinism: Results from 500 pancreatectomies in neonates and children

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Article · Oct 2018 · 1 min read

In brief

In brief

Large retrospective series of 500 pancreatectomies for congenital hyperinsulinism over 20 years, stratifying outcomes by disease subtype: focal, diffuse, atypical (LINE, Beckwith-Wiedemann), and insulinoma. Study compares surgical approaches—partial resection for focal disease versus near-total pancreatectomy for diffuse disease—and their efficacy in controlling severe neonatal and pediatric hypoglycemia.

Written by the GCMD Library team from the article.

Abstract

Background

Congenital Hyperinsulinism (HI) causes severe hypoglycemia in neonates and children. We reviewed our experience with pancreatectomy for the various types of HI.

Methods

From 1998 to 2018, 500 patients with HI underwent pancreatectomy: 246 for focal HI, 202 for diffuse HI, 37 for atypical HI (16 for Localized Islet Nuclear Enlargement [LINE], 21 for Beckwith-Wiedemann Syndrome), and 15 for insulinoma. Focal HI neonates were treated with partial pancreatectomy. Patients with diffuse HI who failed medical management underwent near-total (98%) pancreatectomy. Atypical HI patients had pancreatectomies tailored to the PET scan and biopsy findings.

Results

The vast majority of pancreatectomies for focal HI were 

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