DA90.0

ICD-11 MMS 2026-01

Syndromic diarrhoea

Description

Syndromic diarrhoea (SD), also known as phenotypic diarrhoea (PD) or tricho-hepato-enteric syndrome (THE), is a congenital enteropathy presenting with early-onset of severe diarrhoea requiring parenteral nutrition (PN), associated with facial dysmorphism, woolly and poorly pigmented hair and liver disease, with extensive fibrosis or cirrhosis, in about half of the patients.

Index terms

  • Syndromic diarrhoea
  • Phenotypic diarrhoea
  • THE - [tricho-hepato-enteric] syndrome

Inclusions

  • Phenotypic diarrhoea

Exclusions from above levels

  • DE12.0 – Ileostomy malfunction
  • http://id.who.int/icd/entity/1195691020 – Diseases of duodenum
  • 01 – Certain infectious or parasitic diseases
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  • 02 – Neoplasms
  • 05 – Endocrine, nutritional or metabolic diseases
  • 06 – Mental, behavioural or neurodevelopmental disorders
  • 18 – Complications of pregnancy, childbirth and the puerperium
  • 22 – Injury, poisoning or certain other consequences of external causes

Sibling codes

  • DA90.1 – Congenital intestinal transport defect
  • DA90.2 – Congenital intestinal motility disorders
  • DA90.Y – Other specified nonstructural developmental anomalies of small intestine
  • DA90.Z – Nonstructural developmental anomalies of small intestine, unspecified

Foundation entity

Syndromic diarrhoea – http://id.who.int/icd/entity/1470910753

Allowed postcoordination axes

Part of: DA90: Nonstructural developmental anomalies of small intestine › 13: Diseases of the digestive system