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)

Simultaneous measurements of duodenal and faecal chymotrypsin were made in 30 children aged 3 weeks to 14 years. Apparent chymotrypsin secretion rates measured after stimulation with pancreozymin were compared with the mean faecal chymotrypsin concentration derived from three stool specimens collected at random within 72 hours of the intraduodenal test. In the 25 children who responded to pancreozymin stimulation the mean faecal chymotrypsin concentration was significantly positively correlated with the apparent chymotrypsin secretion rate. Correlation using single specimen stools collected at random was appreciably poorer. In the five children with undetectable or only traces of chymotrypsin in the duodenum after stimulation, the mean faecal chymotrypsin concentrations were only 3-10% of the lower limit of the reference interval. In a second group of 46 children with cystic fibrosis proved by sweat tests and clinical evidence of malabsorption, the chymotrypsin concentration measured in a single stool specimen collected at random was unequivocally subnormal in each case. Faecal chymotrypsin measurement is a rapid, simple, cheap, readily repeated, non-invasive test of high specificity and sensitivity. Faecal chymotrypsin should be measured before contemplating intraduodenal tests of pancreatic function.
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PMID:Faecal chymotrypsin: a reliable index of exocrine pancreatic function. 341 94

The effects of liver disease, fat malabsorption and sunlight exposure on serum vitamin D levels were determined in 21 optimally treated preadolescent cystic fibrosis (CF) children over a 12-month period. Manifest liver disease and fat malabsorption appeared not to affect the vitamin D level. However, the level fell significantly in winter, although not below the normal range, suggesting that sunlight exposure is a more important determinant of vitamin D levels in preadolescent CF children than liver disease and fat malabsorption.
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PMID:Determinants of serum vitamin D levels in preadolescent cystic fibrosis children. 342 14

Methane production was studied in 28 cystic fibrosis patients aged 3-16 years and in 290 healthy children. The percentages of methane producers in the cystic fibrosis and control groups were 60.7 and 20.6%, respectively. In the patient group there was no difference between methane producers and nonproducers with respect to the degree of malabsorption or the administration of pancreatic supplements or antibiotics. We hypothesize that the high proportion of methane producers in cystic fibrosis patients may be associated with the presence of specific substrate(s) (glycoprotein?) in their intestinal contents. Changes of the intestinal microenvironment may favor the development of a methanogenic flora.
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PMID:Methane production in patients with cystic fibrosis. 343 Feb 46

On reassessment of 179 children who had previously been diagnosed as having cystic fibrosis seven (4%) were found not to have the disease. The importance of an accurate sweat test is emphasised as is the necessity to prove malabsorption or pancreatic abnormality to support the diagnosis of cystic fibrosis.
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PMID:Misdiagnosis of cystic fibrosis. 343 63

Three hundred and sixteen patients with cystic fibrosis were seen at the Brompton Hospital during 1965-83; 178 (56.3%) of them were male and 136 female, and their ages ranged from 12 to 51 years. Most patients presented in infancy with respiratory symptoms and malabsorption, but 19 (6%) were diagnosed in adult life, three in their 30s. Pulmonary disease was almost universal (99.7%), being responsible for 97% of all deaths and three quarters of hospital admissions. All patients had developed a productive cough by the age of 21 and over half before the age of 5. Many complained of wheezing, but reversible airflow obstruction was present in only 40% of those tested. Minor haemoptysis was very common (62%), but major episodes less so (10%). Pneumothorax was seen in 61 cases (19%), and was often recurrent. Some irreversible airflow obstruction was present in all patients with pulmonary disease. Two patients have been followed for over 20 years without showing appreciable decline in lung function. Thirty five patients (11%) had no symptoms of malabsorption. Acute meconium ileus equivalent was seen in 16% and a chronic partial obstruction with episodic symptoms in a further 19%. Diabetes mellitus developed in 36 patients, 13 of whom were insulin dependent. Hepatomegaly was common (29%), often occurring without abnormal results in biochemical tests of liver function; only 1% of patients developed portal hypertension with varices and ascites. Skin reactions to at least one common allergen, including Aspergillus fumigatus, were positive in 70%, but very few patients suffered from hay fever or eczema. One hundred and twenty one patients have died, 97% from infection or other pulmonary complications, and 195 were alive in December 1983 (mean age 23 years). Seventy eight per cent of patients were in full time education or full or part time employment, or were housewives, and only 41 were unemployed for reasons for health. Many patients are married and 10 women have borne children. Most patients were admitted to hospital only three or four times during the period of follow up and 50 individuals (16%) have never been in hospital at all. The improvement in prognosis and quality of life for adults with cystic fibrosis should encourage a positive attitude in those who care for them.
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PMID:Cystic fibrosis in adolescents and adults. 343 96

