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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Pediatric nephrology 14 (2000), S. 713-716 
    ISSN: 1432-198X
    Keywords: Key words Heparin-induced thrombocytopenia ; Hemodialysis ; Chest pain ; Respiratory distress ; Danaparoid
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  We report two pediatric patients with end-stage renal failure who developed heparin-induced thrombocytopenia type II (HIT II) on hemodialysis (HD). Both developed acute respiratory distress and chest pain within 30 min of initiating the 5th HD session. The platelets dropped during HD from 168 to 38×109/l and from 248 to 109×109/l, respectively. Marked clots were observed in the dialyzers. Substitution of heparin with the low molecular weight heparin dalteparin had no effect. Switching from anticoagulation to the heparinoid danaparoid resulted in immediate disappearance of all adverse effects, and further long-term HD was uneventful. HIT II was diagnosed clinically; heparin-induced platelet activation test (HIPA) and serum IgG, IgA, and IgM to heparin-platelet factor 4 complexes (HPF4) were both negative. We conclude that HIT II may occur in children on HD. HIT II is essentially a clinical diagnosis, as HIPA and antibodies to HPF4 are not always positive. Once HIT II is suspected, heparin (and low-molecular-weight heparins) should be stopped immediately. Long-term anticoagulation with danaparoid is a valuable option for patients on HD.
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Pediatric nephrology 7 (1993), S. 769-770 
    ISSN: 1432-198X
    Keywords: Pregnancy ; Angiotensin converting enzyme inhibitors ; Neonatal anuria
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Pediatric nephrology 8 (1994), S. 522-524 
    ISSN: 1432-198X
    Keywords: Developing countries ; Paediatric nephrology
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract This paper summarises a symposium concerned with the provision of care for children with kidney disease in developing countries. Better organisation of services is required to prevent waste of resources, with the emphasis on team work between professionals, shared care with local health care personnel remote from the paediatric nephrology unit and good communications. Families need to be educated and provided with appropriate information so that they can care for their child at home. Technology should be simple and robust and the staff using it should be fully trained to maintain it in use. Therapies should be definitive where possible, because long-term supervision of treatment is often difficult. Effective but inexpensive medications should be used where possible. Twinning of developing and richer countries is valuable to transfer technology, help with training and assist in care through the development of personal contacts.
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  • 4
    ISSN: 1432-1440
    Keywords: Essential hypertension ; Renin angiotensin aldosterone system ; Bodyweight ; Blood pressure in childhood ; Essentielle Hypertonie ; Renin-Angiotensin-Aldosteron-System ; Körpergewicht ; Blut-druck beim Kind
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung In der vorliegenden Untersuchung wurden 294 Nachkommen (5–34 Jahre) essentieller Hypertoniker bezüglich Blutdruck, Renin-Angiotensin-Aldosteron-System and anderer kardiovaskulärer Risikofaktoren wie Glukose, Cholesterin und Triglyzeride untersucht und mit 122 Nachkommen (5–34 Jahre) normotoner Eltern verglichen. Dabei zeigte sich, daß Nachkommen essentieller Hypertoniker im Mittel statistisch signifikant höhere systolische und diastolische Blutdruckwerte zeigten als solche normotoner Eltern (p〈0,003 bzw. 0,005). Weiter ließ sich bei Kindern hypertoner Eltern im Mittel ein signifikant höheres Körpergewicht und ein größerer Body-Mass-Index beobachten als im Kontrollkollektiv (p〈0,006 bzw. 0,001). Mit Ausnahme statistisch signifikant niedrigerer Ruhe-Plasma-Aldosteronwerte (p〈0,002) bei Kindern essentieller Hypertoniker ließen sich zwischen beiden Kollektiven keine Unterschiede im Stimulationsaldosteron, in der Ruhe- und Stimulations-Plasma-Renin-Aktivität und im Plasmacortisol nachweisen. Ebenfalls zeigten die beiden Kollektive bezüglich der anderen untersuchten Parametern wie Kreatinin, Glukose, Cholesterin und Triglyzeride keine wesentlichen Unterschiede. Weiterhin wurden in der vorliegenden Untersuchung 41 hypertone Elternteile, 65 (normotone) Ehepartner von Hypertonikern und 47 (normotone) Eltern von Kontrollkindern untersucht. Erwarungsgemäß zeigten Hypertoniker statistisch signifikant höhere Blutdruckwerte als ihre Ehepartner und die Kontrolleltern (p〈0,001). Interessanterweise zeigten die hypertonen Eltern nicht nur einen höheren Body-Mass-Index als Kontrolleltern sondeern auch als Ehepartner (p〈0,01 bzw. 〈0,02). Diese Befunde stützen eine vorwiegend genetische Disposition als zugrundeliegende Ursache des höheren Körpergewichtsverhaltens und lassen eine vermehrte Nahrungsmittelaufnahme in Hypertonikerfamilien eher als unwahrscheinlich erscheinen. Die Ergebnisse der vorliegenden Studie legen es nahe, daß durch eine möglichst frühzeitige Kontrolle des Körpergewichtsverhaltens bei Nachkommen essentieller Hypertoniker ein Beitrag zur Primärprävention der Hochdruckkrankheit geleistet werden kann.
