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  • 1
    Electronic Resource
    Electronic Resource
    Severe atherosclerosis of the aorta and development of peripheral T-cell lymphoma in an adolescent with angiolymphoid hyperplasia with eosinophilia (2005)
    Oxford, UK : Blackwell Science Ltd
    British journal of dermatology 152 (2005), S. 0 
    Details
    ISSN: 1365-2133
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: We report an adolescent girl with a history of angiolymphoid hyperplasia with eosinophilia (ALHE) diagnosed at the age of 10 years. The patient also suffered from chronic persistent multiresistant herpes simplex virus infection. Atherosclerotic occlusive disease of the abdominal aorta and its major branches was observed at the age of 17 years, necessitating vascular surgical intervention 1 year later because of disease progression. Histological examination of the aorta disclosed widespread atherosclerosis and high levels of gene expression of both T-helper cell type (Th) 1- and Th2-derived cytokines. This suggests that a highly stimulated systemic immune response including increased production of both Th1- and Th2-derived cytokines such as interferon-γ and interleukin-4 may result in severe atherosclerotic lesions at a very young age. In addition, the patient developed a peripheral T-cell lymphoma at the age of 18 years. Neither systemic atherosclerosis nor T-cell lymphoma has been reported in association with ALHE. It is suggested that a highly stimulated dysfunctional immune response may play a key role in persistent inflammatory disease and premature development of atherosclerosis as well as malignant transformation of T cells.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1365-2133.2005.06421.x
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  • 2
    Electronic Resource
    Electronic Resource
    Chemotherapy with cytosine arabinoside in a child with Burkitt's lymphoma on maintenance hemodialysis and hemofiltration (1993)
    Springer
    Annals of hematology 67 (1993), S. 37-39 
    Details
    ISSN: 1432-0584
    Keywords: Hemofiltration ; Hemodialysis ; Cytosine arabinoside
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A case of Burkitt's lymphoma (stage IV) in an 8-year-old boy with end-stage renal failure due to hemolytic uremic syndrom is reported. The boy was treated by maintenance hemodialysis (HD) and hemofiltration (HF). During chemotherapy treatment with continuous cytosine arabinoside (Ara-C) infusion (100 mg/m2/d) for 7 days, concentrations of Ara-C and its metabolite uracil arabinoside (Ara-U) were measured in blood, dialysate, and filtrate. Ara-C levels were always below 200 ng/ml and were only qualitatively detectable in blood, dialysate, and filtrate. Ara-U levels were higher than 200 ng/ml after 18 h treatment and were measured quantitatively. Ara-U clearance during 3 h HD was 92 ml/min and the calculated mass removal 14.7 mg/3 h. In contrast, the Ara-U clearance during 3 h HF was 14 ml/min and the mass removal was 6.7 mg/3 h. Ara-C and Ara-U are eliminated by HD and HF in anuric patients. A continuous infusion of 100 mg Ara-C m2/d during HD or HF treatment did not result in a serum concentration above 200 ng/ml.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01709664
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  • 3
    Electronic Resource
    Electronic Resource
    Verbesserung der Nierenfunktion nach langjähriger präterminaler Niereninsuffizienz bei infravesikaler Obstruktion Fallbericht (2000)
    Springer
    Monatsschrift Kinderheilkunde 148 (2000), S. 479-481 
    Details
    ISSN: 1433-0474
    Keywords: Schlüsselwörter Infravesikale Obstruktion ; Posteriore Urethralklappe ; Niereninsuffizienz ; Nierenfunktion ; Key words Lower urinary-tract obstruction ; Posterior urethral valve ; Renal insufficiency ; Renal function
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary Background: In early childhood in the majority of cases due to posterior urethral valves (PUV), lower urinary tract obstructions, in early childhood lead to high pressure and damage to the upper urinary tract. In spite of early urological intervention 25–60% of the boys with PUV develop chronic renal insufficiency. Complete restitution of renal function is very uncommon. Case report: We report a case of a boy now 13 years old, in whom a urethral stricture was diagnosed only at the age of 7 years as the cause of chronic preterminal renal insufficiency. Six years after urethromtomia interna the boy has no complaints, good renal function, no voiding dysfunction and his height approaches the 25th percentile. Discussion: Recovery of renal function usually requires early relief of the obstruction and decompression of the upper urinary tract. However, this case report shows impressively that in spite of long-term preterminal renal insufficiency, restitution can be observed after treatment of bladder outlet obstruction.
    Notes: Zusammenfassung Hintergrund: Hochgradige infravesikale Obstruktionen, im frühen Kindesalter meist durch Urethralklappen hervorgerufen, induzieren eine sekundäre Druckbelastung der oberen Harnwege. Trotz einer in aller Regel frühzeitigen urologischen Intervention entwickeln 25–60% der Jungen mit Rethralklappen eine chronische Niereninsuffizienz. Nur selten wird eine vollständige Restitution der Nierenfunktion beobachtet. Kasuistik: Wir berichten über einen jetzt 13-jährigen Jungen, bei dem erst im Alter von 7 Jahren im Rahmen der Abklärung einer chronischen präterminalen Niereninsuffizienz eine bulbäre Harnröhrenstriktur diagnostiziert wurde. 6 Jahre nach einer Urethrotomia interna ist der Junge beschwerdefrei mit einer guten Nierenfunktion, einer guten Blasenfunktion und einem Längenwachstum entlang der 25. Perzentile. Diskussion: Die frühzeitige Druckentlastung der oberen Harnwege ist Voraussetzung für eine mögliche Erholung der Nierenfunktion. Die Kasuistik zeigt jedoch eindrucksvoll, dass selbst bei einer jahrelangen präterminalen Niereninsuffizienz nach Beseitigung der ursächlichen infravesikalen Obstruktion noch eine Restitution auftreten kann.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s001120050579
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  • 4
    Electronic Resource
    Electronic Resource
    Intravenous iron treatment of renal anemia in children on hemodialysis (1999)
    Springer
    Pediatric nephrology 13 (1999), S. 580-582 
    Details
    ISSN: 1432-198X
    Keywords: Key words Intravenous iron ; Hemodialysis ; Renal anemia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  Treatment of anemia in children with end-stage renal disease (ESRD) has been greatly facilitated by the introduction of recombinant human erythropoietin (rHuEPO). A major limiting factor in the treatment of renal anemia is sufficient iron supplementation. Eight children (aged 10–17 years) receiving hemodialysis were treated with intravenous iron (1 mg/kg per week) for 3 months. Hemoglobin (Hb), hematocrit (Hct), and serum ferritin levels were measured regularly. The mean Hct increased from 25% to 30%, the mean Hb increased from 7.8 g/dl to 9.2 g/dl, and the mean ferritin level from 200 to 395 mg/dl. The mean EPO dosage could be tapered from 6,500 IU to 6,150 IU. No adverse side-effects were noted. Hence, in this uncontrolled study intravenous iron was an effective treatment for iron deficiency during rHuEPO therapy in children with ESRD on hemodialysis.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004670050747
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  • 5
    Electronic Resource
    Electronic Resource
    A stepwise approach to the treatment of early onset nephrotic syndrome (2000)
    Springer
    Pediatric nephrology 14 (2000), S. 1077-1082 
    Details
    ISSN: 1432-198X
    Keywords: Key words Early onset nephrotic syndrome ; Captopril ; Indomethacin ; Growth and development
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  We have retrospectively reviewed our single-center experience of the treatment of early onset nephrotic syndrome (NS). From 1991 to 1998, ten children with NS were treated. Kidney biopsy showed focal sclerosis (n=1), diffuse mesangial sclerosis (n=7), and congenital NS of the Finnish type (n=2). Associated conditions included incomplete Drash syndrome (n=1), Galloway-Mowat syndrome (n=1), and severe mental and motor retardation of unknown origin (n=3). From 1991 to 1997, five children with NS were treated. Bilateral nephrectomy (NX) was performed in three, one patient with severe retardation died at 4 years and NX was not performed in one patient who showed satisfactory growth and development. Three of these children were dialyzed and two were successfully transplanted. One patient was transplanted without previous dialysis. From 1997 to 1998, five children were treated with a regimen that included captopril and indomethacin (CAPTO/INDO). CAPTO/ INDO was successful in increasing serum protein in all patients and producing growth and development in four patients. In two patients CAPTO/INDO was successful only after unilateral NX. Our experience indicates that CAPTO/INDO may be a valuable treatment in patients with early onset NS. An individualized stepwise approach including unilateral NX should be considered to achieve optimal results.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004670000386
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  • 6
    Electronic Resource
    Electronic Resource
    Levamisole treatment in steroid-sensitive and steroid-resistant nephrotic syndrome (1998)
    Springer
    Pediatric nephrology 12 (1998), S. 459-462 
    Details
    ISSN: 1432-198X
    Keywords: Key words: Levamisole ; Steroid-sensitive nephrotic syndrome ; Steroid-resistant nephrotic syndrome
