Fletcher Factor Deficiency: Report of a New Family
- 24 April 2009
- journal article
- research article
- Published by Wiley in Scandinavian Journal of Haematology
- Vol. 21 (2) , 94-98
- https://doi.org/10.1111/j.1600-0609.1978.tb02498.x
Abstract
Three cases of Fletcher factor deficiency in a family not related to the 6 families already published were studied. In the family described here, 3 of 4 siblings had a Fletcher factor level of less than 1% and the 4th had a level of 46%. The Fletcher factor level in the father was 48% and in the mother, 38%. This suggested an autosomal recessive transmission. Clinically they did not present spontaneous bleedings and only 1 sibling required a unit of blood after an amygdalectomy. Three of the siblings suffered from congenital multiple arthrogryposis, and 2 presented the arthrogryposis together with the Fletcher factor deficiency, a circumstance which could have been favored by the consanguinity of the parents. The fact that the family described here is white and of Mediterranean origin contradicts the idea that there exists a special predisposition among members of the black race for this disease.Keywords
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