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An infant with congenital homozygous factor XIII deficiency demonstrated a severe retroperitoneal and intracerebral bleeding with development of a posthemorrhagic hydrocephalus in the first months of life. Factor XIII activity was not measurable by means of enzymatic method and the antiserum inhibition test. Quantitative immunoelectrophoresis according to Laurell presented absence of the subunit A, whereas the concentration of subunit S was reduced to 47% the normal value. After replacement therapy factor XIII activity was estimated at 23% and corresponded to the concentration of the subunit A, concentration of subunit S increased by 20%. The turnover rate of fibrin stabilizing factor could be observed over a period of 39 days. The half life was estimated at 4,7 days. The child developed normally after continous substitution with 250 units of factor XIII concentrate every 6 weeks.
R Zimmermann, and
W Ehlers, and
F Manz, and
W Meinrenken, and
R Egbring, and
H Gemmeke
February 1974,
[Rinsho ketsueki] The Japanese journal of clinical hematology,
R Zimmermann, and
W Ehlers, and
F Manz, and
W Meinrenken, and
R Egbring, and
H Gemmeke
January 1978,
Zahn-, Mund-, und Kieferheilkunde mit Zentralblatt,
R Zimmermann, and
W Ehlers, and
F Manz, and
W Meinrenken, and
R Egbring, and
H Gemmeke
October 2008,
Hawaii medical journal,
R Zimmermann, and
W Ehlers, and
F Manz, and
W Meinrenken, and
R Egbring, and
H Gemmeke
November 1980,
Taiwan yi xue hui za zhi. Journal of the Formosan Medical Association,
R Zimmermann, and
W Ehlers, and
F Manz, and
W Meinrenken, and
R Egbring, and
H Gemmeke
June 1968,
Giornale di clinica medica,
R Zimmermann, and
W Ehlers, and
F Manz, and
W Meinrenken, and
R Egbring, and
H Gemmeke
January 1993,
Pediatrie,
R Zimmermann, and
W Ehlers, and
F Manz, and
W Meinrenken, and
R Egbring, and
H Gemmeke
December 1976,
La Nouvelle presse medicale,
R Zimmermann, and
W Ehlers, and
F Manz, and
W Meinrenken, and
R Egbring, and
H Gemmeke
January 1980,
Zentralblatt fur Gynakologie,
R Zimmermann, and
W Ehlers, and
F Manz, and
W Meinrenken, and
R Egbring, and
H Gemmeke
April 1972,
Revista medica de Chile,
R Zimmermann, and
W Ehlers, and
F Manz, and
W Meinrenken, and
R Egbring, and
H Gemmeke
August 1980,
Problemy gematologii i perelivaniia krovi,
