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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Infection 5 (1977), S. 64-65 
    ISSN: 1439-0973
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Infection 6 (1978), S. 199-200 
    ISSN: 1439-0973
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Infection 6 (1978), S. 95-96 
    ISSN: 1439-0973
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Oxford, UK : Munksgaard International Publishers
    Allergy 57 (2002), S. 0 
    ISSN: 1398-9995
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 5
    ISSN: 1432-1440
    Keywords: Bone marrow transplantation ; Aplastic anaemia ; Knochenmarktransplantation ; Aplastische Anämie
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Im Rahmen der Arbeitsgemeinschaft Knochenmarktransplantation — München (AG-KMT) wurden vom März 1975 bis Mai 1980 insgesamt 12 Patienten wegen schwerer, aplastischer Anämie mit Knochenmarktransplantation (KMT) behandelt. Sechs Patienten überleben derzeit mit normalem Blutbild und Knochenmark zwischen 10 Monaten und mehr als 5 Jahren nach KMT von HLA-identischen Geschwistern, eine Patientin steht noch in ambulanter Behandlung wegen lokalisierter, chronischer Graft-versus-Host Krankheit (GvHK), fünf Patienten sind klinisch gesund. Sechs Patienten starben, ein Patient starb am Tag vor KMT mit Hirnblutung, drei Patienten 32, 40 und 55 Tage nach KMT an den Folgen der Transplantatabstoßung, einer an schwerer GvHK 85 Tage nach KMT und einer 87 Tage nach KMT vermutlich an interstitieller Pneumonie nach Hirnblutung. Drei von 6 Patienten, die nur mit Cyclophosphamid (CY) vorbehandelt waren, starben infolge Abstoßung des Transplantates. Zwei erwachsene Patienten, die mit CY und „total lymphoid irradiation“ vorbehandelt waren, und drei Kinder, die nach KMT unbestrahlte Leukocytenkonzentrate von Knochenmarkspender erhalten hatten, stießen das Transplantat nicht ab. Die Ergebnisse der AG-KMT sind vergleichbar denen großer, spezialisierter Zentren für KMT und zeigen die Möglichkeiten einer Heilung schwerer aplastischer Anämien durch KMT von HLA-identischen Geschwistern. Die Erfolge sind besser bei frühzeitiger KMT.
    Notes: Summary From March 1975 until May 1980 twelve patients with severe aplastic anemia were grafted with bone marrow from HLA-identical siblings by the Munich Cooperative Group for Bone Marrow Transplantation. Six patients are alive between 10 months and more than 5 years after grafting with normal blood values and marrow. One patient is treated as an out patient for chronic localized graft-versus-host disease (GvHD), five patients are well and without treatment. Six patients have died, one patient with a cerebral hemorrhage the day before transplantation, three patients following rejection of grafts 32, 40 and 55 days after grafting, one patient with severe GvHD 85 days after grafting and one patient, probably with interstitial pneumonia, following cerebral hemorrhage. Three of 6 patients who were conditioned with Cyclophosphamide (CY) only died following rejection of the graft. Two adults who were conditioned with CY and “total lymphoid irradiation” and three children, who were given unirradiated leukocyte concentrates from the marrow donor after grafting, did not reject their grafts. The results of the Munich-Cooperative Group for Bone Marrow Transplantation are comparable to those of large, specialized centers for bone marrow transplantation, they indicate possibilities of cure of severe aplastic anemia by marrow grafts from HLA-identical siblings. They confirm that better results are obtained with earlier transplantation in the course of the disease.
    Type of Medium: Electronic Resource
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  • 6
    Electronic Resource
    Electronic Resource
    Springer
    Journal of molecular medicine 54 (1976), S. 1109-1115 
    ISSN: 1432-1440
    Keywords: Primary immunodeficiencies ; Adenosine deaminase deficiency ; Genetics ; Prenatal diagnosis ; Primäre Immundefizienz ; Adenosindesaminasemangel ; Genetik ; Pränatale Diagnostik
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Von 14 Patienten mit schwerer kombinierter Immundefizienz (SCID) wurde ein Mangel an Adenosindesaminase (ADA) in den Erythrozyten berichtet. Die unzureichende Entwicklung des lymphatischen Systems ist möglicherweise Folge des Enzymmangels. ADA kann spektralphotometrisch und elektrophoretisch in verschiedenen Geweben nachgewiesen werden. Der Genort für ADA liegt auf Chromosom 20. Untersuchungen des Enzymdefekts bei verschiedenen Formen primärer Immundefizienzen erlauben die Abgrenzung einer genetisch definierten Krankheitseinheit. Für die Klinik ergeben sich neue Aspekte in der Pathogeneseforschung, pränatalen Diagnostik, genetischen Beratung und möglicherweise kausalen Behandlung der SCID mit ADA-Mangel.
