Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0008370 (cholestasis)
 9,378 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 3.5-month-old white boy was born with meconium ileus, peritonitis, and jejunal atresia from cystic fibrosis. He subsequently developed unrelenting and severe extrahepatic biliary obstruction as demonstrated by liver biopsy showing periportal inflammation, cholestasis, and fibrosis. Surgical exploration confirmed the diagnosis of extrahepatic biliary obstruction by severely inspissated bile. A cholecystostomy tube was left in place. The cholestasis remained unresponsive to conservative medical therapy. The obstruction was relieved by hydrostatic infusion of 2% N-acetylcysteine into the biliary tree over a 6-day period. The child also received concurrently four i.v. injections of synthetic cholecystokinin. This therapeutic modality was thought to be both safe and effective.
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PMID:Biliary infusion therapy in the inspissated bile syndrome of cystic fibrosis. 206 68

A 30-year-old woman in the 36th week of her second pregnancy, suddenly developed jaundice with remarkable liver necrosis, accompanied by generalized bleeding due to disseminated intravascular coagulation (DIC). She underwent a caesarean and a dead foetus was extracted from the uterus. Heparin and frozen plasma infusion resulted in a prompt recovery from the haemostatic disorder. The course of the disease involved the successive appearance of haemorrhagic shock, intestinal ileus and pulmonary embolism all of which she recovered from. The liver biopsy showed severe cholestasis without derangement of the lobular structure. Hypotheses of acute veno-occlusive disease caused by the DIC, and acute fatty liver of pregnancy are discussed.
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PMID:[Disseminated intravascular coagulation and acute hepatic necrosis at the end of pregnancy. A case report]. 262 77

Gallbladder stones remain asymptomatic over a long period. The biliary colic is the typical pain caused by these stones. Dyspeptic symptoms seem to be unrelated to the presence of gallstones. Acute cholecystitis, a serious complication of gallstone disease, spans a wide spectrum of clinical findings. The typical signs are right upper abdominal pain and tenderness, fever, leucocytosis and Murphy's sign. 35% of patients experience gallbladder empyema or perforation. Localized gallbladder perforation, characterized by high fever, severe right upper abdominal pain and tenderness and a palpable mass is often difficult to distinguish from acute cholecystitis. Free perforation into the abdominal cavity causes diffuse peritonitis. Gallbladder perforation into the lumen of an adjacent organ produces fistulas, mostly with minimal symptoms or a pain relief after decompression of the inflamed gallbladder. Air in the bile ducts and on some occasions bile-acid-induced diarrhea may result. Rarely, the perforation of large stones leads to an occlusion of the GI tract and results in a gallstone ileus. Common bile duct stones may be asymptomatic or cause bile duct obstruction with biliary colics and jaundice. Acute bacterial cholangitis characterized by Charcot's triad (pain, jaundice and fever) and the acute biliary pancreatitis with its typical symptoms are the serious complications of common bile duct stones, associated with a high mortality rate. The clinical manifestations of a gallstone disease and its complications reveal important diagnostic features, but the most important diagnostic features, modalities are the imaging procedures. They are decisive for an accurate therapy.
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PMID:[Clinical manifestations of cholelithiasis and its complications]. 776 32

Cystic fibrosis (CF), the most common lethal autosomal recessive disease in white populations, is characterized by dysfunctional chloride ion transport across epithelial surfaces. Although recurrent pulmonary infections and pulmonary insufficiency are the principal causes of morbidity and death, gastrointestinal symptoms commonly precede the pulmonary findings and may suggest the diagnosis in infants and young children. The protean gastrointestinal manifestations of CF result primarily from abnormally viscous luminal secretions within hollow viscera and the ducts of solid organs. Bowel obstruction may be present at birth due to meconium ileus or meconium plug syndrome. Complications of meconium ileus include volvulus, small bowel atresia, perforation, and meconium peritonitis with abdominal calcifications. Older children with CF may present with bowel obstruction due to distal intestinal obstruction syndrome or colonic stricture, and tenacious intestinal residue may serve as a lead point for intussusception or cause recurrent rectal prolapse. Radiologic studies often demonstrate thickened intestinal mucosal folds in older children and uncommonly show colonic pneumatosis, peptic esophageal stricture due to gastroesophageal reflux, and duodenal ulcer. Appendicitis due to inspissated secretions is uncommon. Obstruction of ducts and ductules produces exocrine pancreatic insufficiency, pancreatitis, cholestasis, cholelithiasis, and cirrhosis with portal hypertension. On imaging studies, the pancreas is commonly small and largely replaced by fat, sometimes displays calcifications, and is rarely replaced by macrocysts. Radiologic features of hepatobiliary disease include an enlarged radiolucent liver from steatosis, gallstones, a shrunken nodular liver, splenomegaly, and portosystemic collateral vessels. With the improved survival of CF patients, an increased risk for developing gastrointestinal carcinomas has been established, many occurring as early as the 3rd decade.
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PMID:Gastrointestinal manifestations of cystic fibrosis: radiologic-pathologic correlation. 883 77