    Notes: Summary In the present study, blood pressure, the renin angiotensin aldosterone system, and other cardiovascular risk factors, such as glucose, cholesterine, and triglycerides, were investigated in 294 offspring of essential hypertensives (5–34 years) and in 122 children of normotensive parents (5–34 years). Offspring of essential hypertensives showed statistically significant higher systolic and diastolic blood pressure values than those of normotensive parents (p〈0.003, 〈0.005, respectively). Furthermore, in children of hypertensive parents a statistically significant higher body weight and body mass index than in controls could be observed (p〈0.006, 〈0.001, respectively). With the exception of statistically significant, lower mean supine plasma aldosterone values (p〈0.02) in children of hypertensive parents, no major differences between the two groups were seen in stimulated aldosterone, supine and stimulated plasma renin activity, and plasma cortisol. Furthermore, in the present study, 41 hypertensive parents, 65 (normotensive) spouses of hypertensives, and 47 (normotensive) parents of control children were investigated. As expected, hypertensive parents showed statistically significant higher blood pressure values than parents of control children and their spouses (p〈0.001). Interestingly, hypertensive parents had not only a higher body mass index than control parents but also than their spouses (p〈0.01 and 〈0.02, respectively). These findings support a genetic disposition as being the underlying cause of higher body weight in hypertensives and make it less probable that a higher food intake in hypertensive families is responsible for this phenomenon. The results of the present study indicate that early body weight control in children of hypertensive parents may be an important contribution to the prevention of hypertension.
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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 151 (1992), S. 775-778 
    ISSN: 1432-1076
    Keywords: Nephrotic syndrome ; Steroid-dependent idiopathic nephrotic syndrome ; Cyclosporin A ; Children
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Therapy of steroid-dependent idiopathic nephrotic syndrome is often unsatisfactory. Since 1986 we have treated nine children (six male and three female), aged 3–16 years, with cyclosporin A (CsA) during 2.0–5.2 (median 3.1) years. All had minimal change disease on renal biopsy and had previously received cyclophosphamide. Mean daily dosage of CsA was 4.1 mg/kg (range 2.7–5.8) and mean whole blood trough level was 220ng/ml (range 141–271). The relapse rate decreased from 3.4/patient year before CsA to 0.55 on CsA. Discontinuation of CsA or reduction below 2 mg/kg daily was always followed by a relapse. The overall relapse rate, including the period with very low-dose CsA, was 0.95/patient year. Four patients required additional low-dose alternate-day prednisone. Repeat renal biopsy showed minimal change disease in eight patients and focal segmental glomerulosclerosis in one; CsA-toxicity was mild in two and moderate in one. The latter was the only patient with slightly reduced glomerular filtration rate. Two boys with delayed puberty spontaneously matured and reached expected final height. We conclude that long-term low-dose CsA is very effective and steroid-sparing. Its use is justified in selected patients, particularly in those with numerous relapses and in male patients before and during puberty, as long as renal function and CsA-toxicity are carefully monitored.
