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  • 1
    Electronic Resource
    Electronic Resource
    Anti-glomerular basement membrane antibody-mediated glomerulonephritis due to glue sniffing (1987)
    Springer
    European journal of pediatrics 146 (1987), S. 296-300 
    Details
    ISSN: 1432-1076
    Keywords: Glomerulonephritis, antiglomerular basement membrane antibody-mediated ; Renal failure ; Hydrocarbons ; Sniffing ; Plasmapheresis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A 16-year-old girl developed rapidly progressive glomerulonephritis and renal failure. The disease was associated with high titres of antiglomerular basement membrane antibodies in serum, linear deposits of immunoglobulin G and diffuse epithelial crescents on renal biopsy. Past history revealed heavy smoking and deliberate sniffing of Pattex glue, a mixture of hydrocarbons which possibly may affect the structure of glomerular basement membrane. After treatment by repeated plasmapheresis and drug immunosuppression autoantibodies disappeared from serum but renal function was not influenced. Renal damage is a potential hazard for glue sniffing adolescents.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00716479
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  • 2
    Electronic Resource
    Electronic Resource
    Primary hyperoxaluria type 2 (1997)
    Springer
    European journal of pediatrics 156 (1997), S. 509-512 
    Details
    ISSN: 1432-1076
    Keywords: Key words Nephrocalcinosis ; Primary hyperoxaluria type 1 ; Primary hyperoxaluria type 2 ; Urolithiasis ; Chronic renal failure
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Primary hyperoxaluria type 2 (PH2) is a rare disease with only 24 patients reported in the literature so far. It should be considered in any patient presenting with urolithiasis or nephrocalcinosis due to hyperoxaluria. The metabolic defect is deficiency of d-glycerate dehydrogenase/glyoxylate reductase leading to characteristic hyperoxaluria and excretion of l-glycerate, the cornerstone of diagnosis of PH 2. Although development of terminal renal failure seems to be less prevalent than in PH 1, recent reports indicate that chronic as well as terminal renal insufficiency may occur. Therefore specific therapeutic measures should aim at reduction of urinary calcium oxalate saturation by potassium citrate or pyrophosphate to reduce the incidence of nephrolithiasis and nephrocalcinosis and thus improve renal survival. Secondary complications (obstruction, urinary tract infections and pyelonephritis) must be avoided. In patients with terminal renal failure isolated renal transplantation seems to carry a high risk of disease recurrence. Conclusion PH 2 is a rare but important cause of urolithiasis and nephrocalcinosis; long-term follow up is necessary, since the renal prognosis may be worse than previously anticipated.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004310050649
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  • 3
    Electronic Resource
    Electronic Resource
    Effective treatment of acute hyperkalaemia in childhood by short-term infusion of salbutamol (1996)
    Springer
    European journal of pediatrics 155 (1996), S. 495-497 
    Details
    ISSN: 1432-1076
    Keywords: Hyperkalaemia ; Renal failure ; Salbutamol ; Children
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Hyperkalaemia is a lifethreatening emergency and infusion of glucose with insulin has so far been regarded as the standard treatment of choice. Recently the β-2 stimulatory drug salbutamol has been shown to be an effective agent to treat hyperkalaemia by inducing a shift of potassium into the intracellular compartment. We treated 15 children aged 0.1–14 (mean 5.2) years suffering from acute hyperkalaemia (mean level 6.6±0.54, range 5.9–7.7 mmol/l) with a single infusion of salbutamol (5 μg/kg over 15 min). Serum potassium concentrations decreased significantly within 30 min to levels of 5.74±0.53 and 4.92±0.53 mmol/l after 120 min (P〈0.001, respectively). No side-effects occurred other than a slight increase in heart rate in 3 patients. Conclusion A single intravenous infusion of salbutamol at a dose of 5 μg/kg is a highly effective treatment for hyperkalaemia with minimal clinical side-effects. The effect lasts for at least 120 min and may reverse hyperkalaemia in some patients without further interventions so that salbutamol seems justified as the first choice treatment for this condition in childhood.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01955188
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  • 4
    Electronic Resource
    Electronic Resource
    The role of hyperparathyroidism in the pathogenesis of renal anemia (1983)
    Springer
    European journal of pediatrics 141 (1983), S. 63-65 
    Details
    ISSN: 1432-1076
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00445676
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  • 5
    Electronic Resource
    Electronic Resource
    Changning pattern of chronic renal failure and renal replacement therapy in children and adolescents: a 20-year single centre study (1993)
    Springer
    European journal of pediatrics 152 (1993), S. 166-171 
    Details
    ISSN: 1432-1076
    Keywords: Chronic renal failure ; Haemodialysis ; Continuous ambulatory peritoneal dialysis ; Renal transplantation ; Survival
