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Titolo:
HEMOSTATIC STUDIES IN PATIENTS WITH CARBOHYDRATE-DEFICIENT GLYCOPROTEIN SYNDROME
Autore:
IIJIMA K; MURAKAMI F; NAKAMURA K; IKAWA S; YUASA I; MOTOSUMI H; OHNO K; TAKESHITA K;
Indirizzi:
TOTTORI UNIV HOSP,DEPT CLIN LAB,NISHIMACHI 36-1 YONAGO TOTTORI JAPAN TOTTORI UNIV,FAC MED,DEPT CLIN LAB MED TOTTORI 680 JAPAN TOTTORI UNIV,FAC MED,DEPT LEGAL MED TOTTORI 680 JAPAN TOTTORI UNIV,FAC MED,DEPT PEDIAT TOTTORI 680 JAPAN TOTTORI UNIV,FAC MED,INST NEUROL SCI,DIV CHILD NEUROL TOTTORI 680 JAPAN
Titolo Testata:
Thrombosis research
fascicolo: 2, volume: 76, anno: 1994,
pagine: 193 - 198
SICI:
0049-3848(1994)76:2<193:HSIPWC>2.0.ZU;2-X
Fonte:
ISI
Lingua:
ENG
Soggetto:
PROTEIN-S DEFICIENCY; TRANSFERRIN; VARIANT; SERUM;
Keywords:
CARBOHYDRATE-DEFICIENT GLYCOPROTEIN (CDG) SYNDROME; SUGAR SIDE-CHAIN; THROMBOTIC PREDISPOSITION; ANTITHROMBIN III (AT III); ISOELECTRIC FOCUSING (IEF);
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Science Citation Index Expanded
Citazioni:
18
Recensione:
Indirizzi per estratti:
Citazione:
K. Iijima et al., "HEMOSTATIC STUDIES IN PATIENTS WITH CARBOHYDRATE-DEFICIENT GLYCOPROTEIN SYNDROME", Thrombosis research, 76(2), 1994, pp. 193-198

Abstract

The carbohydrate deficient glycoprotein (CDG) syndrome is a newly described disorder characterized by impaired glycosylated molecules. It has been reported that transient stroke-like episodes appear in half ofthe patients. We performed hemostatic studies on three CDG syndrome patients belonging to two unrelated families. The most characteristic findings were decreases in antithrombin III (AT III), protein C and alpha 2 plasmin inhibitor to nearly half normal levels. Protein S was reduced in two (siblings) patients. Isoelectric focusing of AT III in native plasma revealed decreased intensity of the major band and increased intensity of a minor cathodal band. These minor AT III molecules were considered to lack an oligosaccharide sidechain. A 12-year-old girl defective not only for AT III but also protein C and protein S developed disseminated intravascular coagulation accompanied by arterial thrombosis in her left hand following dyspnea associated with broncial asthma. These findings suggest that thrombotic predisposition in patientswith CDG syndrome is due to decreased levels of major coagulation inhibitors, particularly as a result of impaired glycosylation of AT III.

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Documento generato il 19/09/20 alle ore 12:20:32