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)

Patients with cystic fibrosis (CF) and pancreatic insufficiency usually have decreased linoleic and increased oleic, palmitoleic and eicosatrienoic (20:3 omega 9) acids compared to normal values of blood and tissue lipids. These changes are consistent with early essential fatty acid deficiency and are observed despite the regular use of exogenous pancreatic enzyme supplementation. As part of a study to determine the relative role of malabsorption as the etiology for the altered fatty acid status, the change in total plasma fatty acids and in area percent of plasma linoleic acid was determined in CF patients and control subjects following the ingestion of various lipid supplements, including two safflower oil preparations and two structured lipid preparations. Fasting subjects consumed 36 g of lipid in a milkshake containing 15 g of protein and 45 g of carbohydrate. Plasma samples obtained 0, 2, 4, 6 and 8 hr after the meal showed that the CF patients absorbed all preparations when administered with their regular dose of pancreatic enzyme supplement. Comparison of the patterns of increase for total plasma fatty acids and area percent of plasma linoleic acid following the administration of the different lipid supplements in CF patients and control subjects suggests that malabsorption alone is not the cause of the abnormal fatty acid composition in the lipids of CF patients and that increased caloric intake along with consumption of adequate amounts of linoleic acid should improve the linoleic acid status of CF patients; there may be selectively increased metabolism of certain fatty acids from the ingested lipids in the relatively malnourished CF patient compared to control subjects.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Absorption of safflower oil and structured lipid preparations in patients with cystic fibrosis. 361 73

Fifteen school-age cystic fibrosis children, participating in a year-long nutritional management study, were hospitalized at six-month intervals for balance studies during which they continued "free-choice" diets and their usual enzyme supplementation. Stools were analyzed for fat and protein by conventional methods and for carbohydrate using a recently validated anthrone method. Despite persistent fat and protein malabsorption, less than 1% of ingested carbohydrate was lost intact in the stools. Comparison of baseline and placebo balance studies showed fecal excretion of carbohydrate to be independent of intake, in contrast to the fat and protein results. Using a thin-layer chromatography method capable of detecting microgram quantities of urinary organic acids, no short-chain fatty acids were detected in the stool. Further exploration of carbohydrate as a dietary energy source for this patient group with increased energy demands should be pursued.
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PMID:Carbohydrate malabsorption is minimal in school-age cystic fibrosis children. 365 95

The effect of taurine supplementation on the absorption of a fat meal was evaluated in patients with cystic fibrosis. In a cross-over design study, five patients with cystic fibrosis (12.1 +/- 2.6 years of age) and three control subjects received either placebo or taurine (30 mg/kg/d) for two 1-week periods, a month apart, followed by a fat meal test. Blood samples were drawn 0, 1, 2, 3, 5, 8 hours after the meal. Four patients with cystic fibrosis and severe steatorrhea despite appropriate enzyme therapy showed a significant (P less than .05) improvement in the absorption of triglycerides, total fatty acids, and linoleic acid while receiving taurine supplements. Three control subjects and one child with cystic fibrosis and mild steatorrhea receiving enzyme therapy did not experience such an effect. The difference in triglyceride absorption, when calculated as the area under the curve, receiving and not receiving taurine was significantly (P less than .05) correlated with the degree of steatorrhea. Furthermore, in contrast to control subjects, the fatty acid composition of chylomicrons in these four study patients showed important discrepancies with that of the fat meal and was corrected, in part, by taurine supplementation. These results suggest that taurine supplementation could be a useful adjunct in the management of patients with cystic fibrosis with ongoing fat malabsorption and essential fatty acid deficiency.
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PMID:Taurine improves the absorption of a fat meal in patients with cystic fibrosis. 365 70

Fatty acid composition were determined by gas chromatography in serum triglycerides and cholesterol esters of 32 patients (aged 7-17 years) with cystic fibrosis. According to their exocrine pancreas function (determined with secretin-cholecystokinin test), patients were divided in 3 groups. In comparison with previously measured values in 30 metabolically healthy children, patients with cystic fibrosis showed a significantly increased level of palmitic, palmitoleic, stearic, oleic, and linolenic acid in the triglyceride fraction and a significantly increased level of myristic, myristoleic, palmitoleic, stearic, oleic, and triene acid in the cholesterol ester fraction. However, in both fractions, concentration of linoleic acid was significantly decreased. It seems unlikely that malabsorption of ingested fat is responsible for the abnormalities in fatty acid pattern, because no significant differences could be found between the 3 patient's groups.
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PMID:[Fatty acid pattern in children with mucoviscidosis in relation to the degree of exocrine pancreas insufficiency]. 366 28

