morphine and Liver-Cirrhosis--Biliary

Topics: Adolescent; Adult; Autopsy; Child; Child, Preschool; Cystic Fibrosis; Diabetes Mellitus, Type 1; Female; Humans; Infant; Infant, Newborn; Infertility, Male; Intestinal Obstruction; Liver; Liver Cirrhosis, Biliary; Lung; Male; Meconium; Pancreas; Pregnancy; Sweat

The incidence of cystic fibrosis (CF) is very rare in Japanese, while it is frequent in Caucasians. Here we report on three Japanese cases of CF. One of the patients had consanguineous parents. All three patients initially developed meconium ileus, and hepatobiliary and pancreatic changes became more severe as age increased. The DeltaF508 mutation, the most frequent mutation associated with CF in Caucasians, was not found in these patients. To evaluate the relationship between the severity of hepatic lesions and a history of meconium ileus, we examined hepatic lesions in the present three cases, and we reviewed 22 Japanese autopsied cases of CF in the literature. No correlation was found between the incidence of biliary cirrhosis and a history of meconium ileus, because the cases with meconium ileus showed a high mortality during the neonatal period, before biliary cirrhosis developed. The high incidence of meconium ileus in Japanese CF patients may relate to a clinically severe phenotype and reflect a different genetic background between Caucasians and Japanese.

Topics: Adolescent; Autopsy; Child, Preschool; Consanguinity; Cystic Fibrosis; Fatal Outcome; Female; Humans; Infant; Intestinal Obstruction; Japan; Liver; Liver Cirrhosis, Biliary; Male; Meconium; Pancreas; United States

Although dehydrated obstructing mucus is thought to account for the obstructive pathology involving the lungs, the pancreas, the reproductive system, and the intestinal tract, its relationship with CF-associated liver disease remains largely hypothetical and little is known about possible risk factors. Complete clinical and autopsy records were available in 38 of 73 deaths occurring over a 10-year period. The liver was normal in only five cases, and they were all infants. Steatosis was the only lesion present in 9, hypoxic liver disease was documented in 8, and biliary cirrhosis in 16 (focal in 10 and multilobular in 6). There was no relationship between the presence of cirrhosis, gallbladder abnormalities, age at death, and clinical status recorded during the year precoding their demise. Mucus plugs characterized by amorphous eosinophilic material within proliferated bile ductules were present in 75% of children with focal or multilobular biliary cirrhosis as opposed to 14% in those without (p = 0.015). A history of meconium ileus or its equivalent was recorded more frequently (p = 0.038) in those with cirrhosis. Finally, biliary cirrhosis was invariably present when there was a history of meconium ileus or its equivalent and when mucus plugs were noted. These findings suggest that patients with intestinal obstruction are at greater risk for the development of cirrhosis and that strategies should be developed to increase the detergent capacity of bile and its flow in order to decrease the viscosity of mucus in the biliary tree.

Topics: Bile; Child; Cystic Fibrosis; Female; Humans; Infant, Newborn; Intestinal Obstruction; Liver Cirrhosis, Biliary; Male; Meconium; Retrospective Studies; Risk Factors

Topics: Chlorides; Cystic Fibrosis; Exocrine Pancreatic Insufficiency; Humans; Infant, Newborn; Intestinal Obstruction; Liver Cirrhosis, Biliary; Lung Diseases; Meconium; Sex Factors; Sweat

Topics: Candida; Candidiasis; Child, Preschool; Colon; Colonic Diseases; Cystic Fibrosis; Duodenum; Female; Humans; Hyperplasia; Infant; Infant, Newborn; Intestinal Obstruction; Liver Cirrhosis, Biliary; Male; Meconium; Pancreas; Submandibular Gland

Topics: Autopsy; Bile; Biopsy; Child; Cystic Fibrosis; Female; Humans; Infant; Infant, Newborn; Intestinal Obstruction; Jaundice, Neonatal; Liver; Liver Cirrhosis, Biliary; Male; Meconium