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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Methods in cell science 13 (1991), S. 163-168 
    ISSN: 1573-0603
    Keywords: organ culture ; metanephros ; kidney ; immunohistochemistry
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology
    Notes: Summary A murine metanephric whole-organ culture system is described which maintains normal structural relationships of epithelial and mesenchymal elements and undergoes advanced organotypic differentiation in vitro. Manipulation of the culture conditions permits study of the developmental, immunologic, and biochemical aspects of normal and abnormal renal organogenesis.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-198X
    Keywords: Key words: Polycystic kidney disease ; Taxol ; Renal cysts ; Biliary hyperplasia ; Epidermal growth factor receptor
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract. Polycystic kidney disease (PKD) a is common disease in the human population that can lead to renal failure and death. Taxol has recently been reported to be of therapeutic benefit in the cpk mouse model of PKD. To determine whether these results also apply to other models of PKD, we studied the effects of taxol treatment on the development of renal cysts and biliary hyperplasia/dysplasia/fibrosis in the orpk mouse mutant, a unique murine model for human autosomal recessive PKD. We report no significant differences between the treatment and control groups with respect to weight gain, survival, urine to serum osmolality ratio, and serum concentration of liver enzymes. Moreover, renal cystic development was not affected by taxol treatment in the orpk mutant animals. This was confirmed by lectin staining and morphometric analysis of the renal cysts, which indicated no significant differences between treatment groups. Therefore, while taxol has a positive effect on the cystic kidney disease in cpk mutant mice, this effect is not applicable to all forms of PKD.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-198X
    Keywords: Key words: Peritoneal dialysis ; Infants ; Survival ; Growth ; Morbidity
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract. We reviewed our center’s experience with nightly automated peritoneal dialysis (APD) as maintenance renal replacement therapy (RRT) for infants and children under the age of 5 years and compared it with national dialysis and transplant data. A retrospective chart review of 19 consecutive patients with the onset of end-stage renal disease (ESRD) before 5 years of age (mean = 1.8 years) between June 1988 and June 1994 was performed. All patients received nightly APD, supplemental feedings, calcitriol, erythropoietin, and 10 of 19 were on growth hormone (rhGH) therapy. The growth of our patients was maintained or improved during the study period, with the 10 of 19 on rhGH gaining a mean of one standard deviation in height when followed for 2 years. Our school-age children were all in age-appropriate classes. There were no deaths in our group; the incidence of peritonitis was lower than in national data. We conclude that APD is a realistic option for the treatment of ESRD in the 0- to 5-year-old child. Because of the improved graft and patient survival in older children, APD in a specialized center might be the RRT of choice in this age group, allowing good growth and development while maximizing the chances of an eventual and successful renal transplant.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    Pediatric nephrology 13 (1999), S. 143-147 
    ISSN: 1432-198X
    Keywords: Key words Recombinant human erythropoietin ; Safety and efficacy ; Chronic renal failure
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  A prospective randomized study of the use of recombinant human erythropoietin (rHuEPO) in children with chronic renal disease was conducted to assess dosing requirements and side effects. Forty-four children with chronic renal failure, aged 4 months to 21 years, were studied. Twenty-five patients were pre dialysis, 10 on peritoneal dialysis, and 9 on hemodialysis. Patients received either 150 U/kg per week or 450 U/kg per week divided thrice weekly of rHuEPO for 12 weeks or until target hemoglobin (Hb) was attained. Dose was then adjusted to maintain a normal Hb. Eighty-two percent of patients reached target Hb by 7.9±5.6 weeks (mean±SD); 95% of patients in the high-dose group and 66% in the low-dose group reached target Hb within 12 weeks. The overall median rHuEPO dose at target Hb was 150 U/kg per week. Hemodialysis patients tended to require more rHuEPO to maintain a normal Hb (median 250 U/kg per week). Transfusion requirements and panel-reactive antibody levels decreased during the 12 weeks. Iron deficiency and/or hypertension occurred in 30% of children. In conclusion, rHuEPO at 150 U/kg per week is safe and effective in treating anemia in children with chronic renal disease.
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    Pediatric nephrology 2 (1988), S. 92-99 
    ISSN: 1432-198X
    Keywords: Epithelial differentiation ; Organ culture ; Nephrogenesis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract An in vitro model system for the experimental study of renal epithelial differentiation is described. Fetal murine metanephric tissue consisting of nephrogenic blastema and branched ureteric bud is isolated following 24–36 h of natural embryonic inductive interaction (13±0.4 days gestation) and cultured as an intact organ in a Trowell-type assembly. During 120 h of organ culture incubation in completely defined serum-free medium, advanced organotypic proximal tubular and glomerular epithelial differentiation proceed in the absence of vascularization, perfusion, and urine production. The system thus experimentally separates the processes of three-dimensional organ growth and post-induction renal epithelial differentiation from glomerular filtration, flow-related phenomena, endothelial or mesangial cell interactions, and the effects of growth factors or transport substrates present in mammalian serum or urine. Studies to date in the model system have defined the growth factor requirements of epithelial growth and differentiation and have demonstrated that specific hormonally induced alterations in tubular epithelial cell metabolism and function may lead to specific patterns of tubular maldevelopment. Whole organ metanephric organ culture is thus a valuable in vitro model system for future investigations into the complex processes of normal and abnormal renal epithelial differentiation.
