| syndroom van teleangiëctasie, polycytemie, monoklonale gammopathie, perirenale vochtophoping en intrapulmonale shunting (aandoening) | | syndroom van teleangiëctasie, polycytemie, monoklonale gammopathie, perirenale vochtophoping en intrapulmonale shunting | | syndroom van telangiectasia, erytrocytose, monoklonale gammopathie, perinefritische vochtophoping en intrapulmonale shunting TEMPI-syndroom
| | TEMPI syndrome | | Telangiectasia, erythrocytosis, monoclonal gammopathy, perinephric fluid collections and intrapulmonary shunting syndrome
| | TEMPI syndrome is a rare multi-systemic disease characterized by the presence of Telangiectasias, Erythrocytosis with elevated erythropoietin levels, Monoclonal gammopathy, Perinephric-fluid collections, and Intrapulmonary shunting. |
| | Id | 718614004 | | Status | Primitive |
| DHD Diagnosis thesaurus reference set |
| RIVM authorized national diagnosis thesaurus to ICD10 complex mapping reference set | | Target | Q87.8 | | Term | Overige gespecificeerde congenitale gestoorde-ontwikkelingssyndromen, niet elders geclassificeerd |
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| SNOMED CT to Orphanet simple map | 284227 |
| SNOMED CT to ICD-10 extended map | | Target | D75.1 | | Rule | TRUE | | Advice | ALWAYS D75.1 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION | | Correlation | SNOMED CT source code to target map code correlation not specified |
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