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Vitamin B12-unresponsive methylmalonic acidemia type mut0

ORPHA:289916Clinical subtypeAutosomal recessiveInfancy, Neonatal

Ассоциированные гены (1)

MMUT
methylmalonyl-CoA mutase
Disease-causing germline mutation(s) (loss of function) in
OMIM: 609058

Фенотипы (22)

Очень частый (80–99%)4
HP:0001254Lethargy
HP:0001259Coma
HP:0001510Growth delay
HP:0002017Nausea and vomiting
Частый (30–79%)6
HP:0001249Intellectual disability
HP:0001263Global developmental delay
HP:0001252Hypotonia
HP:0001873Thrombocytopenia
HP:0002098Respiratory distress
HP:0002240Hepatomegaly
Периодический (5–29%)12
HP:0000083Renal insufficiency
HP:0000124Renal tubular dysfunction
HP:0000648Optic atrophy
HP:0001266Choreoathetosis
HP:0001332Dystonia
HP:0001733Pancreatitis
HP:0001875Decreased total neutrophil count
HP:0001903Anemia
HP:0001987Hyperammonemia
HP:0002072Chorea
HP:0004374Hemiplegia/hemiparesis
HP:0100806Sepsis

Эпидемиология (1)

Point prevalence
Unknown
Worldwide

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы