Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0272170 (SDS)
 50,377 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Twenty-eight patients with defective neutrophil mobility were classified, largely on results of parent values, into primary (17)--mainly Shwachman's syndrome--and probable secondary (11). They presented with frequent infections and/or allergy and these symptoms were essentially similar in both groups. Neutropenia was virtually confined. to the patients with Shwachman's syndrome. Diagnoses associated with secondary neutrophil mobility defects included hydrocephalus with shunt, cystic fibrosis, and immunoglobulin deficiency. The neutrophil mobility of nearly nearly all the patients studied increased when levamisole was added in vitro, but there was no clear evidence of clinical benefit when patients were given the drug.
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PMID:Clinicopathological findings in patients with primary and secondary defects of neutrophil mobility. 70 97

Total serum amylase was determined in 83 patients with cystic fibrosis (CF) (14 of whom had no steatorrhea), 21 control patients, and 6 patients with Shwachman's syndrome. Patients with CF who had steatorrhea and therefore lacked pancreatic function had the same serum levels pre- and postprandially as the control subjects, whereas patients without steatorrhea and, therefore, with evidence of pancreatic function, had significantly higher levels. The patients with Shwachman's syndrome had significantly lower total serum amylase than the three other patient groups. Only the patients with Shwachman's syndrome demonstrated a significant postprandial rise in activity. Only the salivary isoenzyme was detected in patients with Shwachman's syndrome or CF and apparently absent pancreatic function, whereas CF patients without steatorrhea had both salivary and pancreatic isoenzymes. These results demonstrate that patients with CF have an increase in circulating salivary amylase isoenzyme whereas patients with Shwachman's syndrome do not. The increase probably reflects an autonomic hyperstimulation of salivary secretion which is primary to CF, and unrelated to the state of pancreatic activity.
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PMID:Salivary amylase in cystic fibrosis: a marker of disordered autonomic function. 72 98

The interrelationship of enterokinase and trypsin activities were investigated in 133 infants and children with a variety of gastrointestinal and pancreatic disorders. Fourteen patients with diarrhea and grade II mucosal injury revealed a significant (P less than 0.01) reduction of enterokinase, trypsin, and disaccharidase activites as compared to 59 children with normal mucosa. Nine patients with cystic fibrosis and pancreatic insufficiency had normal mucosal enterokinase activity and elevated intraluminal enterokinase activity with very low or no trypsin activity. Patients with hypoproteinemia and gastrointestinal protein loss, associated with intestinal lymphangiectasia (4 patients) and intestinal lymphoid nodular hyperplasia (3 patients), had normal or insignificant decrease of enterokinase and trypsin activities. In patients with steatorrhea, a normal sweat test, normal intestinal mucosa, and absent trypsin activity, two entities were defined. One group (3 patients) was diagnosed as Schwachman-Diamond syndrome with pancreatic insufficiency and normal mucosal and intraluminal enterokinase activity. The second group (2 patients) with absent mucosal and intraluminal enterokinase activity and normal lipase and amylase activities was diagnosed as congenital enterokinase deficiency.
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PMID:Enterokinase and trypsin activities in pancreatic insufficiency and diseases of the small intestine. 94 55

Pancreatic function can only be determined exactly via the pancreozymin-secretin test. We conducted this test in two versions: (1) under conditions of continuous perfusion with the possibility of volume correction and (2) as a simple tubing. We compared the results of 86 tubings with the results of 87 examinations under perfusion. For that purpose all patients were classified into four groups: group a) with 46 and 10 examinations, respectively, in patients suffering from cholestasis in early infancy, group b) with 7 and 12 examinations, respectively, in older patients with liver diseases, group c) with 8 and 17 examinations, respectively, in patients suffering from cystic fibrosis or Shwachman's syndrome and group d) with 25 and 48 examinations, respectively, in children with normal pancreatic function. Both examination methods nearly identical mean values of the enzyme activities in all four patient groups. However, mean variations were found to be higher in case of tubing. Therefore the lower limits (x - 2s) of this test were defined at a lower level than those of the tests under perfusion.
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PMID:[Determination of exocrine pancreatic function in childhood with the pancreozymin-secretin test]. 128 98

A characteristic feature of imipenem-resistant strains of Pseudomonas aeruginosa is loss or decreased expression of the outer membrane protein (OMP) D2, whose molecular weight is 45 to 49 kDa. D2 was studied in 15 strains of P. aeruginosa with intermediate susceptibility or resistance to imipenem recovered from the sputum of 15 patients with cystic fibrosis. The OMP was extracted using Sarkosyl and separated by SDS-PAGE electrophoresis. Electrophoresis patterns were compared to those of reference strains 3B and 3C which are resistant and susceptible to imipenem, respectively. Expression of D2 was normal in three strains, weak or very weak in 11 strains and absent in one strain. For 12 strains, the alteration of the D2 protein was consistent with previous reports. However, the finding of normal D2 production in three strains is unusual and suggests the possible presence of another mechanism of resistance.
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PMID:[Pseudomonas aeruginosa and imipenem: correlation between membrane protein D2 and resistance in fifteen strains isolated from patients with mucoviscidosis]. 149 25

