Delayed Resolution of Feeding Problems in Patients With Congenital Hyperinsulinism

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Feeding Problems Are Persistent in Children with Severe Congenital Hyperinsulinism

BACKGROUND Congenital hyperinsulinism (CHI) is a rare but severe disorder of hypoglycemia in children, often complicated by brain injury. In CHI, the long-term prevention of hypoglycemia is dependent on reliable enteral intake of glucose. However, feeding problems (FPs) often impede oral glucose delivery, thereby complicating the management of hypoglycemia. FPs have not been systematically char...

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[Congenital hyperinsulinism].

In the last five years, our knowledge about the heterogenous syndrome of congenital hyperinsulinism (HI) has expanded explosively. HI may be familiar or sporadic, mild or severe, transitory or persistent, and histologically focal or diffuse. At least 63 disease-causing mutations have been found in the genes for the beta cell's ATP-dependent potassium channel, whose elements are the sulphonylure...

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Long-term follow-up of 114 patients with congenital hyperinsulinism.

BACKGROUND The term congenital hyperinsulinism (CHI) comprises a group of different genetic disorders with the common finding of recurrent episodes of hyperinsulinemic hypoglycemia. OBJECTIVE To evaluate the clinical presentation, diagnostic criteria, treatment and long-term follow-up in a large cohort of CHI patients. PATIENTS The data from 114 patients from different hospitals were obtain...

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Clinical and molecular characterisation of 300 patients with congenital hyperinsulinism

BACKGROUND Congenital hyperinsulinism (CHI) is a clinically heterogeneous condition. Mutations in eight genes (ABCC8, KCNJ11, GLUD1, GCK, HADH, SLC16A1, HNF4A and HNF1A) are known to cause CHI. AIM To characterise the clinical and molecular aspects of a large cohort of patients with CHI. METHODOLOGY Three hundred patients were recruited and clinical information was collected before genotypi...

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ژورنال

عنوان ژورنال: Frontiers in Endocrinology

سال: 2020

ISSN: 1664-2392

DOI: 10.3389/fendo.2020.00143