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We analysed the demographic data, clinical course and survival on different forms of renal replacement therapy (RRT) of 374 children and adolescents with chronic renal failure observed between 1969 and 1988 and compared the findings for the four subsequent 5-year periods. The proportion of children below 5 years of age rose from 21% to 47%. With time the incidence of glomerulonephritis increased and that of pyelonephritis decreased. As RRT became more common, more very young children and more adolescents were admitted to the study. In the last 5 years continuous ambulatory peritoncal dialysis (CAPD) and haemodialysis (HD) were performed to the same extent as the initial form of RRT. The time a subject had to wait for a first transplant decreased from 36 to 21 months. Between 1969 and 1988 overall survival on any form of RRT increased to 77% after 10 years of therapy. In the last observation period 2-year patient survival was 100% both on HD and CAPD. First cadaver graft survival after 4 years improved from 25% in 1969–1973 to 69% in 1984–1988.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02072498
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  • 6
    Electronic Resource
    Electronic Resource
    Erythrocyte organic phosphates in the anemia of renal failure in childhood (1978)
    Springer
    European journal of pediatrics 128 (1978), S. 103-111 
    Details
    ISSN: 1432-1076
    Keywords: 2,3-diphosphoglycerate ; Adenosinetriphosphate ; Chronic renal failure ; Hemodialysis ; Anemia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Erythrocyte 2,3-diphosphoglycerate (2,3-DPG) and adenosinetriphosphate (ATP) levels were determined in 43 children with chronic renal failure on conservative treatment (CT), and 12 children on regular hemodialysis (HD) immediately before and after a HD session. The results were compared to non-anemic and anemic controls. In spite of anemia, erythrocyte 2,3-DPG in renal failure was similar to non-anemic controls at normal blood pH, but rose during dialysis as a result of alkalosis. In contrast, ATP levels were high already at a normal blood pH. 2,3-DPG correlated with packed cell volume (PCV) in children with renal failure but at lower concentrations compared to controls. Both organic phosphates in the erythrocytes showed a significant correlation with blood pH. The poor increase of 2,3-DPG, in combination with elevated ATP levels, suggests uremia-induced inhibition of 2,3-DPG synthesis.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00496995
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  • 7
    Electronic Resource
    Electronic Resource
    Autosomal recessive and dominant forms of polycystic kidney disease are not allelic (1987)
    Springer
    Human genetics 〈Berlin〉 77 (1987), S. 221-222 
    Details
    ISSN: 1432-1203
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Summary Linkage analysis has been carried out in 11 kindreds with autosomal recessive polycystic kidney disease (ARPKD) using the genetic marker 3'HVR, closely linked ( $$\hat \theta = 0.05$$ ) to the gene of the autosomal dominant type. Close linkage ( $$\hat \theta \leqq 0.20$$ ) between the locus of the marker and that of ARPKD can be excluded. These data strongly suggest that the loci for the autosomal recessive and dominant forms of polycystic kidney disease are not allelic.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00284473
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  • 8
    Electronic Resource
    Electronic Resource
    IgG2 deficiency in uremic children is not restricted to peritoneal dialysis treatment (1997)
    Springer
    Pediatric nephrology 11 (1997), S. 684-686 
    Details
    ISSN: 1432-198X
    Keywords: Key words:  Chronic renal failure ; IgG subclasses ; Immunoglobulins ; Peritoneal dialysis ; Peritonitis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract.   To date there are no data concerning IgG subclasses in children with preterminal chronic renal failure (CRF), although a reduction of total serum IgG, including its major subclasses IgG1 and IgG2, has been demonstrated in patients on peritoneal dialysis (PD). Therefore we studied total IgG, IgA, IgM, and IgG subclasses in preterminal CRF (n = 25), PD (n = 22) patients, and 13 age-matched healthy children and also compared results with age-related normal values previously established in 226 healthy children. While total IgG, IgA, IgM, and IgG 1 were comparable, there was a significant deficiency of IgG2 in children both with preterminal CRF and on PD compared with controls. Moreover, the prevalence of IgG2 deficiency compared with age-related normal values was significantly increased not only in PD but also in preterminal CRF patients. We conclude that there are alterations in serum IgG subclasses in children with CRF, with IgG2 deficiency not restricted to PD, but being present in the preterminal state as well.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004670050365
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  • 9
    Electronic Resource
    Electronic Resource
    Nephrocalcinosis in a patient with primary hyperoxaluria type 2 (1996)
    Springer
    Pediatric nephrology 10 (1996), S. 442-444 
    Details
    ISSN: 1432-198X
    Keywords: Key words: Nephrocalcinosis ; Primary hyperoxaluria type 1 ; Primary hyperoxaluria type 2 ; Urolithiasis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract. Although nephrocalcinosis is a classical finding in primary hyperoxaluria type 1 (PH 1) associated with a poor renal survival it is exceptional in patients with PH type 2 (PH 2) , characterized by a more favorable outcome . We describe an 8-month-old girl who suffered from recurrent urinary tract infections. Imaging studies revealed a profound corticomedullary nephrocalcinosis with no evidence of calculi. Urinary oxalate and D-glycerate excretion were massively elevated, while urinary glycolate or glyoxylate could not be detected, confirming the diagnosis of PH 2. Although the nephrocalcinosis progressed radiologically, renal function remained stable for over 2 years. Only further follow-up will show whether the associated nephrocalcinosis worsens the prognosis of our patient and of PH 2 in general.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004670050135
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  • 10
    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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