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  • 1
    Electronic Resource
    Electronic Resource
    Chronology of renal scarring in males with Alport syndrome (1998)
    Springer
    Pediatric nephrology 12 (1998), S. 269-274 
    Details
    ISSN: 1432-198X
    Keywords: Key words: Alport syndrome ; Renal scarring ; Cortical interstitial volume fraction ; Global glomerular sclerosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract. We investigated the onset of renal scarring in 62 males (aged 4 – 26 years) with Alport syndrome by measuring cortical interstitial volume fraction [Vv (interstitium/cortex)] and percentage global glomerular sclerosis in kidney biopsies. Male pediatric (n = 9) and adult (n = 7) donor kidneys served as controls. Creatinine clearance at the time of biopsy was available for 43 Alport patients. A statistically insignificant correlation between age and Vv (interstitium/cortex) was observed in normal subjects (r = +0.47, slope = 0.0009, P = 0.07). In the Alport patients, age was significantly correlated with Vv (interstitium/cortex (r = +0.49, slope = 0.01, P = 0.001) and global glomerular sclerosis (r = +0.41, P = 0.01), and inversely correlated with creatinine clearance (r = –0.33, P = 0.04). Creatinine clearance was inversely correlated with Vv (interstitium/cortex) (r = –0.78, P = 0.001) and global glomerular sclerosis (r = –0.74, P = 0.001). The correlation with creatinine clearance was especially strong for Vv (interstitium/cortex) values above the normal range, i. e., 〉0.2 (r = –0.82, P = 0.001), and was absent for Vv (interstitium/cortex) 〈0.2 (r = –0.119, P = 0.55). Creatinine clearance values less than 80 ml/min per 1.73 m2 occurred more frequently in patients with Vv (interstitium/cortex) values 〉0.2 (P 〈0.0001) and in patients with 〉10% globally sclerosed glomeruli (P 〈0.001). Patients ≤ or 〉10 years of age differed in Vv (interstitium/cortex) [0.13±0.09 (mean ±SD) vs. 0.24±0.026, P 〈0.001], the frequency of Vv (interstitium/cortex) 〉0.2 (3/32 vs. 15/31, P 〈0.0001), the frequency of 〉10% globally sclerosed glomeruli (3/33 vs. 11/30, P 〈0.05), mean creatinine clearance (113±7 vs. 84±10 ml/min per 1.73 m2, P = 0.057), and the frequency of creatinine clearance 〈80 ml/min per 1.73 m2 (1/20 vs. 11/23, P 〈0.01). Thus, reduced creatinine clearance in males with Alport syndrome is associated with Vv (interstitium/cortex) 〉0.2 and 〉10% globally sclerosed glomeruli. These are frequently detectable in the 2nd decade. We hypothesize that most Alport males will require intervention during the 1st decade for optimal preservation of kidney function.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004670050451
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  • 2
    Electronic Resource
    Electronic Resource
    Post-transplantation outcome of patients with hemolytic-uremic syndrome: update (1991)
    Springer
    Pediatric nephrology 5 (1991), S. 162-167 
    Details
    ISSN: 1432-198X
    Keywords: Hemolytic-uremic syndrome ; Posttransplantation outcome ; Graft survival
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The outcome of renal transplantation in patients with hemolytic-uremic syndrome (HUS) is variable in reported cases. An update of the previously published series of patients from the University of Minnesota is reported. Seventeen patients with HUS received a renal transplant. Seven patients had recurrent HUS based on strict clinical and histological features and in 4 of these patients grafts were loss from recurrent HUS, with 1 patient losing three successive grafts. Three patients had histological features consistent with HUS but lacked some of the clinical features. Seven patients had no evidence of recurrent HUS post transplantation. The incidence of recurrence of HUS post transplantation in this updated report remains high (7/17 patients). There was no difference in the allografts used (living-related donor grafts were more common in all groups) or in the immunosuppression in the different groups of patients; only 1 patient with recurrent HUS received cyclosporine. The published cases of transplantation in patients with HUS show a variable recurrence rate of 0–25% in different centers with a poor graft outcome in patients with recurrence; a higher incidence of early chronic vascular rejection with decreased graft survival is also reported in patients without recurrence. Patients with HUS post renal transplant are at a variable risk of recurrence of HUS or decreased graft survival, and the factors responsible for this outcome are not known.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00852876
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  • 3
    Electronic Resource
    Electronic Resource
    Improved growth velocity with intensive dialysis. Consequence or coincidence? (2000)
