{"orpha":497188,"name":"Diffuse intrinsic pontine glioma","type":"Disease","group":"Disorder","status":"inactive","flags":["Deprecated entity"],"synonyms":["DIPG"],"definition":null,"note":"This entity has been excluded from the Orphanet nomenclature of rare diseases and moved to Diffuse midline glioma, H3 K27-altered","url":"https://raremap.alftech.space/d/ORPHA-497188/","orphanet_url":"https://www.orpha.net/en/disease/detail/497188","names":{"fr":{"name":"Gliome infiltrant du tronc cérébral","synonyms":["DIPG"]},"de":{"name":"Diffuses intrinsisches Ponsgliom","synonyms":["DIPG"]},"es":{"name":"Glioma pontino intrínseco difuso","synonyms":["GPID"]},"it":{"name":"Glioma pontino intrinseco diffuso"},"nl":{"name":"Diffuus intrinsiek ponsglioom","synonyms":["DIPG"]},"pt":{"name":"Glioma pontino intrínseco difuso"},"pl":{"name":"Rozsiany glejak wewnątrz mostu mózgu","synonyms":["DIPG"]},"cs":{"name":"Difuzní intrinsický pontinní gliom","synonyms":["DIPG"]},"uk":{"name":"Дифузна внутрішня гліома мозолистого тіла","synonyms":["DIPG"],"definition":"A rare glial tumor characterized by a highly aggressive, diffusely infiltrative pontine lesion generally occurring in children, affecting local nerve fiber tracts and spreading contiguously to involve adjacent structures, but also metastasizing within the central nervous system. Patients mostly present with a short history of symptoms, typically including the classic triad of multiple cranial neuropathies, long tract signs, and ataxia. Signs and symptoms of increased intracranial pressure may present due to obstructive hydrocephalus. Prognosis is poor and not related to histological grade."},"zh":{"name":"弥漫性真性脑桥神经胶质","synonyms":["DIPG"],"definition":"一种罕见的胶质瘤，其特点是侵袭性强，弥漫性浸润性的脑桥病变，一般发生在儿童身上，影响局部神经纤维束，并连续蔓延累及邻近结构，也可在中枢神经系统内转移。患者多表现为病史较短的症状，典型的包括典型性多发性颅神经病变、长束征和共济失调三联征。由于阻塞性脑积水可出现颅内压增高的体征和症状。预后不良且与组织学分级无关。"},"tr":{"name":"Difüz intrinsik pontin gliom","synonyms":["DIPG"]}},"xrefs":[{"source":"MONDO","code":"0006033","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D000080443","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C2986658","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"13075","qualifier":"E","icd_relation":null,"validated":true}],"associations":[{"type":"Moved to","orpha":723358,"name":"Diffuse midline glioma, H3 K27-altered"}],"incoming_associations":[],"preferential_parent":null,"parents":[],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[],"phenotypes":null}