Cystic fibrosis is associated with an inspissated bile syndrome producing cholestasis secondary to plugging of macroscopically normal bile ducts. In extreme neonatal forms, with early profound intrahepatic cholestasis, the process can be associated with a marked decrease in ductal diameter, varying from hypoplasia to atresia. From 1990 to 1995 three infants were identified with cystic fibrosis, persistent jaundice, and complete absence of biliary excretion despite expectant and conservative treatment including choleretics and surgical biliary irrigation. Abdominal ultrasounds showed contracted gallbladders and no evidence of dilated ducts. Liver biopsy results in two infants showed portal fibrosis, paucity of bile ducts, and minimal inflammation. The third infant had moderate inflammation, bile duct replication, and plugging. Two infants had undergone intestinal resection followed by hyperalimenation for complications of meconium ileus in the newborn period. Surgical exploration was undertaken at 7 to 12 weeks of age. Gross findings were typical of biliary atresia with microgallbladders and nonpatency of the cystic duct. Cholangiograms failed to document ductal patency in two patients who were then treated with a Kasai portoenterostomy. The third infant had patent hypoplastic ducts and underwent only biliary irrigation. Although bile flow was transiently achieved, jaundice recurred, and at reexploration at 16 weeks of age a Kasai poroenterostomy was performed. Histological review of the biliary specimens showed microscopically patent ducts in two patients and proximal patency and distal atresia in the third. All the ducts had mural fibrosis with cystic changes. Bile drainage was achieved in each instance, although in one patient with hypoplastic ducts scant output of highly concentrated bile proved insufficient to arrest progressive liver failure. The subsequent two patients responded with resolution of hyperbilirubinemia and normalization of liver function. They remain free of biliary complications at 30 and 40 months postoperatively. This manifestation of cystic fibrosis in infants is suggested by prolonged jaundice unresponsive to choleretics, nondilated bile ducts and gallbladder on ultrasound, absent biliary excretion on nuclear scan, and characteristic liver biopsy. Exploration is warranted, and discovery of atrophic bile ducts may be best managed with reconstruction.
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PMID:Biliary obstruction in infants with cystic fibrosis requiring Kasai portoenterostomy. 904 17

Pseudo-obstruction is actually a group of disorders resulting in bowel dysmotility in the absence of anatomic obstruction. Because of varied pathologic abnormalities of the GI tract involved in the disorder, pseudo-obstruction has been reported in the literature under different names, including hypoganglionosis, chronic adynamic ileus, pseudo-Hirschprung's disease, visceral neuropathy, visceral myopathy, and megacystis-microcolon-intestinal hypoperistalsis syndrome. Although a rare condition, its effects are severe, disabling, and life-threatening in pediatric clients. Prognosis is inversely correlated with age and is especially poor in those children with symptoms as newborns. Management involves promoting nutrition adequate for growth, treating symptoms, and preventing complications. Total parenteral nutrition is almost always required, and its long-term use is associated with chronic cholestasis and potential hepatic failure. Nurses may encounter pediatric clients with this disorder and their families in many different healthcare arenas, such as in radiology departments, ambulatory care, and acute and home care settings. The purpose of this article is to review current literature on this disease and summarize information useful to nurses.
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PMID:Understanding pediatric intestinal pseudo-obstruction: implications for nurses. 955 62

