1/19. Effects of clofibrate in primary biliary cirrhosis hypercholesterolemia and gallstones.A patient with primary biliary cirrhosis is reported in whom the administration of clofibrate in a dose of 1 g per day for 2 months resulted in a marked increase in hypercholesterolemia, and endoscopic retrograde cholangiography showed multiple intrahepatic gallstones. The stones disappeared 3 months after stopping clofibrate and starting chenodeoxycholic acid in a dose of 125 mg daily. These observations are discussed in relation to the known effects of clofibrate on bile composition.- - - - - - - - - - ranking = 1keywords = bile (Clic here for more details about this article) |
2/19. Severe hypercholesterolemia mediated by lipoprotein X in patients with chronic graft-versus-host disease of the liver.We describe a series of cases of extreme hypercholesterolemia mediated by lipoprotein X in patients with chronic graft-versus-host disease of the liver after an allogeneic bone marrow transplant. All of the patients presented with a total cholesterol in excess of 1000 mg/dl (25.9 mmol/l). At the time they were also noted to have pseudohyponatremia. Cholesterol appeared to be predominantly carried by lipoprotein X. Intrahepatic cholestasis leading to reflux of bile lipoproteins into the bloodstream and subsequent formation of protein X appears to be the mechanism underlying this phenomenon. Complications, including retinal cholesterol thromboembolism and cholesteroloma of the lung have been seen in the patient with the highest cholesterol levels. Severe hypercholesterolemia is an important, and likely more common than previously reported, long-term complication of allogeneic hematopoietic stem cell transplantation. It is important for clinicians to familiarize themselves with the diagnostic and therapeutic challenges this condition presents.- - - - - - - - - - ranking = 152.01444969574keywords = cholestasis, bile (Clic here for more details about this article) |
3/19. Severe hypercholesterolemia associated with decreased hepatic triglyceride lipase activity and pseudohyponatremia in patients after allogeneic stem cell transplantation.A 55-year-old woman with Ph-negative acute lymphoblastic leukemia in primary induction failure received allogeneic peripheral blood stem cell transplantation from her HLA-compatible sister. Pseudohyponatremia developed due to extreme hypercholesterolemia of 4091 mg/dL accompanied by lipoprotein X and lipoprotein Y.The hypercholesterolemia was caused by cholestasis due to chronic GVHD and ischemic cholangiopathy. In addition, we found that hepatic triglyceride lipase (HTGL) activity was severely decreased, which could be another novel factor causing extreme hypercholesterolemia after allogeneic transplantation. The total cholesterol has been gradually decreasing followed by the improvement of cholestasis with bezafibrate, ursodeoxycholic acid and prednisone treatments, and by a slight increase in HTGL-protein. To our knowledge, this is the first report to describe the association of decreased HTGL with extreme hypercholesterolemia after allogeneic transplantation.- - - - - - - - - - ranking = 302.02889939147keywords = cholestasis (Clic here for more details about this article) |
4/19. Relief of intractable pruritis in alagille syndrome by partial external biliary diversion.BACKGROUND: patients with alagille syndrome (AGS) may develop pruritis, skin hypertrophy, and xanthomas because of chronic cholestasis and hypercholesterolemia. Partial external biliary diversion (PEBD) has been used successfully to treat chronic cholestasis in patients with progressive familial intrahepatic cholestasis (PFIC) and is a potentially useful treatment for patients with severe and intractable pruritis because of AGS. methods: Four children with chronic cholestasis and intractable pruritis were treated with PEBD, 1 by surgical cholecystostomy and 3 by cholecystojejunostomy. RESULTS: Three patients had a known diagnosis of AGS. The fourth was an 11-month-old infant boy with PFIC. The first patient, a 15-month-old boy with AGS, underwent surgical cholecystostomy, which has required frequent tube changes to maintain patency. Three patients underwent PEBD using a segment of jejunum as a conduit between the gallbladder and the skin, where bile is collected in a standard ostomy appliance. Mean follow-up is 15.5 months (range, 9-26 months). All patients experienced rapid and enduring relief of