Nesidioblastosis

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Classification according to ICD-10
D13.7 benign nesidioblastosis
C25.4 malignant nesidioblastosis
ICD-10 online (WHO version 2019)

Familial nesidioblastosis , and islet cell hyperplasia or persistent hyperinsulinemic hypoglycemia of infants ( PHHI of persistent hyperinsulinemic hypoglycemia of infancy ) called, is an inherited islet cell - hyperplasia (enlargement) of the pancreas that are already in newborns in severe hypoglycemia (low blood sugar ) leads.

In children, nesidioblastosis is the most common cause of hypoglycaemia.

etiology

The genetic defect causing the disease is located on chromosome 11 gene locus p15.1. The genetic defect both autosomal - recessive as autosomal dominant inherited. The disease is genetically very heterogeneous and occurs in two pathohistologically different forms: diffuse and focal nesidioblastosis. The two forms cannot be clinically distinguished from one another. The focal form is characterized by focal adenomatous islet cell hyperplasia , while in the diffuse form in the islets of Langerhans all beta cells are hypertrophied - that is, enlarged.

therapy

The drug of choice for treatment is diazoxide . However, much of the patient does not speak to diazoxide, nor to nifedipine or somatostatin - analogue octreotide , or a leucine at-reduced diet.

Individual evidence

  1. ↑ a b Orpha.net: Hypoglycemia, hyperinsulinemic persistent of childhood. Retrieved July 3, 2008

See also

literature

  • A. Raffel et al .: Diffuse nesidioblastosis as a cause of hyperinsulinemic hypoglycemia in adults: a diagnostic and therapeutic challenge. In: Surgery 141/2007, pp. 179-184. PMID 17263973
  • T. Clancy et al: Post-gastric bypass hyperinsulinism with nesidioblastosis: subtotal or total pancreatectomy may be needed to prevent recurrent hypoglycemia. In: Journal of Gastrointestinal Surgery 10/2006, pp. 1116-1119. PMID 16966030
  • A. Raffel et al .: Hyperinsulinemic hypoglycemia due to adult nesidioblastosis in insulin-dependent diabetes. In: World J Gastroenterol 12/2006, pp. 7221-7224. PMID 17131493

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