Kongenitaler Factor VII (SPCA)- Mangel als Ursache einer hämophilieartigen hämorrhagischen Diathese

Jurgens, J

doi:10.1159/000205126

Cited by 18 publications

(2 citation statements)

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“…We showed that, in contrast to certain other cases in the literature (5)(6)(7)(8), the defect in our patient's plasma could not be corrected by Russell viper venom, and that his serum was inactive in the thromboplastin generation test. In this last respect our patient's defect resembled that of some of the previously reported cases (8)(9)(10)(11) but differed from certain others (5,6,(12)(13)(14).…”

supporting

confidence: 79%

Stuart Clotting Defect. II. Genetic Aspects of a `New' Hemorrhagic State

Graham¹,

Barrow²,

Hougie³

1957

J. Clin. Invest.

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supporting

confidence: 79%

Stuart Clotting Defect. II. Genetic Aspects of a `New' Hemorrhagic State

Graham¹,

Barrow²,

Hougie³

1957

J. Clin. Invest.

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“…Although the three terms have been used synonymously since that time, the literature shows no evidence that the identity has been rigorously established. Since the case of Alexander, Goldstein, Landwehr, and Cook (1), 29 other cases (Table I) of a congenital deficiency of these presumably identical factors have been described (5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19)(20)(21)(22)(23)(24). Our present studies indicate that the patient of Crockett, Shotton, Craddock, and Leavell (25) also belongs in the same group.…”

mentioning

confidence: 99%

Stuart Clotting Defect. I. Segregation of an Hereditary Hemorrhagic State from the Heterogeneous Group Heretofore Called “Stable Factor” (SPCA, Proconvertin, Factor VII) Deficiency

Hougie¹,

Barrow²,

Graham³

1957

J. Clin. Invest.

255

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Factor VII deficiency

et al. 1981

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This report describes three patients with factor (F) VII deficiency: two adult siblings and an unrelated 5 1/2-month-old child who succumbed after several central nervous system (CNS) hemorrhages. This event prompted a review of the literature concerning the incidence and characteristics of intracranial hemorrhage in congenital F VII deficiency. Of 138 patients reported to have F VII deficiency, only 75 were considered to have a true deficiency. There was a 1:1 sex distribution with a 19% incidence of consanguinity in the 63 families which these 75 patients represented. CNS hemorrhage occurred in 12 of the 75 proven factor-deficient patients -- an incidence of 16.0%. There was a 1.4:1 female predominance in this group with a 44.4% incidence of consanguinity in their nine families. Except for one patient with hypertension, there was no history of preceding trauma or previous underlying CNS abnormality, though head trauma with a difficult vaginal delivery may have occurred in five infants. Diagnostic lumbar puncture or ventricular tap revealed bloody, xanthochromic cerebrospinal fluid in five. Five patients with F VII deficiency developed a CNS hemorrhage prior to 1 week of age, and none survived. Seven patients older than 1 week of age suffered such an event, and four of these survived. It is concluded that the greatest risk factor for development of CNS hemorrhage is trauma related to the birth process.

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Kongenitaler Factor VII (SPCA)- Mangel als Ursache einer hämophilieartigen hämorrhagischen Diathese

Cited by 18 publications

References 0 publications

Stuart Clotting Defect. II. Genetic Aspects of a `New' Hemorrhagic State

Stuart Clotting Defect. II. Genetic Aspects of a `New' Hemorrhagic State

Stuart Clotting Defect. I. Segregation of an Hereditary Hemorrhagic State from the Heterogeneous Group Heretofore Called “Stable Factor” (SPCA, Proconvertin, Factor VII) Deficiency

Factor VII deficiency

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