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Query: UMLS:C0024523 (malabsorption)
 7,319 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Prevention of malnutrition, a consequence of elevated energy requirements, increased losses and low caloric intake, is one of the main goals in the treatment of cystic fibrosis. Caloric stool losses, catch-up growth and an elevated energy expenditure, even in the absence of overt lung disease and malabsorption, have led to recommendations for a caloric intake of 120-150% of the recommended daily allowances. A high energy intake with a fat content of at least 40 calorie % and adequate pancreatic supplementation has shown to improve growth and median age of survival. As a rational treatment of the fundamental disturbance in cystic fibrosis, a decrease in chloride permeability across epithelia, is not yet available, treatment should be concentrated on adequate nutritional support in combination with optimal correction of those gastrointestinal abnormalities including faecal bile acid loss, small intestinal abnormalities, pancreatic insufficiency, hormonal abnormalities and disturbances in gastrointestinal motility, which may aggravate maldigestion and malabsorption.
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PMID:Gastrointestinal dysfunction and its effects on nutrition in CF. 270 29

Twenty-one pre-adolescent cystic fibrosis (CF) children with good clinical scores had significant (P less than 0.001) deficiencies of arachidonic (mean 3.8% of total plasma fatty acids +/- SD 1.4) and linoleic (18.1 +/- 6.3) acids compared with controls (6.0 +/- 1.0, and 27.6 +/- 3.9, respectively). Despite the presence of pulmonary involvement of varying severity in all the CF children, neither arachidonic nor linoleic acid levels correlated significantly with pulmonary function measured by spirometry. All children had adequate caloric and fat intakes, and the coefficient of fat absorption correlated with none of the deficient fatty acid levels. These findings suggest that deficiencies of arachidonic and linoleic acids are of minor importance in the early development of pulmonary involvement in CF, and that factors other than fat malabsorption and decreased dietary intake probably contribute to fatty acid deficiency.
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PMID:Relationships between essential fatty acid levels, pulmonary function and fat absorption in pre-adolescent cystic fibrosis children with good clinical scores. 270 78

We retrospectively studied 20 patients with cystic fibrosis who underwent surgery for gallbladder disease from 1973 to 1986. A long delay between the onset of symptoms and the diagnosis was noted (mean 7.4 months). This delay was attributed to masking of the symptoms of biliary disease by the malabsorption and pulmonary symptoms seen in this patient population. There was a notable lack of common bile duct disease in our patients as well as in those reported in the literature. This may have been due to a combination of factors: the increased viscosity of the mucus, the small caliber of the gallbladder and ductal system, and the hypotonicity of the gallbladder. We do not recommend routine intraoperative cholangiography in patients with cystic fibrosis and gallbladder disease. Cystic fibrosis is a disease with progressive pulmonary deterioration. Cholecystectomy can be performed in these patients with relative safety if careful preoperative and postoperative care is provided. We recommend early operative intervention in the patient with gallbladder disease and cystic fibrosis.
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PMID:Operative therapy of gallbladder disease in patients with cystic fibrosis. 239 61

Xerophthalmia is a common complication of vitamin A deficiency in communities where malnutrition is found. We report on a 16-month-old infant with severe photophobia and failure to thrive. On examination, her major presenting sign was corneal xerosis, with corneal and conjunctival keratinization, and corneal stromal edema with opacification. Based on these findings, vitamin A deficiency secondary to fat malabsorption was suspected, and a workup confirmed the diagnosis of cystic fibrosis. With parenteral vitamin A supplementation, she had complete resolution of her ocular signs and symptoms. This case illustrates the value of a complete ophthalmic examination in the diagnosis of fat malabsorption syndromes.
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PMID:Advanced xerophthalmia as a presenting sign in cystic fibrosis. 274 92

Polyunsaturated fatty acids are known to affect plasma lipids and lipoproteins but there is no information on the effect of essential fatty acid (EFA) deficiency on lipoprotein composition. The purpose of this study was to characterize lipoproteins from 17 cystic fibrosis (CF) patients in relationship to their EFA status (eicosatrienoic/arachidonic acid ratio) and compare them with those of 10 healthy siblings (SIB) and of 10 unrelated controls. In 7 EFA-deficient (EFAD) and 10 EFA-sufficient (EFAS) patients, hypocholesterolemia was associated with a decrease of HDL-cholesterol and of LDL-cholesterol which was more marked in the EFAD group. Similarly, although triglyceride enrichment of VLDL, LDL, HDL2, and HDL3 with a concomitant reduction of cholesteryl esters from all particles except HDL2 was observed in both CF groups, it was more sizable in the EFAD patients. These changes led to an increase in the particle size of VLDL, LDL, and HDL2 whereas the distribution of HDL3 was skewed to smaller particles. Alterations in the apoprotein composition of particles were greater in EFAD than in EFAS. A decrease of total postheparin lipolytic activity was observed in the two groups of CF patients as well as in siblings. It was entirely accounted for by hepatic lipase (mumol FFA/ml per h) which was more severely diminished in EFAD (2.8 +/- 0.6) than in EFAS (4.4 +/- 0.7) and SIB (5.1 +/- 0.5). Although the two groups of CF children differed in terms of growth, severity of malabsorption, and vitamin E status, these data suggest that disturbance of lipoprotein concentration, composition, size, and metabolism (hepatic lipase) may be in part related to EFA deficiency. Further studies are necessary to explore the effect of EFA deficiency on hepatic lipase activity.
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PMID:Relationship of decreased hepatic lipase activity and lipoprotein abnormalities to essential fatty acid deficiency in cystic fibrosis patients. 276 73

