ICD-11 classes
05 Endocrine, nutritional or metabolic diseases
Endocrine diseases
Other disorders of glucose regulation or pancreatic...
5A45 — Persistent hyperinsulinaemic hypoglycaemia of infancy

ICD-11 5A45 — Persistent hyperinsulinaemic hypoglycaemia of infancy

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.

The diagnosis includes nothing.

The diagnosis excludes nothing.

It has no clarifying diagnoses.