" /> Syndromic diarrhoea - CISMeF





ICD-11 code : DA90.0;

Preferred Label : Syndromic diarrhoea;

ICD-11 definition : 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.;

ICD-11 synonym : Phenotypic diarrhoea;

ICD-11 inclusion : Phenotypic diarrhoea; THE - [tricho-hepato-enteric] syndrome;

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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.

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21/12/2025


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