CRM+Severe Fletcher Factor Deficiency Associated with Graves’ Disease
作者:
P.A. Kyrle,
H. Niessner,
E. Deutsch,
K. Lechner,
C. Korninger,
C. Mannhalter,
期刊:
Pathophysiology of Haemostasis and Thrombosis
(Karger Available online 1984)
卷期:
Volume 14,
issue 4
页码: 302-306
ISSN:1424-8832
年代: 1984
DOI:10.1159/000215079
出版商: S. Karger AG
关键词: Fletcher trait;Cross-reacting material;Heredity;Graves’ disease
数据来源: Karger
摘要:
A 59-year-old male patient with Graves’ disease and severe hereditary Fletcher factor deficiency is described. PKK clotting activity as well as the activity by a chromogenic substrate method (Chromozym PK®) was less then 0.01 U/ml. In contrast to functional tests, the immunological assay (Laurell method) showed a PKK antigen concentration of 0.25 U/ml, indicating the presence of an abnormal nonfunctional PKK molecule (CRM+ variant). An inhibitor was excluded since the patient plasma did not inactivate partially purified PKK. Investigation of 11 family members revealed a reduction of the PKK clotting activity in 9 relatives of the patient. Since Graves’ disease is considered an autoimmune disease, our case represents an example of an association of a severe hereditary deficiency of a contact factor and an autoimmune dis
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