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Congenital dyserythropoietic anemia type III

ORPHA:98870DiseaseAutosomal dominant, Autosomal recessiveAll ages

Ассоциированные гены (2)

KIF23
kinesin family member 23
Disease-causing germline mutation(s) in
OMIM: 605064
RACGAP1
Rac GTPase activating protein 1
Disease-causing germline mutation(s) in
OMIM: 604980

Фенотипы (20)

Очень частый (80–99%)3
HP:0001903Anemia
HP:0004447Poikilocytosis
HP:0011273Anisocytosis
Частый (30–79%)8
HP:0002904Hyperbilirubinemia
HP:0003452Increased serum iron
HP:0005518Increased mean corpuscular volume
HP:0012130Abnormality of cells of the erythroid lineage
HP:0012378Fatigue
HP:0025035Abnormal proerythroblast morphology
HP:0025196Increased total iron binding capacity
HP:0025354Abnormal cellular phenotype
Периодический (5–29%)8
HP:0001877Abnormal erythrocyte morphology
HP:0000225Gingival bleeding
HP:0000980Pallor
HP:0002249Melena
HP:0002315Headache
HP:0002910Elevated circulating hepatic transaminase concentration
HP:0011891Post-partum hemorrhage
HP:0030140Oral cavity bleeding
Очень редкий (1–4%)1
HP:0004322Short stature

Эпидемиология (2)

Cases/families
Worldwide
Point prevalence
<1 / 1 000 000
Worldwide

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы