Persistent hyperinsulinaemic hypoglycaemia of infancy (Q39821): Difference between revisions
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Hiperinsulinismo congênito isolado ou Hipoglicemia hiperinsulinêmica persistente da infância é definida por um excesso de secreção de insulina inapropriado pelo pâncreas endócrino que é responsável por hipoglicemia profunda, que requer tratamento médico e/ou cirúrgico agressivo para prevenir severos e irreversíveis danos cerebrais. É um transtorno geneticamente heterogêneo com dois tipos de lesões histológicas: difuso e focal, que são clinicamente indistinguíveis. | |||
| description / en | description / en | ||
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. | |||
Revision as of 05:22, 13 August 2026
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.
| Language | Label | Description | Also known as |
|---|---|---|---|
| default for all languages | 5A45 |
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| English | 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. |