The clinical histories of 46 adult patients (24 men and 22 women, mean age 20.6 +/- 5.1 years) diagnosed of cystic fibrosis were reviewed evaluating the digestive alterations. The age at diagnosis of cystic fibrosis was 5.63 +/- 5.3 years (range: newborns-19 years). The initial diagnosis was established by ileus meconium, in four, lung disease in 15, steatorrhea in 12, lung disease and steatorrhea in 13 and following the diagnosis of cystic fibrosis in siblings in two. Four patients presented ileus meconium, nine occlusive syndrome of the distal intestine, 42 steatorrhea (20 severe, 12 moderate and 10 mild), with the severity of the steatorrhea not being associated with the severity of the respiratory insufficiency. Two patients presents rectal prolapse, five gastroesophageal reflux syndrome (four with hiatal hernia), six cholelithiasis, one recurrent pancreatitis without detection of biliary lithiasis, one neonatal cholestasis and 10 malnutrition (five severe and five moderate) fundamentally in relation to the severity of the lung disease and, to a lesser degree, liver disease. In 10 patients chronic liver disease was diagnosed corresponding to established cirrhosis in seven, indicating liver transplantation in two. In most cases, the liver disease was already manifest in adolescence even in the cirrhotic stage. Cholangiography by magnetic resonance was useful in the study of liver disease showing abnormalities which imitated primary sclerosing cholangitis. Treatment with ursodesoxicholic acid at a dosis of 20 mg/kg/day led to a significant decrease in the transaminase values and overall of gammaglutamyltranspeptidase but did not avoid complications in the cirrhotic stages. Genetic studies performed in 36 patients detected the delta F508 mutation in 69.4%, being found in almost all of the patients with ileus meconium, occlusive syndrome of the distal intestine, liver disease, cholelithiasis and malnutrition.
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PMID:[Digestive alterations in cystic fibrosis. Retrospective study of a series of 46 adult patients]. 1019 90

Cystic Fibrosis is the most frequent hereditary disease in Caucasians. Its clinical presentation may be very variable. Neonatal cholestasis is a typical but rare primary clinical manifestation that usually occurs in the first 3 weeks of life. It is often associated with meconium ileus. We present the case of an infant with cystic fibrosis whose primary clinical manifestation was cholestasis and liver failure at the age of 6 weeks.
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PMID:[Neonatal cholestasis associated with liver failure as a clinical manifestation of cystic fibrosis]. 1167 9

Liver disease is relatively common during parenteral nutrition (PN). Cholestasis predominates in infants, and ranges in severity from mild increases in plasma conjugated bilirubin to progressive liver failure that results in death of the patient. Severity of liver disease depends primarily on the magnitude of the underlying intestinal problem that indicated PN. Transient ileus resulting from a non-intestinal disorder usually results in trivial, self-limited liver injury. Removal of a large segment of the intestinal tract because of necrotizing enterocolitis or a congenital malformation predicts a more prolonged course with a guarded prognosis, particularly when initially complicated by sepsis. Pathogenesis of PN-associated liver disease is not completely understood. There is no proven treatment short of ending PN through adaptation of remnant intestine or intestinal transplantation, with or without a concurrent liver graft. Effective interventions that are less radical than transplantation are needed. Research that includes prospective trials of novel therapies in PN-associated liver disease is the key to improving outcome.
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PMID:Parenteral nutrition associated liver disease. 1500 Nov 25

Stents and prostheses in the hepatobiliary system are visible by ultrasound examination and can be checked for their function. The reappearance of mechanical cholestasis, stent dislocation or formation of sludge on the stent tip is signs of stent dysfunction. Graduation of the biliary obstruction can help to find the best time for changing the stent. After intervention hematoma, abscess, intraabdominal fluid or gas may occur and can be diagnosed by ultrasound. Tumor stenosis in the gastrointestinal tract can be treated by self expandable metallic stents. Ultrasound helps to localize the stent in the correct position and to observe the reduction of occlusive ileus. Percutaneous and gastrointestinal drainage of pancreatic cysts, necrosis and abscesses are often controlled by ultrasound to evaluate size, echogenicity and localized pain. Many procedures and interventions in gastroenterology can be monitored by an experienced ultrasound examiner in an effective and inexpensive manner.
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PMID:[Abdominal ultrasound after endoscopic or percutaneous stenting and drainage]. 1570 Feb 3


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