    Notes: Summary The occurrence of severe combined immunodeficiency (SCID) with adenosine deaminase (ADA) deficiency in erythrocytes has been reported in 14 patients. Enzyme deficiency may result in early depression of the lymphatic system. ADA is detectable in different tissues by photometric and electrophoretic methods. The gene locus for ADA has been localised on chromosome 20. Studies on the enzyme defect in different forms of primary immunodeficiencies led to the description of a well defined nosological entity. New aspects can be expected in the fields of pathogenesis, prenatal diagnosis, genetic councelling, and possibly therapeutic trials.
    Type of Medium: Electronic Resource
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  • 7
    ISSN: 1432-1440
    Keywords: Hemophiliacs ; Human immunodeficiency virus infection ; Acquired immunodeficiency syndome ; Intravenous immunoglobulin ; Therapy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract In children infected with human immunodeficiency virus (HIV) placebo-controlled trials with intravenous immunoglobulins have resulted in a significant reduction in morbidity; however, the results of small trials in adolescents and adults have been inconsistent. In this study 17 HIV-infected hemophiliacs aged 9–30 years were treated with monthly intravenous immunglobulins for an average of 32 months. At the end of the study, 8 years after the HIV infection, three patients (18%) had progressed to the acquired immunodeficiency syndrome(AIDS), and the average decrease in CD4 cells was 81 cells/μl per year. The natural history of HIV infection in hemophiliacs in this age group shows a manifestation rate of AIDS between 11 and 26% 6–8 years after seroconversion and an average yearly decrease in CD4 lymphocytes of 68–110 cells/μl. In conclusion, we observed no difference either in the manifestation rate of AIDS or in prognostic markers in this small cohort of HIV-infected hemophiliacs treated for more than 30% of their latency period with intravenous immunoglobulins compared to the well-documented natural history of HIV-infected hemophiliacs. However, none of the patients developed severe bacterial infections during the study period.
    Type of Medium: Electronic Resource
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  • 8
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 150 (1991), S. 234-237 
    ISSN: 1432-1076
    Keywords: HIV infection ; AIDS ; IgG subclass deficiency
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Infection with the human immunodeficiency virus (HIV) induces a polyclonal B-cell activation. Despite elevated serum immunoglobulin levels, a significant deterioration of the antigen specific humoral immune response exists in most cases. We studied the influence of HIV infection on the serum levels of IgG subclasses in children. We investigated 76 children (aged 15 months to 18 years) with HIV-1-infection. Most children (88%) showed elevated serum immunoglobulin levels. IgA (87%) and IgM (74%) were more often above normal levels for age than IgG (60%). IgG subclass serum levels were significantly altered. The increase in total IgG was mainly due to a marked augmentation of the IgG1 fraction. In most cases IgG3 was simultaneously elevated. Ten children (13%) had very low IgG4 levels (〈0.03 g/l). Out of 61 patients older than 2 years 8 (13%) had a profound IgG2 deficiency with normal or elevated total IgG. Four of them also had low IgG4 levels (〈0.03 g/l). A correlation between IgG2 deficiency and HIV infection according to the Centres for Disease Control classification for acquired immunodeficiency syndrome could not be demonstrated (three patients with symptomatic and five with asymptomatic infection).
    Type of Medium: Electronic Resource
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  • 9
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 146 (1987), S. 354-359 
    ISSN: 1432-1076
    Keywords: Neonate ; Bacterial infection ; Eye infection ; Endophthalmitis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Invasive bacterial eye infections in the neonate range from perforating keratitis to panophthalmitis. These infections have gained clinical and therapeutic importance since mortality rates are high and prognosis concerning preservation of vision is poor. Effective antibiotics against the infective agents are now available. Risk factors for developing invasive bacterial eye infections are mainly prematurity and colonisation with Pseudomonas aeruginosa.
    Type of Medium: Electronic Resource
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  • 10
    ISSN: 1432-1076
    Keywords: Omenn syndrome ; Hypereosinophilia ; Endomyocardial disease ; Bone marrow transplantation ; Pulmonary vascular obstruction
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A 5-month-old infant presented with severe combined immunodeficiency disease, reticuloendotheliosis, and hypereosinophilia (Omenn syndrome) resulting in recurrent infections and endomyocardial disease. Bone marrow transplantation from an HLA-identical donor after chemotherapeutic conditioning led to both immunological and clinical recovery. Bone marrow transplantation, however, was followed by severe pulmonary occlusive disease. The patient gradually recovered while on increased inspiratory oxygen and the calcium channel blocker nifedipine.
    Type of Medium: Electronic Resource
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