Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0024523 (malabsorption)
 7,319 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Subjects with a variety of enteropathies, hemolytic anemias, acute respiratory distress syndrome, hepatitis, Gaucher's disease as well as those on TPN and hemodialysis, often have low ("deficient") blood levels of vitamin E. A deficiency of vitamin E can be manifested by accelerated red blood cell destruction and neuromuscular deficit. Supplementation of these patients may be advisable. Neurological dysfunction has been observed in adults with prolonged vitamin E deficiency resulting from lipid malabsorption. Long-term treatment with high doses of vitamin E results in improvement. Administration of 800 IU/day of vitamin E to subjects with G6PD deficiency, sickle-cell anemia and beta-thalassemia has resulted in improvement of hematological parameters. Supplementation with 300 IU/day for 3-6 months has resulted in improved walking distances and improved blood flow in patients with intermittent claudication. In a limited number of controlled studies, 300-600 IU/day resulted in improvement in premenstrual syndrome, tardive dyskinesia and also arthritis. Epidemiological studies suggest that high levels of serum vitamin E are associated with lower risk of certain cancers, cardiovascular disease and infections. In some cases the high levels are difficult to obtain by diet alone. High levels of vitamin E are contraindicated in subjects who are receiving vitamin K antagonists as anticoagulant therapy. Except for this interaction with vitamin K, there are no specific side effects associated with high doses of vitamin E. Thus, there are various reasons for supplementations with vitamin E and, with the exception noted, the risk of such supplementation is very low.
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PMID:Use and safety of elevated dosages of vitamin E in adults. 250 7

Nutritional support, whether enteral or parenteral, is an important part of the treatment of IBD. Inadequate oral intake, malabsorption, and increased gastrointestinal losses all contribute to malnutrition. Weight loss, cachexia, abnormal body composition, and multiple micronutrient deficiencies are common. Acute repletion of body weight and correction of specific nutrient deficiencies improve the patients' sense of well-being and decrease morbidity, especially in the perioperative period. If a short period of bowel rest (10 to 14 days) is part of the medical therapy of acute exacerbations of IBD, TPN should be administered to prevent further nutritional deficiencies. Chronic undernutrition, and growth failure in children, usually are best treated by intensive enteral supplementation. Prolonged bowel rest and TPN (4 to 6 weeks) have not been shown to improve outcome but may be appropriate in carefully selected patients. Long-term home TPN may be necessary for patients who have short gut syndrome. The mainstay of treatment for IBD is medical therapy including corticosteroids. Timely and appropriate surgery is equally important and should not be considered a last resort. Careful nutritional management is essential but is adjunctive rather than primary therapy.
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PMID:Nutrition and inflammatory bowel disease. 250 55

Home parenteral nutrition (HPN) provides long-term nutritional support for persons whose absorptive capacity is compromised by a variety of intestinal malabsorption problems. However, the presence of vitamin and mineral deficiency syndromes that normally would not have time to develop in the hospitalized patient receiving total parenteral nutrition has been reported in patients receiving HPN. This study entails a longitudinal survey of plasma concentrations of vitamins A, E, and 1,25-dihydroxyvitamin D, as well as the minerals zinc, copper, and selenium, in patients receiving HPN. Plasma samples from eight patients who had been on HPN for 1-92 months before the study began were obtained once a month over a 12-month period. The blood was drawn immediately before their evening infusion of TPN in order to approximate fasting plasma nutrient concentrations. Patient values were compared to fasting control values and to published norms. Values for vitamin A, 1,25-dihydroxyvitamin D, and zinc all were within the normal range, and there was no evidence of metabolic bone disease. Plasma vitamin E and copper concentrations exceeded the normal range for most of the 12-month period. Of all of the nutrients studied, only plasma selenium concentrations were consistently in the low-normal to below-normal range. Selenium levels in patients on HPN should be monitored regularly, and supplementation may be necessary if clinical conditions warrant.
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PMID:Plasma vitamin and mineral status in home parenteral nutrition patients. 311 95

Cholestasis associated with TPN is now diminishing in frequency. One factor is likely to be the early initiation of oral feeding. One of the effects of oral intake is to restore the enterohepatic circulation of bile acids. This in turn promotes lipid absorption, which leads to improved nutrition and a decreased in mucosal atrophy. The role of the enterohepatic cycling of bile acids has been explored in this review. In infants or children on TPN who have bile acid malabsorption, UDCA can be used to correct decreased secretion of endogenous bile acids. However, controlled studies showing that UDCA actually decreases moribidity and mortality in infants on prolonged TPN have not yet been performed and they are sorely needed. UDCA is nontoxic and acts as a natural bile acid after conjugation. An improved formulation of UDCA with rapid and efficient intestinal absorption has been developed, but the optimal formulation of UDCA is still not available. Cholylsarcosine, a new conjugated bile acid analogue that has been developed as a bile acid replacement agent, improves lipid absorption in animals with steatorrhea caused by bile acid malabsorption and intestinal resection. However, this compound has not been tested clinically.
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PMID:Defective biliary secretion during total parenteral nutrition: probable mechanisms and possible solutions. 763 79

