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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Electrical engineering 79 (1996), S. 365-369 
    ISSN: 1432-0487
    Source: Springer Online Journal Archives 1860-2000
    Topics: Electrical Engineering, Measurement and Control Technology
    Description / Table of Contents: Contents The combination of bipolar and MOS-transistors offers a large potential to develop complex circuits on a single chip. The large transconductance and the high transit frequency of the bipolar transistor allows the realization of precision analog baseband circuits and of RF-circuits capable to operate at frequencies up to several GHz. In contrast the transconductance of the MOS-transistor is lower but it has an insulating gate and yields an excellent bidirectional switch. The BiCMOS-technology combines the benefits of both technologies. This contribution shows some applications of analog BiCMOS circuits which cannot be easily duplicated in other technologies.
    Notes: Übersicht Die Kombination der Bipolar-und der CMOS-Schaltungstechnik auf einem Chip eröffnet dem Schaltungsentwickler ein großes Potential zur Realisierung komplexer Systemkonzepte. Die hohe Steilheit und die hohe Transitfrequenz des Bipolartransistors erlauben die Realisierung von Präzisions-Analogschaltungen für die Basisband-und von Schaltungen für die Hochfrequenzsignalverarbeitung bis zu einigen GHz. Der MOS-Transistor besitzt dagegen eine geringere Steilheit, hat jedoch den Vorteil, daß er keinen Eingangsstrom benötigt und er ist gleichzeitig ein nahezu idealer Schalter. Die BiCMOS-Technologie verbindet die Vorteile beider Technologien. Der folgende Beitrag stellt Applikationen analoger BiCMOS-Schaltungen vor, welche in anderen Technologien nur schwer zu realisieren sind.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 112 (1972), S. 133-141 
    ISSN: 1432-1076
    Keywords: Cystic Fibrosis (Mucoviscidosis) ; Heterozygote Test ; White Blood Cell Culture ; Metachromasia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Es wird über eine modifizierte Kultivierungsmethode weißer Blutzellen berichtet. In transformierten Leukocyten wurde Metachromasie bei Patienten mit Mucoviscidose und heterozygoten Erbträgern nachgewiesen. Da auch bei anderen selteneren angeborenen Stoffwechselstörungen Metachromasie nachgewiesen werden kann, ist der Heterozygotennachweis nur bei Familien mit bekanntem Genstatus anwendbar.
    Notes: Abstract A modified method of white blood cell culturing is described. Metachromasia could be demonstrated in transformed leucocytes of probands homozygotic for cystic fibrosis (CF) and heterozygote carriers of the CF-gene. Since these staining properties are known to occur in other inherited metabolic diseases heterozygote detecting should be restricted to CF-families and not applied in the general population as a screening test.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 135 (1980), S. 31-36 
    ISSN: 1432-1076
    Keywords: Antigen handling ; Coeliac disease ; Family study ; Fluorescent antibody technique ; Gliadin antibodies ; Reticulin antibodies ; Monozygotic twins
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The familial occurence of coeliac disease is well known. In every day practice, however, diagnosis of coeliac disease is not frequently established in the relatives of patients. As it did not seem practicable to biopsy all relatives, several tests were investigated in selecting individuals for intestinal biopsy in a family study. 55 index patients out of 54 families with biopsy-proven coeliac disease and 165 of their first grade relatives underwent the study. Immunofluorescent gliadin and reticulin antibodies were determined, and additionally laboratory tests were done. These included haemoglobin, serum iron, serum protein and albumin, serum immunoglobulins and blood xylose. The immunofluorescent gliadin antibody assay using red cells coated with gliadin proved to be superior to the other methods. False negatives came to 8.7%, and false positives 10.9%, in healthy relatives. Gliadin antibodies could be found five times more frequently in healthy relatives than in normal controls. This finding indicates a genetic predisposition to the formation of gliadin antibodies in coeliac families. Ninety-one percent of index coeliac children had IgG-antigliadin in their sera while on a normal diet. During gluten-free diet, and in adult patients, results were less convincing. All relatives with antigliadin titres greater than 8 have been biopsied, and all with titres above 64 were shown to have coeliac disease. The prevalence of coeliac disease found in this study was 5.5%. In the active state of coeliac disease in children, gliadin antibody determination thus is a valuable diagnostic tool but in selecting relatives for biopsy there are limitations to the wide application of the test. Although reticulin antibodies are more specific for coeliac disease than gliadin antibodies, determination of antireticulin proved to be much less sensitive.
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1432-1076
    Keywords: Hereditary fructose intolerance ; Aldolase activity ; Small intestine ; Malabsorption states
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Determination of aldolase activity in intestinal biopsy material offers a diagnostic alternative to liver biopsy in hereditary fructose intolerance (HFI). This diagnostic method could be validated by analysis of intestinal biopsies from eight patients with HFI. With the substrate fructose-1-phosphate (fru-1-p) we found 0.3±0.3 (x±SD) U/g of protein, with the substrate fructose-1,6-diphosphate (fru-1,6-p) 3.8±2.7 U/g of protein were measured. These results differ clearly from control activities (substrate = fru-1-p: 7.4±1.9 U/g of protein; substrate=fru-1,6-p: 13.9±4.3 U/g of protein). As the performance of the intestinal biopsy—in contrast to the liver biopsy—is virtually free of complications, it is recommended as the diagnostic method of choice in this disease. Aldolase activities were also determined in 40 intestinal biopsies from children with different malabsorption states: celiac disease (n=13), cow's milk protein intolerance (n=9), postinfectious syndrome (n=10), giardiasis (n=8). The activities differed clearly from our results in hereditary fructose intolerance. In celiac disease, cow's milk protein intolerance and postinfectious syndrome characteristic activity patterns were obtained: activities with fru-1,6-p were increased (celiac disease 17.2±5.7 U/g of protein; cow's milk protein intolerance 19.5±7.0 U/g of protein) or in the normal range (postinfectious syndrome 14.0±3.1 U/g or protein); activities with fru-1-p were decreased (celiac disease 2.3±1.0 U/g of protein; cow's milk protein intolerance 3.4±1.3 U/g of protein; postinfectious syndrome 5.0±0.8 U/g of protein). These results are discussed on the basis of aldolase isoenzyme distribution in the small intestinal mucosa.
