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  • 1
    Electronic Resource
    Electronic Resource
    Determination of protease-cleaved p-Aminobenzoic Acid (PABA) in serum after oral administration of N-benzoyl-L-tyrosyl-p-aminobenzoic acid (PABA-peptide) in children (1981)
    Springer
    European journal of pediatrics 135 (1981), S. 277-279 
    Details
    ISSN: 1432-1076
    Keywords: Exocrine pancreas function ; PABA-test (serum) ; Age dependend values ; Cystic fibrosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A modification of the urine PABA test published by Imondi et al. is described. Ninety minutes after oral administration of PABA peptide, PABA was determined in serum. The average concentration in healthy children was 0.42±0.055 mg per 100 ml. Up to that time we recovered in the urine 66.1±6.1% of the substance previously administered. In the children with cystic fibrosis of the pancreas (CF) serum PABA concentrations were less than 0.1 mg per 100 ml. The infants under 2 months old also had clearly low serum concentrations, on average 0.29±0.06 mg per 100 ml, whereas the PABA concentrations in the older babies lay within the range for the older children (0.40±0.07 mg%).
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00442103
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    Paper (German National Licenses)
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  • 2
    Electronic Resource
    Electronic Resource
    Bare lymphocyte syndrome — combined immunodeficiency and neutrophil dysfunction (1990)
    Springer
    European journal of pediatrics 149 (1990), S. 700-704 
    Details
    ISSN: 1432-1076
    Keywords: Bare lymphocyte syndrome ; HLA expression ; Severe combined immunodeficiency syndrome ; Neutrophil dysfunction
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A 4-year-old girl presented with recurrent infections. Immunoglobulin deficiency (serum and secretory IgA, serum IgG3) neutropenia and neutrophil dysfunction (defective spontaneous migration and chemotaxis) were found. T-lymphocyte counts were normal and they responded to phytohaemagglutinin but were not stimulated by Concanavalin A, pokeweed mitogen and microbial antigens in vitro. Delayed cutaneous hypersensitivity testing to purified protein derivative and candidin was negative. Despite bacille Calmette-Guérm vaccination and candidiasis, near normal β-2-microglobulin and human leucocyte antigen (HLA) class I concentrations were detected on mononuclear cells and phytohaemagglutinin-induced lymphoblasts. HLA class II antigens (HLA-DP, −DQ, −DR) were not expressed. These observations indicated a bare lymphocyte syndrome (BLS) type II. This is the first time neutrophil dysfunction has been noted in association with BLS.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01959526
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    Paper (German National Licenses)
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  • 3
    Electronic Resource
    Electronic Resource
    Determination of chymotrypsin in stool by a new photometric method
    Amsterdam : Elsevier
    Clinical Biochemistry 19 (1986), S. 329-332 
    Details
    ISSN: 0009-9120
    Keywords: chymotrypsin ; cystic fibrosis ; fecal ; gastro-intestinal diseases ; photometry
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1016/S0009-9120(86)80004-2
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    Paper (German National Licenses)
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  • 4
    Electronic Resource
    Electronic Resource
    Determination of chymotrypsin in stool by a new photometric method
    Amsterdam : Elsevier
    Clinical Biochemistry 19 (1986), S. 329-332 
    Details
    ISSN: 0009-9120
    Keywords: chymotrypsin ; cystic fibrosis ; fecal ; gastro-intestinal diseases ; photometry
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1016/S0009-9120(86)80004-2
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    Paper (German National Licenses)
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  • 5
    Electronic Resource
    Electronic Resource
    An approach to study the viral safety of plasma-derived products in previously treated, non-infected patients (2001)
    Oxford UK : Blackwell Science Ltd
    Haemophilia 7 (2001), S. 0 
    Details
    ISSN: 1365-2516
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Using the polymerase chain reaction (PCR), we designed a study concept to evaluate the safety of plasma derivatives in previously treated patients who are non-infected by the specific viruses studied. Several product lots can be studied in a single patient, with a study period for each lot of 3 months. In the present study 19 patients were included for treatment with Baxter Hyland Immuno’s PCR-screened factor VIII concentrate Immunate (n=7), factor IX concentrate Immunine (n=10), the by-passing agent FEIBA plus Immunine (n=1), and the protein C concentrate Ceprotin (n=1). PCR testing for hepatitis B, C or HIV genomic material in patient samples was done as well as serological testing. All patients remained negative for the tested markers. All seven Immunate patients completed three treatment periods with three different lots of the study drug. The median study period was 282 days and the median dose 115 000 units, with a median of 115 exposure days. Five of the 10 Immunine patients completed three treatment periods and four patients, two treatment periods. One Immunine patient was discontinued from the study for reasons unrelated to the study drug administration. The median study period was 305 days and the median total dose 82 200 units, with a median of 88 exposure days. Our study presents a new design to approach the evaluation of viral safety of new plasma derivatives in previously treated, non-infected patients (NIPs) and offers several advantages over the currently recommended studies using testing for serological markers of infection in previously untreated patients (PUPs).
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1365-2516.2001.00522.x
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  • 6
    Electronic Resource
    Electronic Resource
    Clinical course of an exacerbated pulmonary infection in a girl with cystic fibrosis (CF) (1987)
    Springer
    Infection 15 (1987), S. 378-380 
    Details
    ISSN: 1439-0973
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Die Behandlung der schweren, akut exazerbierten Lungeninfektion eines jugendlichen oder erwachsenen CF-Patienten hängt vom Alter, Krankheitsverlauf, bakterieller Besiedlung der Lunge, Antibiogramm und Ernährungszustand des Patienten ab. Neben einer gezielten antimikrobiellen Therapie, Enzymsubstitution und einer optimierten Physiotherapie, wird der hochkalorischen Ernährung und der Sauerstoff-Dauerinsufflation große Bedeutung für die Behandlung einer exazerbierten pulmonalen Infektion mit respiratorischer Insuffizienz beigemessen.
    Notes: Summary The treatment of severe pulmonary infection in young CF-adults depends on age, clinical course, bacterial colonization of the lung, susceptibility pattern and state of nutrition. Besides specific antibiotic therapy, enzyme replacement and physiotherapy, high caloric nutrition, continuous oxygen insufflation and early mobilisation are the main tools in the treatment of an exacerbated pulmonary infection with respiratory insufficiency.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01647749
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    Paper (German National Licenses)
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