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Sickle cell anemia

ORPHA:232DiseaseAutosomal recessiveAll ages

Ассоциированные гены (1)

HBB
hemoglobin subunit beta
Disease-causing germline mutation(s) in
OMIM: 141900

Фенотипы (37)

Облигатный (100%)1
HP:0004870Chronic hemolytic anemia
Очень частый (80–99%)3
HP:0001878Hemolytic anemia
HP:0002719Recurrent infections
HP:0012531Pain
Частый (30–79%)13
HP:0000488Retinopathy
HP:0000939Osteoporosis
HP:0000952Jaundice
HP:0001743Abnormality of the spleen
HP:0001891Iron deficiency anemia
HP:0001894Thrombocytosis
HP:0001923Reticulocytosis
HP:0001974Leukocytosis
HP:0002754Osteomyelitis
HP:0010885Avascular necrosis
HP:0011981Pigment gallstones
HP:0012622Chronic kidney disease
HP:0100749Chest pain
Периодический (5–29%)17
HP:0000707Abnormality of the nervous system
HP:0001081Cholelithiasis
HP:0001406Intrahepatic cholestasis
HP:0002092Pulmonary arterial hypertension
HP:0002140Ischemic stroke
HP:0002597Abnormality of the vasculature
HP:0003259Elevated circulating creatinine concentration
HP:0008282Unconjugated hyperbilirubinemia
HP:0011886Hyphema
HP:0011904Persistence of hemoglobin F
HP:0012418Hypoxemia
HP:0025326Retinal arterial occlusion
HP:0025435Increased circulating lactate dehydrogenase concentration
HP:0031090Finger dactylitis
HP:0034336Splenic infarction
HP:0200023Priapism
HP:0200042Skin ulcer
Очень редкий (1–4%)3
HP:0001931Hypochromic anemia
HP:0001935Microcytic anemia
HP:0005518Increased mean corpuscular volume

Эпидемиология (12)

Prevalence at birth
1-5 / 10 000
France
Prevalence at birth
1-5 / 10 000
Germany
Point prevalence
1-5 / 10 000
United States
Prevalence at birth
1-5 / 10 000
Belgium
Prevalence at birth
1-5 / 10 000
United Kingdom
Point prevalence
1-5 / 10 000
Europe
Prevalence at birth
1-5 / 10 000
United States
Prevalence at birth
>1 / 1000
Guadeloupe
Prevalence at birth
>1 / 1000
Martinique
Prevalence at birth
>1 / 1000
Guyana
Prevalence at birth
1-5 / 10 000
Reunion
Annual incidence
1-5 / 10 000
Brazil

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы