Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0847097 (acidity)
 15,165 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The acid concentration and quantity, the pH and the peptic activity of the gastric juice were measured after stimulation with pentagastrin in 10 children with cystic fibrosis between the ages 2 and 12 years and in 20 healthy children of the same age group. Furthermore, the basal, maximal and peak volume outputs (BVO, MVO and PVO), the basal, maximal and peak acid outputs (BAO, MAO and PAO) and the basal, maximal and peak pepsin output (BPO, MPO and PPO) were determined. The statistical calculations were carried out with the help of partial hierarchical analysis of variance, comparison of regression curves, simple analysis of covariance and the t test. After stimulation with pentagastrin, the volume of the gastric juice, the acid quantity and the peptic activity were found to be dependent on age in healthy children as well as in children with cystic fibrosis. The maximal volume of secretion in children with cystic fibrosis is less than that of healthy children; however, the acid quantity and peptic activity show no significant difference in both groups. The volume of the gastric juice, acid quantity and peptic activity in basal and stimulated secretions, expressed in kilograms per body weight or surface area in square meters, are independent of age and show no significant difference between the two groups. In the two groups the curves for the three parameters differ significantly from one to another. There is a significant shift in the time course of the curves that depict the acid secretion and peptic activity. Contrary to the accepted views, the acid and enzyme secretions are not closely interrelated. Based on the acidity and peptic activity, the digestive capacity of the stomach is the same for healthy children and children with cystic fibrosis. In contrast to the pancreas, there is no impairment in the exocrine function of the stomach. The gastric secretions of children with cystic fibrosis are not completely the same as in healthy children.
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PMID:[The acidity and peptic activity of gastric juice in healthy children and in children suffering from cystic fibrosis (author's transl)]. 24 Nov 64

An episode of severe small intestinal hemorrhage occurred in a cystic fibrosis patient having pancreatic insufficiency and receiving timed-release aspirin therapy for disabling hypertrophic pulmonary osteoarthropathy. The increased acidity of small intestinal contents due to decreased bicarbonate secretion observed in patients with pancreatic insufficiency may alter the luminal environment and result in mucosal erosions and/or ulcerations in association with the presence of aspirin. Thus, physicians should be aware of the possibility that timed-release aspirin causes small intestinal hemorrhage in such patients.
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PMID:Possible hazard of timed-release aspirin in a patient with pancreatic insufficiency. 31 79

Acidic glycoproteins having blood group H activity were isolated from the sputum of two patients suffering from chronic bronchitis by reduction of the fibrillar mucus, chromatography on ECTEOLA-cellulose and gel filtration on Sepharose 4-B. These glycoproteins were degraded with alkaline borohydride and the degradation products were fractionated by chromatography on ionexchange resins and by gel filtration. The carbohydrate chains have a wide heterogeneity with regard to acidity and molecular size. Therefore carbohydrate chain heterogeneity which was already observed for bronchial glycoproteins isolated from a patient suffering from cystic fibrosis is not specific for cystic fibrosis.
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PMID:[Heterogeneity of carbohydrate chains of acidic bronchial mucin isolated from the spatum of two subjects with chronic bronchitis]. 89 Sep 79

Sulfated glycoproteins having blood group H activity were isolated from the sputum of a child suffering from cystic fibrosis, by reduction of the fibrillar mucus, chromatography on ECTEOLA-cellulose, and gel filtration on Sepharose 4B. The sulfated glycoproteins were degraded with alkaline borohydride, and the degradation products were fractionated by chromatography on ion exchange resins and by gel filtration. The carbohydrate chains thus obtained have a wide heterogeneity with regard to acidity and molecular size. The neutral chains contain blood group H active oligosaccharides and incomplete chains as short as 1 residue of 2-acetamido-2-deoxy-D-galactose. The minimal size of the neuraminic acid-containing chains is less than that of the sulfated chains, which increases with the degree of sulfation. The sulfate groups are linked at C-6 at the D-galactose residues.
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PMID:Heterogeneity of the carbohydrate chains of sulfated bronchial glycoproteins isolated from a patient suffering from cystic fibrosis. 111 99

A survey is given of the pathophysiology of the main alterations in the small intestine of cystic fibrosis patients. Special attention is paid to the understanding of meconium ileus in the fetus and the newborn, the repercussion of duodenal acidity on the duodenal mucosa and the intraduodenal digestion, and primary and secondary biochemical alterations in the secretory-digestive-absorptive function of the small-intestinal mucosa. The meconium equivalent syndrome and its connection with the atypical course of intussusception and appendiceal perforation with silent pelvic abscess are also discussed.
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PMID:Small-intestinal abnormalities in cystic fibrosis patients. 174 11

Fat malabsorption in patients with chronic alcoholic pancreatitis and cystic fibrosis may lead to vitamin and essential fatty acid deficiency in addition to steatorrhea. In clinical practice it can be difficult to achieve complete correction of malabsorption and elimination of steatorrhea. The earliest treatment methods used the oral administration of porcine pancreatic enzyme preparations. These conventional enzymes, however, were unstable in the acidic intragastric environment. Subsequently, medications to neutralize or reduce gastric acidity (H2-blockers, antacids, or bicarbonate) were added to improve the stability of the conventional enzymes. Enteric-coated enzyme preparations were then developed that would release only in an alkaline milieu, protecting the enzymes from acid denaturation. The newest and potentially most exciting modalities for the treatment of fat malabsorption are acid-stable lipases, obtained either from a fungal source or through the expression of cloned genes for the enzymes utilizing recombinant DNA techniques. The advantages and disadvantages of the various medications for the therapy of fat malabsorption in pancreatic insufficiency are reviewed.
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PMID:Enzyme therapy for malabsorption in exocrine pancreatic insufficiency. 266 33

