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  • 1
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    Journal of clinical periodontology 23 (1996), S. 0 
    ISSN: 1600-051X
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Abstract Oral bacteria play an important role in the causation of oro-facial abscesses. However, they can also be involved in brain, liver and lung abscesses. To persist, it is essential that these bacteria can grow on those sites. The main source of nutrients for growth in abscesses is likely to be tissue exudate, which is rich in serum-derived proteins, and relatively poor in free amino acids and carbohydrates. Degradation of intact proteins seems a crucial step in providing the pep-tides necessary for energy generation. The aim of this study was to investigate the capacity of microorganisms from asscesses to degrade serum proteins, in particular immunoglobulins. To this end, samples were taken by aspiration from 16 odontogenic abscesses. It was found that pus from abscesses differed strongly in the concentration of viable bacterial cells. The ability of the abscess microflora to degrade serum proteins was investigated after growth of the sample in heat–inactivated human serum. The microflora from abscesses with a high concentration (n= 10) of bacteria strongly degraded immunoglobulins, whereas breakdown of immunoglobulins was virtually absent after growth of the microflora from low–bacterial concentration (n= 6) abscesses. Bacteriological analyses revealed the presence of at least one proteinase-producing species, like Porphyromonas, black-pigmented Prevotella species, or Actinomyces meyeri, in abscesses with a high density of bacteria, but not in those with low bacterial density. The results indicate that the capacity lo degrade intact proteins, in particular immunoglobulins, is a major determinant of bacterial growth in abscesses.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    Scandinavian journal of immunology 35 (1992), S. 0 
    ISSN: 1365-3083
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Longitudinal serum immunoglobulin levels were studied in 36 children with selective IgA deficiency during a median follow-up period of 5 years. Twenty-five children were ‘sporadic’ cases, and 11 were ‘familial’. Serum and saliva IgA levels in 23 children remained below 2 mg/1. Eight children with IgA levels above 2 mg/1 showed considerable intra-individual variance in serum IgA, but remained IgA deficient. Five children at various ages developed IgA levels above 50 mg/1 with detectable secretory IgA in saliva. In most of the children IgG subclass levels were found to be rather high, including at younger ages. There were no obvious differences between ‘sporadic’ and ‘familial’ cases except an association between IgD deficiency and serum IgA levels below 2 mg/1, and between serum levels of IgD above 1 1IU/ml and of IgA above 2 mg/1, which was found to be significant in the ‘sporadic’ group but not in the ‘familial’ group.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    Scandinavian journal of immunology 41 (1995), S. 0 
    ISSN: 1365-3083
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: In children with cryptogenic Lennox-Gastaut syndrome we found a functionally impaired humoral immune response to a primary antigen (haemocyanin), despite signs of a triggered immune system consisting of elevated IgG concentrations. This combination of immunological findings, considered to be the expression of a dysbalanced-triggered as well as functionally impaired-immune system, has also been described in an auto-immune disease like systemic lupus erythaematodes in humans, and in genetically epilepsy-prone rats. The interactions between the immune system and the nervous system in Lennox-Gastaut syndrome will be discussed.
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1432-1076
    Keywords: Muscle ; Lipid storage myopathy ; Carnitine ; Morphometria
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract An 18-month-old boy presented with general hypotonia, decreased muscle strength, retarded motor development and stunted growth. The excretion of dicarboxylic acids was enhanced. EMG was normal. A muscle biopsy revealed a lipid storage myopathy. Oral daily supplementation with 2 g D,L-carnitine resulted in: (1) an increase of the growth velocity; (2) increased muscle strength, and (3) a decrease in the lipid fraction of the fibre volume. The carnitine content of the muscle biopsied prior to treatment appeared to be normal.
    Type of Medium: Electronic Resource
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  • 5
    ISSN: 1432-1076
    Keywords: Mitochondrial myopathy ; Electron transport chain ; Metabolic screening
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The clinical identification of patients with defects in the mitochondrial respiratory chain is almost impossible. We describe screening tests that should be performed in order to select those patients in whom a skeletal muscle biopsy should be carried out for more specific biochemical assays. The importance of performing in vivo function tests is stressed. The biochemical diagnosis in disorders of the respiratory chain is presented and the application of immunological methods discussed.
