aandoening van mucosa van tractus gastrointestinalis	aandoening van weke delen van romp	afwijkend weefsel van mucosa	afwijkend weefsel van weke delen	autosomaal recessieve hereditaire aandoening	congenitale afwijking van duodenum	congenitale afwijking van jejunum	congenitale dysplasie	hereditaire aandoening van spijsverteringsstelsel	hereditaire ontwikkelingsstoornis
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congenitale intestinale epitheliale dysplasie (aandoening)
	congenitale intestinale epitheliale dysplasie
	Intestinal epithelial dysplasia
	Congenital epithelial dysplasia of intestine Congenital tufting enteropathy Tufting enteropathy
	A congenital enteropathy presenting with early-onset severe intractable diarrhea sometimes causing irreversible intestinal failure. Infants develop a watery diarrhea within the first days after birth and the diarrhea persists in spite of bowel rest and parenteral nutrition. Some infants are reported to have associated choanal, rectal or esophageal atresia. Autosomal recessive transmission but the causative gene has not been yet identified.

Id	715669000
Status	Primitive

Associated morphology	epitheliale dysplasie
Finding site	structuur van mucosa van jejunum
Occurrence	congenitaal
Pathological process	proces van pathologische ontwikkeling

Associated morphology	epitheliale dysplasie
Finding site	structuur van mucosa van duodenum
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	Q43.8
Term	Overige gespecificeerde congenitale misvormingen van darm

SNOMED CT to Orphanet simple map

92050

SNOMED CT to ICD-10 extended map

Target	Q43.8
Rule	TRUE
Advice	ALWAYS Q43.8
Correlation	SNOMED CT source code to target map code correlation not specified