aandoening van mucosa van tractus gastrointestinalis	aandoening van weke delen van romp	congenitale afwijking van darm
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congenitale enterocyt-heparansulfaatdeficiëntie (aandoening)
	congenitale enterocyt-heparansulfaatdeficiëntie
	Congenital enterocyte heparan sulfate deficiency
	Disease that is characterized by massive enteric protein loss, secretory diarrhea and intolerance to enteral feeds during the first few weeks of life. It has been described in three male infants. Histochemical studies revealed a complete absence of enterocyte heparan sulfate. All three infants required total parenteral nutrition and repeated albumin infusions.

Id	725591002
Status	Primitive

Associated morphology	morfologische afwijking
Finding site	structuur van tunica mucosa intestini
Occurrence	congenitaal
Pathological process	proces van pathologische ontwikkeling

DHD Diagnosis thesaurus reference set

RIVM authorized national diagnosis thesaurus to ICD10 complex mapping reference set

Target	P78.3
Term	Niet-infectieuze neonatale diarree

SNOMED CT to Orphanet simple map

103910

SNOMED CT to ICD-10 extended map

Target	P78.3
Rule	TRUE
Advice	ALWAYS P78.3 \| POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
Correlation	SNOMED CT source code to target map code correlation not specified