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  • 1
    ISSN: 1432-1076
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-1076
    Keywords: Interstitial nephritis ; Polyuric renal failure ; Renal tubular dysfunction ; Uveitis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Three children aged 11 to 14 years with acute interstitial nephritis (AIN) are presented. In one patient AIN developed following antibiotic treatment with trimethoprim/sulfamethoxazole (TMP/SMX). In two patients no infection, drug, or toxin could be implicated. Severe polyuric renal failure without elevation of blood pressure was the predominant clinical feature. Uveitis occurred either simultaneously with the nephritic symptoms or several weeks after complete recovery of renal function. Renal functions were characteristically altered and led to suspicion of AIN even prior to renal biopsy. Renal plasma flow was relatively more reduced than glomerular filtration rate (GFR) with an accordingly increased filtration fraction. Quantitative evaluation of selective tubular functions revealed significant transport deficiencies for glucose, amino acids, inorganic phosphate and low molecular weight proteins. In two patients GFR increased rapidly following initiation of steroid treatment and tubular symptoms simultaneously disappeared. In one patient spontaneous remission occurred. We conclude that—in contrast to adults—the prognosis of AIN in childhood is favorable. Although general clinical features are rather nonspecific, symptoms of decreased tubular reabsorption ability provide a good indication of the diagnosis and may contribute to enhanced recognition of this disease.
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  • 3
    ISSN: 1432-1076
    Keywords: Renal transplantation ; Children ; Cyclosporin A ; Azathioprine ; Hypomagnesaemia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We investigated the renal handling of magnesium in 12 transplanted children under cyclosporin A treatment during the early period after transplantation. We also studied 30 children treated with cyclosporin A 2 years after renal transplantation and compared the results with those of 22 children treated with azathioprine and prednisolone 2–4 years after transplantation. Twenty-two children with chronic renal failure and 10 healthy children served as controls. During the 1st week after transplantation, the mean serum magnesium level dropped to 0.54±0.14 mmol/l and was accompanied by a high fractional magnesium clearance. During this period two patients showed generalized convulsions. After magnesium substitution, serum levels increased gradually and normalized 4 months later. Fractional magnesium clearance remained slightly elevated due to lower glomerular filtration rates. One to four years after transplantation there were no statistically significant differences in serum levels, clearance, excretion and fractional clearance of magnesium between patients treated with cyclosporin A and those receiving azathioprine. There was a linear regression between magnesium clearance and creatinine clearance and an inverse exponential correlation between fractional magnesium clearance and creatinine clearance. We conclude that cyclosporin A treatment after transplantation has no major effect on the renal handling of magnesium in kidney transplants. The observed changes in serum magnesium levels and fractional clearance are probably due to tubular cell damage early after transplantation and to high doses of furosemide.
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  • 4
    ISSN: 1432-1076
    Keywords: Key words Kidney transplantation ; Graft survival ; Infant ; Children preschool
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Between 1975 and 1994, 46 children under 6 years of age received a total of 52 renal transplants. Obstructive uropathy and dysplasia accounted for most causes of terminal renal failure (17 and 12 cases respectively). Four patients required a second, 1 patient a third transplantation. Cadaveric organs were used on 33 occasions; 19 patients received a living-related donor kidney. Immunosuppression was performed with azathioprine in 5, with cyclosporine A in 21 and combined azathioprine/cyclosporine therapy in 20 cases. After 1 year, graft survival was 81%, and after 5 years 78%. Creatinine clearance declined slightly between 1 and 5 years from 69 to 56 ml/min per 1.73 m2. Main causes of graft failure were thrombotic complications in 6 cases and death with functioning graft in 5 cases. Graft thrombosis occurred only in grafts from young donors under the age of 7 years and after vascular anastomosis to the iliac vessels. Only two transplants were lost in rejection episodes. Patient survival was 94% after 1 and 90% after 5 years. Two patients died due to septiacemia, 1 died of a ruptured aortic aneurysm, 1 of cerebral ischaemia and 1 suddenly of unknown cause. Patient and graft survival was not different compared with 204 patients aged 6–16 years who received a renal transplantation during the same time period at our institution. After transplantation the patients receiving cyclosporine A showed a marked catch-up growth in the 1st year. The median standard deviation score (SDS) of body length improved from −2.63 to −1.39 standard deviations. Conclusion Renal transplantation is the treatment of choice in end-stage renal failure in children under 6 years.