The comparison of distribution of glycopeptides of sputa from patients suffering from various chronic hypersecretions has already shown an increased acidity with a decreased proportion of neutral glycopeptides in the respiratory secretions of patients suffering from cystic fibrosis, as compared to those of patients with chronic bronchitis. In order to find out whether this decrease is specific to cystic fibrosis mucins or whether it is due to a degradation of mucus by Pseudomonas aeruginosa, which infects most of the sputa from patients with this disease, mucus glycopeptides from patients with different chronic bronchial disorders, infected by Pseudomonas or not, were prepared and fractionated by ion-exchange chromatography. The neutral fraction, which has never been studied in detail, was gel-filtered, and provided two fractions, one containing true mucin glycopeptides and the other containing a mixture of peptides and glycopeptides with a lower molecular mass. In the Pseudomonas-infected samples, the true mucin glycopeptide fraction was greatly diminished as compared to this same fraction in non-Pseudomonas-infected samples; this was not specific to cystic fibrosis secretions. In contrast, the glycopeptide fraction with a lower molecular mass was greatly increased in all the Pseudomonas-infected samples. Polyacrylamide gel electrophoresis of this second fraction showed unique glycopeptide bands between 40-50 kDa in the Pseudomonas-infected samples, regardless of the origin of the samples. These bands were revealed by an antibody directed against whole cystic fibrosis mucin. Infected chronic bronchitis sputa and cystic fibrosis samples without P. aeruginosa did not show these bands. These studies therefore suggest that there are P. aeruginosa-associated changes in mucins which may result from degradation of mucins.
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PMID:Evidence for the in vivo degradation of human respiratory mucins during Pseudomonas aeruginosa infection. 275 44

Actual acidity of the skin surface as well as the capacity to neutralize topically applied 0,01 n NaOH were measured by means of a potentiometric procedure. 50 children without diseases of their skin showed pH values between 4,5 and 6,1 (mean pH = 5,24) when tested at the volar forearms. A significant shift towards higher acidity became apparent by pH readings (4,1--5,7; mean 4,73) in 25 patients suffering from mucoviscidosis. In both groups no difference could be found in regeneration of the skin acidity after exposure to NaOH. Our results favour the assumption that elevated skin surface acidity in patients with mucoviscidosis is due to qualitative or quantitative differences in the biochemical composition of the epidermis.
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PMID:[Potentiometric measurements and skin surface acidity in health children and children with mucoviscidosis]. 663 11

Tracheae, bronchi, nasal epithelial, and nasal polyp tissue slices were incubated in tissue culture with [3H]-glucosamine, and the rate of secretion of labeled mucus glycoproteins was measured. Secretion rates were at least 3- to 6-fold higher for all of the samples from nine patients with cystic fibrosis (CF) who were studied, as compared with values for tissue slices from eight young subjects not affected with this disease. The secreted glycoproteins were further purified into one neutral and three acidic fractions by ion-exchange chromatography on DEAE-cellulose. The glycoproteins secreted by respiratory epithelial tissue from cystic fibrosis subjects contained relatively more of two acidic glycoprotein fractions. Double-label experiments with both [3H]-glucosamine and [35S]-sulfate as mucus glycoprotein precursors further substantiated the shift to more acidic components in the purified mucus glycoproteins and, in addition, suggested a higher level of sulfation of these same two acidic glycoprotein fractions. All four of the labeled glycoprotein fractions secreted by cultured human bronchi cochromatographed with authentic mucus glycoproteins purified from sputum of cystic fibrosis subjects by the same techniques. The differences between mucus glycoproteins from cultured CF airway tissue and mucus glycoproteins from other patients' tissue included relatively increased rates of production, level of sulfation, and greater acidity. Further applications of these in vitro techniques should allow the determination of the enzymatic and biochemical causes of these observed differences in the absence of such potentially confounding variables as concurrent airway infection or of oropharyngeal secretions.
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PMID:Mucus glycoproteins secreted by respiratory epithelial tissue from cystic fibrosis patients. 683 12

CFTR, or cystic fibrosis transmembrane conductance regulator, the gene product that is defective in cystic fibrosis, is present in the apical membrane of the epithelial cells from the stomach to the colon. In the foregut, the clinical manifestations are not directly related to the primary defect of the CFTR chloride channel. The most troublesome complaints and symptoms originate from the oesophagus as peptic oesophagitis or oesophageal varices. In the small intestinal wall, the clinical expression of CF depends largely on the decreased secretion of fluid and chloride ions, the increased permeability of the paracellular space between adjacent enterocytes and the sticky mucous cover over the enterocytes. As a rule, the brush border enzyme activities are normal and there is some enhanced active transport as shown for glucose and alanine. The results of continuous enteral feeding of CF patients clearly show that the small intestinal mucosa, in the daily situation, is not functioning at maximal capacity. Although CFTR expression in the colon is lower, the large intestine may be the site of several serious complications such as rectal prolapse, meconium ileus equivalent, intussusception, volvulus and silent appendicitis. In recent years colonic strictures, after the use of high-dose pancreatic enzymes, are being increasingly reported; the condition has recently been called CF fibrosing colonopathy. The CF gastrointestinal content itself differs mainly from the normal condition by the lower acidity in the foregut and the accretion of mucins and proteins, eventually resulting in intestinal obstruction, in the ileum and colon. Better understanding of the CF gastrointestinal phenotype may contribute to improvement of the overall wellbeing of these patients.
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PMID:Gastrointestinal manifestations in cystic fibrosis. 886 67


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