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  • 1
    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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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 150 (1991), S. 665-668 
    ISSN: 1432-1076
    Keywords: Vitamin D resistant rickets ; Extramedullary haematopoiesis ; Intravenous calcium ; Neutrophil chemotaxis ; Lipid A antibodies
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We present a new patient with vitamin D dependent rickets type II. A 20-month-old Arabian boy whose parents are first cousins showed florid rickets, myelofibrosis and recurrent septicaemia. In addition to absent specific binding for 1,25-dihydroxyvitamin D3 (1,25(OH)2D3). 25-Hydroxyvitamin D3-24-hydroxylase activity could not be induced in cultured fibroblasts. The patient did not respond to 99 μg 1,25(OH)2D3 per day, but skeletal and haematological abnormalities improved with daily infusion of 100 mg/kg calcium, as serum parathyroid hormone levels fell to normal values. At the age of 7 years, he died from pneumonia. The improvement of haematological abnormalities with calcium infusions but not with 1.25(OH)2D3 suggests a pathogenetic relationship of myelofibrosis and hyperparathyroidism. Having antilipid A IgM antibody titres up to 1∶10.000 after Gram negative septicaemias, the patient never produced corresponding IgG antibodies. His neutrophil chemotaxis was persistently reduced to 57%±3% of age-matched controls (P〈0.028). The patient showed two pathological immune functions considered to contribute to the well-known susceptibility to infection in rickets.
    Type of Medium: Electronic Resource
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  • 3
    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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  • 4
    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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  • 5
    ISSN: 1439-0973
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 6
    ISSN: 1439-0973
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Die schwere fokale virale Enzephalitis wird meist durch Herpes-simplex-Viren verursacht, aber auch andere Viren kommen ätiologisch in Betracht. Die Standardtherapie der Herpes-simplex-Enzephalitis ist Acyclovir (ACV), wobei die Letalität von 28% und Defekt-Heilungsrate von 35% immer noch unbefriedigend ist. Darüberhinaus hat Acyclovir mit Ausnahme von Varizella-zoster-Virus (VZV) keinen Effekt bei Enzephalitiden durch andere Erreger. β-Interferon (β-IFN) hat ein breites antivirales Spektrum und hatin vitro in der Kombination mit Acyclovir eine synergistische Wirkung gegen HSV. Wir führten deshalb eine retrospektive Studie mit der Fragestellung durch, ob die Kombination von ACV und β-IFN in der Behandlung der viralen Enzephalitis einen therapeutischen Vorteil gegenüber der Monotherapie mit ACV erbringt. Hierzu wurde ein Fragebogen an alle 278 westdeutschen Kinderkliniken versandt. Die Rücklaufquote betrug 78%, 301 Patienten wurden mitgeteilt. 214/301 Patienten erhielten eine antivirale Therapie, wobei 179 mit ACV-Monotherapie und 35 mit der Kombinations-therapie (ACV plus β-IFN) behandelt wurden. In der Gesamtgruppe der behandelten Patienten wurden keine Unterschiede hinsichtlich Morbidität und Letalität zwischen der Mono- und der Kombinationstherapie beobachtet. In einer Untergruppe von 41 Patienten allerdings (ACV n=30, ACV plus β-IFN n=11), die in der cranialen Computertomographie hypodense Zonen in den Temporallappen aufwiesen (vereinbar mit fokaler Enzephalitis), war die Defekt-Heilungsrate und die Letalität in der Patientengruppe mit Kombinationstherapie signifikant niedriger (p=0,014) als mit ACV-Monotherapie. Diese Beobachtung könnte ein erster Hinweis sein, daß eine Kombinationstherapie bei der fokalen viralen Enzephalitis einen therapeutischen Vorteil bringen könnte und sollte in einer kontrollierten prospektiven Studie geprüft werden.
    Notes: Summary Severe focal viral encephalitis is most commonly caused by herpes simplex virus (HSV), but other viruses may act as etiologic agents as well. Acyclovir (ACV) is the standard therapy for HSV encephalitis, but the mortality of 28% and defect healing rate of about 35% are still unsatisfactory. Furthermore, ACV has virtually no effect on other pathogens of viral encephalitis, except for varicella-zoster virus (VZV). It is well known that β-interferon (β-IFN) has a broad antiviral spectrum, and it has been demonstratedin vitro that β-IFN in combination with acyclovir has synergistic inhibitory effects on HSV. To investigate if the combination of ACV with and without β-IFN might also be of significance for the treatment of severe viral encephalitis, we performed a retrospective study. A case record form was sent to all 278 West German children's hospitals. The response rate was 78%. A total of 301 patients were reported, of whom 214 received specific antiviral therapy with either ACV alone (n=179) or ACV plus β-IFN (n=35). No overall differences between ACV monotherapy and the combination therapy were observed. However, in a subgroup of 41 patients (ACV n=30, ACV plus β-IFN n=11) who had low-density areas of the temporal lobes on cranial computed tomography scans, compatible with severe focal encephalitis, sequelae due to defect formation and mortality were significantly (p=0.014) reduced in patients who had received combination therapy. These data are the first indication that combination treatment with ACV and β-IFN may be of advantage in patients with focal viral encephalitis and should be controlled in a prospective trial.
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