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Titolo:
THE HEART AND PERICARDIAL-EFFUSIONS IN CDGS-I (CARBOHYDRATE-DEFICIENTGLYCOPROTEIN SYNDROME TYPE-I)
Autore:
KRISTIANSSON B; STIBLER H; CONRADI N; ERIKSSON BO; RYD W;
Indirizzi:
SAHLGRENS UNIV HOSP,DEPT PEDIAT S-41685 GOTHENBURG SWEDEN UNIV GOTHENBURG,DEPT PEDIAT GOTHENBURG SWEDEN UNIV GOTHENBURG,DEPT PATHOL GOTHENBURG SWEDEN
Titolo Testata:
Journal of inherited metabolic disease
fascicolo: 2, volume: 21, anno: 1998,
pagine: 112 - 124
SICI:
0141-8955(1998)21:2<112:THAPIC>2.0.ZU;2-3
Fonte:
ISI
Lingua:
ENG
Soggetto:
OLIVOPONTOCEREBELLAR ATROPHY; NEONATAL ONSET; ABNORMALITIES; DISORDERS; SERUM;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Science Citation Index Expanded
Citazioni:
28
Recensione:
Indirizzi per estratti:
Citazione:
B. Kristiansson et al., "THE HEART AND PERICARDIAL-EFFUSIONS IN CDGS-I (CARBOHYDRATE-DEFICIENTGLYCOPROTEIN SYNDROME TYPE-I)", Journal of inherited metabolic disease, 21(2), 1998, pp. 112-124

Abstract

Pericardial effusions were found in 6 of 10 children with carbohydrate-deficient glycoprotein syndrome type I (CDGS-I). In three cases pericardectomy was necessary. Blood concentrations of several glycoproteins and albumin were low. Similar abnormal isoforms of four glycoproteins were found in blood (B) and pericardial fluid (PF). There was a significant negative correlation between the mean concentration ratio PF/Band the molecular mass (MW) of 11 proteins. For proteins with MW < 100 kDa there were significant correlations in the controls, but not in the patients, between the PF/B ratio and both the MW and the sialic acid contents in the (glyco-)proteins. The pericardium exhibited focal mixed inflammatory changes with mesothelial proliferation, with widenedendoplasmic reticulum and flocculent and/or lamellated material. Damage to a pericardial protein barrier is suggested to be involved in pericardial effusion in CDGS-I.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 25/11/20 alle ore 09:58:23