ArticleJournal of pediatric surgery2013
Pancreatic surgery in infants with Beckwith-Wiedemann syndrome and hyperinsulinism.
Article in Journal of pediatric surgery, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT01468454 (A Phase II Safety and Efficacy Study of 18F-L-Fluoro-DOPA PET/CT Scan Localization of Focal Pancreatic Lesions in Children With Hyperinsulinemic Hypoglycemia), which is not on this map. Cited by 16 papers.
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A Phase II Safety and Efficacy Study of 18F-L-Fluoro-DOPA PET/CT Scan Localization of Focal Pancreatic Lesions in Children With Hyperinsulinemic Hypoglycemia
Who cites it
16 citing papers in PubMed, 39 citations in OpenAlex.
- Determinants of hyperinsulinism severity in children with Beckwith-Wiedemann syndrome.The Journal of clinical endocrinology and metabolism · 2026Article
- Functional evaluation of pancreatic islets from patients with Beckwith-Wiedemann syndrome and congenital hyperinsulinism.The Journal of clinical endocrinology and metabolism · 2026Article
- Genotype-histotype-phenotype correlations in hyperinsulinemic hypoglycemia.Histology and histopathology · 2024Review
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- Article
- Syndromic forms of congenital hyperinsulinism.Frontiers in endocrinology · 2023Review
- Coexistence of paternally-inheritedInternational journal of pediatric endocrinology · 2020Article
- Preclinical and Clinical Epigenetic-Based Reconsideration of Beckwith-Wiedemann Syndrome.Frontiers in genetics · 2020Review
- Diagnosis and treatment of pancreatoblastoma in children: a retrospective study in a single pediatric center.Pediatric surgery international · 2019Article
- Beckwith-Wiedemann syndrome in diverse populations.American journal of medical genetics. Part A · 2019Article
- Surgical treatment of congenital hyperinsulinism: Results from 500 pancreatectomies in neonates and children.Journal of pediatric surgery · 2019Article
- Nutrient sensing in pancreatic islets: lessons from congenital hyperinsulinism and monogenic diabetes.Annals of the New York Academy of Sciences · 2018Review
- Pancreatoblastoma With Metastatic Retroperitoneal Lymph Node and PET/CT.Clinical nuclear medicine · 2017Article
- Congenital hyperinsulinism in children with paternal 11p uniparental isodisomy and Beckwith-Wiedemann syndrome.Journal of medical genetics · 2016Article
- A novel variant in CDKN1C is associated with intrauterine growth restriction, short stature, and early-adulthood-onset diabetes.The Journal of clinical endocrinology and metabolism · 2014Article
- Successful use of long acting octreotide in two cases with Beckwith-Wiedemann syndrome and severe hypoglycemia.International journal of pediatric endocrinology · 2014Article
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Authors and funding
6 authors at 1 institution in 1 country.
Funding
Abstract
purposeTo present our experience in the care of infants with Beckwith-Wiedemann syndrome (BWS) who required pancreatectomy for the management of severe Congenital Hyperinsulinism (HI).
methodsWe did a retrospective chart review of patients with BWS who underwent pancreatectomy between 2009 and 2012.
resultsFour patients with BWS and severe HI underwent pancreatectomy, 3 females and one male. Eight other BWS patients with HI could be managed medically. The diagnosis of BWS was established by the presence of mosaic 11p15 loss of heterozygosity and uniparental disomy in peripheral blood and/or pancreatic tissue. All patients had hypoglycemia since birth that did not respond to medical management with diazoxide or octreotide, and required glucose infusion rates of up to 30 mg/kg/min. Preoperative 18-F-DOPA PET/CT scans showed diffuse uptake of the radiotracer throughout an enlarged pancreas in three patients and a normal sized pancreas with a large area of focal uptake in the pancreatic body in one patient. None of the patients had mutations in the ABCC8 or KCNJ1 genes that are typically associated with diazoxide-resistant HI. Age at surgery was 1, 2, 4, and 12 months and the procedures were 85%, 95%, 90%, and 75% pancreatectomy, respectively, with the pancreatectomy extent tailored to HI severity. Pathologic analysis revealed marked diffuse endocrine proliferation throughout the pancreas that occupied up to 80% of the parenchyma with scattered islet cell nucleomegaly. One patient had a small pancreatoblastoma in the pancreatectomy specimen. The HI improved in all cases after the pancreatectomy, with patients being able to fast safely for more than 8 h. All patients are under close surveillance for embryonal tumors. One patient developed a hepatoblastoma at age 2.
conclusionThe pathophysiology of HI in BWS patients is likely multifactorial and is associated with a dramatic increase in pancreatic endocrine tissue. Severe cases of HI that do not respond to medical therapy improve when the mass of endocrine tissue is reduced by subtotal or near-total pancreatectomy.
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