[CLINICAL EXPERIENCE OF TRANSURETHRAL INJECTION USING DEXTRANOMER-HYALURONIC ACID COPOLYMER (Deflux®) FOR CASES OF SECONDARY VESICOURETERAL REFLUX AFTER URETEROCYSTONEOSTOMY].

(Objective) We reviewed the clinical efficacy of transurethral Deflux® injection therapy (Deflux® injection therapy) in patients with secondary vesicoureteral reflux (VUR) after ureterocystoneostomy. (Methods) We retrospectively evaluated data for Deflux® injection therapy in 4 patients after ureterocystoneostomy with secondary VUR due to lower urinary tract dysfunction treated at this hospital from 2010 through 2018. The indications for surgery were febrile urinary tract infection or new renal scarring, and the outcomes of treatment with Deflux® injection therapy were evaluated. (Results) There were 2 male and 2 female patients. Three patients had unexplained lower urinary tract dysfunction, all patients were using clean intermittent catherization, and 3 patients had operated by the Cohen method for VUR. Preoperative examinations revealed that all patients had unilateral VUR, and reflux was assessed as Grade III in 1 patient and Grade IV in 3 patients. The median age at initial surgery was 12.8 years, and Deflux® injection therapy was performed unilaterally in all patients. Deflux® injection therapy was performed by a combined HIT/STING technique in 2 patients and by the HIT technique alone in 2 patients, with a median injection volume of 2.4 ml. The therapeutic outcome for initial Deflux® injection therapy was resolution of VUR in 1 patient (25%) and persistence of VUR in 3 patients. Treatment was repeated for 2 of the 3 patients with persistent VUR, and resolution was reported for 1 of these patients. (Conclusion) The initial success rate for Deflux® injection therapy in VUR with bladder dysfunction was a low 25%, suggesting that caution is required in choosing this treatment option.

Ureterosciatic Hernia Treated with Laparoscopic Intraperitonization of the Ureter.

Background: Ureterosciatic hernia (USH) is a rare benign disease. We report a case of USH treated with laparoscopic intraperitonization of the ureter. Case Presentation: A 70-year-old woman was admitted to our hospital with right abdominal pain lasting for 2 months. CT showed right hydronephrosis and invagination of the right ureter into the right sciatic foramen. She underwent retrograde ureterography, which revealed abnormal tortuosity of the right lower ureter, and was found to have USH. We performed laparoscopic intraperitonization of the ureter and she presented good postoperative course. Conclusion: Laparoscopic intraperitonization of the ureter can be a useful treatment for USH.

Long-term outcomes of pediatric kidney transplantation: A single-center experience over the past 34 years in Japan.

OBJECTIVES: To evaluate long-term outcomes and risk factors for graft loss in pediatric kidney transplantation over a 30-year period. METHODS: We retrospectively assessed 400 consecutive kidney transplants carried out in 377 children during 1975-2009. Patients were stratified according to the immunosuppressive regimen (era 1: methylprednisolone and azathioprine; era 2: calcineurin inhibitor-based therapy, including methylprednisolone and azathioprine or mizoribine; era 3: basiliximab induction therapy, including calcineurin inhibitors, methylprednisolone and mycophenolate mofetil). RESULTS: The median age and bodyweight at transplantation were 9.7 years and 20.6 kg, respectively. In total, 364 (91.0%) children received a living related donor transplantation. The acute rejection rate within 1 year post-transplant decreased significantly from 61.0% in era 1 to 14.5% in era 3 (P < 0.001). For transplant eras 1-3, 1-year graft survival was 81%, 93% and 95%; 5-year graft survival was 66%, 86% and 93%; and 10-year graft survival was 47%, 79% and 89%, respectively. The overall 5-, 10- and 20-year patient survival rates were 96%, 93% and 88%, respectively. A Cox multivariate analysis identified cold ischemia time (hazard ratio 1.385, 95% confidence interval 1.251-1.603), acute rejection (hazard ratio 1.682, 95% confidence interval 1.547-3.842), re-transplant (hazard ratio 2.680, 95% confidence interval 1.759-3.982) and donor type (hazard ratio 2.957, 95% confidence interval 1.754-4.691) as independent risk factors for graft loss at 10 years post-transplant. CONCLUSIONS: The progress of immunosuppressive therapy has led to a low incidence of acute rejection and a high graft survival rate across 30 years of pediatric transplantation.

Compound heterozygous splice site variants in the SCLT1 gene highlight an additional candidate locus for Senior-Løken syndrome.

Senior Løken syndrome (SLS) is a heterogeneous disorder characterized by severe retinal degenerations and juvenile-onset nephronophthisis. Genetic variants in ten different genes have been reported as the causes of SLS. Clinical evaluation of a patient with SLS and her unaffected parents revealed that the patient had infantile-onset retinal dystrophy and juvenile-onset nephronophthisis. Other systemic abnormalities included hepatic dysfunction, megacystis, mild learning disability, autism, obesity, and hyperinsulinemia. Whole-exome sequencing identified compound heterozygous SCLT1 variants (c.1218 + 3insT and c.1631A > G) in the patient. The unaffected parents were heterozygous for each variant. Transcript analysis using reverse transcription PCR demonstrated that the c.1218 + 3insT variant leads to exon 14 skipping (p.V383_M406del), while the other variant (c.1631A > G) primarily leads to exon 17 skipping (p.D480EfsX11) as well as minor amounts of two transcripts (6 bps deletion in the last of exon 17 [p.V543_K544del] and exons 17 and 18 skipping [p.D480E, S481_K610del]). Immunohistochemical analysis demonstrated that the Sclt1 protein was localized to the distal appendage of the photoreceptor basal body, indicating a ciliary protein. In conclusion, we identified compound heterozygous splice site variants of SCLT1 in a patient with a new form of ciliopathies that exhibits clinical features of SLS.

