ABSTRACT
OBJECTIVE: To assess the feasibility and outcomes of laparo-endoscopic single site (LESS) partial nephrectomy (PN) in infants and small children for upper urinary tract duplication anomalies. MATERIALS AND METHODS: The medical records of all patients undergoing LESS PN at a single pediatric institution were retrospectively reviewed for patient demographics, perioperative details, and outcomes. A cystoscopy was initially performed to place an externalized catheter into the ureter of the ipsilateral normal renal moiety. An Olympus TriPort, an Olympus Endoeye flexible tip laparoscope, standard 3- or 5-mm instrumentation, and a LigaSure Blunt were utilized. RESULTS: Four children (two boys, two girls) underwent LESS PN. Three patients underwent upper pole PN and one underwent lower pole PN. All procedures were performed for poorly functioning obstructed renal moieties (one ureterocele, one ureteropelvic junction obstruction and vesicoureteral reflux, and two ectopic ureters). Median age was 6.2 months (range 2.5-16.4 months). Median weight was 7.7 kg (range 6.1-12.6 kg). Median operative time was 126 min (range 97-180 min). No patient received inpatient postoperative narcotics. Median follow-up was 9.9 months (range 6.2-19.1 months). No postoperative complications were noted. Postoperative renal ultrasound demonstrated successful resection in all patients. CONCLUSIONS: LESS PN is technically feasible, safe, and effective for upper urinary tract duplication anomalies in infants and small children.
Subject(s)
Laparoscopy/methods , Nephrectomy/methods , Ureteral Obstruction/surgery , Ureterocele/surgery , Urinary Tract/abnormalities , Vesico-Ureteral Reflux/surgery , Age Factors , Child, Preschool , Cystoscopy , Feasibility Studies , Female , Humans , Infant , Male , Operative Time , Retrospective Studies , Treatment Outcome , Ureteral Obstruction/diagnosis , Ureteral Obstruction/etiology , Ureterocele/diagnosis , Ureterocele/etiology , Vesico-Ureteral Reflux/diagnosis , Vesico-Ureteral Reflux/etiologyABSTRACT
The t(4;14) translocation in multiple myeloma (MM) simultaneously dysregulates two apparent oncogenes: fibroblast growth factor receptor 3 (FGFR3) controlled by the 3' immunoglobulin heavy chain enhancer on der(14) and MMSET controlled by the intronic Emu enhancer on der(4). Although all MM tumors and cell lines with a t(4;14) translocation have dysregulated MMSET, about 25% do not express FGFR3. Therefore, the function of dysregulated wild-type (WT) FGFR3 in the pathogenesis of MM remains unclear. We developed a murine transgenic (TG) model in which WT FGFR3 is overexpressed in B lymphoid cells. Although high levels of FGFR3 resulted in lymphoid hyperplasia in about one-third of older mice, no increase in tumorigenesis was observed. However, double TG FGFR3/Myc mice develop mature B lymphoma tumors that occur with a higher penetrance and shorter latency than in single TG Myc mice (P=0.006). We conclude that expression of high levels of WT FGFR3 can be oncogenic and cooperate with MYC to generate B lymphoid tumors. This suggests that dysregulated FGFR3 expression is likely to be essential at least for the early stages of pathogenesis of MM tumors that have a t(4;14) translocation.
