Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0042961 (volvulus)
 4,305 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Glucose-6-phosphate dehydrogenase (E.C. 1.1.1.49) was partially purified from the extracts of adult Onchocerca volvulus by affinity chromatography on 2'5'ADP-Sepharose-4B. Kinetic studies revealed a typical bell-shaped pH profile with an optimum lying between pH 7.3 and 7.8. The apparent Km for glucose-6-phosphate was 5.66 x 10(-5) M, whereas that for NADP was 2.17 x 10(-6) M. Suramin, a filaricidal drug, inhibited the enzyme competitively with respect to NADP as a substrate: the apparent Ki values were 2.23 x 10(-6) M and 4.21 x 10(-7) M, respectively, for the crude and purified enzyme preparations. Glucose-6-phosphate dehydrogenase therefore, could be one of the targets of suramin in vivo.
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PMID:Studies on glucose-6-phosphate dehydrogenase from the human parasite, Onchocerca volvulus. 338 9

NADP-linked malic enzyme (malate dehydrogenase (oxaloacetate-decarboxylating) NADP+, EC 1.1.1.40) has been partially purified from adult Onchocerca volvulus and Dirofilaria immitis. Suramin was found to inhibit the activity of malic enzyme from both filarial worms. The inhibition constants for suramin were calculated to be 0.011 microM and 0.015 microM for the enzymes from O. volvulus and D. immitis, respectively. In the case of NADP-linked malic enzyme from Trypanosoma brucei and chicken liver the inhibition by suramin was less pronounced. The inhibition constants were found to be 0.8 microM and 2.5 microM for the protozoan and vertebrate enzymes, respectively. The type of inhibition was competitive with respect to malate. The Michaelis constants for malate and pyruvate were determined to be 0.9 and 4.5 mM for O. volvulus and 0.85 and 5.0 mM for D. immitis, respectively. The low Km values for malate compared to those for pyruvate and the about 15-fold greater turnover in the direction of decarboxylation compared to carboxylation indicated that malic enzyme from both filarial sources might be involved in an alternative pathway leading from phosphoenolpyruvate via oxaleacetate, malate and pyruvate to lactate. It is suggested, that the inhibition of malic enzyme activity from O. volvulus by suramin might interfere with the generation of NADPH for biosynthetic reactions.
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PMID:Inhibition of NADP-linked malic enzyme from Onchocerca volvulus and Dirofilaria immitis by suramin. 732 87

While small-bowel transplantation remains an option for the management of short-bowel syndrome (SBS), every effort must be made to optimize the function of the native bowel. This report describes a patient with SBS who dramatically improved after a Bianchi procedure. The patient was born with type IIIb jejunal atresia, complicated by volvulus of the terminal ileum. He was left with 40 cm of small bowel and his ileocecal valve. During the first 20 months of his life, he was always hospitalized except for 3 months. He was on parenteral nutrition (TPN) and suffered multiple episodes of central line sepsis. Massive small-bowel dilatation caused a functional bowel obstruction with bacterial overgrowth, and villous atrophy of the jejunal mucosa was discovered on biopsies done by colonoscopy. In March 1994, we elected to proceed with a Bianchi procedure. Thirty cm of jejunum were divided longitudinally. During the following year, his enteral tolerance steadily improved to the point where TPN could be discontinued. We conclude that pediatric surgeons should not hesitate to use this procedure in the management of SBS.
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PMID:The Bianchi procedure in a patient with jejunal atresia. 949 89

We analyzed clinical, biochemical, and histo- logic parameters of ten infants with parenteral nutrition-induced hepatobiliary dysfunction. The data were compared with the results of a rabbit model. All infants were born prematurely with low birth weight. Their clinical diagnoses were necrotizing enterocolitis (6), gastroschisis (1), intrauterine volvulus (1), and lung hypoplasia (2). All required total (TPN) or partial parenteral nutrition for at least 8 weeks. All had repeated episodes of infections or sepsis. A rise in bilirubin and aminotransferase levels occurred after a minimum of 5 weeks; peak bilirubin levels ranged from 4 to 14 mg% and aminotransferases from 40 to 140 IU/l. One child later developed gallstones. Liver biopsies after 1 to 24 months showed fibrosis, bile-duct proliferation, cholestasis, and hydropic degeneration. All of the above-mentioned clinical factors have been accused of causing the observed biochemical and histologic changes. In our rabbit model we were able to produce almost identical symptoms by TPN alone: gallbladder distension, sludge, and stones developed after 1-4 weeks of TPN as well as uncharacteristic changes in aminotransferases and bilirubin after 4 weeks. Liver histology revealed severe hydropic degeneration of zone 3 as early as 1 week after beginning TPN. A rise of fibrosis and bile-duct proliferation after 1 to 4 weeks of infusion was statistically significant. Cholestasis, as was observed in the infants, could not be detected. In our model, all alterations observed could be attributed exclusively to TPN. We therefore assume that TPN was the true cause of the dysfunction. In a second experimental series infusions were reduced to 80% PN and free access to lab chow. These animals produced normal feces, indicating physiologic enteral stimulation. They developed the same degenerative and proliferative histologic changes, whereas gallbladder distension, sludge, and stones were not noted. We conclude that: (1) The TPN solution itself is responsible for the histologic changes in the liver, which is supported by the fact that hydropic degeneration of zone 3 is typical of a direct toxic effect; and (2) Complete enteral starvation with an absence of enteral stimulation causes disease of the lower biliary tract.
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PMID:Parenteral nutrition-induced hepatobiliary dysfunction in infants and prepubertal rabbits. 1052 3

Short bowel syndrome is a spectrum of malnutrition resulting from inadequate bowel length. In infant and pediatric patients, the most common causes are necrotizing enterocolitis, abdominal wall defects, jejunal ileal atresia, and mid gut volvulus. There appear to be regional variations in etiology. Since the publication of Wilmore's classic monograph in 1972, there have been significant improvements in monitoring and nutritional support. In the modern era, survival rate ranges from 80% to 94%, and the presence or absence the ileal cecal valve appears to not impact on mortality rate, but does significantly affect the length of time on total parenteral nutrition TPN. The most common morbidities remain sepsis, both central line related and bacterial overgrowth, and TPN cholestasis. Long-term recovery of these children often is remarkably normal, but there is a 10% to 15% incidence of neurologic and developmental defects. The clinical and ethical considerations around the care of infants with 20 to 40 cm of residual bowel remains controversial, as does the place of intestinal transplantation, especially in patients developing gut failure in infancy. Perioperative surgical decision making plays a critical role in the long-term outcome of these patients. This chapter presents an overview of the current status of care and outcome in this difficult population; these topics are further expanded in subsequent chapters.
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PMID:Short bowel syndrome in infants and children: an overview. 1132 5

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

Management of severe Short Bowel Syndrome (SBS) is still one of the largest challenges of the medicine. Vast majority of the short bowel patients are children, the conditions that lead to this possible outcome most often are necrotizing enterocolitis (NEC), small intestinal volvulus as a result of intestinal malrotation, gastroschisis and the "apple peel" syndrome. Therefore, paediatricians and paediatric surgeons face this challenge most often. The nontransplant treatment appears to be effective using surgical procedure to increase absorptive surface and to reduce the transit time, but in some cases these procedures are enough to weaning of TPN. The aim of this review was to summarize the modern non-stransplant surgical management of short bowel syndrome.
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PMID:Non-transplant Surgical Management of Short Bowel Syndrome in Children: An Overview. 3049 16