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  • 1
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    Cardiovascular drug reviews 20 (2002), S. 0 
    ISSN: 1527-3466
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Sitosterolemia was first described 28 years ago in two sisters. They had tendon xanthomas, normal plasma cholesterol levels, and elevated plant sterol levels. The high plant sterol levels were shown to be due to the increased absorption and delayed removal of plant sterols from the body. The increased absorption of plant sterols does not affect cholesterol absorption in these patients. However, cholesterol biosynthesis pathway is downregulated and turnover rates are reduced. Bile acid binding resins and ileal bypass surgery are effective treatments for sitosterolemic patients, whereas statins are ineffective. Sitosterolemia is inherited as a recessive trait. Recent studies have shown that mutations in ABCG5 and ABCG8 genes contribute to sitosterolemia. Most likely, ABCG5 and ABCG8 proteins function as obligate heterodimers and play a role in the absorption of plant sterol or control their rate of absorption.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Pediatric surgery international 5 (1990), S. 91-93 
    ISSN: 1437-9813
    Keywords: Biliary atresia ; Malabsorption ; Bile acids ; Kasai portoenterostomy ; Malnutrition
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Accelerated weight-gain velocity was observed in five infants with extraheptic biliary atresia following closure of the exteriorized conduit. Despite excellent bile drainage (daily volumes 150–900 ml) and refeeding of this fluid to the efferent limb, steatorrhea occurred in three patients and was suspected in the other two. Total bile-acid concentrations in the bile collected prior to refeeding into the jejunum were below the critical micellar concentration in most of the specimens tesred. The nutritional consequences of bile diversion need to be balanced against the beneficial effects in diminishing the incidence of postoperative cholangitis. We postulate that fat malabsorption occured in these children because of inadequate bile-acid concentrations and inefficient fat emulsification. Bile acids were normally conjugated with glycine and taurine.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1546-1718
    Source: Nature Archives 1869 - 2009
    Topics: Biology , Medicine
    Notes: [Auszug] Familial hypercholanemia (FHC) is characterized by elevated serum bile acid concentrations, itching, and fat malabsorption. We show here that FHC in Amish individuals is associated with mutations in tight junction protein 2 (encoded by TJP2, also known as ZO-2) and bile acid Coenzyme A: amino acid ...
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1546-1718
    Source: Nature Archives 1869 - 2009
    Topics: Biology , Medicine
    Notes: [Auszug] The molecular mechanisms regulating the amount of dietary cholesterol retained in the body, as well as the body's ability to exclude selectively other dietary sterols, are poorly understood. An average western diet will contain about 250–500 mg of dietary cholesterol and about 200–400 ...
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    Digestive diseases and sciences 34 (1989), S. S4 
    ISSN: 1573-2568
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 6
    Electronic Resource
    Electronic Resource
    Springer
    Digestive diseases and sciences 34 (1989), S. S39 
    ISSN: 1573-2568
    Keywords: cholesterol gallstones ; HMG CoA reductase ; cholesterol 7α-hydroxylase ; ursodiol
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The balance between the synthesis of cholesterol and bile acids in the liver is a key factor in the formation of gallstones. Patients with cholesterol gallstones have been shown to have higher rates of hepatic cholesterol synthesis and lower rates of bile acid synthesis than control subjects, as measured by the activity of the respective rate-controlling enzymes. Treatment with ursodiol reduced the high levels of hydroxymethylglutaryl coenzyme A reductase in gallstone patients treated for approximately one year. Such treatment did not appear to inhibit endogenous synthesis of bile acids. Ursodiol is distinct from chenodeoxycholic acid in that no significant abnormalities of liver function have been reported during its use. Bacterial 7-dehydroxylation is more active for chenodeoxycholic acid than ursodiol; thus less lithocholic acid is formed with ursodiol. Patients receiving the highest dose of ursodiol often have been shown to have the lowest percentage of lithocholic acid in the bile. During treatment with ursodiol for dissolution of gallstones, symptoms of biliary distress began to improve after three to six weeks. Gallstones will recur in about 50% of patients, but no consensus exists on management of patients after dissolution of their stones. Overall, ursodiol is a safe and effective litholytic agent.
    Type of Medium: Electronic Resource
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  • 7
    ISSN: 1573-2568
    Keywords: external shock-wave lithotripsy ; gallstones ; multicenter trial
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A prospective multicenter trial was performed to evaluate the use of external shockwave lithotripsy (ESL) and adjuvant medical therapy for the treatment of gallstones. A Medstone STS lithotripter was used together with ursodiol. Two hundred twenty-three patients were treated under general anesthesia (75%) or with intravenous analgesia (25%). Initial treatments were on an inpatient basis, but as centers gained experience, outpatient treatments became more common. Stone fragmentation and clearance were greatest in patients with solitary gallstones 〈2 cm in diameter. In this group of patients, stone fragmentation occurred in 97% of patients, and the cumulative stone-free rates at three and six months were 54% and 90%, respectively. These results indicate that fragmentation of gallstones can be achieved by a dry shock-wave lithotripter and that stone clearance is induced more rapidly by external shock-wave lithotripsy and adjuvant ursodiol therapy than by ursodiol therapy alone.
    Type of Medium: Electronic Resource
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  • 8
    Electronic Resource
    Electronic Resource
    Springer
    Digestive diseases and sciences 19 (1974), S. 920-929 
    ISSN: 1573-2568
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 9
    Electronic Resource
    Electronic Resource
    Springer
    Digestive diseases and sciences 14 (1969), S. 170-181 
    ISSN: 1573-2568
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Seven patients with small-intestinal diverticulosis were studied by means of intestinal absorptive tests, biopsies, and bacterial cultures of small-intestinal aspirates. Aerobic bacterial cultures were obtained in all patients, while anaerobic cultures with a modified Hungate roll-tube technic were performed in 3. Three of the patients manifested steatorrhea and were studied over prolonged periods of time. The bacterial flora varied over a wide quantitative range, with highest total counts in excess of 109 colony-forming units per milliliter found in the 2 patients with the most severe malabsorption. Despite multiple diverticulosis, 1 patient without steatorrhea had no recoverable aerobic or anaerobic bacterial flora. E. coli predominated among the recovered aerobic organisms in this series, but A. aerogenes and various streptococci also were encountered repeatedly. Patients treated with antibiotics directed at the aerobic flora showed prompt improvement in absorption, and reduction in bacterial counts. Repeated short courses of antibiotics were needed to treat early bacteriologic and clinical relapses. After several such courses, all 3 patients treated maintained their clinical and metabolic improvement for periods of close observation ranging from 2 to 15 months without additional antibiotic treatment, and the clinical remissions were accompanied by gradual reductions in total bacterial counts to “borderline normal” limits. Of the 3 patients, 1 has now been followed for 5 years without relapse. It is apparent that the mere presence of diverticulosis does not lead necessarily to malabsorption. Malabsorption of fat, Vitamin B12, and other nutrients was found only in patients with excessive bacterial flora, emphasizing the crucial role bacteria play in the pathogenesis of malabsorption in diverticulosis of the small intestine.
    Type of Medium: Electronic Resource
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