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

The authors present a case of cystic fibrosis in a 3 months old infant. Clinically, the first manifestation was a severe anemia secondary to iron deperdition through a hemorrhage due to an acquired trouble of thrombin formation. The late was explained by the vitamin K malabsorption and by the hepatocellular insufficiency.
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PMID:[The diagnostic difficulties in a case of a hemorrhagic syndrome in an infant]. 133 78

Cystic fibrosis (CF) is the most common hereditary disease amongst Caucasians with a potentially lethal outcome. The basic defect is a dysregulation of the chloride channels leading to a relative dehydration of the luminal surface of the exocrine cells. The prognosis of CF patients is predominantly related to the progression of their pulmonary disease. This prognosis has improved dramatically during the last decennia and many patients live into adulthood. Gastrointestinal disturbances and, consequently, malabsorption of nutrients represent a major therapeutic problem in CF. A better understanding of the nutritional problems, including the treatment of pancreatic insufficiency, appears to contribute to an improved prognosis. However, the nutritional status of many patients is still poor and studies to optimise treatment are of paramount importance. In addition, with the increased life span, hepatobiliary complications will contribute to the morbidity of these patients and preventive treatment may be warranted. The aim of this Symposium was to improve our knowledge in this important field by considering the interesting and up-to-date contributions of many experts.
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PMID:Gastroenterological Aspects of Cystic Fibrosis. Symposium proceedings. The Hague, The Netherlands, 10 April 1992. 136 64

Previous studies from our groups have demonstrated improvements in biochemical markers of liver function when cystic fibrosis patients with associated liver disease were administered oral ursodeoxycholic acid. The magnitude of the response was somewhat less than that found when comparable doses (10 to 15 mg/kg body wt/day) of ursodeoxycholic acid are given to other liver disease patients; this may be explained by the bile acid malabsorption that is characteristic of the disease. For this reason a dose-response study was carried out in nine cystic fibrosis patients with liver disease to establish whether improved efficacy could be obtained with higher doses. Ursodeoxycholic acid in doses of 5, 10 and 15 mg/kg body wt/day was given orally for consecutive 2-mo periods in a replicated Latin-square design. After this, all patients received 20 mg/kg body wt/day. Liver function, individual serum bile acids and biliary bile acid composition were determined at entry and at the end of each treatment period. Our data demonstrate that the magnitude of the biochemical improvement in serum liver enzymes was significantly greater with higher doses of ursodeoxycholic acid; at 20 mg/kg body wt/day it was similar to that reported for patients with other liver diseases administered lower doses. Biliary ursodeoxycholic acid enrichment increased with increasing doses, attaining 42% +/- 6% of the total biliary bile acids with the highest dose. Fasting serum ursodeoxycholic acid concentrations increased during ursodeoxycholic acid administration but were variable and correlated poorly with the dose of ursodeoxycholic acid administered, whereas no correlation was found between serum ursodeoxycholic acid concentration and the proportion of ursodeoxycholic acid in bile.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Ursodeoxycholic acid therapy in cystic fibrosis-associated liver disease: a dose-response study. 139 98

Patients with cystic fibrosis (CF) often exhibit malabsorption despite the use of supplemental pancreatic enzymes. Unabsorbed carbohydrates and amino acids can serve as substrates for large intestine anaerobic fermentation, thus increasing excretion of short-chain fatty acids (SCFA) in the feces. Nine patients with CF on regular pancreatic enzyme supplementations in the age range of 5-11 years and one older patient were studied. Three-day stool samples were collected, as were 72-h food records. Stools were analyzed for gross energy, total nitrogen, fat content, and SCFA concentration. A significant difference was found between CF and normal controls in total caloric excretion due to fat malabsorption. No significant difference was found between CF and normal controls in protein or SCFA excretion. Fat excretion as percentage of fat intake was significantly increased in CF patients: 35.3 +/- 10.2% versus 8.0 +/- 3.0%, respectively. These data suggest that carbohydrate supplementation could be more widely used to increase caloric intake in CF patients without causing secondary osmotic diarrhea.
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PMID:Short-chain fatty acid absorption in patients with cystic fibrosis. 140 62

