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Titolo:
Clinical aspects on neonatal cholestasis based on observations at a Swedish tertiary referral centre
Autore:
Fischler, B; Papadogiannakis, N; Nemeth, A;
Indirizzi:
Karolinska Inst, Huddinge Univ Hosp, Dept Paediat, SE-14186 Huddinge, Sweden Karolinska Inst Huddinge Sweden SE-14186 diat, SE-14186 Huddinge, Sweden Karolinska Inst, Huddinge Univ Hosp, Dept Pathol, SE-14186 Huddinge, Sweden Karolinska Inst Huddinge Sweden SE-14186 thol, SE-14186 Huddinge, Sweden
Titolo Testata:
ACTA PAEDIATRICA
fascicolo: 2, volume: 90, anno: 2001,
pagine: 171 - 178
SICI:
0803-5253(200102)90:2<171:CAONCB>2.0.ZU;2-D
Fonte:
ISI
Lingua:
ENG
Soggetto:
FAMILIAL INTRAHEPATIC CHOLESTASIS; BILIARY ATRESIA; LIVER-DISEASE; DEFICIENCY; SURGERY; INFANTS; SWEDEN;
Keywords:
aetiology; extrahepatic biliary atresia; neonatal cholestasis; outcome; progressive familial intrahepatic cholestasis;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Life Sciences
Citazioni:
28
Recensione:
Indirizzi per estratti:
Indirizzo: Fischler, B Karolinska Inst, Huddinge Univ Hosp, Dept Paediat, SE-14186 Huddinge, Sweden Karolinska Inst Huddinge Sweden SE-14186 86 Huddinge, Sweden
Citazione:
B. Fischler et al., "Clinical aspects on neonatal cholestasis based on observations at a Swedish tertiary referral centre", ACT PAEDIAT, 90(2), 2001, pp. 171-178

Abstract

The aim of the study was to investigate the clinical aspects of neonatal cholestasis. The medical records of 85 cholestatic infants were retrospectively reviewed. A majority of the patients were referred from other parts of the country. The most common diagnoses were extrahepatic biliary atresia (n= 30 patients), alpha (1)-antitrypsin deficiency (n = 11) and progressive familial intrahepatic cholestasis (n = 11). On presentation, the biliary atresia group had higher mean serum values of bilirubin. G-GT and cholesterolthan the patients with intrahepatic cholestasis, with no significant differences noticed for any other biochemical parameter. A lack of excretion on hepatobiliary scintigraphy was noticed in all investigated patients with biliary atresia, but also in 9 of 34 patients with intrahepatic neonatal cholestasis. There was no statistical correlation between the age at portoenterostomy and the outcome in patients with biliary atresia. However, both the detection of a partial flow on perioperative cholangiogram and the establishment of a non-icteric phase within 6 mo after the portoenterostomy correlated to a good outcome. Eight of 11 patients with progressive familial intrahepatic cholestasis were treated with a biliary diversion procedure, five of eight experienced a sustained cholestatic remission. Conclusions: Progressive familial intrahepatic cholestasis may be a more common cause of neonatal cholestasis in Sweden than reported elsewhere and that the experience with biliary diversion is positive. While early referralin patients with extrahepatic biliary atresia remains important, a portoenterostomy should be attempted also in patients referred after 3 mo of age.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 14/07/20 alle ore 03:24:58