    Type of Medium: Electronic Resource
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  • 5
    ISSN: 1432-1076
    Keywords: Cystic fibrosis ; Ambroxol ; N-acetylcysteine ; Pulmonary function test
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The therapeutic efficacy of oral N-acetylcysteine (NAC) and ambroxol as compared with the effect of placebos was studied in 36 cystic fibrosis (CF) patients with mild to moderate pulmonary disease. The patients were randomly assigned to one of three regimens, matched on the basis of age and Chrispin-Norman scores. The trial was conducted over a period of 12 weeks. Patients were assessed clinically and by extensive pulmonary function techniques (body-plethysmography, maximal expiratory flow-volume curves, trapped air determination). Although no clinical differences could be observed between the three groups, significant impairment in the placebo group was found for trapped air and FEV1 when compared to the active groups, suggesting a therapeutic effect of ambroxol and NAC in CF.
    Type of Medium: Electronic Resource
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  • 6
    ISSN: 1432-1076
    Keywords: Fatty acid oxidation ; Cardiomyopathy ; Hypoketotic hypoglycaemia ; 3-Hydroxyacyl-CoA dehydrogenase deficiency
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract 3-Hydroxyacyl-CoA dehydrogenase deficiency is a newly recognised fatty acid oxidation disorder with a usually fatal outcome. We present a further patient who presented with hypoketotic hypoglycaemia, hepatopathy, secondary carnitine deficiency and increased plasma long-chain acylcarnitines. 3-Hydroxydicarboxylic aciduria was present and the diagnosis confirmed in cultured skin fibroblasts. Our patient is compared with those reported in the literature with respect to clinical symptoms, differential diagnosis and possible therapeutic regimens.
    Type of Medium: Electronic Resource
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  • 7
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 113 (1972), S. 111-121 
    ISSN: 1432-1076
    Keywords: Croup Syndrome ; Air Pollution ; SO2-Concentration ; Meteorological Factors
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Der Erkrankungsbeginn von 576 Kindern mit Croup-Syndrom wurde in 4 2/3 Jahren mit den Parametern SO2, Staub und Kontinentalluft in Beziehung gesetzt. Es wurde ein deutlich signifikanter Anstieg von Croupfällen ab 80 ppb beobachtet. Die Daten wurden durch Computeranalyse erarbeitet.
    Notes: Abstract 576 children with croup syndrome were studied in correlation with SO2, dust and continental air masses over a period of 4 2/3 years (1967–1971). A definite increase of morbidity related to SO2-concentrations higher than 80 ppb was demonstrated. These data were evaluated by computer analysis.
    Type of Medium: Electronic Resource
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  • 8
    ISSN: 1432-1076
    Keywords: Tobramycin ; Pulmonary disease ; Pseudomonas aeruginosa ; Cystic fibrosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract To nine cystic fibrosis patients with chronic bronchopulmonary infection of severely damaged lungs invaded by Pseudomonas aeruginosa, eleven courses of prolonged tobramycin treatment (5 mg/kg/day) for four to 16 weeks were administered. Pulmonary symptoms improved and a better quality of life was achieved in all but one patient. Objective parameters (chest X-ray, pulmonary function tests) changed to a lesser extent. In only one patient was Pseudomonas eradicated from the sputum but reappeared after discontinuation of therapy. In the rest of the patients Pseudomonas was significantly suppressed or replaced by other pathogens. Four patients showed rises of antibody titres to Candida and two to Aspergillus fumigatus. No nephrotoxic side effects were observed, but vestibular function was reversibly impaired in one patient without corresponding clinical symptoms. No bacterial resistance to tobramycin was observed during therapy.
    Type of Medium: Electronic Resource
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  • 9
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 143 (1984), S. 135-139 
    ISSN: 1432-1076
    Keywords: Hydrops fetalis ; Lysosomal storage disease ; Mucolipidosis I ; Neuraminidase deficiency
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A newborn infant with oedema, ascites and hepatosplenomegaly is described. In ascites fluid foamy macrophages were found, in a liver biopsy cytoplasmic inclusions and membrane-bound vacuoles were seen. Furthermore the child excreted excessive amounts of sialic acid-rich oligosaccharides in the urine, and therefore a neurovisceral degenerative disorder was assumed. The diagnosis of sialidosis was confirmed by enzymatic assay in cultured fibroblasts, in which a complete deficiency of the lysosomal enzyme neuraminidase could be demonstrated. After recurrent septicaemias the child became dystrophic and died at the age of 6 months. Our case is compared with sialidoses observed by other authors, the wide phenotypic diversity within this biochemical defect is emphasised. The occurrence of hydrops fetalis in lysosomal storage diseases is discussed.
    Type of Medium: Electronic Resource
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  • 10
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    BJOG 57 (1950), S. 0 
    ISSN: 1471-0528
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Type of Medium: Electronic Resource
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