Hypofibrinogenaemia is the commonest congenital fibrinogen abnormality in north India.
Congenital abnormalities of fibrinogen are rare disorders and all the cases reported in the literature indicate that the incidence of afibrinogenaemia is much higher than hypofibrinogenaemia. Of the total of 20 cases reported from other parts of India only one was congenital hypofibrinogenaemia. In contrast, the present study showed eight patients with congenital hypofibrinogenaemia among a total of nine unrelated North Indian patients with a fibrinogen abnormality. This disproportionately high incidence of hypofibrinogenaemia suggests the existence of a distinct genetic defect in the North Indian population.
Pati
H P
HP
Department of Haematology, All India Institute of Medical Sciences, New Delhi.
Sharma
M C
MC
Ahlawat
S
S
Choudhry
V P
VP
D000293:Adolescent; D000347:Afibrinogenemia / Q000097:blood / Q000151:congenital / Q000453:epidemiology* / Q000235:genetics; D001780:Blood Coagulation Tests; D002648:Child; D002675:Child, Preschool; D005260:Female; D005340:Fibrinogen / Q000235:genetics; D006474:Hemorrhagic Disorders / Q000453:epidemiology / Q000235:genetics; D006801:Humans; D015994:Incidence; D007194:India / Q000453:epidemiology; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D010976:Platelet Count
D005340:Fibrinogen