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  • 1
    Electronic Resource
    Electronic Resource
    s.l. : American Chemical Society
    Organometallics 11 (1992), S. 298-310 
    ISSN: 1520-6041
    Source: ACS Legacy Archives
    Topics: Chemistry and Pharmacology
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-0533
    Keywords: KeyWordsInfantile multiple system atrophy Ubiquitin ; Neuronal intranuclear hyaline inclusion disease
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract This report presents a case of infantile multiple system atrophy with probably autosomal recessive inheritance. The female patient developed generalized muscular hypotonia, myoclonias and tonic-clonic seizures at the age of 8 months, followed by gradual development of choreoathetotic hyperkinesia and increasing psychomotor retardation. Metabolic disease was ruled out and the child died of aspiration pneumonia at the age of 5 years. General autopsy was unremarkable, but neuropathological examination showed degeneration of cerebellum, inferior olives, medial thalamus, Clarke's nucleus, anterior horn cells, corticospinal, spinocerebellar tracts, and posterior columns. Immunohistochemically many neurons contained intranuclear and intracytoplasmic ubiquitin-positive inclusions, which did not contain neurofilament or tau epitopes and ultrastructurally consisted of granulofilamentous material. We tentatively classify this case as a form of infantile multiple system atrophy linked to neuronal intranuclear hyaline inclusion disease.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-0533
    Keywords: Infantile multiple system atrophy ; Ubiquitin ; Neuronal intraunclear hyaline inclusion disease
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract This report presents a case of infantile multiple system atrophy with probably autosomal recessive inheritance. The female patient developed generalized muscular hypotonia, myoclonias and tonic-clonic seizures at the age of 8 months, followed by gradual development of choreoathetotic hyperkinesia and increasing psychomotor retardation. Metabolic disease was ruled out and the child died of aspiration pneumonia at the age of 5 years. General autopsy was unremarkable, but neuropathological examination showed degeneration of cerebellum, inferior olives, medial thalamus, Clarke's nucleus, anterior horn cells, corticospinal, spinocerebellar tracts, and posterior columns. Immunohistochemically many neurons contained intranuclear and intracytoplasmic ubiquitin-positive inclusions, which did not contain neurofilament or tau epitopes and ultrastructurally consisted of granulofilamentous material. We tentatively classify this case as a form of infantile multiple system atrophy linked to neuronal intranuclear hyaline inclusion disease.
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1432-1076
    Keywords: Cystic fibrosis ; Mutation frequency ; Polymerase chain reaction ; Molecular genetics
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Since the characterization of the gene encoding the cystic fibrosis transmembrane conductance regulator protein and identification of its main mutation, ΔF508, causing cystic fibrosis (CF), more than 150 mutations in this gene have been reported, most of them only in a few or even single CF patients. Attempts to use mutation analysis in genetic counselling or for the diagnosis of CF depends on prevalence data of certain mutations in the respective population, and considerable ethnic differences have been reported. In this study we determined the prevalence of the mutations ΔF508, G551D, R553X, and G542X and of genotypes defined by these mutations in 239 CF patients (444 independent CF chromosomes) seen in our clinic. The analysis for those four mutations alone now permits identification of approximately 75% of all mutations in our CF patients. The complete genotype can be resolved in approximately 63% of patients. This represents the diagnostic sensitivity which can be achieved by mutation analysis in patients without a family history of CF. We conclude that in situations where conventional diagnostic tests are not feasible or difficult to interpret, mutation analysis using a limited set of mutations can contribute significantly to an early and specific diagnosis of CF.
    Type of Medium: Electronic Resource
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  • 5
    ISSN: 1432-1076
    Keywords: Surfactant ; Intracranial haemorrhage ; Respiratory distress syndrome ; Infants ; Neonates
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Within a randomized European multicentre trial the time of onset, severity and progression of intracerebral haemorrhages (ICH) were investigated prospectively by serial cranial ultrasonography in 343 ventilated infants with severe respiratory distress syndrome (RDS) following instillation of single or multiple doses of a natural porcine surfactant (Curosurf). In 148/343 infants (43%) ICH was diagnosed (grade I or II: 22%, grade III or IV: 21%). In 26 cases (8%) ICH was present on the ultrasound scan prior to surfactant instillation at a median age of 6h. Incidence and severity of ICH was similar after single- or multiple-dose surfactant treatment. Using a logistic regression model the following risk factors predictive of ICH were defined: low birth weight, allocation to certain hospitals, vaginal delivery, Apgar score≤6, rectal temperature on admission ≤36°C, primary anaemia, acidosis prior to treatment, RDS grade IV in pre-treatment chest films and poor response to surfactant treatment Our study provides supportive evidence that multiple doses of Curosurf do not increase the risk for ICH as compared to single-dose administration.
    Type of Medium: Electronic Resource
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  • 6
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 146 (1987), S. 508-511 
    ISSN: 1432-1076
    Keywords: Pancreas ; Ultrasound ; Haemosiderosis ; Cystic Fibrosis ; Pancreatitis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract An increased echogenicity of the pancreas (“white pancreas”) was sonographically found in 25 children with various pancreatic and systemic diseases. Fifteen patients with cystic fibrosis had a small white pancreas. Five patients with haemosiderosis, two with pancreatitis and one with Shwachman-syndrome presented with a normal-sized or slightly enlarged pancreas. Fatty infiltration and calcifications of the pancreas can also increase its echogenicity.
    Type of Medium: Electronic Resource
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  • 7
    ISSN: 1432-1076
    Keywords: Key words     Surfactant ; Intracranial haemorrhage ; Respiratory distress syndrome ; Infants ; Neonates
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract      Within a randomized European multicentre trial the time of onset, severity and progression of intracerebral haemorrhages (ICH) were investigated prospectively by serial cranial ultrasonography in 343 ventilated infants with severe respiratory distress syndrome (RDS) following instillation of single or multiple doses of a natural porcine surfactant (Curosurf). In 148/343 infants (43%) ICH was diagnosed (grade I or II: 22%, grade III or IV: 21%). In 26 cases (8%) ICH was present on the ultrasound scan prior to surfactant instillation at a median age of 6 h. Incidence and severity of ICH was similar after single- or multiple-dose surfactant treatment. Using a logistic regression model the following risk factors predictive of ICH were defined: low birth weight, allocation to certain hospitals, vaginal delivery, Apgar score £ 6, rectal temperature on admission £ 36°C, primary anaemia, acidosis prior to treatment, RDS grade IV in pre-treatment chest films and poor response to surfactant treatment. Conclusion     Our study provides supportive evidence that multiple doses of Curosurf do not increase the risk for ICH as compared to single-dose administration.
    Type of Medium: Electronic Resource
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  • 8
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 114 (1973), S. 221-232 
    ISSN: 1432-1076
    Keywords: Celiac disease ; Oral d-xylose test ; Small bowel morphology
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung 179 d-Xylose-Teste (d-XT) von 145 Kindern im Alter von 3 Monaten bis 131/2 Jahren wurden mit den stereomikroskopischen Befunden bioptisch gewonnener Dünndarmmucosa verglichen. Mit einer Ausnahme waren alle 68 subtotalen und 3 der 18 partiellen Zottenatrophien gluteninduziert. Da im Alter bis zu 3 Jahren bei subtotaler Zottenatrophie die Mehrzahl (57%) der d-XT in den Bereich von 1–9% field und sich dieses Ergebnis für diesen Bereich, nicht aber mehr zwischen 10 und 15% d-Xylose-Wiederfindung von den beiden anderen morphologischen Gruppen-normale Mucosa und partielle Zottenatrophie-signifikant unterschied, legt nur ein d-XT unter 10% den Verdacht auf eine Cöliakie nahe. Ein Teil der d-XT über 15% bei Kindern mit subtotaler Zottenatrophie war auf eine bewußte oder unbewußte glutenfreie Ernährung vor der Biopsie zurückzuführen; die Mucosaregeneration im oberen Dünndarm verläuft langsamer als die Normalisierung des d-XT. In der Erstbehandlungsphase eines cöliakiekranken Säuglings oder Kleinkindes hat der d-XT eine Bedeutung im frühen Nachweis eines Therapieerfolges. Zottenatrophien des älteren Kindes mit Cöliakie, die nach Wiedereinführung der Normalkost auftreten, lassen sich mit dem d-XT nicht mehr aufspüren. Das Verteilungsmuster der d-XT bei partieller Zottenatrophie unterscheidet sich nicht von dem bei normaler Mucosa. Bei normaler Mucosa nahm in Bestätigung anderer Angaben die d-Xylose-Wiederfindung mit dem Alter der Kinder zu.
    Notes: Abstract The results of 179 oral d-xylose tests (d-xt) performed in 145 children aged 3 months to 131/2 years were compared with the dissecting microscope findings of the jejunal biopsy specimens. All but one of 68 subtotal villous atrophies and 3 out of 18 partial villous atrophies were gluten-induced. The majority of the d-xt (57%) of children in the age group of 3 months to 3 years with subtotal villous atrophy were found to yield 1–9% of recovered d-xylose, with a significant difference from the two other morphological groups, partial villous atrophy and normal mucosa. All three groups overlapped in the range of 10 to 15% d-xt. This means that only a d-xt of less than 10% is indicative of celiac disease. Some d-xt values above 15% in children under 3 years of age with subtotal villous atrophy were the result of a consciously or unconsciously given gluten-free diet prior to biopsy. The regeneration of the mucosa in the upper small intestine proceeds more slowly than the normalization of the d-xt. In early childhood the d-xt may be of value in that it may provide evidence of successful gluten-free diet in the first phase of therapy. Villous atrophy in the older child with celiac disease recurring after reintroduction of gluten was never detected with the d-xt. There is no difference between the distribution of d-xt in children with normal mucosa and those with partial villous atrophy. The d-xylose recovery in the urine of children with normal mucosa increased with the children's age.
    Type of Medium: Electronic Resource
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  • 9
    ISSN: 1432-1076
    Keywords: Intractable diarrhea ; Celiac disease ; Treatment ; Casein hydrolisate ; Amino acids
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Ten infants with intractable diarrhea, celiac disease and small bowel resection were treated with a special dietetic regimen called “Baustein” principle. The three major food constituents were added to the formula stepwise: first glucose and maltodextrin followed by protein and vegetable oil or MCT oil. The protein source was a newly developed casein hydrolisate also containing minerals, trace elements and vitamins.
    Type of Medium: Electronic Resource
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  • 10
    Electronic Resource
    Electronic Resource
    Amsterdam : Elsevier
    Physica B: Physics of Condensed Matter 165-166 (1990), S. 353-354 
    ISSN: 0921-4526
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Physics
    Type of Medium: Electronic Resource
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