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Propionic acidemia

ORPHA:35DiseaseAutosomal recessiveInfancy, Neonatal

Ассоциированные гены (2)

PCCA
propionyl-CoA carboxylase subunit alpha
Disease-causing germline mutation(s) in
OMIM: 232000
PCCB
propionyl-CoA carboxylase subunit beta
Disease-causing germline mutation(s) in
OMIM: 232050

Фенотипы (11)

Очень частый (80–99%)5
HP:0001943Hypoglycemia
HP:0001987Hyperammonemia
HP:0001992Organic aciduria
HP:0002019Constipation
HP:0003353Propionyl-CoA carboxylase deficiency
Частый (30–79%)5
HP:0001249Intellectual disability
HP:0001263Global developmental delay
HP:0002240Hepatomegaly
HP:0010978Abnormality of immune system physiology
HP:0011675Arrhythmia
Периодический (5–29%)1
HP:0001638Cardiomyopathy

Эпидемиология (9)

Prevalence at birth
>1 / 1000
Canada
Annual incidence
1-9 / 100 000
Worldwide
Point prevalence
1-9 / 1 000 000
Europe
Prevalence at birth
1-9 / 1 000 000
Italy
Prevalence at birth
1-9 / 100 000
Germany
Prevalence at birth
1-5 / 10 000
Saudi Arabia
Prevalence at birth
1-9 / 100 000
Japan
Prevalence at birth
1-9 / 100 000
Korea, Republic of
Point prevalence
<1 / 1 000 000
China

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы