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  • 1
    Electronic Resource
    Electronic Resource
    Vitamin A, Carotin, retinolbindendes Protein und Präalbumin im Serum von Patienten mit cystischer Fibrose (1975)
    Springer
    European journal of pediatrics 119 (1975), S. 279-291 
    Details
    ISSN: 1432-1076
    Keywords: Vitamin A ; Carotene ; Retinol-binding protein ; Prealbumin ; Cystic fibrosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Bei 42 Patienten mit cystischer Fibrose (CF) und bei 92 Normalpersonen wurden die Serumkonzentrationen von Vitamin A, Carotin, retinolbindendem Protein (RBP) und Präalbumin (PA) bestimmt. Alle Patienten mit CF erhielten Vitamin A-Palmitat in Form eines Multivitaminpräparates in der doppelten empfohlenen Dosis für Gesunde. Bei der statistischen Prüfung waren die Serumkonzentrationen von Vitamin A, Carotin und RBP bei den Patienten mit CF im Vergleich zu den Normalpersonen erniedrigt (P〈0,001). Für das PA ließ sich zwischen beiden Gruppen ein statistisch signifikanter Unterschied nicht sichern. Bei den Normalpersonen fand sich nur für das PA ein altersabhängiger Anstieg (r=0,455, P〈0,001). Bei den Patienten mit CF nahmen die Serumkonzentrationen von Vitamin A in Korrelation zum Lebensalter ab (r=−0,423, P〈0,01). PA und RBP sowie RBP und Vitamin A waren bei beiden Gruppen signifikant gleichsinnig korreliert (P〈0,001). Bei den Normalpersonen ergab sich auch zwischen Vitamin A und Carotin eine positive Korrelation (r s=0,606, P〈0.001), während bei den Patienten mit CF diese Beziehung schwächer signifikant war (r s=0,311, P〈0,02).
    Notes: Abstract Vitamin A, carotene, retinol-binding protein (RBP), and prealbumin (PA) have been measured in 42 children and adolescents with cystic fibrosis (CF) and in 92 normal controls. All patients with CF were on vitamin A palmitate in twice the dose for normals. For statistical analysis U-test of Wilcoxon, Mann and Whitney, parametric correlation coefficient and Spearman's rank correlation coefficient were used. Compared with those in normal controls mean serum concentrations of vitamin A, carotene and RBP were depressed in patients with CF (P〈0.001), whereas PA levels did not differ significantly from those of normal individuals. In normal controls there was only in serum concentration of PA an elevation with age (r=0.455, P〈0.001). In patients with CF, serum concentration of vitamin A decreased in correlation with age (r=−0.423,P〈0.01). PA and RBP as well as RBP and vitamin A were positively related in both groups (P〈0.001). In normal individuals there was a highly significant correlation between serum concentrations of vitamin A and carotene (r s=0.606, P〈0.001), whereas in patients with CF this relationship was less significant (r s=0.311, P〈0.02).
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00443512
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  • 2
    Electronic Resource
    Electronic Resource
    Cyclic AMP in pancreatic and biliary secretion of children with chronic intrahepatic cholestasis and cystic fibrosis (1980)
    Springer
    European journal of pediatrics 134 (1980), S. 217-225 
    Details
    ISSN: 1432-1076
    Keywords: Pancreatic and biliary secretion ; Secretin ; Cholecystokinin ; cAMP ; Chronic intrahepatic cholestasis ; Cystic fibrosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The intracellular transmitter cAMP enters the extracellular space and can be found in the duodenal fluid. The role of this messenger was investigated in response to secretin and CCK in children with secretory insufficiency, i.e. 7 with chronic intrahepatic cholestasis, 7 with cystic fibrosis, and 6 controls. Duodenal juice was collected in 10 min aliquots before and after stimulation with 2 U/kg secretin and subsequently 2 U/kg CCK. cAMP, bicarbonate, Ca++, Na+, K+, bilirubin, protein, amylase, trypsin and lipase were determined. Controls. After the injection of secretin the cAMP concentration increased 2.5-fold, the output 6-fold. Compared to cAMP, the time-concentration curve of bicarbonate and Na+, as well as volume output, were slightly delayed after secretin, whereas Ca++ and bilirubin concentrations decreased. CCK stimulation resulted in an increase of volume, bicarbonate-Na+, Ca++-, bilirubin-, protein- and hydrolase concentration. cAMP concentration increased 1.7-fold and the output was doubled. Chronic Intrahepatic Cholestasis. Following