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syndroom van longfibrose, leverhyperplasie en beenmerghypoplasie (aandoening)
syndroom van longfibrose, leverhyperplasie en beenmerghypoplasie
Pulmonary fibrosis, hepatic hyperplasia, bone marrow hypoplasia syndrome
Trimorphic syndrome
A rare disease with three inherited morbidities; idiopathic pulmonary fibrosis, hepatic nodular regenerative hyperplasia leading to portal hypertension and thrombocytopenia due to bone marrow hypoplasia. The condition is associated with 100% mortality.
Id723829000
StatusPrimitive
Associated morphologyhypoplasie
Finding sitestructuur van beenmerg
Associated morphologyfibrose
Finding sitestructuur van long
Clinical coursechronisch
DHD Diagnosis thesaurus reference set
RIVM authorized national diagnosis thesaurus to ICD10 complex mapping reference set
TargetJ84.1
TermOverige interstitiƫle longziekten met fibrose
TargetQ44.7
TermOverige congenitale misvormingen van lever
TargetD61.9
TermAplastische anemie, niet gespecificeerd
SNOMED CT to Orphanet simple map210136
SNOMED CT to ICD-10 extended map
TargetJ84.1
RuleTRUE
AdviceALWAYS J84.1 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
CorrelationSNOMED CT source code to target map code correlation not specified
TargetQ44.7
RuleTRUE
AdviceALWAYS Q44.7
CorrelationSNOMED CT source code to target map code correlation not specified
TargetD61.9
RuleTRUE
AdviceALWAYS D61.9
CorrelationSNOMED CT source code to target map code correlation not specified