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Atypical teratoid rhabdoid tumor

ORPHA:99966DiseaseNot applicableAntenatal, Childhood, Infancy, Neonatal

Ассоциированные гены (1)

SMARCB1
SWI/SNF related BAF chromatin remodeling complex subunit B1
Disease-causing somatic mutation(s) in
OMIM: 601607

Фенотипы (16)

Очень частый (80–99%)4
HP:0000737Irritability
HP:0000741Apathy
HP:0002017Nausea and vomiting
HP:0100836Malignant neoplasm of the central nervous system
Частый (30–79%)9
HP:0000238Hydrocephalus
HP:0000256Macrocephaly
HP:0001250Seizure
HP:0001251Ataxia
HP:0001324Muscle weakness
HP:0001376Limitation of joint mobility
HP:0002076Migraine
HP:0004372Reduced consciousness/confusion
HP:0004374Hemiplegia/hemiparesis
Периодический (5–29%)3
HP:0002514Cerebral calcification
HP:0006824Cranial nerve paralysis
HP:0100021Cerebral palsy

Эпидемиология (2)

Point prevalence
Unknown
Worldwide
Annual incidence
1-9 / 100 000
Austria

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы