ICD-11 code : 5A45;
Preferred Label : Persistent hyperinsulinaemic hypoglycaemia of infancy;
ICD-11 definition : Congenital isolated hyperinsulinism, or Persistent hyperinsulinemic hypoglycemia of
infancy (PHHI) is defined by an inappropriate oversecretion of insulin by the endocrine
pancreas that is responsible for profound hypoglycemia, 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.;
ICD-11 synonym : PHHI - [Persistent hyperinsulinaemic hypoglycaemia of infancy]; Persistent hyperinsulinaemic hypoglycaemia of infancy;
ICD-11 acronym : PHHI;
Origin ID : 402589098;
Automatic exact mappings (from CISMeF team)
- Currated CISMeF NLP mapping
- ICD-10 Mapping
Congenital isolated hyperinsulinism, or Persistent hyperinsulinemic hypoglycemia of
infancy (PHHI) is defined by an inappropriate oversecretion of insulin by the endocrine
pancreas that is responsible for profound hypoglycemia, 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.
