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Titolo:
HEMOSTATIC STUDIES IN CARBOHYDRATE-DEFICIENT GLYCOPROTEIN SYNDROME TYPE-I
Autore:
FIUMARA A; BARONE R; BUTTITTA P; MUSSO R; PAVONE L; NIGRO F; JAEKEN J;
Indirizzi:
UNIV HOSP GASTHUISBERG,DEPT PAEDIAT,HERESTR 49 B-3000 LOUVAIN BELGIUM UNIV CATANIA,DEPT PAEDIAT CATANIA ITALY G CRISTINA HOSP,DEPT NEONATOL PALERMO ITALY UNIV CATANIA,DEPT HEMATOL,HEMOPHILIA SECT CATANIA ITALY UNIV LEUVEN,DEPT PAEDIAT LOUVAIN BELGIUM
Titolo Testata:
Thrombosis and haemostasis
fascicolo: 4, volume: 76, anno: 1996,
pagine: 502 - 504
SICI:
0340-6245(1996)76:4<502:HSICGS>2.0.ZU;2-0
Fonte:
ISI
Lingua:
ENG
Soggetto:
ADULTS; GOLGI;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Science Citation Index Expanded
Science Citation Index Expanded
Citazioni:
20
Recensione:
Indirizzi per estratti:
Citazione:
A. Fiumara et al., "HEMOSTATIC STUDIES IN CARBOHYDRATE-DEFICIENT GLYCOPROTEIN SYNDROME TYPE-I", Thrombosis and haemostasis, 76(4), 1996, pp. 502-504

Abstract

CDG syndrome (CDGS) type I is the most frequent form of a group of metabolic disorders characterised by a defect of the carbohydrate moietyof glycoproteins. A large number of plasma glycoproteins, including clotting factors and inhibitors, an decreased and stroke-like episodes have been described in about half of the reported patients. We studiedblood coagulation factors, inhibitors and D-dimer plasma levels in four subjects, aged 12-23 years, with CDGS type I. Factors VIII, XI, antithrombin III activity, antigen plasma levels of antithrombin Iii, free protein S and protein C were decreased whereas protein C as activitywas normal. In addition two patients had reduction of factors II, V, VIII IX, X reflecting the phenotypic heterogeneity associated with CDGS type I. D-dimer plasma concentrations were elevated in all subjects. The hypercoagulable state as consequence of the combined deficienciesof coagulation inhibitors could contribute to the stroke-like phenomena in CDGS type I.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 27/11/20 alle ore 16:04:28