pruritis. Two adolescents with AGS had significant improvement of the hypertrophic skin of their hands. There was one significant complication: the infant with PFIC required reoperation for bleeding from the jejunal anastomosis 1 week after PEBD; he has subsequently done well. One adolescent girl with AGS initially had difficulty with her ostomy because of poor site placement and partial retraction, but nevertheless has managed quite well. The patient treated by cholecystostomy has had excellent relief of his pruritis and is being considered for conversion to cholecystojejunostomy. CONCLUSIONS: Chronic cholestasis caused by AGS can cause debilitating symptoms that are resistant to medical therapy. Partial external biliary diversion is a safe and technically straightforward operation that may be effective for the relief of intractable pruritis and other symptoms in patients with AGS.- - - - - - - - - - ranking = 756.07224847868keywords = cholestasis, bile (Clic here for more details about this article) |
5/19. The cholesterol turnover, synthesis, and absorption in two sisters with familial hypercholesterolemia (type IIa).To explore the mechanisms of the profound plasma cholesterol elevations in familial homozygous hypercholesterolemia (type IIa), cholesterol turnover, sterol balance, cholesterol absorption, and low density lipoprotein studies were carried out under controlled dietary conditions in two sisters (aged 13 and 16). Cholesterol turnover was prolonged. The half-life of the first exponential of the plasma cholesterol specific activity decay curve was double that of normal adults. The rate constants for the removal of cholesterol from pool A (KAA = 0.0652) and for the excretion of cholesterol from the system (Kaa = 0.0197) were less than half of normal. The production rates of cholesterol were low, only 6.30 and 6.86 mg/kg per day as measured by cholesterol turnover and sterol balance techniques, respectively. Fecal neutral steroid and bile acid excretion were 5.22 and 1.64 mg/kg per day, which is remarkably low in comparison to those of normal and heterozygous children. Cholesterol absorption was within the upper limit of the values reported for normal adults. THE HDL cholesterol values were extremely low (27 mg/dl) in contrast to profoundly elevated LDL levels. The fractional catabolic rate of LDL (0.127 per day) and the rate of synthesis and catabolism of apo-LDL (15 mg/kg per day) were low in comparison to previously reported values in homozygotes. These composite data indicated that the definable metabolic defects of these two sisters with homozygous familial hypercholesterolemia were the sluggish clearance of cholesterol from the body coupled with low total body synthesis of cholesterol.- - - - - - - - - - ranking = 1keywords = bile (Clic here for more details about this article) |
6/19. Hyperviscosity syndrome in a hypercholesterolemic patient with primary biliary cirrhosis.A 45-yr-old woman with primary biliary cirrhosis, xanthomatosis, and marked hypercholesterolemia developed symptoms of the hyperviscosity syndrome on three separate occasions. On presentation, she had a plasma total cholesterol concentration of 53.40 mM (2065 mg/dl) and a relative serum viscosity of 2.9. Following three courses of plasma exchange in a 5-day period, the total cholesterol level decreased to 6.75 mM (261 mg/dl) and the viscosity to 1.3. The cutaneous xanthomata were markedly diminished 1 wk following plasma exchange. Despite therapy with colestipol (30 g/d), the hyperviscosity syndrome developed 147 days later. This cycle recurred again 137 days after colestipol was discontinued. serum viscosity and total cholesterol concentration were highly correlated during the postexchange or accumulation phases (R = 0.95, 95% CI: 0.85, 0.98) and during the exchange or interventional phases (R = 0.95, 95% CI: 0.84, 0.99). serum viscosity was less significantly correlated with total serum protein (R = 0.84; 95% CI: 0.55, 0.95) or with plasma triglyceride (R = 0.63; 95% CI: 0.26, 0.81). There were no significant correlations of red cell mass, plasma fibrinogen levels, or serum bile salts with viscosity. Subfractionation of plasma into lipoprotein classes showed 45% of total cholesterol in the lipoprotein X fraction and a presumptive slow alpha-lipoprotein species. It is postulated that both the hyperviscosity syndrome and rapid resolution of xanthomata in the patient may be attributable to the physiology of her abnormal lipoprotein particles.- - - - - - - - - - ranking = 1keywords = bile (Clic here for more details about this article) |
