Whole-exome sequencing in the molecular diagnosis of individuals with congenital anomalies of the kidney and urinary tract and identification of a new causative gene.

PURPOSE: To investigate the utility of whole-exome sequencing (WES) to define a molecular diagnosis for patients clinically diagnosed with congenital anomalies of kidney and urinary tract (CAKUT). METHODS: WES was performed in 62 families with CAKUT. WES data were analyzed for single-nucleotide variants (SNVs) in 35 known CAKUT genes, putatively deleterious sequence changes in new candidate genes, and potentially disease-associated copy-number variants (CNVs). RESULTS: In approximately 5% of families, pathogenic SNVs were identified in PAX2, HNF1B, and EYA1. Observed phenotypes in these families expand the current understanding about the role of these genes in CAKUT. Four pathogenic CNVs were also identified using two CNV detection tools. In addition, we found one deleterious de novo SNV in FOXP1 among the 62 families with CAKUT. The clinical database of the Baylor Miraca Genetics laboratory was queried and seven additional unrelated individuals with novel de novo SNVs in FOXP1 were identified. Six of these eight individuals with FOXP1 SNVs have syndromic urinary tract defects, implicating this gene in urinary tract development. CONCLUSION: We conclude that WES can be used to identify molecular etiology (SNVs, CNVs) in a subset of individuals with CAKUT. WES can also help identify novel CAKUT genes.Genet Med 19 4, 412-420.

Complications following Staged Hypospadias Repair Using Transposed Preputial Skin Flaps.

PURPOSE: Proximal hypospadias repair using a staged approach is a complex reconstructive operation with the potential for significant complications requiring repeat surgery. We report outcomes of staged hypospadias repair using transposed preputial skin flaps and factors predictive of postoperative complications. MATERIALS AND METHODS: We retrospectively analyzed patients who underwent staged proximal hypospadias repair using transposed preputial skin flaps between 2002 and 2013. Patient demographics, operative details, complications, reoperations and factors predictive of complications were reviewed. RESULTS: A total of 56 patients were identified with a mean age of 14.1 months (median 14.3) at first stage. Mean followup was 38.6 months (median 34.1). Complications requiring additional unplanned operation(s) were observed in 38 patients (68%), including fistulas in 32 (57%), diverticula in 8 (14%), meatal stenosis in 5 (9%), urethral stricture in 8 (14%) and glans dehiscence in 3 (5%). In addition, redo first stage repair was performed in 4 patients (7%). Since some patients had more than 1 complication, the total number of complications is greater than the number of patients undergoing a redo operation. On univariate analyses the use of small intestinal submucosa was significantly associated with an increased risk of fistula (91% vs 49%, p = 0.02) and urethral diverticulum (64% vs 24%, p = 0.04). Incision of the tunica albuginea of the corpora was associated with an increased likelihood of fistula (77% vs 44%, p = 0.03). Finally, patients with glans dehiscence were significantly younger at first stage (5.8 vs 14.8 months, p = 0.01). CONCLUSIONS: The reoperation rate for complications in children undergoing staged hypospadias repair using transposed preputial skin flaps is higher than previously reported.

Outcomes of endoscopic incision for the treatment of ureterocele in children at a single institution.

PURPOSE: We assessed outcomes in children with ureterocele after transurethral incision at our institution between 2001 and 2014, focusing on end points of vesicoureteral reflux, improvement of hydronephrosis and need for second surgery. MATERIALS AND METHODS: We performed chart reviews of 83 patients, collecting information including age at transurethral incision, gender, renal anatomy, ureterocele location, indication for transurethral incision, and preincision and postincision vesicoureteral reflux and hydronephrosis status. Patients were divided into those with single system and duplex system ureteroceles, and intravesical and extravesical location for analysis. Statistical evaluations were performed with the Wilcoxon rank test and Fisher exact test. RESULTS: Transurethral incision was performed at a mean age of 34.2 months in patients with single system ureterocele and 8.9 months in those with duplex system ureterocele (p <0.0001). Cure rates (improvement of hydronephrosis and absence of vesicoureteral reflux) were 55.6% in patients with single system ureterocele and 14.9% in those with duplex system ureterocele (p = 0.0031). Rates of de novo reflux into the ureterocele moiety were 27.8% for patients with single system ureterocele and 56.2% for those with duplex system ureterocele (p = 0.0773). Patients with single system ureterocele required significantly fewer second surgeries (3.8%) than those with duplex system ureterocele (73.7%, p <0.0001). CONCLUSIONS: Patients with single system ureterocele may benefit from endoscopic incision. Transurethral incision was definitive in all such patients except 1 in our study. Although most patients with duplex system ureterocele will need a second operation, transurethral incision remains of value in this population in instances of sepsis or bladder outlet obstruction, or to facilitate planned reconstruction when the child is older.

The impact of surgical approach and urinary diversion on patient outcomes in pediatric pyeloplasty.

