Congenital Factor XIII Deficiency: Observation of Two New Cases in the Newborn Period

We treated two cases of fibrin stabilizing factor (FSF) deficiency in the newborn period and an additional case at 6 months of age. The presenting symptom of all these patients was bleeding from the cord stump. The parents and a sibling of the first patient were shown to be heterozygotes by assays for this factor. The genetics of this factor deficiency appeared to be autosomal recessive in all. When cord stump bleeding occurs in the early newborn period, especially in babies who have received vitamin K, factor XIII deficiency should be remembered.

[1]  H. Godal.  An inhibitor to fibrin stabilizing factor (FSF, factor XIII). , 2009, Scandinavian journal of haematology.

[2]  Mary Hanna.  CONGENITAL DEFICIENCY OF FACTOR XIII: REPORT OF A FAMILY FROM NEWFOUNDLAND WITH ASSOCIATED MILD DEFICIENCY OF FACTOR XII , 1970, Pediatrics.

[3]  Ş. Ozsoylu,et al.  Congenital deficiency of factor XIII. , 1969, The Turkish journal of pediatrics.

[4]  O. Egeberg.  New Families with Hereditary Hemorrhagic Trait due to Deficiency of Fibrin Stabilizing Factor (F. XIII) , 1968, Thrombosis and Haemostasis.

[5]  M. Hilden,et al.  Treatment of Factor XIII Deficiency with Cryoprecipitate , 1968, Thrombosis and Haemostasis.

[6]  Ç. Altay,et al.  Factor XIII deficiency: Concentrations inrelatives of patients and in normal infants , 1968 .

[7]  A. Britten.  Congenital deficiency of factor 13 (fibrin-stabilizing factor): Report of a case and review of the literature. , 1967, The American journal of medicine.

[8]  H. Strauss,et al.  Hemophilia in the first year of life. , 1966, The New England journal of medicine.

[9]  M. Rikover,et al.  Factor 13 deficiency with severe hemorrhagic diathesis. , 1966, Blood.

[10]  Hampton Jw,et al.  DEFECTIVE FIBRINASE ACTIVITY IN TWO BROTHERS. , 1965 .

[11]  B. Morse,et al.  PLASMA FIBRIN STABILIZING FACTOR ACTIVITY IN VARIOUS DISEASES. , 1964, Blood.

[12]  J. Graham,et al.  Diagnosis and control of the hemophilioid states with the partial thromboplastin time (PTT) test. , 1958, American journal of clinical pathology.

[13]  A. S. Douglas,et al.  The Thromboplastin Generation Test , 1953, Journal of clinical pathology.

[14]  O. Ratnoff,et al.  A new method for the determination of fibrinogen in small samples of plasma. , 1951, The Journal of laboratory and clinical medicine.

[15]  L. Lorand,et al.  Inheritance of deficiency of fibrin-stabilizing factor (factor 13). , 1970, American journal of human genetics.

[16]  G. Broccia.  Haemorrhagic disease due to hereditary deficiency of factor XIII. Description of a family. , 1970, Acta haematologica.

[17]  F. Duckert,et al.  A Hitherto Undescribed Congenital Haemorrhagic Diathesis Probably Due to Fibrin Stabilizing Factor Deficiency , 1961, Thrombosis and Haemostasis.