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Surgical treatment of congenital hyperinsulinism
Seminars in Pediatric Surgery, 2020A multidisciplinary approach to patients with congenital hyperinsulinism (HI) can distinguish focal from diffuse HI, localize focal lesions, and permit partial pancreatectomy with cure in almost all focal patients. Surgery does not cure diffuse disease but can help prevent severe hypoglycemia and brain damage. Surgery can be curative for insulinoma and
Scott Adzick
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Congenital Hyperinsulinism and Cardiomyopathy
Fetal and Pediatric Pathology, 2012We read with great interest the article by Bulbul et al [1] appearing in Volume 29, Issue 3, 2010.They presented a babywith hypertrophic cardiomyopathy and hyperinsulinemic hypoglycemia resistant tomedical treatment.Themutation analysis could not been performed in this case, however the disease clearly was due to an ATP-sensitivepotassium (KATP ...
Teoman, Akcay +3 more
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Increased Plasma Incretin Concentrations Identifies a Subset of Patients with Persistent Congenital Hyperinsulinism without KATP Channel Gene Defects [PDF]
Congenital hyperinsulinism causes profound hypoglycemia, which may persist or resolve spontaneously. Among 13 children with congenital hyperinsulinism, elevated incretin hormone concentrations were detected in 2 with atypical, persistent disease.
Raja Padidela +2 more
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Seminars in Fetal and Neonatal Medicine, 2005
Congenital hyperinsulinism is a cause of persistent hypoglycaemia in the neonatal period. It is a heterogeneous disease with respect to clinical presentation, molecular biology, genetic aetiology and response to medical therapy. The clinical heterogeneity may range from severe life-threatening disease to very mild clinical symptoms.
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Congenital hyperinsulinism is a cause of persistent hypoglycaemia in the neonatal period. It is a heterogeneous disease with respect to clinical presentation, molecular biology, genetic aetiology and response to medical therapy. The clinical heterogeneity may range from severe life-threatening disease to very mild clinical symptoms.
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New Tools for Congenital Hyperinsulinism
Clinical Pediatrics, 2021N ...
Da Lozzo, Prisca +8 more
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Congenital Hyperinsulinism: An Historical Perspective
Hormone Research in Paediatrics, 2022Congenital hyperinsulinism is the most common cause of persistent hypoglycemia in neonates, infants, and children. Since the first case descriptions in the 1950s, the field has advanced significantly. It was the development of the insulin radioimmunoassay by Yalow and Berson a decade later that made it possible to demonstrate that this form of ...
Paul S. Thornton +2 more
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Insights in Congenital Hyperinsulinism
2007Congenital hyperinsulinism is characterized by the unregulated secretion of insulin from pancreatic Beta-cells. The inappropriate insulin secretion causes severe and persistent hypoglycaemia, which is a potent cause of brain damage if inappropriately managed.
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A syndrome of congenital hyperinsulinism and hyperammonemia
The Journal of Pediatrics, 1997This report describes two patients from unrelated families with an unusual syndrome of hyperinsulinism plus hyperammonemia. The diagnosis of hyperinsulinism was based on the demonstration of fasting hypoglycemia with inappropriately elevated insulin levels, inappropriately low beta-hydroxybutyrate and free fatty acid levels, and inappropriately large ...
S A, Weinzimer +5 more
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Rare forms of congenital hyperinsulinism
Seminars in Pediatric Surgery, 2011Rare forms of congenital hyperinsulinism (CHI) are caused by mutations in GLUD1 (encoding glutamate dehydrogenase), GCK (encoding glucokinase), HADH (encoding for L-3-hydroxyacyl-CoA dehydrogenase), SLC16A1 (encoding the monocarboxylat transporter 1), HNF4A (encoding hepatocyte nuclear factor 4α) or UCP2 (encoding mitochondrial uncoupling protein 2 ...
Jan, Marquard +4 more
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Investigation and management of congenital hyperinsulinism
British Journal of Nursing, 2009Insulin is a powerful hormone produced by the beta-cells in the pancreas. Its major function is to regulate blood glucose levels, facilitating the transport of glucose into the body’s cells. Congenital hyperinsulinism is characterized by the presence of insulin that is inappropriately high for the concentration of blood glucose. Because high levels of
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