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  • 1
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    Scandinavian journal of immunology 36 (1992), S. 0 
    ISSN: 1365-3083
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: The total kappa/lambda immunoglobulin light chain ratio and the kappa/lambda ratios within each of the serum immunoglobulin classes G. A. and M were measured in thirteen patients with humoral immunological disorders. Of those patients, eight had common variable immunodeficiency whereas live patients had other forms of humoral immunological deficiencies. Eleven patients had abnormal antibody response in vivo. All but three of the thirteen patients had clearly abnormal light chain ratios in one or more of the immunoglobulin classes.We conclude that humoral immunological disorders, usually characterized by abnormal heavy chain production and a disturbed antibody response, may frequently have a concomitant abnormal synthesis of the light chains resulting in an abnormal kappa/lambda light chain ratio.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 155 (1996), S. 362-367 
    ISSN: 1432-1076
    Keywords: Key words Hypothyroidism ; Growth ; Catch-up growth
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract This report presents an analysis of four patients who suffered from longstanding untreated hypothyroidism, with special attention to the phase of catch-up growth after the start of L-thyroxine treatment. Although a permanent height loss could not be prevented, the capacity to establish a remarkable catch-up growth spurt proved to be still intact, even after a long period of thyroid dysfunction. Conclusion Catch-up growth in hypothyroidism may be incomplete if treatment has been started shortly before or during puberty.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-1076
    Keywords: Arteriovenous aneurysm of the great cerebral vein of Galen ; Ultrasound duplexcanning ; Transfontanellar cerebral echography ; Doppler flow (velocity) measurements
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Using combined echoencephalography and Doppler flow determination the diagnosis Arteriovenous aneurysm of the great cerebral vein of Galen could be made in two infants. Without vascular surgery one patient died, the other recovered completely. CT scanning confirmed the diagnosis. Invasive methods such as cerebral angiography were avoided. Case histories, neuropathological findings, ultrasound method and results are presented. Pathogenesis, clinical signs, treatment and prognosis are discussed. With the ultrasound method presented, the nature and location of the vascular anomaly were demonstrable, thus additional higher risk diagnostic methods could be avoided or planned more purposefully.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 155 (1996), S. 362-367 
    ISSN: 1432-1076
    Keywords: Hypothyroidism ; Growth ; Catch-up growth
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract This report presents an analysis of four patients who suffered from longstanding untreated hypothyroidism, with special attention to the start of L-thyroxine treatment. Although a permanent height loss could not be prevented, the capacity to establish a remarkable catch-up growth spurt proved to be still intact, even after a long period of thyroid dysfunction. Conclusion Catch-up growth in hypothyroidism may be incomplete if treatment has been started shortly before or during puberty.
    Type of Medium: Electronic Resource
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  • 5
    ISSN: 1432-1076
    Keywords: Mulibrey nanism ; Immunodeficiency ; Light chains ; Growth hormone deficiency
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A combination of humoral immunodeficiency and isolated growth hormone deficiency was observed in a girl with Mulibrey nanism. The humoral immunodeficiency consisted of subnormal concentration of serum IgG, in particular IgG2 and IgG4, and low concentration of serum IgM. Serum IgA and IgD were elevated, IgE was absent. Antibody response in vivo was very low or absent and opsonization in vitro was defective. Total B-cell number was low. In addition, the serum kappa/lambda light chain ratios within the immunoglobulin classes G, A, and M were abnormal. The defective anti-body response may be linked to the abnormal kappa/lambda light chain ratios. Endocrine functions were normal except for isolated growth hormone deficiency. Therapy with human growth hormone resulted in increased growth velocity but did not improve humoral immune functions.
    Type of Medium: Electronic Resource
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  • 6
    ISSN: 1433-0350
    Keywords: Brain stem auditory evoked potentials ; Somatosensory evoked potentials ; Intracranial tumors ; Childhood
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Somatosensory and brainstem auditory evoked potentials (SSEPs and BAEPs) provide sensitive measures of the central conduction functions of the auditory and somatosensory input systems at different levels of the central nervous system. The contribution of SSEPs and BAEPs in the primary diagnosis and follow-up of 26 children with infra- and supratentorial tumors were reviewed. The SSEPs, especially the specific complex, showed a latency increase in patients with supratentorial and brainstem mass lesions involving directly or distantly the somatosensory tracts. The BAEPs were sensitive for supratentorial pressure effects and for local and distant posterior fossa tumor effects. In the follow-up of children, evoked potentials offer a good method of detecting tumor recurrence, whereas neuroradiological procedures may be obscured by surgical or radiation artifacts.
    Type of Medium: Electronic Resource
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  • 7
    Electronic Resource
    Electronic Resource
    Springer
    Journal of clinical immunology 12 (1992), S. 424-428 
    ISSN: 1573-2592
    Keywords: Immunoglobulin D (IgD) ; IgG3 ; clinical symptoms ; light chains
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Five patients with hyper-immunoglobulin D syndrome (hyper-IgD syndrome) were followed up for 3 to 8 years. In all patients studied, serum IgG3 was high. IgM decreased during the follow-up in all patients. In four of the patients serum IgA was elevated. In four patients the serum IgD κ/λ ratio was measured and was found to be raised in all. However, the serum total light-chain ratio and IgG, IgA, and IgM κ/λ ratios separately were virtually normal. In two of the patients, clinical symptoms preceded the increase in serum IgD. All patients had a history of severe reactions on immunizations in early childhood. We conclude that in hyper-IgD syndrome, other immunoglobulins may also be affected, in particular, IgA, IgM, and IgG3. The IgD light-chain ratio is also disturbed. We emphasize that clinical symptoms may herald immunological changes. This may be the result of an underlying factor causing both the clinical symptoms and, later, the increasing serum IgD levels.
    Type of Medium: Electronic Resource
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  • 8
    ISSN: 1573-6857
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology
    Notes: Abstract A patient is described with some features of the Turner syndrome (dwarfism, pterygium colli, hypoplasia mandibulae and syndactylism) but with normal genital development.* In the skin 9.6% of the nuclei were Barr-positive. Skin and blood-cultures revealed that she was a mosaic with an XO and an X-ring cell-line. The ring-chromosome is assumed to be derived from an X-chromosome. A possible explanation for the existence of patients with some features of the Turner syndrome and normal genital development is discussed.
    Type of Medium: Electronic Resource
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