auto-immuunenteropathie	congenitale enteropathie	congenitale immunodeficiëntie	endocriene auto-immuunziekte	hereditaire aandoening van endocrien systeem	hereditaire aandoening van immuunsysteem	hereditaire aandoening van spijsverteringsstelsel	immunodeficiëntie bij chromosomale afwijking	X-gebonden hereditaire recessieve aandoening
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syndroom van X-gebonden immunodisregulatie, polyendocrinopathie en enteropathie (aandoening)
	syndroom van X-gebonden immunodisregulatie, polyendocrinopathie en enteropathie
	IPEX-syndroom X-gebonden auto-immuunenteropathie auto-immuunenteropathie type 1 X-gebonden immuundisregulatie-polyendocrinopathie-enteropathie-syndroom
	X-linked immune dysregulation, polyendocrinopathy, enteropathy syndrome
	Autoimmune enteropathy type 1
	A rare immunodysregulatory disease characterized by refractory diarrhea, endocrinopathies, cutaneous involvement, and infections.

Id	724276006
Status	Primitive

Associated with

chromosomale aandoening

Finding site	structuur van endocrien systeem
Occurrence	congenitaal
Pathological process	auto-immuunproces

Finding site	structuur van intestinum tenue
Occurrence	congenitaal
Pathological process	auto-immuunproces

Global Patient Set

DHD Diagnosis thesaurus reference set

RIVM authorized national diagnosis thesaurus to ICD10 complex mapping reference set

Target	E31.0
Term	Auto-immune polyglandulaire insufficiëntie

SNOMED CT to Orphanet simple map

37042

SNOMED CT to ICD-10 extended map

Target	E31.0
Rule	TRUE
Advice	ALWAYS E31.0 \| POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
Correlation	SNOMED CT source code to target map code correlation not specified