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Background: Atypical hemolytic uremic syndrome (aHUS) is a rare, mostly complement-mediated thrombotic microangiopathy. The majority of patients are infants. In contrast to infantile-onset aHUS, the clinical and genetic characteristics of adolescence-onset aHUS have not been sufficiently addressed to date. Methods: A total of 28 patients (21 girls, 7 boys) who were diagnosed as aHUS between the ages of ≥10 years and <18 years were included in this study. All available data in the Turkish Pediatric aHUS registry were collected and analyzed. Results: The mean age at diagnosis was 12.8±2.3 years. Extra-renal involvement was noted in 13 patients (46.4%); neurological involvement was the most common (32%). A total of 21 patients (75%) required kidney replacement therapy. Five patients (17.8%) received only plasma therapy and 23 (82%) of the patients received eculizumab. Hematologic remission and renal remission were achieved in 25 (89.3%) and 17 (60.7%) of the patients, respectively. Compared with the infantile-onset aHUS patients, adolescent patients had a lower complete remission rate during the first episode (p = 0.002). Genetic analyses were performed in all and a genetic variant was detected in 39.3% of the patients. The mean follow-up duration was 4.9±2.6 years. At the last visit, adolescent patients had lower eGFR levels (p = 0.03) and higher rates of chronic kidney disease stage 5 when compared to infantile-onset aHUS patients (p = 0.04). Conclusions: Adolescence-onset aHUS is a rare disease but tends to cause more permanent renal dysfunction than infantile-onset aHUS. These results may modify the management approaches in these patients. [ABSTRACT FROM AUTHOR]

AbstractBackground and Purpose:In children with end-stage reflux nephropathy, nephroureterectomy can be performed either open or laparoscopically. It is not common to use a three-trocar retroperitoneal approach for nephroureterectomy with complete ureteral excision in the pediatric age group. We report the results of pediatric retroperitoneoscopic nephroureterectomies by using three trocars only.Patients and Methods:Between August 2003 and November 2009, 13 children—seven boys and six girls—with end-stage reflux nephropathy underwent retroperitoneoscopic nephroureterectomy. The operations were performed by using three trocars with the patient in a flank position under general anesthesia. Renal blood vessels were individually dissected and divided followed by the transection of the ureter at the level of the ureterovesical junction.Results:The mean age of the patients was 6.5 ± 4.4 years (15 months–14 years). Eight of the operations were performed on the left and five on the right side. Mean operative time was 137 ± 47 minutes (75–230 min). Estimated blood losses were minimal. Mean hospitalization time was 2.2 ± 0.9 days (2–5 d). No major intraoperative complication was seen. Overall postoperative analgesic requirement was minimal. Cosmetic results were excellent at the initial postoperative visit.Conclusion:Retroperitoneoscopic nephroureterectomy by using three trocars is feasible and safe with excellent outcomes in pediatric patients with end-stage reflux nephropathy. [ABSTRACT FROM AUTHOR]