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  • 6
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 150 (1991), S. 509-512 
    ISSN: 1432-1076
    Keywords: Renal anaemia ; Human recominant erythropoietin ; Epoetin alfa ; Renal hypertension ; Hyperkalaemia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Eighteen patients aged 5–18 years on regular dialysis had a packed cell volume (PCV)〈0.27. On treatment with epoetin alfa (EA) PCV increased by 0.05 or more in all patients. Iron supplementation was necessary in 13 patients with a ferritinaemia 〈300 μg/l before study. During treatment, plasma potassium increased significantly and more vigorous antihypertensive measures were required in 8 patients, 5 of them being already on antihypertensive drugs before EA. Iliofemoral thrombosis occurred in 1 patient 10 days after renal transplant. The data indicate that EA ameliorates the anaemia of chronic renal disease. The main concerns arising during treatment with EA are hyperkalaemia, arterial hypertension and possibly thrombosis.
    Type of Medium: Electronic Resource
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  • 7
    ISSN: 1432-1076
    Keywords: Key words Tubulo-interstitial nephropathy ; Cholestatic liver disease ; Primary sclerosing cholangitis ; Familial
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We describe two siblings (female and male) with progressive tubulo-interstitial nephropathy and cholestatic liver disease. The main characteristics were progressive renal failure and elevated liver enzymes (AST, ALT and γ-GT). Dialysis was started at the age of 1.9 and 6.5 years, respectively. Renal histology disclosed sclerosed glomeruli and atrophic tubules; the interstitium was fibrotic and infiltrated by lymphocytes. Endoscopic retrograde cholangiopancreatography revealed segmental irregularities and narrowing of the intrahepatic bile ducts, consistent with early primary sclerosing cholangitis. Liver histology showed enlarged portal triads, mild proliferation and inflammation of bile ducts, and fibrosis. At 5.9 years the girl underwent a successful renal transplantation whereas the boy is still on dialysis. Conclusion The association of progressive tubulo-interstitial nephropathy and cholestatic liver disease, consistent with early primary sclerosing cholangitis, constitutes a distinct autosomal recessive entity.
    Type of Medium: Electronic Resource
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  • 8
    ISSN: 1432-1076
    Keywords: Key words Renal-hepatic-pancreatic dysplasia ; Congenital ; hepatic fibrosis ; Liver cirrhosis ; Combined liver-kidney ; transplantation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We report two brothers with renal dysplasia and congenital hepatic fibrosis. One patient died shortly after birth of lung hypoplasia. The second developed end-stage renal failure at 14 months. The hepatic fibrosis progressed to cirrhosis and hepatic failure. Pancreatic function was normal, but increased echogenicity was seen on ultrasound. At age 3 years and 9 months a successful combined liver-kidney transplantation was performed. The features of our patients are compatible with the “renal-hepatic-pancreatic dysplasia” syndrome. Conclusion Renal-hepatic-pancreatic dysplasia is an autosomal recessive disorder with variable expression. Combined liver-kidney transplantation offers a new therapeutic option.
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  • 9
    Electronic Resource
    Electronic Resource
    Springer
    Intensive care medicine 17 (1991), S. 311-312 
    ISSN: 1432-1238
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 10
    ISSN: 1432-1076
    Keywords: Renal-hepaticpancreatic dysplasia ; Congenital hepatic fibrosis ; Liver cirrhosis ; Combined liver-kidney transplantation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Abstract We report two brothers with renal dysplasia and congenital hepatic fibrosis. One patient died shortly after birth of lung hypoplasia. The second developed end-stage renal failure at 14 months. The hepatic fibrosis progressed to cirrhosis and hepatic failure. Pancreatic function was normal, but increased echogenicity was seen on ultrasound. At age 3 years and 9 months a successful combined liver-kidney transplantation was performed. The features of our patients are compatible with the “renal-hepatic-pancreatic dysplasia” syndrome. Conclusion Renal-hepatic-pancreatic dysplasia is an autosomal recessive disorder with variable expression. Combined liver-kidney transplantation offers a new therapeutic option.
    Type of Medium: Electronic Resource
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