5A45

ICD-11 MMS 2026-01

Persistent hyperinsulinaemic hypoglycaemia of infancy

Description

Congenital isolated hyperinsulinism, or Persistent hyperinsulinaemic hypoglycaemia of infancy (PHHI) is defined by an inappropriate oversecretion of insulin by the endocrine pancreas that is responsible for profound hypoglycaemia, which requires aggressive medical and/or surgical treatment to prevent severe and irreversible brain damage. PHHI is a genetically heterogeneous disorder with two types of histological lesions: diffuse (DiPHHI) and focal (FoPHHI) which are clinically indistinguishable.

Index terms

  • Persistent hyperinsulinaemic hypoglycaemia of infancy
  • PHHI - [Persistent hyperinsulinaemic hypoglycaemia of infancy]
  • hypoglycaemia of infancy
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  • infantile hypoglycaemia
  • infantile spontaneous hypoglycaemia
  • Diffuse hyperinsulinism
  • Nesidioblastosis of pancreas
  • Diffuse hyperinsulinism, diazoxide resistant
  • Diffuse hyperinsulinism, diazoxide sensitive
  • Exercise-induced hyperinsulinism
  • Hyperinsulinism due to focal adenomatous hyperplasia

Exclusions from above levels

  • 2C10 – Malignant neoplasm of pancreas
  • 2E92.9 – Benign neoplasm of endocrine pancreas
  • 2F7A.0 – Multiple endocrine neoplasia type 1
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  • 18 – Pregnancy, childbirth or the puerperium
  • http://id.who.int/icd/entity/1004987305 – Transitory endocrine or metabolic disorders specific to fetus or newborn

Sibling codes

  • 5A40 – Intermediate hyperglycaemia
  • 5A41 – Hypoglycaemia without associated diabetes
  • 5A42 – Increased secretion of glucagon
  • 5A43 – Abnormal secretion of gastrin
  • 5A44 – Insulin-resistance syndromes
  • 5A4Y – Other specified disorders of glucose regulation or pancreatic internal secretion
  • 5A4Z – Disorders of glucose regulation or pancreatic internal secretion, unspecified

Foundation entity

Persistent hyperinsulinaemic hypoglycaemia of infancy – http://id.who.int/icd/entity/402589098

Part of: 05: Endocrine, nutritional or metabolic diseases