Patients with malabsorption and histological findings consistent with celiac disease, who are unresponsive to gluten free diet, and in whom other causes of flat mucosa have been excluded, are considered to suffer from so called unclassified or refractory sprue. Although the true nature of this condition needs to be further elucidated, it is known to represent a difficult therapeutical problem with potentially fatal course. Herein, we report a patient with refractory sprue-like disease who after failing to respond to corticosteroids and TPN was in a critical condition. He responded promptly to cyclosporine and made a remarkable recovery. In contrast to previous reports, the cyclosporine treatment in this patient was pursued only for 1 month, whereupon the patient turned responsive to steroids. Subsequent treatment with azathioprine allowed corticosteroids to be reduced to a low maintenance dose and eventually all drugs could be discontinued without reappearance of symptoms. Cyclosporine therapy might be lifesaving in occasional patients with refractory sprue-like disease and it may result in reversal of steroid resistance. Moreover, azathioprine seems to have a steroid sparing effect in this setting. Short term immunosuppressive treatment may have an advantage of lower risk for drug related side effects.
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PMID:Role of immunosuppressive therapy in refractory sprue-like disease. 993 59

This study examined the prevalence of vitamin K deficiency in children pre-bone marrow transplantation (BMT). Vitamin K status was measured by the PIVKA-II assay and prothrombin times. Blood samples were obtained before vitamin-containing TPN was infused. Results indicated that eight of 26 patients (31%) were vitamin K deficient; four cases were attributed to drug antagonism (phenytoin) and four were due to inadequate vitamin K intake, synthesis or malabsorption. Only one patient had a prolonged prothrombin time. Prothrombin time, in our study, is shown to be an ineffective screening tool to determine vitamin K status. All patients receiving phenytoin and chemotherapy are at increased risk of vitamin K deficiency.
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PMID:Vitamin K deficiency in children pre-bone marrow transplantation. 1628 12

Short bowel syndrome (SBS) is a malabsorptive state occuring as a result of surgical resection or congenital disease of a significant portion of the small intestine . The amount of resection or remaining bowel generally dictates the degree of malabsorption and consequentely the need for specialized enteral nutrition or parenteral nutrition (PN). Intestinal failure in the context of SBS is defined as a dependence on PN to maintain minimal energy and fluid requirement for growth in children. Common causes of SBS in infants and children include necrotizing enterocolitis, midgut volvulus, intestinal atresia, and gastroschisis. Early identification of patients at risk for long-term PN dependency is the first step toward avoiding severe complications. Close monitoring of nutritional status, steady and early introduction of enteral nutrition, and aggressive prevention, diagnosis, and treatment of infections such as central venous catheter sepsis and bacterial overgrowth can significantly improve the prognosis. Intestinal transplantation is an emerging treatment that may be considered when intestinal failure is irreversible and children are experiencing serious complications related to TPN administration.
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PMID:Overview of pediatric short bowel syndrome. 1866 16

Only a dozen cases of pancytopenia caused by copper deficiency have been reported. We report the case of an 81-year-old man who underwent total gastrectomy for gastric cancer. He received total parenteral nutrition without trace element supplementation for 6 months. Serum levels of copper and ceruloplasmin were low, but serum zinc was normal. The administration of copper into TPN led to rapid improvement in anemia and neutropenia. We review 11 previous cases of copper-deficient cytopenia, categorized into two groups according to etiology, and define the characteristic symptom of copper malabsorption caused by excess Zn as peripheral neuropathy.
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PMID:Pancytopenia complicated with peripheral neuropathy due to copper deficiency: clinical diagnostic review. 1904 62

Congenital Glucose Galactose malabsorption (CGGM) is a rare disorder with limited data from the Arab world. We report the first case of CGGM in Oman.B.S.A two years old female who presented with chronic osmotic diarrhea since birth with hypernatraemic dehydration. B.S was found to have Glucose Galactose Malabsorption based on clinical trial of ORS and elemental formula. Symptoms resolved on introduction of Carbohydrate free formula. The patient developed many complications while on TPN including rickets and nephrogenic diabetes insipidus. These complications have not been reported earlier in CGGM.
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PMID:Glucose Galactose Malabsorption complicated with Rickets and Nephrogenic Diabetes Insipidus. 2235 15