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5A45 Persistent hyperinsulinaemic hypoglycaemia of infancy

International Classification of Diseases for Mortality and Morbidity Statistics, 11th Revision, v2024-01

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.

synonyms

• Persistent hyperinsulinaemic hypoglycaemia of infancy
• PHHI - [Persistent hyperinsulinaemic hypoglycaemia of infancy]
• hypoglycaemia of infancy
• 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

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