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract. Since 1992 we have treated 11 children with frequently relapsing steroid-sensitive (n=6) or steroid-resistant (n=5) nephrotic syndrome with levamisole. All had been non-responsive to other immunosuppressive medication before levamisole treatment. All steroid-sensitive patients had signs of steroid toxicity. At least 1 kidney biopsy had been performed prior to study in each patient. Five children had minimal glomerular changes and the other 6 focal segmental glomerular sclerosis. The patients were treated with levamisole (2.5 mg/kg per 48 h) for at least 2 months (up to 18 months, median 10 months). Two patients had additional immunosuppression (cyclosporine A) during levamisole treatment. All patients with steroid-sensitive nephrotic syndrome became free of proteinuria within 2 months and have remained in remission after discontinuation of levamisole (follow-up time 8–50 months, median 24 months). None of the children with steroid-resistant nephrotic syndrome experienced a remission. Side effects were observed in 2 patients and included a granulocytopenia and a severe psoriasis-like cutaneous reaction; both were reversible after discontinuation of levamisole. We conclude that levamisole is of benefit in steroid-sensitive nephrotic syndrome but not in steroid-resistant nephrotic syndrome.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004670050487
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  • 7
    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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  • 8
    Electronic Resource
    Electronic Resource
    Renale tubuläre Dysgenesie mit fetaler Nierenvenenthrombose (1995)
    Springer
    Der Pathologe 16 (1995), S. 143-147 
    Details
    ISSN: 1432-1963
    Keywords: Schlüsselwörter Renale tubuläre Dysgenesie ; Autosomal-rezessiv ; Oligohydramnion ; Potter-Sequenz ; Frühfetale Nierenvenenthrombose ; Key words Renal tubular dysgenesis ; Autosomal recessive disorder ; Oligohydramnios ; Potter sequence ; Fetal renal vein thrombosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary Renal tubular dysgenesis is a rarely recognized condition characterized by oligohydramnios, Potter's sequence and congenital anuria leading to stillbirth or neonatal death from respiratory failure. It is thought to be inherited in an autosomal recessive manner. Definitive diagnosis is based on renal histology, revealing the lack of proximal tubule differentiation. Two additional cases of affected sibs in a family with parental consanguinity are reported. Lectin and immunohistochemical studies confirmed structural and functional immaturity of the proximal tubule. Further findings include bilateral renal vein thrombosis. The clinical and morphological parameters defining this disorder and the possible mechanisms of pathogenesis are discussed.
    Notes: Zusammenfassung Die renale tubuläre Dysgenesie ist ein selten diagnostiziertes, autosomal-rezessiv vererbtes Krankheitsbild, das nach Entwicklung eines Oligohydramnion mit Potter-Sequenz zur Totgeburt führt oder infolge kongenitaler Niereninsuffizienz und Lungenhypoplasie einen letalen Ausgang nimmt. Die Diagnose basiert im wesentlichen auf dem charakteristischen histologischen Befund in der Niere, der eine Differenzierungsstörung des tubulären Systems, insbesondere des proximalen Tubulus aufdeckt. In Ergänzung zu den bisher publizierten Kasuistiken werden 2 Geschwister einer weiteren Familie mit blutsverwandten Eltern beschrieben. Lektin- und immunhistochemische Untersuchungen bestätigen die Annahme einer strukturellen und funktionellen Unreife des proximalen Tubulus bei generalisierter Differenzierungsstörung im tubulären System. Zusätzliche Befunde umfassen u. a. eine ausgedehnte ältere Nierenvenenthrombose beidseits mit fraglicher pathogenetischer Relevanz. Das Spektrum der klinischen und morphologischen Befunde dieser abnormen Entwicklung der Niere und die Vorstellungen zur Pathogenese werden diskutiert.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s002920050087
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  • 9
    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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  • 10
    Electronic Resource
    Electronic Resource
    Hyperlipidemia in pediatric patients undergoing peritoneal dialysis (1988)
    Springer
    Pediatric nephrology 2 (1988), S. 447-452 
    Details
    ISSN: 1432-198X
    Keywords: Hyperlipidemia ; Triglycerides ; Cholesterol ; Peritoneal dialysis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We evaluated serial measurements of serum lipid levels in 68 patients aged 12.6±4.7 years undergoing treatment with continuous ambulatory peritoneal dialysis/continuous cycling peritoneal dialysis (CAPD/CCPD). Fasting mean levels of triglycerides (TG) and cholesterol (C) were elevated above the 95th percentile of published normal values by 102% and 19%, respectively, at the start of dialysis. Except for a shortterm decrease in TG levels at 6 and 9 months, no significant change in mean lipid levels was observed during a follow-up period of 2 years. At initiation of dialysis, elevated TG and C levels were present in 90% and 69% of the patients, respectively. The prevalence of hyperlipidemia (HL) varied between 63% and 88% (TG) and 61% and 93% (C), respectively, during the follow-up period. TG and C levels were not correlated with caloric intake (evaluated in 17 patients), serum albumin levels, treatment modality (CAPD or CCPD), a history of the nephrotic syndrome, or previous treatment with hemodialysis or transplantation. However, a significant inverse correlation was observed between age and serum lipids at the initiation of dialysis treatment and after 1 year (TG:r=−0.40; C:r=−0.44). Our data indicate a high prevalence of HL but no significant change of serum lipid levels during 2 years of treatment with CAPD/CCPD.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00853440
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