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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 139 (1982), S. 296-298 
    ISSN: 1432-1076
    Keywords: Hyperparathyroidism ; Chronic renal failure ; Anemia ; Dialysis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The clinical course of an adolescent on chronic hemodialysis with severe hyperparathyroidism, osteodystrophy and profound anemia is described. After 1,25,dihydroxycholecalciferol was instituted for therapy, bone disease improved as evidenced by radiographic findings. Transfusion requirements diminished and bone marrow fibrosis on serial biopsies decreased significantly. These therapeutic results link hyperparathyroidism with the pathogenesis of anemia in chronic renal failure.
    Type of Medium: Electronic Resource
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  • 2
    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
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  • 3
    ISSN: 1432-1998
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Of 60 renal transplant patients who underwent 134 duplex Doppler sonograms, 38 had normal frequency shift profiles and 8 showed only minimal disturbances, within normal post-transplantation limits. Fourteen patients had hypertension and showed a very abnormal profile. In 3 of the 14 the hypertension was controllable medically, and the remaining 11 underwent angiography. Duplex Doppler sonography showed gross turbulence (spectral broadening, bidirectional flow) and high frequency shift confirmed on angiography to represent 50 to 75% main renal artery stenosis in 8 of these 11, and major renal intrahilar vessel stenosis in 2. In the remaining patient, no Doppler signal was found and 95% stenosis was present. In 1 patient with intrahilar stenosis, arterial signals detected in the renal veins indicated the presence of a post-biopsy arteriovenous fistula. One patient with main renal artery stenosis also had a fistula, which was masked by a 70% stenosis. All patients with angiographically proven renal artery stenosis had a distinctive abnormality of the Doppler arterial flow pattern.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    Pediatric nephrology 13 (1999), S. 401-403 
    ISSN: 1432-198X
    Keywords: Key words Hemodialysis ; End-stage renal disease ; Infants
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  Hemodialysis (HD) of infants with end-stage renal disease (ESRD) is technically difficult and labor intensive, although there are few data in the literature to document the outcomes of this treatment. We retrospectively reviewed all patients with ESRD who received HD between 1983 and 1997 who weighed 〈10 kg at the beginning of HD. A total of ten patients aged 2–27 months, weighing 3.5–9.5 kg, were identified. All patients were dialyzed through a central venous line; three had a failed sapheno-femoral loop and one a failed brachial shunt. Line clot was observed in nine and line sepsis in six patinets. Subclavian vein stenosis was documented in one patient following removal of a clotted subclavian line. The mean urea reduction ratios calculated during the 1st and 3rd month of HD were only 54% and 49%, respectively. Anemia was a frequent problem, despite the use of erythropoietin in seven of the infants. Outcomes included: successful renal transplant in four, switch back to peritoneal dialysis in two, improved renal function and dialysis discontinuation in one, and death after withdrawal of treatment in three patients. All three patients who died were 〈5 months of age, weighed 〈5 kg, and were anuric; two of the three had congenital nephrotic syndrome. In conclusion, successful HD is possible in small children with ESRD, but morbidity is substantial and mortality is high.
    Type of Medium: Electronic Resource
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