secretin the cAMP concentration hardly differed from the control values; the output of cAMP, bicarbonate and Na+ was enhanced. Compared to the controls CCK was less effective—the concentration and output of cAMP, bilirubin, K+ and Ca++ were diminished. Cystic Fibrosis. After both hormones high concentrations of cAMP, Na+, K+, Ca++ and bilirubin were found. Due to the reduced secretion volume the output of these parameters were significantly decreased. The Results Indicate that essentially more cAMP is found in the duodenal juice after secretin stimulation than after CCK. cAMP in response to secretin seems to be mainly of pancreatic origin, that after CCK of hepatic origin. One of the first steps of stimulus-secretion coupling—the activation of the membrane bound adenylate cyclase system by secretin and CCK —seems to be intact in cystic fibrosis. The defect of this disease is probably beyond this mechanism.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00441476
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  • 3
    Electronic Resource
    Electronic Resource
    Serum alpha1-fetoprotein in cystic fibrosis (1976)
    Springer
    European journal of pediatrics 122 (1976), S. 241-248 
    Details
    ISSN: 1432-1076
    Keywords: Alpha1-fetoprotein ; Cystic fibrosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Bei 38 Kindern und Jugendlichen mit cystischer Fibrose, 19 gesunden Kindern und 62 gesunden Erwachsenen wurden die Serumkonzentrationen des Alpha1-Feto-proteins im Radioimmunoassay bestimmt. 97,5% der Alpha1-Fetoprotein-Werte der Patienten mit cystischer Fibrose und 95% der Meßwerte der gesunden Kinder lagen innerhalb des Normbereiches (1–9 ng/ml), der für gesunde Erwachsene ermittelt worden war. Aufgrund unserer eigenen Untersuchungsergebnisse und bei kritischer Bewertung der bisher publizierten Befunde erscheint die Alpha1-Fetoprotein-Bestimmung im Serum zur Erfassung der homozygoten und heterozygoten Genträger der cystischen Fibrose nicht geeignet.
    Notes: Abstract In 38 children and adolescents with cystic fibrosis, in 19 normal children and 62 healthy adults the serum α1-fetoprotein concentrations were measured by radioimmunoassay. In cystic fibrosis patients 97.5% and in normal children 95% of the α1-fetoprotein values were within the normal range for healthy adults (1–9 ng/ml). Critical judgement of the reported findings in literature and our own results demonstrate that the investigation of α1-fetoprotein in the serum cannot serve for detecting homozygotes of cystic fibrosis genes or heterozygote carriers.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00481503
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    Paper (German National Licenses)
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  • 4
    Electronic Resource
    Electronic Resource
    Serum immunoreactive trypsin and pancreatic lipase in cystic fibrosis (1985)
    Springer
    European journal of pediatrics 144 (1985), S. 167-170 
    Details
    ISSN: 1432-1076
    Keywords: Cystic fibrosis ; Trypsin ; Lipase
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Serum immunoreactive trypsin (IRT) and pancreatic lipase have been measured in 59 patients with cystic fibrosis (age 1 month-27 years). Follow-up values were obtained from 49 patients. Their serum enzyme levels were compared to those of 120 healthy children of all age groups. Faecal fat excretion was determined in selected patients (n=23) to elucidate the relationship between serum enzyme levels and pancreatic exocrine function. In cystic fibrosis IRT and lipase showed a very similar agecorrelated pattern: in infancy levels were markedly elevated. During the following years the concentrations of both enzymes decreased rapidly and were found to be far below the normal range after the 10th year of life. Elevated enzyme levels in infancy as well as low levels in all age groups coincided with steatorrhaea. Older patients (11–27 years) without severe pancreatic insufficiency however, had IRT and lipase levels in or above the normal range. In healthy children there was no age dependency of IRT levels, whereas in the first 12 months of life lipase levels were significantly lower than in later childhood.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00451906
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    Paper (German National Licenses)
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