Sputum samples from seven patients with cystic fibrosis and chronic P. aeruginosa lung infection were investigated for immune complexes by PEG precipitation and in two different complement binding assays. All seven patients were immune complex positive. The components involved in immune complex formation were identified by SDS-PAGE and immunoblotting. We found P. aeruginosa lipopolysaccharide as a major antigen. Both core and O-specific saccharide antigens could be demonstrated. IgG and IgA were the immunoglobulins involved, with IgG2 as the dominating IgG subclass. Lipopolysaccharide has a number of biological activities and its presence in sputum may have consequences for the pathogenesis of lung disease in cystic fibrosis.
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PMID:Lipopolysaccharide is present in immune complexes isolated from sputum in patients with cystic fibrosis and chronic Pseudomonas aeruginosa lung infection. 155 93

Increased intestinal permeability to lactulose has been reported in patients with cystic fibrosis (CF). To determine whether this finding is unique to CF or whether it is related to accompanying exocrine pancreatic dysfunction, we evaluated 31 patients with CF and 10 with Shwachman syndrome who had variable degrees of pancreatic dysfunction, together with 17 healthy control subjects. There was no significant difference in the mean urinary lactulose excretion, expressed as the percentage of dose recovered, between CF and non-CF patients with pancreatic insufficiency (2.1% +/- 1.2% and 1.9% +/- 0.8, respectively) or between CF and non-CF patients with pancreatic sufficiency (0.6% +/- 0.5% and 0.6% +/- 0.3%, respectively). However, there was a significant difference in mean lactulose excretion between the pancreatic-insufficient and the pancreatic-sufficient patients (both CF and non-CF groups; p less than 0.001 and p less than 0.013, respectively). We further analyzed the results from 26 of the 41 patients (16 patients with CF and 10 non-CF patients) with pancreatic dysfunction who had previously undergone quantitative pancreatic function testing. A nonlinear, inverse relationship was found between urinary lactulose excretion and exocrine pancreatic function determined by duodenal trypsin output. These data confirm a direct relationship between intestinal lactulose permeability and the degree of exocrine pancreatic dysfunction, unrelated to the cause of the pancreatic disease.
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PMID:Correlation of intestinal lactulose permeability with exocrine pancreatic dysfunction. 157 3

The purpose of this investigation was to demonstrate the presence of different species (subpopulations) in the purified human tracheobronchial mucin (HTM-1). Mucin was highly purified from sputum specimens collected from a cystic fibrosis (CF) patient using a protocol involving sequential chromatography on Bio-Gel A-5m and hydroxylapatite columns. SDS-composite gel electrophoresis followed by periodic acid-Schiff's reagent staining was unable to detect mucin species. However, using enzyme-linked immunoelectrotransfer blot (EITB) method and polyclonal antibodies raised against HTM-1, at least four different migrating mucin species were detected. Further immunological characterization of these mucin species was carried out using a library of 16 monoclonal antibodies (MAbs) developed against the purified mucin. Nine MAbs belonged to the IgM class, two MAbs were IgG1, one IgG2a and remaining four were of the IgG3 subclass. Periodate oxidation of the mucin antigen was used to establish the nature of the mucin epitopes recognized by the MAbs. 11 MAbs recognized carbohydrate epitopes in the mucin molecule that were sensitive to periodate, while five MAbs reacted with periodate resistant carbohydrate epitopes or the protein portion of the mucin molecule. Enzyme-linked immunoelectrotransfer blot analysis of the MAbs against HTM-1 showed the presence of at least three distinct mucin species. Chromatography of the mucin on immunoaffinity columns (MAbs H(13.3), M(33.3) and CCK 061 conjugated to CNBr-activated Sepharose 4B), followed by ELISA and EITB analyses, established the mucin species recognized by the antibodies. These experiments further indicated that both unique and shared epitopes were present in the mucin species. These monoclonal antibodies may provide a promising approach to differentiate the secretory products of the tracheobronchial tree.
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PMID:Detection of distinct species in purified human respiratory mucin using monoclonal antibodies. 164 66

Twelve children and young adults with Shwachman syndrome were compared with their unaffected siblings and with controls suffering from cystic fibrosis in terms of intellectual ability, motor skills, and behaviour. There were highly significant differences in intelligence quotient between those with Shwachman syndrome and the other two groups. Four of the index subjects but none of the control subjects were below the normal range. The differences between groups on other tests of cognitive and motor skills were not significant, though those with Shwachman syndrome tended to have the lowest scores. There was no evidence that those with Shwachman syndrome had more behavioural difficulties than the control subjects. We suggest that the intellectual difficulties of patients with Shwachman syndrome may be of neurological rather than social origin and that they may originate before birth.
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PMID:Psychological characteristics of children with Shwachman syndrome. 170 66

The linear growth of Swedish children with cystic fibrosis (CF), is described using the infancy-childhood-puberty (ICP) growth model. Length/height was studied in 51 patients during their first 8 years of life. The median age at diagnosis was 0.4 years (range 0.0-6.1 years). At birth, their mean length was close to normal, but the gain in length over the first 0.25 years of life was significantly below normal, resulting in a mean length SDS of -1.3. Length remained subnormal up to 1.0 year of age. Thereafter, catch-up growth occurred, resulting in almost normal height (mean SDS-0.3) at 5.0 years of age. Between 5.0 and 8.0 years of age growth was normal. The mean age at onset of the childhood component was not significantly different from the controls. Hence, the catch-up growth did not occur until after the onset of the childhood component. This study shows that the postnatal linear growth rate is retarded in children with CF during the first months of life. This is almost completely compensated for by a supranormal growth rate that starts at the end of the first year of life.
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PMID:Linear growth in children with cystic fibrosis. I. Birth to 8 years of age. 187 73


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