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  • 5
    ISSN: 1432-1076
    Keywords: Key words Renal venous thrombosis ; Neonate ; Incidence ; Therapy ; Outcome
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Renal venous thrombosis (RVT) occurs mainly in the neonatal period and bears a dismal prognosis for individual kidney function. There is no generally accepted therapeutic regimen as controlled clinical trials are lacking. Over the last few years, thrombolytic therapy has been successfully employed by single centres. The present study set out to gather up-to-date information on the incidence, therapy and outcome of neonatal RVT as part of a prospective nation wide survey on neonatal thrombosis in Germany to serve as a basis for therapeutic trials in the future. Between 1992 and 1994 the minimum incidence of symptomatic neonatal RVT in Germany was 2.2 per 100,000 live births (95% confidence interval 1.4–3.3). Out of 35 cases, RVT occurred in 15 premature babies (incidence 13 per 100,000 live births (95% confidence interval 5.9–24.8). Ten babies had associated caval occlusion. Therapy was supportive in 8, low dose heparin was used in 14, full heparinization in 9 and thrombolytic therapy in 4 children. On follow-up after a median time of 11.5 months, renal atrophy was present in 26 out of 39 affected kidneys. Conclusion Neonatal renal venous thrombosis still leads to irreversible kidney damage in the majority of cases. Because of the low incidence a multi-national multi-centre therapeutic trial over a long period has to be considered in order to determine the optimal therapeutic approach.
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  • 6
    ISSN: 1432-1076
    Keywords: Paediatric renal transplantation ; Pre-emptive transplantation ; Nephropathic cystinosis ; Growth development ; Long-term outcome
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract From 1970 to 1991 a total of 244 renal transplantations were performed in 203 children at the Medical School in Hannover. The mean patient age was 10.4 years with a range between 11 months and 16.9 years. Fifty-nine children received a living donor graft from one parent and 144 received cadaveric grafts. Forty-two children were transplanted without prior dialysis treatment. After 20 years the overall survival rates were 86% for the patients and 39% for the first grafts. Grafts from donors below 5 years of age had a less favourable survival (44% after 5 years). Pre-emptive transplantation yielded comparable results with the benefit of a shorter period of uraemia. Hypertension developed in 80% of transplanted patients. Only children with living related donor grafts had significantly less hypertensive problems independent of the immunosuppressive regimen. Post-transplantational growth improved under cyclosporin. Children with nephropathic cystinosis also showed catch up growth after transplantation under cyclosporin. The long-term outcome and rehabilitation of grown-up recipients were encouraging.
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  • 7
    ISSN: 1433-0474
    Keywords: Schlüsselwörter Nierentransplantation ; Varizellen ; Immunsuppression ; Key words Kidney transplantation ; Immunosuppression ; Varicella ; Chickenpox
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary We report the case of a seven year old girl presenting with varicella infection 14 days after kidney transplantation. Cramp-like abdominal pain and a subileus were the first symptoms. Enemas did not relieve the symptoms, and the following laparotomoy with Tenckhoff catheter explantation was performed which did not reveal any pathologic abnormalities. Two days after the beginning of abdominal pain an unspecific papular rash developed. Further twelve hours later skin vesicles appeared and varicella-zoster virus was isolated from their fluid. Despite high dose administration of aciclovir and varicella-zoster immune globulin further visceral dissemination occurred and finally lead to liver failure and intravascular coagulopathy. The patient died 18 days after kidney transplantation from multiorgan failure. Discussion: On the background of this lethal varicella infection we emphasize the complications and unusual manifestations in immunocompromised children. Therefore, active immunization is indicated in seronegative patients awaiting organ transplantation.
