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  • 1
    ISSN: 1432-0843
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Assessment of the toxicity caused by chemotherapy in children with cancer has become more important as the number of long-term survivors has continued to increase. It is vital to monitor both acute life-threatening adverse effects and long-term toxicity that may impair the child's development and cause permanent morbidity. Renal damage may follow treatment with cytotoxic drugs, especially cisplatin or ifosfamide, and lead to glomerular, proximal tubular or distal tubular impairment or to any combination of these. Greater understanding of nephrotoxicity and of its prevention may enable the use of more intensive schedules or of higher doses of potentially nephrotoxic chemotherapy. However, the evaluation of cytotoxic drug-induced nephrotoxicity has frequently depended mainly on measurement of the plasma creatinine concentration, which may remain normal despite substantial glomerular impairment or severe tubular dysfunction. Detailed assessment of nephrotoxicity depends on an understanding of normal renal physiology and requires evaluation of all aspects of function. A comprehensive but simple investigatory protocol that enables assessment of the nature and severity of nephrotoxicity in children is described, which can be performed without admission to hospital.Glomerular function is assessed by measurement of the glomerular filtration rate from the plasma clearance of [51Cr]-ethylenediaminetetraacetic acid ([51Cr]-EDTA).Proximal nephron function is evaluated in three ways: by measurement of the concentration of calcium, magnesium, phosphate, glucose and urate in blood and urine along with calculations of their fractional excretion and of the renal threshold for phosphate; by determination of the excretion in urine of low-molecular-weight proteins (e.g. retinol-binding protein); and by investigation of urinary bicarbonate excretion in patients who are acidotic.Distal nephron function is initially investigated by examination of the concentration (osmolality) and acidification (pH) of an early morning sample of urine. Finally, a group of general investigations is performed, including quantitation of urinary excretion of renal tubular enzymes (e.g.N-acetylglucosaminidase) and measurement of blood pressure.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-198X
    Keywords: D+haemolytic uraemic syndrome ; Severity ; Identical twins
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-198X
    Keywords: Fanconi syndrome ; Lactic acidosis ; Mitochondria ; Respiratory chain ; Complex III
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Fanconi syndrome is an important presentation of respiratory chain disease. We report three patients who presented in the neonatal period with Fanconi syndrome, lactic acidosis and intrauterine growth retardation. In all three patients the major biochemical defect was in complex III of the mitochondrial respiratory chain, a relatively uncommon defect. The diagnosis could only be made by muscle biopsy as the defect was not expressed in cultured skin fibroblasts. Treatment with vitamins C and K3 and ubiquinone did not alter the course of the disease and all patients died before the age of 4 months.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    Pediatric nephrology 1 (1987), S. 605-607 
    ISSN: 1432-198X
    Keywords: Haemolytic-uraemic syndrome ; Glucose intolerance ; Peritoneal dialysis ; Diabetes mellitus
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A 3-year-old girl developed glucose intolerance during treatment with peritoneal dialysis for haemolytic-uraemic syndrome and became insulin dependent for almost a month. Diabetes mellitus in this condition is a rare but potentially serious complication which should be sought by regular blood sugar measurement during the acute stage of the illness.
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    Pediatric nephrology 14 (2000), S. 997-1001 
    ISSN: 1432-198X
    Keywords: Key words Chronic renal failure ; Renal transplant ; Dental health
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  Thirty-eight children (aged 2–16 years) attending a regional kidney unit had a full clinical and radiological dental examination. Twenty had previously undergone a renal transplant, 11 had chronic renal failure and 7 had other renal diseases. Periodontal disease was uncommon The presence of gingival hyperplasia (gum overgrowth), as recorded in 22 of the children, did not show any relationship with the use of immunosuppressant therapy. However, gingival overgrowth was so excessive in 2 patients that surgical removal was required. The prevalence of dental caries was low. Enamel defects were common, and of an unusual pattern, with a much higher prevalence of diffuse opacities and enamel hypoplasia than in the normal child population, 83% and 22%, respectively. This increased prevalence is probably due to disordered calcium and phosphate metabolism. The prevalence of these defects may reflect an early onset of renal disease, since there were a number of very young children in the programme. Dental and medical care should be closely integrated for children with renal disease to avoid the undesirable dental sequelae of, in particular, gingival overgrowth, carcinoma and enamel hypoplasia.
    Type of Medium: Electronic Resource
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  • 6
    ISSN: 1432-198X
    Keywords: Erythropoietin ; Anaemia ; Iron deficiency ; Kidney failure
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Iron deficiency severely limits the efficacy of recombinant human erythropoietin (EPO). In order to determine how best to identify and monitor children at risk of developing iron deficiency, we serially measured several parameters of iron status in nine children before and during a 24-week period of EPO therapy. Serum ferritin was the best predictor of development of iron deficiency, five of the nine children developed iron deficiency, characterised by a poor haemoglobin response or evidence of microcytosis and hypochromia; all had a serum ferritin of 60 μg/l or less at the start of EPO. Haemoglobin response was also related to change in mean red cell volume (MCV); a falling MCV, irrespective of absolute value, accompanying a poor response to EPO. Iron treatment in five children resulted in significant improvements in haemoglobin and iron status parameters. Although MCV remained low, there was a marked increase in red cell volume distribution width after iron, which may be of value in monitoring the response to iron therapy. We suggest that children with a serum ferritin of 60 μg/l or less and those who develop a falling MCV during EPO treatment should receive high-dose oral iron supplementation before and during treatment with EPO.
    Type of Medium: Electronic Resource
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  • 7
    Electronic Resource
    Electronic Resource
    Springer
    Pediatric nephrology 3 (1989), S. 451-453 
    ISSN: 1432-198X
    Keywords: Congenital nephrotic syndrome ; Finnish ; Unilateral nephrectomy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Finnish congenital nephrotic syndrome is fatal unless managed aggressively; treatment in hospital with albumin infusions and subsequent bilateral nephrectomy allows survival. However, it consistently results in protein malnutrition which may lead to serious infections and severe physical and developmental retardation, as well as the subsequent burden of dialysing an anephric child. We treated a boy by unilateral nephrectomy at 3 months. The reduced protein loss allowed home management with albumin infusions and resulted in a substantial rise in his plasma IgG levels. The glomerular filtration rate declined fairly rapidly after unilateral nephrectomy, so he needed peritoneal dialysis by 16 months, which was straightforward as he still maintained a good urine output. Management by unilateral nephrectomy has allowed this boy to grow and develop normally. The time spent in hospital was minimal and the very high cost of providing albumin preparations was reduced considerably.
    Type of Medium: Electronic Resource
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  • 8
    Electronic Resource
    Electronic Resource
    Springer
    Pediatric nephrology 3 (1989), S. 225-226 
    ISSN: 1432-198X
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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