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  • 1
    Electronic Resource
    Electronic Resource
    Glucocorticoid receptors in idiopathic nephrotic syndrome (1999)
    Springer
    Pediatric nephrology 13 (1999), S. 653-656 
    Details
    ISSN: 1432-198X
    Keywords: Key words Nephrotic syndrome ; Glucocorticoid receptors ; Glucocorticoids
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  The variable response of patients with idiopathic nephrotic syndrome (NS) to glucocorticoid (GC) treatment has not been explained. Earlier studies indicated that the response is limited by cellular GC receptors. We investigated these receptors in mononuclear leukocytes of 28 pediatric patients with NS divided into three groups: steroid-sensitive in relapse, steroid-sensitive in remission, and steroid-resistant. Density and binding affinity of GC receptors were determined by a dexamethasone binding assay; no significant differences were found between the three patient groups and between these and healthy controls, although a few patient values fell outside the range of controls. Total and free plasma concentrations of cortisol were low in all three patient groups. A weak positive correlation was found between the number of GC receptors and total plasma cortisol (r=0.36, P=0.03). The results suggest that factors other than GC receptors that mediate the cellular effects of GC are involved in the variable response of NS patients to GC.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004670050675
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  • 2
    Electronic Resource
    Electronic Resource
    Clinical outcome of Schönlein-Henoch purpura nephritis in children (1999)
    Springer
    Pediatric nephrology 13 (1999), S. 816-823 
    Details
    ISSN: 1432-198X
    Keywords: Key words Schönlein-Henoch purpura ; Glomerulonephritis ; Hypertension ; Proteinuria ; Renal failure ; Plasma exchange
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  We studied the long-term outcome of 64 children with biopsy-proven Schönlein-Henoch purpura (SHP) nephritis over 1–23 years of follow-up. Overall renal survival 10 years after onset was 73%. Multivariate logistic regression analysis identified initial renal insufficiency (P=0.004), nephrotic syndrome (P=0.037), and the severity of histological alterations, as defined by the proportion of glomerular crescents (P=0.051), as significant independent predictors of progressive renal failure. Four patients followed for more than 19 years showed glomerular damage after transient recovery. Eight children with crescentic nephritis associated with a rapidly progressive course and/or persistent nephrotic syndrome were treated by at least seven sessions of plasma exchange (PE) within 16 weeks of onset of purpura. During treatment serum creatinine levels dropped in each patient from a mean of 2.3 to 1.1 mg/dl, followed by a rebound increase. Repeated courses of PE in 5 patients produced comparable responses. Four patients undergoing PE reached end-stage renal disease at 1.2.–3.7 years after onset, whilst 3 finally were in preterminal renal failure (creatinine 3.2–6.1 mg/dl after 7–13.5 years), and 1 patient reached a normal glomerular filtration rate. Our experience suggests that initial renal insufficiency is the best single predictor of the further clinical course in children with SHP nephritis. Early PE appears to delay the progression in some patients with severe, rapidly progressive forms of the disease.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004670050707
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    Paper (German National Licenses)
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  • 3
    Electronic Resource
    Electronic Resource
    Body growth of children with steroid-resistant nephrotic syndrome (1999)
    Springer
    Pediatric nephrology 13 (1999), S. 828-834 
    Details
    ISSN: 1432-198X
    Keywords: Key words Steroid-resistant nephrotic syndrome ; Growth ; Corticosteroids ; Protein metabolism ; Chronic renal insufficiency
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  Whilst it is assumed that body growth is retarded in children with steroid-resistant nephrotic syndrome (NS), the degree of growth failure and the pathomechanisms involved are poorly understood. We collected serial growth data in 45 children (24 males) with steroid-resistant NS usually from onset to end-stage renal disease (ESRD) during childhood (n=10) or until final height was attained (n=27). Mean follow-up time was 9 (2–19) years. Mean initial standardized height was –0.3±1.2 standard deviation scores (SDS). Mean final height was +0.4 SDS in males and –1.0 SDS in females (sex difference not significant). In 16 patients with serum creatinine levels consistently 〈1.2 mg/dl, mean final height SDS was 0.3 SDS higher than that obtained within 6 months of onset. In contrast, 9 children who entered ESRD lost an average of 1.3 SDS from the initial record to ESRD (P=0.017). In prepubertal