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)

Primary biliary cirrhosis is a chronic, progressive, cholestatic liver disease thought to be related to abnormalities in immune regulation. The disease is associated with granulomatous bile duct destruction, cholestasis, hepatic copper overloading and the development of hepatic fibrosis or cirrhosis or both. There have been numerous therapeutic trials evaluating immunosuppressive, antifibrotic and cupruretic agents. Prednisolone, D-penicillamine, azathioprine, colchicine and chlorambucil have been evaluated in controlled clinical trials, and biochemical improvement of liver function has been noted with all of the agents, except D-penicillamine. Improved survival has also been reported in patients treated long-term with azathioprine and colchicine. However, none of the therapeutic agents has been demonstrated to halt histologic progression of the disease or to induce a complete clinical, biochemical and histologic remission as has been reported in patients with autoimmune chronic active hepatitis treated with corticosteroids. Many of the trials did not use a double-blind design, failed to use the "intent to treat" rule or failed to define an objective time to analyze results. Many of the studies involved small numbers of patients with short-term follow-up and thus potentially were inadequate to appreciate drug effects that might be of clinical benefit. Currently, there is no totally effective therapy for primary biliary cirrhosis. We believe that well-designed clinical trials can provide important information to better understand this disease until a totally effective therapy is available. New clinical trials should use well-established methodologic guidelines in study design and well-accepted statistical standards in the analysis and interpretation of results.
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PMID:Clinical and statistical analyses of new and evolving therapies for primary biliary cirrhosis. 328 61

The main complications of endoscopic retrograde cholangiography and sphincterotomy are bleeding, pancreatitis, perforation and sepsis. Two cases of unexplained prolonged cholestatic jaundice in patients who underwent endoscopic retrograde cholangiography (ERC) for biliary obstruction due to choledocholithiasis are reported. The patients were admitted because of right upper quadrant pain, vomiting and jaundice. Laboratory tests showed increased levels of total and conjugated serum bilirubin and increased alkaline phosphatase. Ultrasound examination showed cholelithiasis and choledocholithiasis with bile duct dilatation. ERC with sphincterotomy was performed and gallstones obstructing the common bile duct were removed endoscopically. Following ERC and despite complete patency of the biliary tree, a progressive increase of total and conjugated bilirubin and of alkaline phosphatase was noted, associated with itching and total stool discoloration. The insertion of nasobiliary drain did not improve the jaundice. Prednisolone treatment for 12 days was associated with progressive restoration of serum bilirubin alkaline phosphatase to normal levels. It was postulated that the radiocontrast material used may have acted toxically on the liver with disruption of the canalicular plasma membrane. It is proposed that intrahepatic cholestasis should be added in the list of complications of endoscopic retrograde cholangiography.
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PMID:Prolonged cholestatic jaundice after endoscopic retrograde cholangiography. 922 70

We experienced a 6-year-old girl diagnosed with mevalonate kinase deficiency (MKD) who had cholestasis, anemia, and elevated inflammatory markers in neonatal period. She was admitted to our hospital because of fever and elevated inflammatory markers at 5 years 11months of age. Without using antibiotics, the fever and the inflammatory markers were spontaneously resolved. MKD was suspected from elevated serum IgD level and the recurrent febrile attacks. The genetic test revealed heterozygous mutation of p.Leu51Phe known as causative gene of MKD and p.Met 282Thr which is the novel mutation. In addition, urinary mevalonate levels increased both in afebrile and febrile periods, and mevalonate kinase activity level was very low. Prednisolone was administered on each attack, and her febrile attack has been controlled well since she was diagnosed with MKD. Fetal edema, cholestasis, anemia, elevation of inflammatory markers in her neonatal period are considered to be complications of MKD. Recurrent fever attacks compromise quality of life in patients with MKD. Children with unexplained cholestasis and anemia in neonatal period, or recurrent fever attacks with elevated inflammatory markers should be examined for MKD.
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PMID:A 6-year-old girl diagnosed with mevalonate kinase deficiency who had hydrops fetalis and neonatal-onset cholestasis. 2860 4

Cholestasis following hepatitis A affects around 0.8% of hepatitis A patients. It is considered a distressing complication in spite of its good prognosis. Despite being subject to multiple studies, causes of cholestasis are still controversial. Many treatments (discussed later) have shown some improvements of the accompanied pruritus. In the following article, we present two cholestatic hepatitis A patients who suffered from severe pruritus. Prednisolone was administered via two different methods: prolonged and pulsed. Both showed great improvement of the pruritus in a short time frame. To the best of our knowledge, our management using pulsed corticosteroid therapy in treatment of pruritus in cholestatic hepatitis A is considered the first experimental management in medical literature. The importance of this experimental case lies in reducing the doses and the duration of steroid intake, thus reducing steroid side effects as far as possible.
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PMID:Two case reports of corticosteroid administration-prolonged and pulsed therapy-in treatment of pruritus in cholestatic hepatitis A patients. 3177 47

A 60-year-old man was hospitalised with persistent fever, arm pain, dry cough and cholestasis. Diagnostic workup was remarkable for elevated inflammatory markers. Infectious diseases and autoimmune screening were negative. Imaging modalities excluded a neoplastic aetiology. Liver biopsy was negative for granulomatous or lymphomatous infiltrations. Giant cell arteritis (GCA) was suspected, but temporal artery Doppler ultrasound and biopsy were non-diagnostic. A positron emission tomography scan showed intense metabolic uptake in large vessels suggesting the diagnosis of GCA. Prednisolone was initiated with clinical and analytical improvement. At 1-year follow-up, there were no relapses and the patient remains symptom free.
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PMID:Boundaries of a systemic disease: a protean presentation of giant cell arteritis. 3221 59