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Titolo:
HEPATOBILIARY DISEASE IN CYSTIC-FIBROSIS PATIENTS WITH PANCREATIC SUFFICIENCY
Autore:
WATERS DL; DORNEY SFA; GRUCA MA; MARTIN HCO; HOWMANGILES R; KAN AE; DESILVA M; GASKIN KJ;
Indirizzi:
ROYAL ALEXANDRA HOSP CHILDREN,JAMES FAIRFAX INST,POB 34 CAMPERDOWN 2050 NSW AUSTRALIA ROYAL ALEXANDRA HOSP CHILDREN,DEPT GASTROENTEROL CAMPERDOWN 2050 NSW AUSTRALIA ROYAL ALEXANDRA HOSP CHILDREN,DEPT SURG CAMPERDOWN 2050 NSW AUSTRALIA ROYAL ALEXANDRA HOSP CHILDREN,DEPT NUCL MED CAMPERDOWN 2050 NSW AUSTRALIA ROYAL ALEXANDRA HOSP CHILDREN,DEPT HISTOPATHOL CAMPERDOWN 2050 NSW AUSTRALIA ROYAL ALEXANDRA HOSP CHILDREN,DEPT RADIOL CAMPERDOWN 2050 NSW AUSTRALIA
Titolo Testata:
Hepatology
fascicolo: 4, volume: 21, anno: 1995,
pagine: 963 - 969
SICI:
0270-9139(1995)21:4<963:HDICPW>2.0.ZU;2-F
Fonte:
ISI
Lingua:
ENG
Soggetto:
COMMON-BILE-DUCT; GASTROINTESTINAL MANIFESTATIONS; ACID MALABSORPTION; LIVER-DISEASE; INSUFFICIENCY; COMPRESSION; INFANTS; ADULT; CFTR;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Citazioni:
36
Recensione:
Indirizzi per estratti:
Citazione:
D.L. Waters et al., "HEPATOBILIARY DISEASE IN CYSTIC-FIBROSIS PATIENTS WITH PANCREATIC SUFFICIENCY", Hepatology, 21(4), 1995, pp. 963-969

Abstract

Focal and multilobular biliary cirrhosis are considered pathognomonicof cystic fibrosis (CF) and almost invariably have been reported in patients with steatorrhea. In contrast, patients with pancreatic sufficiency and normal absorption are considered less Likely to develop Liver or biliary tract problems. The authors report three patients with CFand pancreatic sufficiency, presenting with recurrent abdominal pain (unrelated to pancreatitis). All had common bile duct disease, one with multilobular cirrhosis and portal hypertension. Pancreatic sufficiency was proven by quantitative pancreatic stimulation tests, 3-day fecal fat analyses, and serum pancreatic isoamylases. All three patients had mild lung disease. Two were homozygous for the common Delta F508 mutation, and the other, a Delta F508 compound heterozygote. Hepatobiliary structure and function were determined by serial hepatobiliary scintigraphy, percutaneous transhepatic cholecystography, and biochemical liver function tests. Patients 1 and 3 had mild hepatomegaly, normal liver biochemistry, and distal common bile duct strictures. Patient 2 had a firm nodular liver with splenomegaly, abnormal Liver biochemistry, and a cholangiographic appearance of sclerosing cholangitis. All have undergone operative treatment for persistent abdominal pain. These cases confirm the occurrence of common bile duct pathology and liver disease in patients with CF and pancreatic sufficiency. They demonstratethat liver and biliary tract disease can occur independently of the underlying disease severity and the presence of steatorrhea. Further, they suggest that obstruction of the biliary tract may be an additionalfactor in the evolution of liver disease in CF.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 01/10/20 alle ore 05:52:26