patients without renal insufficiency, mean height SDS decreased during corticosteroid treatment by 0.3 SDS, followed by a partial catch-up after discontinuation of treatment; the change from initial to final height SDS was inversely correlated with the total prednisone dose given (r=–0.50, P=0.03). In 16 prepubertal children with serial height and serum protein measurements who were off steroids and maintained normal creatinine levels, mean individual albumin concentrations correlated with the change in height SDS per year (r=0.65, P=0.0006) and in boys with final height (r=0.73, P=0.03). In conclusion, growth in steroid-resistant NS depends on the preservation of renal function, the cumulative dose of steroids applied, and the severity of hypoproteinemia.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004670050709
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  • 4
    Electronic Resource
    Electronic Resource
    Probucol for treatment of hyperlipidemia in persistent childhood nephrotic syndrome (1999)
    Springer
    Pediatric nephrology 13 (1999), S. 7-12 
    Details
    ISSN: 1432-198X
    Keywords: Key words Probucol ; Nephrotic syndrome ; Hyperlipidemia ; Antioxidants
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  In a prospective, uncontrolled multicenter study, we have evaluated the effects of probucol on hyperlipidemia, proteinuria, and glomerular filtration rate (GFR) in hyperlipidemic children with persistent nephrotic syndrome. Probucol was started for a total of 12 weeks in 8 children and for 24 weeks in 14 children. Lipoprotein profiles, serum malondialdehyde (MDA) levels, proteinuria, renal function, and electrocardiogram were monitored every 4 weeks. Side effects were recorded by questionnaire. Treatment was completed by 7 of 8 patients for 12 weeks and by 7 of 14 children for 24 weeks. After 12 weeks, the mean serum concentrations of triglycerides (−15%), total cholesterol (−25%), very low-density lipoprotein-cholesterol (−27%), low-density lipoprotein-cholesterol (−23%), and high-density lipoprotein-cholesterol (−24%), as well as apolipoprotein (apo) A-I (−19%), apo B (−21%), and MDA (−32%) were reduced. The positive effects of probucol on the lipoprotein profile persisted over 24 weeks; however, there was no significant effect on either proteinuria or GFR. In conclusion, probucol had beneficial effects on lipoproteins and lipid peroxidation, but improved neither proteinuria nor GFR. The drug was generally tolerated well, but had to be discontinued because of a prolonged QT interval in 4 of 22 patients.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004670050554
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    Paper (German National Licenses)
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  • 5
    Electronic Resource
    Electronic Resource
    Cardiac function and structure in patients with chronic renal failure (1999)
    Springer
    Pediatric nephrology 13 (1999), S. 951-965 
    Details
    ISSN: 1432-198X
    Keywords: Key words Cardiac complications ; Chronic renal failure ; End-stage renal disease ; Left ventricular function ; Left ventricular hypertrophy ; Renal transplantation ; Echocardiography
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  The long-term consequences of cardiac alterations in children with chronic renal failure (CRF) and after renal transplantation (TX) are largely unknown. Studies in adults with end-stage renal disease (ESRD) assume that the fate of many pediatric patients is determined by a high cardiovascular morbidity and mortality. This review describes clinical manifestations, pathophysiology, cardiac function and structure, and management of heart disease in children with CRF and post transplant. Echocardiography and Doppler ultrasonography allow differentiation of three functional disturbances: hypercirculation, systolic left ventricular (LV) dysfunction, and diastolic LV dysfunction, in addition to analysis of LV size and myocardial mass. From adult studies LV hypertrophy is recognized as an early prognostic marker of cardiovascular disease. It is present in about half of children with ESRD and after TX. It may regress, at least in part, by control of hypertension, hypervolemia, and anemia. Experimental studies have shown that, independent of these hemodynamic complications, uremia is associated with structural abnormalities of the heart, which were also described in adult patients with ESRD. These lesions consist mainly of hypertrophy of cardiomyocytes, interstitial fibrosis, and vascular changes (rarefied capillaries, thickened arteriolar walls). Cardiac complications in children with CRF and after TX deserve regular clinical and echocardiographic monitoring in order to minimize later cardiovascular morbidity by appropriate treatment.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004670050737
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    Paper (German National Licenses)
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