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Titolo:
AUTOSOMAL RECESSIVE POLYCYSTIC KIDNEY-DISEASE - LONG-TERM OUTCOME OF NEONATAL SURVIVORS
Autore:
ROY S; DILLON MJ; TROMPETER RS; BARRATT TM;
Indirizzi:
INST CHILD HLTH,NEPHROUROL UNIT,30 GUILFORD ST LONDON WC1N 1EH ENGLAND GREAT ORMOND ST HOSP CHILDREN,RENAL UNIT LONDON WC1N 3JH ENGLAND
Titolo Testata:
Pediatric nephrology
fascicolo: 3, volume: 11, anno: 1997,
pagine: 302 - 306
SICI:
0931-041X(1997)11:3<302:ARPK-L>2.0.ZU;2-H
Fonte:
ISI
Lingua:
ENG
Soggetto:
CONGENITAL HEPATIC-FIBROSIS; CLINICAL PICTURE; LIVER; CHILDHOOD; DIAGNOSIS;
Keywords:
CONGENITAL HEPATIC FIBROSIS; HYPERTENSION; PORTAL HYPERTENSION; END-STAGE RENAL FAILURE;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Science Citation Index Expanded
Citazioni:
28
Recensione:
Indirizzi per estratti:
Citazione:
S. Roy et al., "AUTOSOMAL RECESSIVE POLYCYSTIC KIDNEY-DISEASE - LONG-TERM OUTCOME OF NEONATAL SURVIVORS", Pediatric nephrology, 11(3), 1997, pp. 302-306

Abstract

Autosomal recessive polycystic kidney disease causes renal and hepatic dysfunction in childhood. We describe the clinical outcome of 52 children with this diagnosis born between 1950 and 1993. Currently 23 arealive, 24 dead and 5 have been lost to follow-up; 1 has been dialysedand 7 transplanted. Life-table analysis of the patients surviving theIst month of life revealed an actuarial renal survival of 86% at 1 year and 67% at 15 years. The probability of requiring anti-hypertensivetreatment was 39% at 1 year and 60% at 15 years of age. Bleeding fromgastro-oesophageal varices occurred in 8 patients at a mean age of 12.5 years, and was preceded by haematological evidence of hypersplenismin 6 of them. The study indicates a relatively good prognosis for patients with this condition who survive the neonatal period and emphasises the importance of early detection and appropriate management of systemic and portal hypertension.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 30/09/20 alle ore 05:35:54