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Titolo:
North American Indian cirrhosis in children: A review of 30 cases
Autore:
Drouin, E; Russo, P; Tuchweber, B; Mitchell, G; Rasquin-Weber, A;
Indirizzi:
Hop St Justine, Dept Pediat, Montreal, PQ H3T 1C5, Canada Hop St Justine Montreal PQ Canada H3T 1C5 t, Montreal, PQ H3T 1C5, Canada Hop St Justine, Dept Pathol, Montreal, PQ H3T 1C5, Canada Hop St Justine Montreal PQ Canada H3T 1C5 l, Montreal, PQ H3T 1C5, Canada Univ Montreal, Dept Nutr, Montreal, PQ, Canada Univ Montreal Montreal PQ Canada ntreal, Dept Nutr, Montreal, PQ, Canada
Titolo Testata:
JOURNAL OF PEDIATRIC GASTROENTEROLOGY AND NUTRITION
fascicolo: 4, volume: 31, anno: 2000,
pagine: 395 - 404
SICI:
0277-2116(200010)31:4<395:NAICIC>2.0.ZU;2-O
Fonte:
ISI
Lingua:
ENG
Soggetto:
FAMILIAL INTRAHEPATIC CHOLESTASIS; PRIMARY SCLEROSING CHOLANGITIS; INTERLOBULAR BILE-DUCTS; ARTERIOHEPATIC DYSPLASIA; INFANTILE CIRRHOSIS; CHILDHOOD CIRRHOSIS; ALAGILLE-SYNDROME; BILIARY ATRESIA; DISEASE; LIVER;
Keywords:
liver transplantation; neonatal cholestasis; progressive familial intrahepatic cholestasis; sclerosing cholangitis;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Life Sciences
Citazioni:
47
Recensione:
Indirizzi per estratti:
Indirizzo: Rasquin-Weber, A Hop St Justine, Div Gastroenterol, 3175 Cote Ste Catherine, Montreal, PQ H3T 1C5, Canada Hop St Justine 3175 Cote Ste Catherine Montreal PQ Canada H3T 1C5
Citazione:
E. Drouin et al., "North American Indian cirrhosis in children: A review of 30 cases", J PED GASTR, 31(4), 2000, pp. 395-404

Abstract

Background: North American Indian childhood cirrhosis (NAIC) is a distinct, rapidly evolving form of familial cholestasis found in aboriginal children from northwestern Quebec. This is a retrospective review of the 30 patients treated in Quebec since the discovery of NAIC in 1970. Methods: The clinical records and histologic samples from 30 patients werereviewed. Extensive metabolic, biochemical, viral, genetic, and radiologicstudies were performed in most patients. Results: Genetic analysis suggests autosomal recessive inheritance and a carrier frequency of 10% in this population. Gene mapping studies showed that the NAIC gene is located on chromosome 16q22. Typically, patients have neonatal cholestatic jaundice (70%) or hepatosplenomegaly (20%) with resolution of clinical jaundice by age 1 year but persistent direct hyperbilirubinemia. Portal hypertension was documented in 29 patients (91%). Variceal bleeding (15 patients, 50%) occurred as early as age 10 months. Surgical portosystemic shunting was performed in 13 of these 15 patients (87%); 4 (31%) rebled after 1 to 5 years. Fourteen patients died (47%). In 10 (71%), liver disease was the cause. Four children died of liver failure before liver transplantation became available. In transplanted livers, no recurrence of NAICwas observed after 1 to 10 years. Recognized infectious, metabolic, toxic,autoimmune, and obstructive causes of cirrhosis have been eliminated. The histologic features of NAIC show early bile duct proliferation and rapid development of portal fibrosis and biliary cirrhosis, suggesting a cholangiopathic phenomenon. Conclusion: Together with gene mapping studies showing that the NAIC gene is different from those of other familial cholestases, these observations suggest that NAIC is a distinct entity that could be classified as "progressive familial cholangiopathy."

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 19/09/20 alle ore 18:32:15