[Two cases of cystic fibrosis of the pancreas].
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Serotyping of 30 mucoid strains isolated from cystic fibrosis patients was carried out by slide agglutination tests with both live and heat-killed cells and by tube agglutination test with heat-killed cells. Comparison of the results obtained by these 2 methods revealed that tube agglutination with heat-killed cells was the superior method. More than half the strains were found to be Homma's serotype 15 (group M in the new schema [2]). Slide agglutination with live cells did not give clear results: some strains showed occasionally positive or negative agglutinations against the same serotype serum. Changes in serotypes (groups in the new schema [2]) were found in some strains, although the number was very small.
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UNLABELLED: 46 sweat-samples from 32 children with cystic fibrosis of the pancreas (C.F.) and 35 samples from 23 control-children were collected with glass micro-capillaries. Protein was determined by ultramicro-electrophoresis. RESULTS: 1. Protein was detected in 48% of the samples (C.F. and controls). 2. At pH 2.3, C.F.-sweat showed at least one more band than control-sweat. 3. At pH 8.9, C.F.-sweat occasionally showed one more band than the control-group. 4. At pH 2.3, more protein was found at the electrophoresis start point using C.F.-sweat, whereas at pH 8.9 the opposite was found. 5. At pH 2.3, protein was found in fewer samples than at pH 8.9. The "C.F.-factor" is postulated to represent a basic polyelectrolyte which induces the following pathogenic mechanisms: a) Aggregation of proteins giving rise to a high viscosity of secretions, such as saliva. b) Binding to the cell-membrane of the glandular epithelium, thus inducing a disturbance of active NaCl-reabsorption (e.g. by reduction of luminal passive Na+-influx).
Serum alphafetoprotein concentrations were measured by three different types of radioimmunoassay in 30 patients with cystic fibrosis of the pancreas and in 55 controls. The highest value obtained in cystic patient was 10.2 ng/ml and in a control 10.8 ng/ml. These are within published normal limits. Previously reported large increases in serum AFP in patients with cystic fibrosis and in heterozygote carriers have not been confirmed.
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BACKGROUND: Cross-sectional study of bone mineral density (BMD) in children and adolescents with cystic fibrosis of the pancreas. The relationship of BMD values with nutritional status, respiratory function and the cystic transmembrane regulator genotype was also evaluated. PATIENTS AND METHODS: BMD expressed as grams of hydroxyapatite/cm2 was measured by dual-energy X-ray absorptiometry in the lumbar spine (L2-L4) in 41 patients (21 males and 20 women; age range: 4-21 years) with cystic fibrosis of the pancreas and compared with that of 471 normal controls (256 males and 215 women; age range: 1-20 years). Twenty patients were prepubertal, 9 pubertal and 12 young adults. RESULTS: Clinical repercussion of the disease evaluated by clinical and anthropometric data (weight, height and body mass index) and respiratory function was considered moderate. Height z score (mean [MSE]) was -0.53 (0.28), weight -0.81 (0.21) and body mass index -0.82 (0.12) BMD z score values (mean [MSE]) were -1.14 (0.17) and differed significantly (p < 0.001) from those of normal age- and sex-matched controls. No significant differences were observed between males and women or among prepubertal, pubertal and young adult patients. BMD z score values less than-1 z score were found in 53% and under -2 z score in 8%. Cystic transmembrane regulator genotype was studied in 36 patients (17 were F508/-, 10 F508/F508, 5 G542X/- and 4 diverse) and did not predict bone mineral status. A statistically significant correlation was found between BMD z score values and height z score, weight z score, body mass index z score and clinical assessment according to Shwachman criteria. A negative and statistically significant correlation was observed between BMD z score and functional score. CONCLUSIONS: The decrease in BMD values in CF patients begins early in life and appears to be related to the degree of clinical expression of the disease.
A study has been made of the effect of saliva from children with cystic fibrosis of the pancreas (CFP) on various components of the ATP hydrolyzing enzyme system. The ouabain-sensitive ATPase activity of erythrocyte membranes prepared from intact erythrocytes preincubated with CFP saliva was 35 +/- 4 nmol Pi/mg fry wt membrane suspension/hr, compared with 48 +/- 7 nmol Pi/mg dry wt membrane suspension/hr when the erythrocytes were preincubated with control saliva. A calcium-activated component of ATPase was decreased from 202 +/- 30 nmol Pi/mg dry wt membrane suspension/hr in erythrocytes preincubated with control saliva, to 151 +/- 17 nmol Pi/mg dry wt membrane suspension/hr when the incubation was carried out with CFP saliva. In a second series of experiments, ultrafiltered saliva was added directly to an ATPase assay. The saliva from children with CFP brought about a mean decrease in ouabain-sensitive ATPase of 16% compared with control saliva.