Microdissection-point count morphometric study of the myenteric (Auerbach) plexus or esophagus, small intestine, and colon was done for infants and children with acardia (2), ataxia-telangiectasia (5), cystic fibrosis of the pancreas (CFP) (25), extrahepatic biliary atresia (EBA) (17), pediatric AIDS (10), and Werdnig-Hoffmann disease (WHD) (8). Values for fractional area of neural tissue in the plane of the plexus were compared to those of control patients in same age range as those in each disease category by t-test. Statistically abnormal values included low values for small intestine and colon in Werdnig-Hoffmann disease, high values for small intestine and colon in biliary atresia, and high value for colon but a low value for small intestine in cystic fibrosis. Values for all three loci were within the normal range for ataxia telangiectasia and pediatric AIDS. The mechanisms of the low value for small and large intestines in WHD, which causes chronic constipation as a result of skeletal muscle weakness, and of the high values for colon in CFP and EBA, both causing malabsorption with bulky stools, are unclear. The value for small intestine in acardia was normal for term but lower than expected for fetal bowel of the same size, possibly because of reduced neural crest inflow to the fetal bowel.
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PMID:Microdissection study of the myenteric plexus in acardia, ataxia-telangiectasia, cystic fibrosis, extrahepatic biliary atresia, pediatric AIDS and Werdnig-Hoffmann disease. 140 39

Cystic fibrosis is an inherited, multisystem disorder characterized by an abnormality in exocrine gland function. It leads to chronic pulmonary disease in most cases and pancreatic insufficiency in 85 percent of patients. Although this disease is not uncommon in Caucasians, it has been considered very rare among Japanese. The majority of patients are diagnosed in infancy or childhood. The patient in this case report was a 45-year-old Japanese man who had not been diagnosed as having cystic fibrosis. This patient had recurrent episodes of pulmonary infection that started in childhood, and plain films of the chest showed increased interstitial markings, hyperaeration, and bronchiectasis. CT of the upper abdomen showed a generally enlarged pancreas with complete fatty replacement. Serum and urine pancreatic enzyme levels were low, suggesting pancreatic insufficiency. Repeated sweat tests were positive. A roentgenologic skeletal survey showed general demineralization, which may be multifactorial. In this case, it was concluded that vitamin D deficiency caused by vitamin D malabsorption and/or insufficient sunlight exposure was mainly responsible for the demineralization and that chronic respiratory acidosis might also be partially responsible.
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PMID:A Japanese adult case of cystic fibrosis causing bone demineralization. 141 May 64

An individual who has cystic fibrosis (CF) may suffer from gastrointestinal problems related to inadequately controlled intestinal absorption secondary to the pancreatic insufficiency. These include neonatal meconium ileus, distal intestinal obstruction syndrome (DIOS), constipation and acquired megacolon, rectal prolapse and rarely pancreatitis. If the intestinal malabsorption is well controlled with an effective pancreatic enzyme preparation, DIOS, constipation and rectal prolapse are infrequent. Persisting gastrointestinal symptoms should be investigated thoroughly to exclude other disorders not directly related to the cystic fibrosis; these include cows' milk intolerance, coeliac disease, giardiasis, Crohn's disease and intra-abdominal malignancy. Both appendicitis and intussusception may cause difficult diagnostic problems particularly in patients who may also have distal ileal obstruction syndrome.
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PMID:Cystic fibrosis: gastrointestinal complications. 145 4

