Congenital factor XIII deficiency. A family report.
Four sisters born to consanguineous muslim parents with a bleeding disorder since birth are presented. They also had prolonged umbilical cord bleeding and history of delayed wound healing. Since childhood, they have been developing spontaneous ecchymotic spots. Three out of four sisters had Factor XIII deficiency. Their mother's sisters, who have been developing ecchymotic spots were found to have normal clot stability and Factor XIII levels. Family study indicates autosomal recessive mode of inheritance of the congenital Factor XIII deficiency.
Thavaraj
V
V
Department of Haematology, All India Institute of Medical Sciences, New Delhi.
Singh
Y
Y
Choudhary
V P
VP
Saraya
A K
AK
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003241:Consanguinity; D005177:Factor XIII Deficiency / Q000151:congenital* / Q000235:genetics; D005260:Female; D005808:Genes, Recessive; D006801:Humans; D010375:Pedigree