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  • 1
    Digitale Medien
    Digitale Medien
    Thrombotisch-thrombozytopenische Purpura mit akuter Niereninsuffizienz bei einem Knaben mit Lupus-ähnlicher Systemerkrankung (1997)
    Springer
    Monatsschrift Kinderheilkunde 145 (1997), S. 1176-1181 
    Details
    ISSN: 1433-0474
    Schlagwort(e): Schlüsselwörter Thrombotisch-thrombozytopenische Purpura ; Hämolytisch-urämisches Syndrom ; Systemischer Lupus erythematodes ; Plasmapherese ; Key words Thrombotic thrombocytopenic purpura ; Hemolytic uremic syndrome ; Systemic lupus erythematosus ; Lupus-like-syndrome ; Plasma exchange
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Beschreibung / Inhaltsverzeichnis: Summary A 139/12 year old boy presented with the clinical and laboratory signs of a systemic lupus erythematosus (SLE)-like illness. Three months later he developed acute renal failure associated with hemolytic anemia and thrombocytopenia while being treated with prednisone and a non-steroidal antiphlogistic agent. Kidney biopsy revealed endotheliotropic microangiopathy. A thrombocytopenic purpura/hemolytic uremic syndrome was diagnosed according to clinical and histological findings. Plasma exchange, hemodialysis and immunosuppressive therapy with prednisone and cyclophosphamide resulted in a normalization of the hematologic findings, and the kidney function improved. The cyclophosphamide therapy was continued over 21/2 years. Three years after the acute episode the boy is free of symptoms with glomerular filtration rate of 71 ml/min/1.73 qm. Discussion. The association of SLE with thrombotic thrombocytopenic purpura is rare, has predominantly been described in adults and has a high mortality rate. Our case demonstrates the success of an early aggressive therapy including plasma exchange, hemodialysis, prednisolone and cyclophosphamide.
    Notizen: Zusammenfassung Ein 139/12 Jahre alter Knabe entwickelte 3 Monate nach Beginn einer Lupus-ähnlichen Systemerkrankung unter Prednisontherapie ein akutes Nierenversagen mit hämolytischer Anämie und Thrombozytopenie. Die Nierenbiopsie zeigte eine thrombotische Mikroangiopathie. Unter Behandlung mit Plasmaaustausch, immunsuppressiver Therapie und Hämodialyse normalisierten sich die hämatologischen Befunde, und die Nierenfunktion besserte sich. Die Cyclophosphamidtherapie wurde über 21/2 Jahre fortgesetzt. Drei Jahre nach dem akuten Nierenversagen ist der Junge unter antihypertensiver Behandlung symptomfrei und hat eine normale glomeruläre Filtrationsrate. Diskussion. Die Assoziation eines systemischen Lupus erythematodes mit einer thrombotisch-thrombozytopenischen Purpura ist in der Literatur bisher nur selten und fast nur bei Erwachsenen beschrieben worden; sie hat eine hohe Mortalität. Der dargestellte Fall demonstriert den Erfolg einer frühzeitigen aggressiven Therapie.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1007/s001120050216
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  • 2
    Digitale Medien
    Digitale Medien
    Low-dose cyclosporin A therapy in cadaver renal transplantation in children (1989)
    Springer
    Transplant international 2 (1989), S. 203-208 
    Details
    ISSN: 1432-2277
    Schlagwort(e): Pediatric renal transplantation ; Cyclosporin, low-dose, in children ; Growth, cyclosporin, in kidney transplantation
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract Fifty-one pediatric patients undergoing a first cadaveric kidney transplantation were followed for at least 2 years after grafting. They were divided into two groups: those treated with methylprednisolone plus azathioprine (AZA) and those treated with methylprednisolone plus low-dose cyclosporin A (CyA; median dose 109 mg/m2 per day ≙ 3.4 mg/kg per day after 1 year). The steroid dosage given was significantly lower in the second group. The 4-year graft survival rate was 68% for the AZA group and 78% for the CyA group. Renal function did not differ significantly in the two groups; after 1, 2, and 3 years, the median 24-h creatinine clearance was 79, 69, and 51 ml/min/1.73 m2, respectively, for the AZA group and 78, 63, and 68 ml/min/1.73 m2, respectively, for the CyA group. Linear growth was similar in the two groups. We conclude that in pediatric patients the results of low-dose CyA immunosuppression do not differ significantly from those obtained with AZA in terms of graft survival, renal function, or growth.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1007/BF02414535
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  • 3
    Digitale Medien
    Digitale Medien
    Impact of recurrent nephrotic syndrome after renal transplantation in young patients (1998)
    Springer
    Pediatric nephrology 12 (1998), S. 529-533 
    Details
    ISSN: 1432-198X
    Schlagwort(e): Key words: Nephrotic syndrome ; Focal segmental glomerulosclerosis ; Renal transplantation ; Recurrent disease ; Plasma exchange