Finger clubbing was measured in 73 of 105 patients with cystic fibrosis undergoing full assessment. The sign correlated well with the chest x ray score and indices of pulmonary function and infection but not with weight, height, age, liver function, or degree of fat malabsorption. The presence of clubbing suggests appreciable pulmonary involvement. Most probably its progression indicates a deterioration in pulmonary state. In both instances increased efforts should be made to treat the infection.
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PMID:Finger clubbing in cystic fibrosis. 348 32

The prevalence of pancreatic diseases as the cause for dyspepsia differs in clinical materials between 0 and 25-30%. In parallel, the incidence rate of chronic pancreatitis varies between 0.7 and 10 per 100,000 inhabitants per year. The correct figures are unsettled. The main reason for the great variability in figures for frequency of chronic pancreatitis is probably the different clinical awareness and variable practice for performing morphological and functional studies of the pancreas in patients with dyspepsia. Epidemiologic data indicate, but do not prove, an increasing frequency of chronic pancreatitis at least valid for the alcoholic chronic pancreatitis. Pancreatic function and pancreatic disease are probably connected to different gastro-intestinal diseases (duodenal ulcer, inflammatory bowel diseases, malabsorption syndromes, subtotal and total gastrectomy and to some extent in patients with hepatobiliary diseases). The prevalence of chronic pancreatitis can be calculated to around 70 per 100,000 inhabitants in the Western world. Around one-third of these present with exocrine pancreatic insufficiency. The demand for enzyme substitution based on marked exocrine pancreatic insufficiency in patients with chronic pancreatitis, pancreatic cancer and mucoviscidosis can be calculated to approximately 150 patients per 1 million inhabitants. The question concerning the analgetic effect of pancreatic enzyme substitution is still unsettled.
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PMID:Exocrine pancreatic function in dyspepsia. 349 32

A sandwich enzyme immunoassay has been developed for human pancreatic lipase using polystyrene balls coated with specific IgG as the first antibody and peroxidase-labelled IgG as the second antibody. The detection limit was 0.5 microgram/l. Good parallelism was observed with the curves obtained from standard lipase and lipase present in serum, pancreatic juice and duodenal contents, demonstrating that the assay may be used to measure the level of the protein in different biological fluids. Mean values of lipase in human sera were 12.3 +/- 6.8 micrograms/l in adults and 4.5 +/- 2.7 in newborns. In all cases a good correlation was found in serum between the catalytic activity and the enzyme immunoassay. Lipase is detectable in amniotic fluids at the 18th week of pregnancy but at a very low level (0.95 +/- 0.32 microgram/l). In pancreatic juices, lipase concentration was 14.6% of the total protein content. A study on cystic fibrosis patients showed a poor correlation between blood pancreatic lipase concentration and fat malabsorption underlying the difficulty in assessing pancreatic function by the measurement of serum pancreatic enzymes. The use of the lipase assay in duodenal contents would permit better assessment of pancreatic function in patients presenting a severe or borderline defect in fat digestion and absorption.
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PMID:Assay of human pancreatic lipase in biological fluids using a non-competitive enzyme immunoassay. 354 6

The fecal chymotrypsin (FC) levels in samples collected over 24 h were determined by a new commercial colorimetric method from Boehringer Mannheim in 82 children suffering from various pancreatic disorders. The patients were divided into 4 groups, in accordance with the following etiologies: cystic fibrosis of the pancreas (CFP), chronic severe hepatic disorders (CSH), primary malabsorption syndrome (PMS) and malnutrition due to nondigestive causes (M). The control group comprised 48 children of similar ages. The 24th FC levels as U/g (mean +/- SD) were: 34 +/- 6 in the control group, 2 +/- 2 in the CFP group, 15 +/- 6 in the M group, 19 +/- 9 in the CSH group and 43 +/- 13 in the PMS group. The differences between the CFP patients and all the other groups were statistically significant. These results indicate that the FC levels may be suitable as a diagnostic indication of CFP and capable of differentiating between this disorder and other causes of pancreatic insufficiency.
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PMID:Fecal chymotrypsin levels in children with pancreatic insufficiency. 358 67

We report the cases of two adult patients with cystic fibrosis affecting the pancreas and liver, who also had severe vitamin E deficiency and neurologic disease. The most prominent clinical features were abnormal eye movements, diminished reflexes, decreased vibratory and position sense, ataxia, and muscle weakness. Treatment with intramuscular injections of vitamin E partially corrected the neurologic deficits. Vitamin E absorption tests documented severe malabsorption, which was later alleviated by the addition of dessicated ox bile to the regimen of alpha-tocopheryl acetate. These studies suggest that a decreased intraluminal concentration of bile salts is an important factor in the development of severe vitamin E deficiency and in the poor response to oral replacement therapy that is seen in some patients with cystic fibrosis.
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PMID:Vitamin E deficiency and neurologic disease in adults with cystic fibrosis. 359 48


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