    Notes: Zusammenfassung Wir berichten über ein 7 Jahre altes Mädchen, das 14 Tage nach einer Nierentransplantation an Varizellen erkrankte. Erste Symptome waren krampfartige Bauchschmerzen mit radiologischen Hinweisen auf einen Obturationssubileus bei Obstipation. Als nach abführenden Maßnahmen keine Besserung auftrat, wurde eine Probelaparotomie mit Entfernung des Tenckhoff-Dialysekatheters durchgeführt. Dabei zeigte sich kein pathologischer Befund, insbesondere fanden sich keine Briden. Zwei Tage nach Beginn der abdominalen Symptomatik trat ein zunächst uncharakteristisches Exanthem auf. Aus den sich in den nächsten 12 h entwickelnden Bläschen konnte Varicella-Zoster-Virus isoliert werden. Trotz hochdosierter Gabe von Aciclovir und Varicella-Zoster-Immunglobulin entwickelte sich ein fulminantes Leberversagen mit Verbrauchskoagulopathie. Die Patientin verstarb am 18. postoperativen Tag trotz intensiver supportiver Therapie im Multiorganversagen. Diskussion: Vor dem Hintergrund dieser letalen Varizelleninfektion wird auf die Komplikationen und die teilweise untypischen Verläufe von Windpocken bei immunsupprimierten Kindern hingewiesen. Eine aktive Impfung ist daher bei seronegativen Patienten vor einer geplanten Organtransplantation zu fordern.
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  • 8
    ISSN: 1433-0474
    Keywords: Schlüsselwörter Wilms-Tumor ; Sonographie ; Nephroblastomatose ; Key words Wilms tumors ; Nephroblastoma ; Sonography ; Nephroblastomatosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary Introduction: Current publications focus on the sonographic features of large nephroblastomas. Since high resolution ultrasound transducers are available, detection of small nephroblastomas became possible. The aim of the study was the definition of sonographic features of small renal tumors in children. Methods: Nephroblastomas with maximum diameter of 4 cm or less were studied. 9 patients with 12 renal tumors were investigated. Results of high-resolution ultrasound were compared to intraoperative findings and to the results of computed tomography and magnetic resonance. Results: Characteristic features of small nephroblastomas are discreet hyperechogenity, clear demarcation from renal parenchyma and disruption of the renal shape. The results of ultrasound examination were confirmed during operation for 8 of 12 renal tumors. Two nephrogenic rests and 2 small nephroblastomas localized near the renal capsule were missed by sonography, 1 of these lesions was also missed by NMR. Conclusion: Ultrasound can detect even very small nephroblastomas. Superficial subcapsular lesions may not be detected by ultrasound nor MRT. To detect small nephroblastomas and nephrogenic rests the combination of imaging methods still remains useful.
    Notes: Zusammenfassung Fragestellung: Ziel der vorliegenden Untersuchung ist die Definition sonomorphologischer Kriterien zur Diagnose kleiner Wilms-Tumoren. Dazu wurden Tumoren mit einem Durchmesser von bis zu 4 cm analysiert. Das sonographische Bild bei klinisch diagnostizierten Wilms-Tumoren ist in der Literatur gut bekannt. Durch die Verbreitung der hochauflösenden Sonographie sind auch die Befunde bei kleinen Wilms-Tumoren von besonderem Interesse. Methode: Die Befunde der hochauflösenden Sonographie wurden mit dem intraoperativen Befund und den Ergebnissen von CT und NMR verglichen. Ergebnisse: 9 Patienten mit 12 Nierentumoren wurden untersucht. Als wichtige sonographische Kriterien konnten die deutliche Abgrenzbarkeit zum Nierenparenchym, die Überschreitung der Nierenkontur und eine diskrete Echogenitätserhöhung definiert werden. In 8 der 12 Fälle bestand eine gute Korrelation zwischen sonographischem, radiologischem und intraoperativem Befund. Bei den 4 nicht korrekt diagnostizierten Tumoren handelte es sich um 2 nephrogene Reste und 2 kleine Wilms-Tumoren, die oberflächlich und nahe der Nierenkapsel lokalisiert waren. Davon war 1 auch in der vorausgegangenen NMR-Untersuchung nicht nachweisbar. Schlußfolgerung: Die Sonographie ist auch zum Nachweis kleiner Wilms-Tumoren geeignet. Tumoren nahe der Nierenkapsel wurden weder sonographisch noch durch MRT in allen Fällen sicher erfaßt. Zur Diagnose kleiner Wilms-Tumoren und nephrogener Reste erscheint die Kombination verschiedener bildgebender Verfahren weiterhin sinnvoll.
