" /> Syndromic diarrhoea - CISMeF





ICD-11 code : DA90.0;

Preferred Label : Syndromic diarrhoea;

ICD-11 definition : Syndromic diarrhea (SD), also known as phenotypic diarrhea (PD) or tricho-hepato-enteric syndrome (THE), is a congenital enteropathy presenting with early-onset of severe diarrhea 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 inclusion : Phenotypic diarrhoea; THE - [tricho-hepato-enteric] syndrome;

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Syndromic diarrhea (SD), also known as phenotypic diarrhea (PD) or tricho-hepato-enteric syndrome (THE), is a congenital enteropathy presenting with early-onset of severe diarrhea 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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07/05/2025


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