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. |
| Id | 723829000 | Status | Primitive |
DHD Diagnosis thesaurus reference set |
RIVM authorized national diagnosis thesaurus to ICD10 complex mapping reference set | Target | J84.1 | Term | Overige interstitiƫle longziekten met fibrose |
Target | Q44.7 | Term | Overige congenitale misvormingen van lever |
Target | D61.9 | Term | Aplastische anemie, niet gespecificeerd |
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SNOMED CT to Orphanet simple map | 210136 |
SNOMED CT to ICD-10 extended map | Target | J84.1 | Rule | TRUE | Advice | ALWAYS J84.1 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION | Correlation | SNOMED CT source code to target map code correlation not specified |
Target | Q44.7 | Rule | TRUE | Advice | ALWAYS Q44.7 | Correlation | SNOMED CT source code to target map code correlation not specified |
Target | D61.9 | Rule | TRUE | Advice | ALWAYS D61.9 | Correlation | SNOMED CT source code to target map code correlation not specified |
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