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  • 9
    ISSN: 1433-0474
    Keywords: Schlüsselwörter Primäre pulmonale Hypertension ; Leber ; Zirrhose ; Kinder ; Lebertransplantation ; Key words Primary pulmonary hypertension ; Liver ; Cirrhosis ; Children ; Liver transplantation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary A child who suffered from primary sclerosing cholangitis presented with severe portal hypertension over years. At age eleven, symptoms of right ventricular decompensation were noted. After stabilization, orthotopic liver transplantation was performed and resulted in severe decompensation with an increase of right ventricular pressure up to 95 mmHg. At further investigation, primary pulmonary hypertension with plexiform pulmonary arteriopathy was diagnosed. After ten months under oxygen and nifedipine treatment, the patient reached a stable cardiopulmonary state comparable to the pre-transplant period (right ventricular pressure 45 mmHg) but did not improve further. Discussion: Primary pulmonary hypertension in children can occur in association with severe portal hypertension. As this complication will considerably alter prognosis and management of children with chronic liver disorders, it must be actively searched for and monitored with regular ECG, chest X-ray and echocardiography. To prove the diagnosis and evaluate treatment response, cardiac catheterization is required.
    Notes: Zusammenfassung Bei einem an einer primär sklerosierenden Cholangitis erkrankten Mädchen bestand über Jahre ein schwerer portaler Hypertonus. Mit 11 Jahren traten klinische Symptome einer Rechtsherzdekompensation auf. Die orthotope Lebertransplantation, 1 Jahr später durchgeführt, führte zu einer Dekompensation mit Anstieg des rechtsventrikulären Drucks bis 95 mmHg. Die Diagnostik ergab eine primäre pulmonale Hypertonie mit plexiformer pulmonaler Arteriopathie. Unter Sauerstofftherapie und Nifedipin kam es im Verlauf von 10 Monaten zu einer Besserung der rechtsventrikulären Belastung etwa auf den Status vor der Transplantation (rechts-ventrikulärer Druck um 45 mmHg), jedoch nicht zu einer weitergehenden Remission. Diskussion: Eine primäre pulmonale Hypertonie in Assoziation mit einer portalen Hypertension kann auch im Kindesalter auftreten und hat erhebliche Bedeutung für die Prognose und das therapeutische Vorgehen. In der Betreuung chronisch leberkranker Kinder muß bei regelmäßigen Verlaufskontrollen von EKG, Röntgenthorax und Echokardiografie bewußt auf diese oft zunächst asymptomatische Komplikation geachtet werden. Im Verdachtsfall ist eine Herzkatheteruntersuchung zur Diagnosesicherung und zum Austesten des Therapieansprechens erforderlich.
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  • 10
    ISSN: 1432-1440
    Keywords: Cyclosporin ; minimal change nephrotic syndrome ; focal segmental glomerulosclerosis ; children
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary In a pilot study 23 children with nephrotic syndrome were treated with cyclosporin A (Cs) for 6–45 months. 8 children suffered from steroid dependent minimal change nephrotic syndrome (MCNS) and had experienced at least one course with cytotoxic drugs, but had relapsed thereafter. 2 children had diabetes mellitus type I with nephrotic syndrome and 13 children had steroid resistant focal segmental glomerulosclerosis (FSGS). Cs was started with 100 mg/m2/day in two doses and increased stepwise to obtain a Cs whole blood trough level of 200–400 ng/ml. In steroid dependent MCNS treatment with Cs reduced relapse rate significantly, and prednisone therapy could be stopped completely. After discontinuation of Cs, relapses reoccurred as frequently as before. Renal function remained unimpaired despite repeated Cs treatment courses up to 38 months. In cases of nephrotic syndrome with diabetes type I Cs treatment led to complete remission without changing the insulin requirement. However, after discontinuation of Cs relapses reoccurred. In steroid resistant FSGS 6 children benefited from Cs treatment: 4 went into complete remission, 2 into partial remission. The 2 children with complete remission relapsed but remained Cs responsive. The remaining 7 children with FSGS did not respond to Cs but continued the course of their disease, with two patients rapidly progressing to terminal renal failure. Side-effects of Cs treatment were mild. It is concluded that Cs is an effective agent in steroid dependent MCNS and can be used as an alternative drug in specific cases like steroid toxicity or diabetes mellitus. In steroid resistant FSGS a trial with Cs seems to be warranted since some cases do respond favorably. To avoid nephrotoxicity treatment with Cs should always be monitored closely by determination of blood levels and renal function.
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