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract. Recurrent disease is a frequent complication of patients transplanted for steroid-resistant nephrotic syndrome associated with focal segmental glomerulosclerosis. Its long-term prognosis has rarely been studied. We examined 39 patients aged 4–25 (mean 13.5) years at the time of first transplantation (TX). Twelve of these (30%) developed nephrotic syndrome after the first TX and 2 of 8 after the second TX. The mean observation period from first TX to last observation with a functioning graft or graft loss was 5.4 (0.1–19.3) years. We confirmed that recurrent disease is associated with older age at onset of the primary disease, shorter time from onset to end-stage renal disease, and diffuse mesangial proliferation in the initial kidney biopsy. Remissions occurred in all 3 children undergoing early repeated plasma exchange and in 1 adolescent following introduction of cyclosporin A 7 years after TX. At last observation 42% of relapsing and 48% of non-relapsing patients with a similar follow-up period had a functioning first graft. Median first graft survival was almost identical in the relapsing and the non-relapsing patients (4.3 vs. 4.2 years). Histological lesions of focal glomerulosclerosis were detected in the posttranplant biopsies of only 3 patients. In conclusion, young patients with nephrotic syndrome associated with focal segmental sclerosis have a similar graft survival with and without recurrence of the nephrotic syndrome.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1007/s004670050499
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  • 4
    Digitale Medien
    Digitale Medien
    Progression to end-stage renal disease in children with posterior urethral valves (1998)
    Springer
    Pediatric nephrology 12 (1998), S. 630-636 
    Details
    ISSN: 1432-198X
    Schlagwort(e): Key words: Posterior urethral valves ; Chronic renal failure ; Obstructive uropathy ; Serum creatinine ; Renal function ; Renal dysplasia/hypoplasia ; Body growth
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract. Diagnostic and therapeutic strategies in boys with congenital posterior urethral valves (PUV) have much improved in past decades, but the impact of these changes on the progression to end-stage renal disease (ESRD) has rarely been investigated. We followed renal function in 20 boys with PUV from diagnosis to ESRD. From the first observation period (1969–1978) to the second period (1979–1992) we found a marked drop in age at diagnosis, at valve resection, at first increase of serum creatinine (SCr), and at onset of ESRD. The progression was analyzed by calculating the slope of 1/SCr and the probability of renal survival. In all patients combined, renal survival at the age of 10 years was 35%. In children undergoing valve resection in the 1st year of life, renal survival was worse than in those undergoing later surgery (15% vs. 65% after 10 years, P=0.006). Patients with a SCr〉1.2 mg/dl before the age of 12 months progressed more rapidly to ESRD than those attaining this level later. The lower the minimum level of SCr observed after initial surgery, the older the patient at the onset of ESRD. The presence of renal dysplasia or hypoplasia, but not of vesicoureteric reflux, was associated with a more rapid progression. Mean body height at ESRD was −2.3±1.3 standard deviation score compared with controls, and was lower if PUV was diagnosed before the age of 6 months.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1007/s004670050517
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  • 5
    Digitale Medien
    Digitale Medien
    Atrial natriuretic peptide and sodium homeostasis in chronic renal failure (1989)
    Springer
    Pediatric nephrology 3 (1989), S. 397-400 
    Details
    ISSN: 1432-198X
    Schlagwort(e): Aldosterone ; Atrial natriuretic peptide ; Chronic renal failure ; Dopamine ; Noradrenaline ; Sodium homeostasis
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract In order to evaluate the possible role of vasoactive hormones in the mechanism of exaggerated sodium loss due to reduced renal mass we measured plasma concentration of atrial natriuretic peptide (ANP), aldosterone, plasma renin activity (PRA), plasma noradrenaline, and dopamine, in 12 children with advanced chronic renal failure (mean CIn17.8-2.6,x± SEM, CPAH93.5±17 ml/min per 1.73 m2, FENa7.0±0.95%). No patient had clinical signs of volume overload. Plasma concentrations of ANP were not significantly different from those of healthy agematched controls (29.2±7.2 vs 23.2±3.1 fmol/ml) and did not correlate with urinary sodium excretion. Plasma concentrations of aldosterone, PRA and noradrenaline, were also within the physiological range, while plasma dopamine levels were elevated (260±36 vs 98±11 pg/ml, 〈0.001). Our data do not support the notion that ANP or the renin-aldosterone axis play a major role in the adaptation of remaining nephrons to maintain long-term sodium balance in normotensive children with chronic renal failure.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1007/BF00850214
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  • 6
    Digitale Medien
    Digitale Medien
    Glucocorticoid receptors in idiopathic nephrotic syndrome (1999)
    Springer
    Pediatric nephrology 13 (1999), S. 653-656 