    Type of Medium: Electronic Resource
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  • 6
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 150 (1991), S. 419-422 
    ISSN: 1432-1076
    Keywords: Hypopigmentation ; Immunodeficiency ; Griscelli disease
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A 9-month-old Turkish boy was diagnosed as having Griscelli disease (Chediak-Higashi-like syndrome). Clinical signs consisted of silver-grey hair and a relatively light skin colour, recurrent episodes of fever, with or without detectable infections, increasing hepatosplenomegaly, hypotonia and motor retardation. Laboratory studies showed pancytopenia of varying degree but neither inclusion bodies nor vacuoles were seen in his leucocytes. Serum immunoglobulin levels were normal except for a IgG2 deficiency. In the mixed lymphocyte reaction the stimulation capacity of the leucocytes was decreased. Microscopic examination of his hair and electron-microscopic examination of a skin biopsy further confirmed the diagnosis. Shortly before the diagnosis was made, the child developed cerebral symptoms with hemiparesis and convulsions. A CT scan suggested cell infiltration of the brain. A few weeks later the boy died of an infection.
    Type of Medium: Electronic Resource
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  • 7
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 151 (1992), S. 271-273 
    ISSN: 1432-1076
    Keywords: Acute non-lymphoblastic leukaemia ; Immunosuppressive drugs ; IgG2 deficiency
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A girl with acute non-lymphoblastic leukaemia was treated with immunosuppressive chemotherapy. After cessation of therapy she had three consecutive episodes of infection due toStreptococcus pneumoniae from which she recovered and was shown to have developed a combined deficiency of both IgG2 and IgG4. The patient eventually relapsed and died 3 years after the initial diagnosis. The importance of measuring IgG subclasses in patients treated with immunosuppressive chemotherapy is discussed.
    Type of Medium: Electronic Resource
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  • 8
    ISSN: 1432-1076
    Keywords: Key words CD4 deficiency ; Myelodysplastic syndrome ; Monosomy 7 ; Immunodeficiency
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We describe a patient with myelodysplastic syndrome with monosomy 7 presenting with a T-cell defect. He suffered from infections from the age of 10 years, when a CD4 deficiency and impaired lymphoproliferative responses in vitro were found. The only symptom of a myelodysplastic syndrome at that time was thrombocytopenia with giant platelets. Monosomy 7 was found in the bone marrow cells. At the age of 11 years he developed other characteristics of monosomy 7 including splenomegaly and anaemia. Some months later leukaemia was diagnosed. Conclusion In non-HIV CD4 deficiency myelodysplastic syndrome has to be considered.
    Type of Medium: Electronic Resource
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  • 9
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 155 (1996), S. 96-98 
    ISSN: 1432-1076
    Keywords: CD4-deficiency ; Myelodysplastic syndrome ; Monosomy 7 ; Immunodeficiency
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Abstract We describe a patient with myelodysplastic syndrome with monosomy 7 presenting with a T-cell defect. He suffered from infections from the age of 10 years, when a CD4 deficiency and impaired lymphoproliferative responses in vitro were found. The only symptom of a myelodysplastic syndrome at that time was thrombocytopenia with giant platelets. Monosomy 7 was found in the bone marrow cells. At the age of 11 years he developed other characteristics of monosomy 7 including splenomegaly and anaemia. Some months later leukaemia was diagnosed. Conclusion In non-HIV CD4 deficiency myelodysplastic syndrome has to be considered.
    Type of Medium: Electronic Resource
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  • 10
    ISSN: 1432-1076
    Keywords: Organic aciduria ; Hypoxia ; Prematures ; Gas chromatography ; Mass spectrometry
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A very unusual pattern of organic acid excretion was established in the urine of two premature newborns with severe respiratory distress and cerebral haemorrhages. By combined gas chromatography/mass spectrometry the following acids were identified: lactic acid, α-hydroxy-butyric acid, β-hydroxy-butyric acid, α-hydroxy-isovaleric acid, and p-hydroxy-phenyllactic acid. Calculation of the concentration revealed an excessive excretion of lactic acid and also very high excretion of the other acids. A post mortem blood sample from one of the patients revealed a comparable pattern. The abnormal urinary organic acid excretion pattern was most probably caused by severe tissue hypoxia.
    Type of Medium: Electronic Resource
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