    Springer
    Pediatric nephrology 14 (2000), S. 710-712 
    Details
    ISSN: 1432-198X
    Keywords: Key words Dialysis adequacy ; Infant growth
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  Growth failure in children with end-stage renal disease remains a difficult problem. A 2.5-month-old baby in renal failure due to primary hyperoxaluria type I received intensive dialysis aimed at decreasing oxalate tissue accretion. Over 5.5 months, while awaiting transplantation, his growth velocity was 29 cm/year compared with an average 4 cm/year in infants on hemodialysis and 22 cm/year in normal infants of this age. This remarkable growth rate, which could have represented catch-up growth, is hypothesized to be related to the delivered dialysis dose. It is suggested that this relationship be evaluated in a prospective randomized trial.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/PL00013422
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  • 4
    Electronic Resource
    Electronic Resource
    Delayed acute renal failure in post-transplant period in young children from unexplained etiology (1997)
    Springer
    Pediatric nephrology 11 (1997), S. 531-536 
    Details
    ISSN: 1432-198X
    Keywords: Key words: Delayed acute renal failure ; Post-transplant period ; Unexplained etiology
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract. This report describes six young children (5 male) who developed delayed acute renal failure (DARF) in the early post-kidney-transplant (Tx) period in the absence of acute rejection (AR) or other diagnosable conditions. These young children, aged 16.5 ± 3.1 (12–21) months [mean ± SD, (range)] and weighing 8.5 ± 1.7 (7.1 – 11.4) kg received a primary renal Tx (5 living-related donor, 1 cadaver) between 1984 and 1992. Immunosuppression included prednisone, azathioprine, and Minnesota antilymphocyte globulin (MALG, n = 5); one patient received cyclosporine and no MALG. Initially, all patients had good urine output (UO). They became systemically ill and abruptly developed diminished UO on post-operative day (POD) 6.5 ± 1 (4 – 8). DARF was accompanied by fever (39.1 – 40.4°C, n = 6), thrombocytopenia (platelets 〈100,000/mm3, n = 6), leukocytosis, or leukopenia (white cell count 〉20,000/mm3, n = 4 or 〈1,000/mm3, n = 1). Four patients had diarrhea. Three had ascites and one was surgically explored for suspected urinary leak. None showed significant urinary obstruction by renal ultrasound. Renograms showed intact blood flow. Renal biopsy showed tubular ectasia (n = 6), vascular congestion (n = 5), focal glomerular endothelial swelling (n = 4), and capillary thrombi (n = 3). None showed AR. Five patients required dialysis for 11 ± 4 (7 – 15) days. All patients survived. One patient, treated for suspected AR with the monoclonal antibody OKT3, developed shock and lost her graft on POD 12 due to vascular thrombosis. Renal functional recovery in the remaining five patients took 14 ± 5 (6 – 20) days and their serum creatinine at discharge was 0.7 ± 0.5 (0.3 – 1.6) mg/dl. We report DARF from undetermined etiology occurring in the first 2 weeks of renal Tx in young children. Treatment is supportive care including dialysis. Recognition of this complication will help avoid risky investigations or unnecessary treatment for rejection.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004670050332
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  • 5
    Electronic Resource
    Electronic Resource
    Hemodialysis catheter placement and recirculation in treatment of hyperammonemia (1998)
    Springer
    Pediatric nephrology 12 (1998), S. 592-595 
    Details
    ISSN: 1432-198X
    Keywords: Key words: Hyperammonemia ; Carbamoyl phosphate synthetase deficiency ; Hemodialysis ; Recirculation ; Clearance
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract. A 2-year-old girl with carbamoyl phosphate synthetase deficiency underwent emergency hemodialysis (HD) for treatment of acute life-threatening hyperammonemia. HD was performed via catheters placed in each femoral vein serving as vascular access. The tip of one of the catheters (aspirating line) was in the left external iliac vein and the tip of the other catheter (the return line) was in the inferior vena cava (IVC). High blood flow rates were used in order to rapidly lower the blood ammonia (NH3) levels. However, unanticipated marked recirculation in the IVC, between the dialysis aspirating and return catheters, was encountered, preventing significant reduction in blood NH3. The recognition of this problem, suggested solutions, and prevention are described.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004670050512
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