    Details
    ISSN: 1432-198X
    Schlagwort(e): Key words Nephrotic syndrome ; Glucocorticoid receptors ; Glucocorticoids
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: 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.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1007/s004670050675
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  • 7
    Digitale Medien
    Digitale Medien
    Clinical outcome of Schönlein-Henoch purpura nephritis in children (1999)
    Springer
    Pediatric nephrology 13 (1999), S. 816-823 
    Details
    ISSN: 1432-198X
    Schlagwort(e): Key words Schönlein-Henoch purpura ; Glomerulonephritis ; Hypertension ; Proteinuria ; Renal failure ; Plasma exchange
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: 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.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1007/s004670050707
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  • 8
    Digitale Medien
    Digitale Medien
    Body growth of children with steroid-resistant nephrotic syndrome (1999)
    Springer
    Pediatric nephrology 13 (1999), S. 828-834 
    Details
    ISSN: 1432-198X
    Schlagwort(e): Key words Steroid-resistant nephrotic syndrome ; Growth ; Corticosteroids ; Protein metabolism ; Chronic renal insufficiency
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: 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.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1007/s004670050709
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  • 9
    Digitale Medien
    Digitale Medien
    Oligotyping for HLA-DQA, -DQB, and -DPB in idiopathic nephrotic syndrome (1997)
    Springer
    Pediatric nephrology 11 (1997), S. 291-295 
    Details
    ISSN: 1432-198X
    Schlagwort(e): Key words: Nephrotic syndrome ; HLA associations ; Immunogenetics
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract. Associations of human leukocyte antigens (HLA) with the idiopathic nephrotic syndrome (NS) have mainly been described for alleles of the HLA-DR locus. In the present study the polymorphism of HLA-DQ and -DP at the molecular level was investigated in 167 children with NS (129 steroid-sensitive) using the polymerase chain reaction and sequence-specific oligonucleotides in a French and a German cohort. HLA-DR typing was also performed by classical serology. In steroid-sensitive patients we observed an increased frequency of the alleles HLA-DQA1*0201 and -DQB1*0201 in both populations with relative risks ranging from 3.8 to 8.5 (P b 〈0.01 to P b 〈0.00001 after Bonferoni’s correction). In contrast, the frequency of HLA-DQA1*0102 and DQB1*0602 was significantly decreased. In children with frequent relapses the HLA associations were generally more pronounced than in those with infrequent or no relapses. Applying logistic regression analysis, a nephrotic child bearing DQA1*0201 or DR7 was five times more likely to be in the steroid-sensitive group of patients than in the steroid-resistant group compared with nephrotic children not bearing one of these alleles. These HLA alleles therefore seem to be useful indicators of a steroid-sensitive frequently relapsing course of NS. No associations with DPB alleles were observed, which narrows the region genetically involved in the disease susceptibility to the DR-DQ region. Steroid-resistant NS was not associated with HLA.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1007/s004670050279
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  • 10
    Digitale Medien
    Digitale Medien
    Urinary excretion of urodilatin in healthy children and children with renal disease (1998)
    Springer
    Pediatric nephrology 12 (1998), S. 55-59 
    Details
    ISSN: 1432-198X
    Schlagwort(e): Key words: Urodilatin ; Acute renal failure ; Chronic renal failure ; Congential tubulopathies ; Sodium excretion
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract. Urodilatin (URO) is a natriuretic peptide isolated from human urine which is thought to be produced by distal tubular cells. We measured urinary URO excretion in 50 healthy children and 23 children with acute (ARF), chronic renal failure (CRF), or hereditary tubular disorders, using a specific radioimmunoassay. The mean URO excreted in these four groups was 56, 45, 94, and 121 fmol/min per 1.73 m2, respectively (differences between first three groups not significant). The variation in URO excretion was larger in patients with kidney disease than in controls. There were significant correlations between urinary URO and sodium excretion in controls and CRF, but not in ARF. URO excretion also correlated with urine flow rate in CRF. Although no correlation was found between URO excretion and creatinine clearance, urinary URO was increased in some patients with advanced CRF, which suggests stimulated tubular production to compensate for reduced sodium excretion. In view of the therapeutic potential of URO in renal insufficiency, further study of the renal handling of URO is warranted.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1007/s004670050403
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