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  • 1
    ISSN: 1432-198X
    Keywords: Nephrotic syndrome ; Kidney transplantation ; Focal glomerulosclerosis ; Plasmaphaeresis ; Cyclophosphamide
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Steroid-resistant nephrotic syndrome (NS) with focal glomerulosclerosis (FGS) and its recurrence after transplantation are mainly seen in children. The recurrence rate approximates 30% and the graft loss is about half this. Several therapeutic regimens have been proposed, giving conflicting results. In an attempt to remove a putative circulating factor and inhibit its production by lymphocytes, three patients with biopsy-proven FGS in the native kidney were included in a prospective uncontrolled trial using early plasmaphaeresis followed by substitutive immunoglobulins in association with methylprednisolone pulses and cyclophosphamide instead of azathioprine over a 2-month period. The patients were girls, aged 6.5, 13.3 and 15.8 years, who received a cadaveric transplant; concomitant immunosuppression included prednisone and cyclosporine A. All three patients exhibited early recurrence of the NS and were treated 5–10 days after the onset of proteinuria. Rapid and sustained remission was achieved in all patients within 12–24 days on therapy. One patient experienced a late acute but steroid-sensitive rejection episode; another suffered from septic ankle arthritis as a complication of reinforced immunosuppression. The latter girl had a second late recurrence of proteinuria that was controlled within 7 weeks. With a 18-to 27-month follow-up, all three patients have normal renal function, normal blood pressure and no proteinuria. We conclude that intensive therapy using plasmaphaeresis, steroid pulses and cyclophosphamide over a 2-month period can induce complete remission in children with early recurrence of NS after transplantation.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-198X
    Keywords: Bone mineral density ; Bone mineral content ; Body composition ; Chronic renal failure ; Dual energy X-ray absorptiometry ; Growth hormone ; Hemodialysis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Dual energy X-ray absorptiometry (DEXA) is a non-invasive accurate method which estimates bone mineral content and density (BMD), as well as fat (FM) and lean (LM) body mass. This method was used in control children in order to establish normal values for BMD of lumbar spine and whole body composition {logistic curves, general equation E=k+K/[1+αexp(-βA)]}. In children with chronic renal failure (CRF), LM correlated with the urinary excretion of creatinine (r=0.97,P=0.0001) independently from glomerular filtration rate. However, the assessment of LM by DEXA must take into account the hydration level, since there is a positive correlation between fluid loss and reduction in LM in children on hemodialysis (r=0.98,P=0.0001). After renal transplantation, a significant loss of BMD (median −9.2%), was observed at 6 months which returned to 95% of pretransplant values by the end of the 1 st year. Maximal changes in LM and FM occurred during the first 3 months (−7.8% and +7.2%, respectively) and may be due to steroids; these should be influenced by physical activity since FM correlated inversely with maximal oxygen consumption (r=0.69P=0.0001). Recombinant growth hormone treatment could also increase LM and decrease FM, as shown in 9 patients. DEXA appears therefore to be a reliable method for evaluating therapeutic interventions affecting nutritional status in children with CRF.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-198X
    Keywords: Key words: Bone mineral density   ;   Bone mineral content   ;   Body composition   ;   Chronic renal failure   ;   Dual energy X-ray absorptiometry   ;   Growth hormone   ;   Hemodialysis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract. Dual energy X-ray absorptiometry (DEXA) is a non-invasive accurate method which estimates bone mineral content and density (BMD), as well as fat (FM) and lean (LM) body mass. This method was used in control children in order to establish normal values for BMD of lumbar spine and whole body composition {logistic curves, general equation E = k+K/[1+αexp( – βA)]}. In children with chronic renal failure (CRF), LM correlated with the urinary excretion of creatinine (r = 0.97, P = 0.0001) independently from glomerular filtration rate. However, the assessment of LM by DEXA must take into account the hydration level, since there is a positive correlation between fluid loss and reduction in LM in children on hemodialysis (r = 0.98, P = 0.0001). After renal transplantation, a significant loss of BMD (median  – 9.2%) was observed at 6 months which returned to 95% of pretransplant values by the end of the 1st year. Maximal changes in LM and FM occurred during the first 3 months ( – 7.8% and +7.2%, respectively) and may be due to steroids; these should be influenced by physical activity since FM correlated inversely with maximal oxygen consumption (r = 0.69, P = 0.0001). Recombinant growth hormone treatment could also increase LM and decrease FM, as shown in 9 patients. DEXA appears therefore to be a reliable method for evaluating therapeutic interventions affecting nutritional status in children with CRF.
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1432-198X
    Keywords: Key words Nephritis ; Post-infectious glomerulonephritis ; Nephrotic syndrome ; Acute renal failure ; Mycoplasma pneumoniae
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  Mycoplasma pneumoniae infection is a rare cause of acute nephritis. Six children (2 girls) aged 5–10 years, admitted for nephritis, had serological tests showing recent Mycoplasma pneumoniae infection. The diagnosis of Mycoplasma pneumoniae infection was based on the presence of serum IgM, detected either by immunofluorescence (IF) (n=1) or enzyme-linked immunosorbent assay (n=5). Four children had a renal biospy, with analysis of parenchymal Mycoplasma pneumoniae components by indirect IF and polymerase chain reaction. Extrarenal symptoms were: respiratory (n=3), ear, nose, and throat (n=2), gastrointestinal (n=3), hepatic (n=1), neurological (n=1), articular (n=1), and hematological (n=3). The patients presented with acute nephritis (1 had a nephrotic syndrome) or with acute renal failure and proteinuria. Pathological findings included type 1 membranoproliferative glomerulonephritis (MPGN, n=1), proliferative endocapillary glomerulonephritis (n=2), and minimal change disease (n=1). The patient with type 1 MPGN progressed rapidly towards end-stage renal failure because of a congenital solitary kidney. Among the patients with endocapillary glomerulonephritis, 1 relapsed 6 months later and remained proteinuric, while the other recovered, as did the child with minimal change disease. The search for Mycoplasma pneumoniae antigens and nucleic acids in renal tissue was negative. However, the absence of the microorganism in the kidney is a common feature of post-streptococcal glomerulonephritis. We conclude that Mycoplasma pneumoniae is a rare yet potential cause of acute glomerulonephritis.
    Type of Medium: Electronic Resource
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  • 5
    ISSN: 1432-198X
    Keywords: Infantile nephrotic syndrome ; Miosis ; Cataract ; Glomerular basement membrane
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Two siblings born from consanguineous parents experienced infantile nephrotic syndrome with ocular and neurological abnormalities; the boy also had a micropenis; both patients died before age 1 year. An initial renal biopsy followed by a two-step binephrectomy allowed good histological assessment of disease progression in one patient. The progression of the lesions was characterized by mesangial involvement, then an extensive extracapillary proliferation and tubular dilatations with a high mitotic activity of the epithelium and nuclei of unequal size. The main features involved major ultrastructural changes of the glomerular basement membrane. These two patients may represent a new disease entity or a severe form of diffuse mesangial sclerosis, with autosomal recessive inheritance.
    Type of Medium: Electronic Resource
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  • 6
    ISSN: 1432-198X
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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