[ACUTE SCROTUM IN THE PEDIATRIC EMERGENCY ROOM: A SINGLE CENTER RETROSPECTIVE STUDY].

(Objective) The objective of this study was to survey cases of acute scrotum examined at the pediatric emergency room (ER). (Subjects and methods) All patients examined for acute scrotum at the emergency outpatient unit between March 2010 and March 2014 were designated subjects of the study. Medical records were used to retrospectively investigate the final diagnoses including testicular torsion (TT), torsion of the testicular appendage, epididymitis, orchitis, incarcerated inguinal hernia, idiopathic scrotal edema, Schönlein-Henoch purpura, hydrocele, external trauma, as well as those with no confirmed diagnosis. (Results) A total of 257 patients presented with acute scrotum at the emergency outpatient unit. The patients had an average age of 6.7 years, with symptoms present on the right side in 120 cases (47%), left side in 108 cases (42%), and both sides in 29 cases (11%). Observing the frequency of each condition, TT was present in 33 cases (13%), torsion of the testicular appendage in eight cases (3%), epididymitis in 85 cases (33%), orchitis in four cases (2%), incarcerated inguinal hernia in 59 cases (23%), idiopathic scrotal edema in six cases (2%), Schönlein-Henoch purpura in nine cases (4%), hydrocele in 10 patients (4%), external trauma in 12 patients (5%), and unconfirmed diagnosis in 31 cases (12%). Average time between onset and presentation at the ER was 0.6 days for TT, with patients having previously been examined at other hospitals before coming to this institution in 12 cases (36%). Emergency urological surgery was performed in 31 cases (12%), with TT comprising 28 cases (90%), and orchiectomy performed in six cases (21%). (Conclusion) Of all patients examined for acute scrotum, epididymitis were high and TT in 13% at the pediatric ER. Average time for TT patients from symptom onset to ER presentation were longer, we must ensure that patients present at earliest possible opportunity requires educating the general population.

Color Doppler Ultrasound Evaluation of Ureteral Jet Angle to Detect Vesicoureteral Reflux in Children.

PURPOSE: Vesicoureteral reflux is the most common urinary tract abnormality in children. Although voiding cystourethrography is the gold standard for diagnosing reflux, this approach is invasive. We evaluated color Doppler ultrasound measurement of ureteral jet angle as a noninvasive screening tool for detecting reflux. MATERIALS AND METHODS: We retrospectively evaluated 125 children (250 renal units) who presented with urinary tract infections or hydronephrosis. All patients underwent color Doppler ultrasonography and voiding cystourethrography. Ureteral jet angle was measured as angle between the direction of the ureteral jet and interureteral ridge. RESULTS: Reflux was diagnosed in 80 patients (117 renal units). Mean ± SD ureteral jet angle was significantly greater in refluxing units (67.9 ± 18.7 degrees) than in nonrefluxing units (47.8 ± 17.3 degrees, p <0.0001). Angle value in each reflux grade became significantly greater according to grade (p <0.0001). Overall ability of ureteral jet angle to detect grade III to V and grade IV/V reflux, as given by the area under the receiver operating characteristic curve, was 0.81 and 0.88, respectively. A cutoff angle of 55 degrees or greater detected grade III to V and grade IV/V reflux with a sensitivity of 85.5% and 94.7%, respectively. A cutoff angle of 70 degrees or greater diagnosed grade IV/V reflux with a sensitivity of 81.6% and a specificity of 82.7%. CONCLUSIONS: Color Doppler ultrasound measurement of ureteral jet angle represents a simple and noninvasive method that is applicable as a screening tool to detect high grade vesicoureteral reflux in children.

[Clinical characteristics and outcomes of congenital midureteral stricture detected by fetal ultrasonography].

OBJECTIVES: Congenital midureteral stricture (CMS), which develops from obstructive lesion between pyeloureteral junction and ureterovesical junction, is relatively rare and its clinical condition and therapeutic strategy have not yet been established. We analyzed the clinical characteristics and surgical outcomes of CMS. PATIENTS AND METHODS: From November 2006 to December 2012, out of 137 patients presented with congenital hydrohephrosis, we identified 4 pediatric patients diagnosed with CMS at our institutions. We retrospectively investigated clinical characteristics and surgical outcomes in these 4 patients. RESULTS: Three boys and one girl were identified in this study. All patients were detected hydronephrosis by fetal ultrasonography. The median age at the diagnosis of CMS was 1 year and 11 months. Three patients had obstructive lesion in left side and 1 patient in right. CMSs were located at the level of L4 in 2 patients, and at the level of L5 and S1 in each 1. Split renal function was decreased less than 45% in 3 of 4 patients. Ipsilateral pyeloureteral junction obstruction and ipsilateral hypoplastic kidney were identified in 2 and 1 patient, respectively. One patient developed urosepsis and underwent nephrostomy. Partial ureterectomy and ureteroureterostomy, pyeloplasty were performed in 3 and 1 patient, respectively. Extrinsic obstruction was detected in just 1 patient intraoperatively. In all patients, there were no protruded lesion and atrophied, fibrotic and ischemic muscles were not detected in pathological finding. Neither urinary tract infection nor recurrence of obstructive lesion was detected in all patients at the mean follow-up period of 3 years and 1 month. CONCLUSION: We analyzed the clinical characteristics of 4 pediatric patients with CMS. In order to prevent critical infection and maintain renal function, it could be considered that surgical intervention is undertaken just after making the diagnosis of CMS.