COEXISTENCE OF CONGENITAL AFIBRINOGENEMIA AND PROTEIN-C DEFICIENCY IN A PATIENT

被引:19
作者
HANANO, M
TAKAHASHI, H
ITOH, M
SHIBATA, A
机构
[1] NIIGATA UNIV,DEPT INTERNAL MED 1,1-757 ASAHIMACHI,NIIGATA 951,JAPAN
[2] MUIKAMACHI PREFECTURAL HOSP,DIV INTERNAL MED,NIIGATA,JAPAN
关键词
FIBRINOGEN; PROTEIN-C; BLOOD COAGULATION; ARTERIAL OCCLUSIVE DISEASE;
D O I
10.1002/ajh.2830410111
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
A rare association of congenital afibrinogenemia and hereditary protein C deficiency is described in a 37-year-old female who suffered from ischemic necrosis in the left first toe. The diagnosis of afibrinogenemia was assessed by the absence of fibrinogen in clotting and immunological assays. The diagnosis of hereditary heterozygous type I protein C deficiency was based on the evidence of proportional decreases of activity and antigen of plasma protein C in the propositus, her mother, and two maternal aunts.
引用
收藏
页码:57 / 60
页数:4
相关论文
共 19 条
[1]  
AWBREY BJ, 1979, J BIOL CHEM, V254, P4092
[2]  
BIGGS R, 1976, HUMAN BLOOD COAGULAT, P657
[3]   CONGENITAL PROTEIN-C DEFICIENCY AND VENOUS THROMBOEMBOLISM - A STUDY OF 3 DUTCH FAMILIES [J].
BROEKMANS, AW ;
VELTKAMP, JJ ;
BERTINA, RM .
NEW ENGLAND JOURNAL OF MEDICINE, 1983, 309 (06) :340-344
[4]  
Broekmans AW, 1988, PROTEIN C RELATED PR, V3, P160
[5]   THE REGULATION OF HEMOSTASIS - THE PROTEIN-C SYSTEM [J].
CLOUSE, LH ;
COMP, PC .
NEW ENGLAND JOURNAL OF MEDICINE, 1986, 314 (20) :1298-1304
[6]   ASSOCIATION OF INHERITED DYSFIBRINOGENEMIA AND PROTEIN C DEFICIENCY IN 2 UNRELATED FAMILIES [J].
GANDRILLE, S ;
PRIOLLET, P ;
CAPRON, L ;
RONCATO, M ;
FIESSINGER, JN ;
AIACH, M .
BRITISH JOURNAL OF HAEMATOLOGY, 1988, 68 (03) :329-337
[7]  
GRALNICK HR, 1990, HEMATOLOGY, P1474
[8]   DEFICIENCY OF PROTEIN-C IN CONGENITAL THROMBOTIC DISEASE [J].
GRIFFIN, JH ;
EVATT, B ;
ZIMMERMAN, TS ;
KLEISS, AJ ;
WIDEMAN, C .
JOURNAL OF CLINICAL INVESTIGATION, 1981, 68 (05) :1370-1373
[9]  
HORLELOU MH, 1984, BRIT MED J, V289, P125
[10]   FIBRINOGEN AND RISK OF CARDIOVASCULAR-DISEASE - THE FRAMINGHAM-STUDY [J].
KANNEL, WB ;
WOLF, PA ;
CASTELLI, WP ;
DAGOSTINO, RB .
JAMA-JOURNAL OF THE AMERICAN MEDICAL ASSOCIATION, 1987, 258 (09) :1183-1186