Congenital Deficiency of Fibrin-Stabilizing Factor (Factor XIII)

Arends, Tulio; Guevara, J M; Rangel, J R

doi:10.1159/000208923

Acta Haematol

1968

DOI: 10.1159/000208923

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Congenital Deficiency of Fibrin-Stabilizing Factor (Factor XIII)

Tulio Arends¹,

J M Guevara²,

J R Rangel³

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Cited by 6 publications

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“…In the same year L orand et al [18] published data con cerning the inheritance of the condition and referred to 44 patients be longing to 30 families. A few additional cases not considered in such re views have been published before and in 1970, and a few more after that year [2,3,5,15,17]. In 1972, R atnoff and Steimberg [21] gathered 63 1 This study was supported in part by a grant from the CNR, Rome (CT 75.00992.04) and by a grant from the Anna Villa Rusconi Foundation, Varese, Italy.…”

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confidence: 97%

Congenital Deficiency of Factor XIII with Normal Subunit S and Lack of Subunit A

Girolami¹,

Burul²,

Sticchi³

1977

Acta Haematol

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Two sisters born from a nonconsanguineous marriage were found to have congenital factor XIII deficiency. In the electroimmunoassay system, using an anti-subunit S antiserum, two distinct peaks or rockets were seen in normal plasma and serum whereas only one peak was present in the propositae plasma or serum. In the bidimensional immunoelectrophoresis system, using the anti-subunit S antiserum, two major peaks were seen in normal plasma whereas only one peak was seen in the propositae plasma. Using an anti-subunit A antiserum no peak or precipitate was seen in our propositae in the electroimmunoassay or in the bidimensional immunoelectrophoresis systems. Both the parents and the children of our two propositae showed a normal coagulation pattern. Therefore, the heredity appears to be autosomal recessive. These data indicate that the defect is characterized by a normal factor XIII subunit S (support) and a lack of factor XIII subunit A (activity)

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mentioning

confidence: 97%

Congenital Deficiency of Factor XIII with Normal Subunit S and Lack of Subunit A

Girolami¹,

Burul²,

Sticchi³

1977

Acta Haematol

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Spontaneous intracerebral hematoma in an adolescent with factor XIII deficiency. Case report

Giraldi¹,

Creta²,

Martini³

et al. 1989

Ital J Neuro Sci

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Spontaneous intracerebral hematoma in an adolescent with factor XIII deficiency. Case report

Giraldi¹,

Creta²,

Martini³

et al. 1989

Ital J Neuro Sci

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Congenital Deficiency of Fibrin-Stabilizing Factor (Factor XIII)

Cited by 6 publications

References 8 publications

Congenital Deficiency of Factor XIII with Normal Subunit S and Lack of Subunit A

Congenital Deficiency of Factor XIII with Normal Subunit S and Lack of Subunit A

Spontaneous intracerebral hematoma in an adolescent with factor XIII deficiency. Case report

Spontaneous intracerebral hematoma in an adolescent with factor XIII deficiency. Case report

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