7/19. cholesterol ester storage disease: clinical, biochemical, and pathological studies of four new cases.cholesterol ester storage disease (CESD) is infrequent in children. Four new cases in two nonrelated families are presented. Acid lipase deficiency in the leukocytes of the patients and reduced activity (50%) in those of parents were demonstrated. Clinical manifestations varied from neonatal cholestasis to asymptomatic hepatomegaly. Hepatic histology showed lipid vacuoles and cholesterol ester storage in hepatocytes and kupffer cells. Increased levels of cholesterol and hepatomegaly were the first findings. There is as yet no specific treatment for CESD; however, the early detection of cases would make possible the timely control of complications.- - - - - - - - - - ranking = 151.01444969574keywords = cholestasis (Clic here for more details about this article) |
8/19. hypertriglyceridemia: a contraindication to the use of bile acid binding resins.bile acid binding resins are indicated for treatment of elevated plasma low-density lipoprotein cholesterol concentrations. In normoglyceridemic patients, plasma concentrations of low-density lipoprotein cholesterol and total cholesterol are closely linked, and for this reason resin treatment of elevated total cholesterol levels in these patients is rational and warranted. Hypertriglyceridemic patients may also have elevated total cholesterol concentrations, and several such patients have been treated with resin. However, resin therapy in these patients is hazardous, since the elevated cholesterol level mainly results from increased plasma concentrations of very-low-density lipoproteins or chylomicrons rather than low-density lipoproteins. In these patients, resin treatment has either no effect or an adverse effect on plasma triglyceride and cholesterol concentrations. The data presented show that fewer than 5 percent of patients whose total plasma triglyceride concentration exceeded 500 mg/dl had elevated low-density lipoprotein cholesterol concentrations, irrespective of total plasma cholesterol concentrations, and no patient whose plasma triglyceride concentration exceeded 1,000 mg/dl had elevated low-density lipoprotein cholesterol levels. Treatment with bile acid binding resins in hypercholesterolemic patients is contraindicated in the presence of hypertriglyceridemia.- - - - - - - - - - ranking = 5keywords = bile (Clic here for more details about this article) |
9/19. Gastric xanthomatosis and cholestasis. A causal relationship.We report two cases of gastric xanthomatosis which developed in patients with marked cholestasis. In both cases, one with acute and one with chronic cholestasis, the gastric xanthomas disappeared with resolution of the cholestasis. A review of the literature is also provided.- - - - - - - - - - ranking = 1057.1011478702keywords = cholestasis (Clic here for more details about this article) |
10/19. hypercholesterolemia associated with alpha-1 antitrypsin deficiency and hepatitis: lipoprotein and apoprotein determinations, sterol balance and treatment.lipid metabolism was investigated in a 4-year-old boy with alpha-1 antitrypsin deficiency (ZZ phenotype) and liver disease. Plasma cholesterol and triglyceride levels were 604 mg/dl and 336 mg/dl respectively. Both parents had normal plasma lipids. Lipoprotein X was present at a concentration of 855 mg/dl and levels of apoproteins A-I, A-II, B and C-III were elevated. The plasma free fatty acid pattern was normal. Plasma cholesterol esterification was greatly depressed. Cholesterol absorption on two occasions was reduced about 13% compared with adult controls. Neutral and total steroid excretion was normal with increased excretion of bile acids. A low-cholesterol, low-fat diet reduced plasma cholesterol to 374 mg/dl and triglyceride to 236 mg/dl in two months. Cholesterol and lipoprotein X concentrations were elevated far out of proportion to the severity of the liver disease (total bilirubin 3.7 mg/dl, SGOT 280 IU/L). This suggests that lipoprotein metabolism in patients with this disorder is unusual and may differ from the derangements seen in other forms of liver disease.- - - - - - - - - - ranking = 1keywords = bile (Clic here for more details about this article) |
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