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  • 1
    Electronic Resource
    Electronic Resource
    Obstructive airway disease caused by Moraxella catarrhalis after renal transplantation (2000)
    Springer
    Pediatric nephrology 14 (2000), S. 707-709 
    Details
    ISSN: 1432-198X
    Keywords: Key words Biedl-Bardet syndrome ; Moraxella catarrhalis ; Pulmonary obstructive disease ; Renal transplantation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  We report a case of severe acute obstructive airway disease 2 months after renal transplantation in a 16-year-old patient with Biedl-Bardet syndrome who was transplanted for end-stage renal failure secondary to cystic kidney disease. Symptoms of severe obstructive airway disease developed 2 months after transplantation under immunosuppression with prednisone, azathioprine, and tacrolimus. The patient did not develop signs of infection; progressive shortness of breath remained the only symptom for several weeks. After extensive diagnostic evaluation, bronchoalveolar lavage revealed Moraxella catarrhalis as the single infectious agent. After 3 weeks of appropriate antibiotic therapy, symptoms of obstructive airway disease were completely relieved. This atypical presentation of Moraxella infection in an immunocompromised host represents a rare complication of renal transplantation, especially in young patients. Special aspects such as frequency, diagnosis, differential diagnosis, and management of this rare complication of renal transplantation in a pediatric patient are discussed.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/PL00013421
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  • 2
    Electronic Resource
    Electronic Resource
    Does growth hormone therapy harmonize distorted morphology and body composition in chronic renal failure? (2000)
    Springer
    Pediatric nephrology 15 (2000), S. 229-235 
    Details
    ISSN: 1432-198X
    Keywords: Key words Growth hormone ; Chronic renal failure ; Morphological disproportion ; Harmonization ; Anthrometry ; Body shape
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  Recombinant human growth hormone (rhGH) therapy of growth retardation in chronic renal failure (CRF) has become well established. While there are ample data about its effectiveness in restoring longitudinal growth delay, data on complex anthropometry are scarce. Twenty-three children with CRF (6 after renal transplantation) were investigated using 24 different parameters. The analysis revealed disproportionate growth in CRF. While parameters of the trunk and transverse dimension of the head were preserved, the extremities were affected more severely. Bone dimensions of the legs and arms as well as muscle mass were affected the most. RhGH therapy was effective in restoring impaired longitudinal dimensions of the body in CRF. The restoration of growth retardation occurred predominantly in the extremities. This was accompanied by an increase in transversal dimensions, circumferences and soft tissue of the extremities, as well as an increase in bone and muscle volume, indicating harmonization of the total body shape. The dimensions of trunk, forehead diameter and morphological face height remained within the normal range. There was no evidence of acromegaly in children with CRF and rhGH therapy. We conclude that rhGH therapy at a dose of 28 iU/m2/week is effective in restoring previously disproportional body structure and shape in children with CRF.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004670000478
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    Paper (German National Licenses)
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  • 3
    Electronic Resource
    Electronic Resource
    Manifestations and treatment of Schimke immuno-osseous dysplasia: 14 new cases and a review of the literature (2000)
    Springer
    European journal of pediatrics 159 (2000), S. 1-7 
    Details
    ISSN: 1432-1076
    Keywords: Key words Osteochondrodysplasia ; Cerebral ischaemia ; Immunologic deficiency syndrome ; Kidney failure ; Hypothyroidism ; Focal glomerulosclerosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Schimke immuno-osseous dysplasia (SIOD) is a rare autosomal recessive spondylo-epiphyseal dysplasia. The characteristic features of SIOD include 1) short stature with hyperpigmented macules and an unusual facies, 2) proteinuria with progressive renal failure, 3) lymphopenia with recurrent infections, and 4) cerebral ischaemia. Although 25 patients have been reported with this disorder, the clinical course and phenotype of SIOD are not well characterized. This report summarizes the clinical findings, course and treatment of reported patients and includes 14 additional patients with SIOD. We emphasize the high incidence of cerebral ischaemia and ocular abnormalities, define the high incidence of thyroid dysfunction and blood cytopenia, and confirm the absence of effective and durable medical therapies. Conclusion Schimke immuno-osseous dysplasia is a multi-system autosomal recessive disorder with variable expression that affects the skeletal, renal, immune, vascular, and haematopoietic systems. Medical therapy is limited especially for more severely affected individuals.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004310050001
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    Paper (German National Licenses)
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