    Type of Medium: Electronic Resource
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  • 6
    Electronic Resource
    Electronic Resource
    Springer
    Pediatric nephrology 7 (1993), S. 129-129 
    ISSN: 1432-198X
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 7
    ISSN: 1432-198X
    Keywords: End-stage renal disease ; Renal transplantation ; Dialysis ; Graft survival ; Patient survival
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The 1993 North American Pediatric Renal Transplant Cooperative Study annual report summarizes data voluntarily contributed by 82 participating centers on 3,223 pediatric patients who received 2,819 renal transplants from January 1987 through January 1993 and 999 independent courses of dialysis from January 1992 through January 1993. In addition to updating information regarding trends and outcomes in pediatric renal transplantation presented in previous annual reports, 1st-year registry data are presented regarding current practices and trends in chronic dialysis therapy for children and adolescents in North America. Living donor graft (LDG) survival rate was 90% at 1 years 85% at 2 years and 75% at 5 years post transplant. Cadaver graft (CG) survival rates were 76%, 71% and 62% at 1, 2 and 5 years post transplant, respectively. Overall mortality post transplantation continues to be low (CG 6.8%, LDG 4%), mortality remains high in young infants. The dialysis cohort was generally younger than the transplantation cohort. In all age groups, peritoneal dialysis was utilized in the majority of pediatric patients and the overall incidence of peritonitis was 1 episode per 8.2 patient months. External percutaneous catheters were utilized as the predominant chronic hemodialysis access in the study, and access site infections ranged from 6.9% at 1 month to 13.5% at 6 months.
    Type of Medium: Electronic Resource
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  • 8
    ISSN: 1432-198X
    Keywords: Cystic kidney disease ; Animal models ; Nephron microdissection ; CPK mouse
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract In the current study, the ontogeny of tubular cyst formation was studied in the CPK mouse, a murine strain with autosomal recessive polycystic kidney disease. Utilizing the technique of intact nephron microdissection in addition to standard light and transmission electron microscopy, the earliest morphologic alterations in CPK kidneys were localized in fetal tissue at 17 days of gestation to the distal portion of developing proximal tubules. During disease progression, from birth to 21 days of postnatal age, there was a shift in the site of cystic nephron involvement from proximal tubule to collecting tubules without involvement of other nephron segments. Cysts were enlarged tubular segments which remained in continuity with other portions of the nephron and were not associated with abnormalities in the overall pattern of nephron growth or differentiation. Analysis suggested that alterations in transtubular transport in abnormally shortened proximal tubular segments of juxtamedullary nephrons may have pathogenic importance in the early stages of cyst formation, and that epithelial hyperplasia and cytoskeletal alterations may have a role in progressive proximal tubular cystic enlargement. Cellular hyperplasia of epithelial walls of normally formed tubules was a prominent feature of cyst formation and progressive enlargement in collecting tubules. Such data form the basis for future studies into specific pathophysiological processes which may be operative in specific nephron segments during different stages of cyst formation in the CPK mouse.
    Type of Medium: Electronic Resource
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  • 9
    ISSN: 1432-198X
    Keywords: Key words:  Hemolytic uremic syndrome ; Escherichia coli O157 : H7 ; Cholelithiasis ; Prognosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract. Sequelae of Escherichia coli O157 : H7-associated hemolytic uremic syndrome (HUS) 2 – 3 years following an outbreak in Washington State have been prospectively studied to identify predictors of adverse sequelae. Logistic regression analysis was used to examine associations between findings in the acute course and long-term renal and gastrointestinal outcomes. Twenty-one percent of patients had gastrointestinal sequelae, which included cholelithiasis resulting in cholecystectomy (3/29), persistent pancreatitis (2/29), late colon stricture (1/29), and/or glucose intolerance (1/29). Logistic regression analysis found long-term gastrointestinal sequelae were higher in patients who, during HUS, had hypertension [odds ratio (OR) = 21.2, 95% confidence interval (CI) = 1.9 – 164.4, P = 0.01] or gastrointestinal complications (OR = 21.2, 95% CI = 1.9 – 164.4, P = 0.01). Renal sequelae were seen in 35% of patients. One patient (4%) had persistent hypertension and 9 (31%) had minor urinary findings (hematuria or proteinuria). Thrombocytopenia lasting longer than 10 days during the acute illness was associated with a risk for subsequent renal sequelae (OR = 15.0, 95% CI = 1.98 – 1,703.0, P = 0.009). We conclude a high incidence of gastrointestinal sequelae, especially cholelithiasis presenting long after the acute illness, may be seen with HUS. The short follow-up period may underestimate the extent and severity of eventual renal sequelae.
    Type of Medium: Electronic Resource
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  • 10
    ISSN: 1432-198X
    Keywords: Key words: Outpatient ; Percutaneous renal biopsy ; Complications
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract. Although several retrospective reports suggest that pediatric outpatient renal biopsies may be done in a safe and cost-effective manner, risk factors and the natural history of major complications following this procedure have not been clearly delineated. In an effort to determine the minimal observation period required to detect major post-renal biopsy complications in children and to establish clinical parameters predictive of these complications, a retrospective review of 177 percutaneous renal biopsies was performed. The overall major complication rate was 3.4%, while the minor complication rate was 14.1%. The mean percentage change in hemoglobin 4 – 10 h postbiopsy in patients with major bleeding complications was significantly greater than patients with minor bleeding complications. Using a 16% drop in hemoglobin 4 – 10 h postbiopsy, the sensitivity and specificity of identifying a major bleeding complication was 100% and 98%, respectively, while the positive and negative predictive value was 68% and 100%, respectively. All patients with major complications due to excess sedation or immediate bleeding were diagnosed within 11 h of the biopsy. Automated renal biopsies offered several safety and efficiency advantages compared with non-automated methods. Our results suggest that outpatient pediatric renal biopsies should be encouraged provided certain precautions are taken to reduce the risk of developing major complications.
    Type of Medium: Electronic Resource
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