Many approaches have been proposed to differentiate between steatorrhea due to pancreatic insufficiency and intestinal disease. Bo-Linn and Fordtran recently suggested that fecal fat concentration (FFC) is a useful screening test for this distinction. Our aim was to validate their result in a large group of patients. Fecal fat concentrations were calculated for 613 fecal fat tests in 538 patients. Included were 88 patients with pancreatic steatorrhea (13 pancreatic carcinoma, 6 cystic fibrosis, and 69 chronic pancreatitis) and 525 with nonpancreatic steatorrhea. The mean FFC of patients with pancreatic disease (15.0 +/- 1.9 g%, mean +/- SEM) was significantly higher than that of patients with other diseases causing malabsorption (8.9 +/- 0.3 g%, p less than 0.001). Forty-two percent of patients with pancreatic steatorrhea had an FFC below 10 g%. The overlapping of the FFC of steatorrhea due to pancreatic disease and that produced by celiac disease, gastric resection, and other conditions suggests that this approach does not differentiate between pancreatic and intestinal steatorrhea.
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PMID:Fecal fat concentration in the differential diagnosis of steatorrhea. 291 27

A high energy intake, compensating for malabsorption, and the energy cost of lung disease, lung infections, and the underlying metabolic abnormality, is required to ensure normal growth in patients with cystic fibrosis. This goal can be readily achieved by adherence to a high quantity, normally balanced diet (with 40% of the energy as triglycerides of long-chain fatty acids). In contrast, this goal cannot be reached in the majority of patients adhering to low-fat and thus low-energy-containing diets, which almost inevitably lead to malnutrition with wasting and stunting. The prevention of malnutrition may well have considerably enhanced the prognosis of patients at one clinic. Further work is needed to define the interrelationship of nutrition and lung disease, and to define the appropriate nutrient requirements induced by the lung disease per se, recurrent infections, and the underlying disease process.
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PMID:The impact of nutrition in cystic fibrosis: a review. 304 37

Food intake is often low in cystic fibrosis (CF), although the patient usually needs more than the standard recommended daily allowance (RDA). Clinics giving food supplementation from an early age report improved survival and nutritional status. Nutritional improvement has been facilitated by improved forms of pancreatin. An additional calorie intake in CF is required to compensate for losses due to malabsorption and to allow for catch-up growth when necessary. With advanced pulmonary disease there are additional requirements for infection and increased work of breathing. There is also evidence for an increased basal metabolic activity in CF, perhaps related to the fundamental intracellular biochemical disorder. Together these factors add to a daily need for 120%-150% RDA for optimum growth and homeostasis.
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PMID:Nutritional requirements in cystic fibrosis: a review. 304 41

Misoprostol, a synthetic prostaglandin that is known to reduce gastric acid production and stimulate duodenal bicarbonate production, was evaluated in 22 patients with cystic fibrosis. In those patients who had greater than 10% fat malabsorption while taking pancrease the addition of misoprostol significantly reduced the degree of fat malabsorption.
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PMID:Effect of misoprostol on fat malabsorption in cystic fibrosis. 263 1

Natural abundance in vivo carbon-13 topical magnetic resonance (TMR) spectroscopy was used to assess human adipose tissue stores of essential (polyunsaturated) fatty acids. TMR spectra were obtained from 17 normal volunteers and nine cystic fibrosis patients using an Oxford TMR-32 with a surface coil that sampled tissue less than 1 cm below the surface of an extremity. Spectra were taken of lower leg adipose tissue. Polyunsaturated fatty acid content was determined by comparing peak heights of the polyunsaturated peak (internal unsaturated carbons, 128 ppm) to C-1 carboxyl groups (173 ppm). Monounsaturated fatty acid content was determined by subtracting the polyunsaturated peak from the peak observed for all unsaturated carbons (external unsaturated carbon, 130 ppm) and dividing this ratio by the carboxyl peak. In vivo TMR of normal volunteers resulted in observed polyunsaturated fatty acid content of 17.8 +/- 2.1% and a monounsaturated content of 44.8 +/- 3.8%. The polyunsaturated and monounsaturated fatty acid content of adipose tissue from the cystic fibrosis patients was 15.0 +/- 2.0% (p less than 0.005 versus normal volunteers) and 47.8 +/- 6.5% (NS), respectively. One cystic fibrosis patient without fat malabsorption had decreased adipose polyunsaturates, whereas another patient on high calorie gastrostomy feeds had normal levels. Carbon-13 TMR spectroscopy is a sensitive, noninvasive technique for determining essential fatty acid status in subcutaneous adipose tissue of patients with cystic fibrosis.
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PMID:Adipose tissue abnormalities in cystic fibrosis: noninvasive determination of mono- and polyunsaturated fatty acids by carbon-13 topical magnetic resonance spectroscopy. 318 35


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