Pancreatic insufficiency is the second most important pathophysiological expression of cystic fibrosis (CF) and occurs in the majority of patients. It leads to fat malabsorption and high energy losses in the stools and is one of the major causes of malnutrition often seen in CF. Although the development of enteric-coated enzyme preparations offers a dramatic improvement in therapy, it is still difficult to achieve complete correction of fat malabsorption. The cause for this treatment failure is the relative acidic environment in the duodenum induced by a decreased pancreatic bicarbonate output. To improve the efficacy of enteric-coated preparations the dissolution of these preparations in the duodenum must be optimised in order to achieve a high intraduodenal enzyme concentration. With the aim to increase intraduodenal pH, additional therapy with H2-antagonists and oral prostaglandins has been tested without unequivocal success. Omeprazole, a gastric acid inhibitor with more potency and duration of action compared to H2-antagonists, improves the efficacy of enteric-coated capsules of pancreatin dramatically. With a daily dose of 20 mg in addition to Pancrease (3 x 4 capsules) near normalization of faecal fat excretion will be reached in most CF patients with persistent steatorrhoea.
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PMID:New modalities in the treatment of exocrine pancreatic insufficiency in cystic fibrosis. 147 Feb 78

To evaluate the impact of early pancreatic insufficiency on growth and nutritional status in cystic fibrosis, we studied 49 infants identified by a newborn screening program. Pancreatic insufficiency, determined by increased 72-hour fecal fat excretion, was present in 59% (23/39) of infants at diagnosis (7.0 +/- 0.8 weeks; mean +/- SEM). Before initiation of pancreatic enzyme replacement, growth and nutritional status of pancreatic-insufficient (n = 16) and pancreatic-sufficient (n = 13) infants were compared. Pancreatic-insufficient infants gained less weight from birth to diagnosis (13.4 +/- 3.4 vs 22.3 +/- 4.0 gm/day; p = 0.05), had decreased triceps skin-fold thicknesses (4.5 +/- 0.3 vs 6.1 +/- 0.4 mm; p less than 0.005), and had lower blood urea nitrogen (3.07 +/- 0.42 vs 4.62 +/- 0.65 mg/dl; p = 0.02) and albumin (2.99 +/- 0.14 vs 3.54 +/- 0.14 gm/dl; p less than 0.01) levels despite higher gross calorie (154 +/- 8 vs 116 +/- 13 kcal/kg per day; p less than 0.01) and protein intakes (2.81 +/- 0.21 vs 2.14 +/- 0.33 gm/kg per day; p = 0.03). Fecal nitrogen loss was correlated with fat loss (r = 0.79; p less than 0.001). Fat malabsorption was present in 79% (30/38) and 92% (33/36) of infants tested at 6 months and 12 months of age, respectively, indicating that pancreatic insufficiency persists and increases in frequency throughout infancy. We conclude that pancreatic insufficiency is prevalent in young infants with cystic fibrosis and has a significant impact on growth and nutrition.
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PMID:Pancreatic insufficiency, growth, and nutrition in infants identified by newborn screening as having cystic fibrosis. 155 90

The exocrine pancreas is a gland which secretes water, enzymes and electrolytes into the intestinal lunar. These enzymes for the normal digestion of food and a deficit, whether due to a seduced secretion (chronic pancreatitis, cystic fibrosis), or dysfunction in the chronology of their secretion (following truncal vagotomy) will cause malabsorption which often develops, in clinical terms, into malnutrition sometimes this functional deficit is secondary to other pathologies or surgical operations which alter the digestive tract physiology, preventing the correct combination of nutrients and pancreatic enzymes together with the biliary salts. The outcome is malabsorption, mainly of fast and fat soluble vitamins. As the onset is slow and forms part of a chronic, pathology, diagnosis is difficult, for which reason it is included in the general group of malabsorption due to pancreatic insufficiency or surgical sequelae, finally resulting in pancreatic failure or problems following surgery. The purpose of this publication is to review one by one all the situations in which there is an alteration in the function of the pancreatic enzymes, with emphasis on cases in which a defined malabsorption syndrome will result, and in which the prescription of exogenous pancreatic enzymes will imposiue the picture.
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PMID:[Exocrine pancreatic insufficiency. Organic and functional deficiencies]. 157 5


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