loading...| Preferred Name | letterer-Siwe disease | |
| Synonyms |
L-S disease histiocytosis X, acute disseminated multifocal multisystem Langerhans cell histiocytosis acute and disseminated Langerhans cell histiocytosis acute disseminated Langerhans cell histiocytosis letterer-Siwe disease |
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| Definitions |
A multifocal, multisystem form of Langerhans-cell histiocytosis. There is involvement of multiple organ systems including the bones, skin, liver, spleen, and lymph nodes. Patients are usually infants presenting with fever, hepatosplenomegaly, lymphadenopathy, bone and skin lesions, and pancytopenia. |
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| ID |
http://purl.obolibrary.org/obo/MONDO_0009519 |
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| database_cross_reference |
MedDRA:10024265 icd11.foundation:1827474596 OMIM:246400 MEDGEN:7311 NCIT:C3160 Orphanet:99870 ICDO:9754/3 UMLS:C0023381 |
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| definition |
A multifocal, multisystem form of Langerhans-cell histiocytosis. There is involvement of multiple organ systems including the bones, skin, liver, spleen, and lymph nodes. Patients are usually infants presenting with fever, hepatosplenomegaly, lymphadenopathy, bone and skin lesions, and pancytopenia. |
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| excluded_from_qc_check |
http://purl.obolibrary.org/obo/mondo/sparql/qc/mondo/qc-omim-subsumption.sparql |
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| has_exact_synonym |
multifocal multisystem Langerhans cell histiocytosis acute and disseminated Langerhans cell histiocytosis acute disseminated Langerhans cell histiocytosis letterer-Siwe disease |
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| has_related_synonym |
L-S disease histiocytosis X, acute disseminated |
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| id |
MONDO:0009519 |
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| in_subset | ||
| label |
letterer-Siwe disease |
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| notation |
MONDO:0009519 |
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| prefLabel |
letterer-Siwe disease |
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| skos_closeMatch | ||
| skos_exactMatch |
http://linkedlifedata.com/resource/umls/id/C0023381 http://purl.obolibrary.org/obo/mondo/sources/icd11foundation/1827474596 |
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| treeView | ||
| subClassOf |