The gastric pentagastrin-stimulated secretions of acid (peak acid output) and of unsaturated intrinsic factor in eight cystic fibrosis patients (1.4 +/- 0.5 mEq/kg/h and 0.27 +/- 0.12 nmol/kg/h, respectively) were significantly enhanced (p less than 0.05) when compared with six normal controls (0.27 +/- 0.16 mEq/kg/h and 0.10 +/- 0.02 nmol/kg/h, respectively). Despite the gastric hypersecretion of intrinsic factor, no significant physicochemical modification of this glycoprotein was observed in cystic fibrosis when using gel filtration and isoelectrofocusing. Haptocorrin (a cobalamin glycoproteic binder that does not promote the assimilation of cobalamin) also increased in gastric juice after stimulation. Since the sequestration of cobalamin to haptocorrin is pH dependent, the gastric acid hypersecretion observed in cystic fibrosis may explain that the malabsorption of crystalline cobalamin is much more frequent in cystic fibrosis than in chronic pancreatitis.
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PMID:Gastric intrinsic factor hypersecretion stimulated by pentagastrin in cystic fibrosis. 368 75

Serum calcium, magnesium, iron, copper and zinc were measured in 117 patients with cystic fibrosis. Apart from serum iron levels, all the other essential element levels were well maintained and there was no evidence of a need for supplementation. Serum iron was frequently low and the need for iron supplements must be considered after further investigation. The subjects studied covered a wide age range, had widely varying fat malabsorption and clinical grading, and also varying degrees of pulmonary involvement. We found no evidence that any of these parameters directly influenced essential element status as assessed by serum levels.
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PMID:Essential element nutritional status in cystic fibrosis. 370 Jan 39

Serum immunoreactive cationic trypsinogen levels were determined in 99 control subjects and 381 cystic fibrosis (CF) patients. To evaluate the status of the exocrine pancreas all CF patients had previously undergone fecal fat balance studies and/or pancreatic stimulation tests. Three hundred fourteen CF patients had fat malabsorption and/or had inadequate pancreatic enzyme secretion (pancreatic insufficiency) requiring oral pancreatic enzyme supplements with meals. Sixty-seven CF patients did not have fat malabsorption and/or had adequate enzyme secretion (pancreatic sufficiency) and were not receiving pancreatic enzyme supplements with meals. Mean serum trypsinogen in 99 control subjects was 31.4 +/- 14.8 micrograms/liter (+/- 2 SD) and levels did not vary with age or sex. In CF infants (less than 2 yr) with pancreatic insufficiency, mean serum trypsinogen was significantly above the non-CF values (p less than 0.001). Ninety-one percent of the CF infants had elevated levels. Serum trypsinogen values in the pancreatic insufficient group declined steeply up to 5 years, reaching subnormal values by age 6. An equation was developed which described these age-related changes very accurately. Only six CF patients with pancreatic insufficiency had serum trypsinogen levels above the 95% confidence limits of this equation. In contrast, there was no age related decline in serum trypsinogen among the CF group with pancreatic sufficiency. Under 7 yr, serum trypsinogen failed to distinguish the two groups. In those over 7 yr of age, however, serum trypsinogen was significantly higher than the CF group with pancreatic insufficiency (p less than 0.001), and 93% had values within or above the control range.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Age-related alterations of immunoreactive pancreatic cationic trypsinogen in sera from cystic fibrosis patients with and without pancreatic insufficiency. 370 9

Cystic fibrosis (CF) is an autosomal recessive condition affecting one in 2,000 live births in the UK. There are few reports of malignant tumours in this condition probably because, until recently, the majority died before the age of 30 years as a result of recurrent and chronic bronchopulmonary infection with impaired growth and development and resistance to infection due to pancreatic malabsorption. We describe an adult male with CF who died from an adenocarcinoma affecting the ileocaecal region of the bowel.
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PMID:Bowel adenocarcinoma in a patient with cystic fibrosis. 373 68

From a systematic neonatal screening for cystic fibrosis in the Basse-Normandie area and to prevent disorders of the intestinal transit related to malabsorption, neonates then infants were given a semi-elemental hypercaloric diet, with supplements in nitrogen, MCT, minerals, vitamins and low in LCT. Diets were adjusted every month during a consultation using clinical and biological parameters. Results in the first 14 children showed that clinically as well as biologically, these children may remain within the normal range, avoiding the previously reported growth retardation and mineral or vitamin deficiencies. This procedure should allow an improvement in the quality of life and prognosis of such children, by maintaining adequate nutritional status.
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PMID:[Nutritional management of neonates and infants with cystic fibrosis of the pancreas detected at birth]. 376 82

An oral solution containing lactulose and L-rhamnose was administered to six patients with cystic fibrosis. Intestinal absorption of both sugars was assessed by measurement of their urinary excretion. The lactulose: L-rhamnose excretion ratio was raised, supporting the hypothesis of an intestinal component to the malabsorption of cystic fibrosis.
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PMID:Intestinal malabsorption in cystic fibrosis. 378 93


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