PURPOSE: Recent comparisons of the impact of the surgical approach on pediatric pyeloplasty outcomes have generally incorporated a form of internal ureteral drainage. We hypothesized that the surgical approach does not affect outcomes in children who undergo unstented pyeloplasty and stenting offers no long-term benefit in those with pediatric pyeloplasty. MATERIALS AND METHODS: After receiving institutional review board approval we examined the records of all children who underwent initial pyeloplasty from December 2001 to December 2009. We compared unstented and stented pyeloplasties, and each surgical approach in the unstented group. RESULTS: During the study period 367 pyeloplasties were performed, including 231 unstented and 136 stented procedures. When comparing unstented to stented pyeloplasties, there was no difference in the complication or failure rate. Of unstented pyeloplasties 71, 67 and 93 were done using a transperitoneal laparoscopic approach, a flank approach and dorsal lumbotomy, respectively. There were 5 failures, of which 2 were laparoscopic, 2 used a flank approach and 1 used dorsal lumbotomy (p = 0.51). A total of 31 patients, including 10 treated with a laparoscopic approach, 3 with a flank approach and 18 with dorsal lumbotomy (p = 0.02), required second procedures, of which 24 were temporary drainage for a urine leak. Univariate analysis of factors associated with secondary procedures in the unstented pyeloplasty group showed that only surgical approach was significant (p = 0.05). CONCLUSIONS: In pediatric pyeloplasty there is no significant difference in outcome between stented and unstented repairs. In unstented repairs complications may vary by surgical approach. Regardless of the approach unstented pyeloplasty is safe and effective in the pediatric population.

Cross-sectional evaluation of parental decision making factors for vesicoureteral reflux management in children.

PURPOSE: Parental decision making in children with vesicoureteral reflux has potentially become more complex with the evolution of ethnic diversity in the United States, the Internet, the publication of contradictory clinical data and the emergence of minimally invasive surgery. We performed a cross-sectional study of parental management for pediatric vesicoureteral reflux. MATERIALS AND METHODS: We administered a 26-item questionnaire to parents of children with vesicoureteral reflux seen at Texas Children's Hospital urology offices or undergoing antireflux surgery at that institution. Univariate and multivariate analysis was done on patient disease characteristics, demographics, predicted reflux duration, surgery success rate, antibiotic cessation, complication risk, financial considerations, urologist recommendations, Internet information, friend recommendations, and postoperative voiding cystourethrography, renal ultrasound and recovery. RESULTS: Enrolled in the study were 15 boys and 49 girls with a mean age of 3.5 years and a mean reflux grade of 2.8. Of the cases 37 were bilateral. Parents chose endoscopic treatment in 38 children, open ureteroneocystostomy in 9, antibiotic prophylaxis in 14 and observation without antibiotics in 3. Univariate analysis suggested that Hispanic parents rated ultrasound and financial considerations as more important than white parents (p <0.05). Multivariate analysis revealed that differences seen on univariate analysis may have been due to an association between race and income. Finally, 93.6% of parents rated urologist opinion as very or extremely important. CONCLUSIONS: Data indicate that the parents of our patients highly value the opinion of the pediatric urologist when choosing treatment for their children with vesicoureteral reflux. Despite social changes the physician-parental relationship remains critical. Differences in parental decision making may be linked to associations between race and income.

Factors associated with delayed treatment of acute testicular torsion-do demographics or interhospital transfer matter?

PURPOSE: Testicular torsion is a true urological emergency. We determined whether a delay in treatment due to hospital transfer or socioeconomic factors would impact the orchiectomy rate in children with this condition. MATERIALS AND METHODS: We retrospectively evaluated the records of boys seen at a single institution emergency department who proceeded to surgery for a diagnosis of acute testicular torsion from 2003 to 2008. Charts were reviewed for transfer status, symptom duration, race, insurance presence or absence and distance from the hospital. Orchiectomy specimens were evaluated for histological confirmation of nonviability. RESULTS: We reviewed 97 records. The orchiectomy rate in patients who were vs were not transferred to the emergency department was 47.8% vs 68.9%, respectively (p = 0.07). Symptom duration was greater in the orchiectomy group with a mean difference of 47.9 hours (p <0.01). The mean transfer delay was 1 hour 15 minutes longer in the orchiectomy group (p = 0.01). Boys who underwent orchiectomy were 2.2 years younger than those who avoided orchiectomy (p = 0.01). Multivariate analysis showed that symptom duration and distance from the hospital were the strongest predictors of orchiectomy. CONCLUSIONS: Data suggest that torsion is a time dependent event and factors that delay time to treatment lead to poorer outcomes. These factors include distance from the hospital and the time delay associated with hospital transfer.

Evaluating Outcomes of Differential Surgical Management of Nonfunctioning Upper Pole Renal Moieties in Duplex Collecting Systems.

OBJECTIVE: To evaluate the management and clinical outcomes of nonfunctioning upper pole moieties treated with either upper pole heminephrectomy or upper pole preservation with lower ureteral reconstruction at a single tertiary institution. METHODS: After Institutional Review Board (IRB) approval, patients with duplicated systems undergoing upper pole heminephrectomy, ureteroureterostomy, or common sheath ureteral reimplantation from 2012-2017 were identified. Only patients with a nonfunctioning upper pole moiety on ultrasound or renal scan were included. Patients undergoing upper pole heminephrectomy were compared to those undergoing upper pole preservation with respect to demographics, anatomic variations preoperatively, and postoperative outcomes. RESULTS: Twenty-seven (57%) patients underwent upper pole preservation with lower ureteral reconstruction; 20 (43%) patients underwent upper pole heminephrectomy. Patients undergoing lower ureteral reconstruction were older (1.63 vs 2.76 years, P = .018) and more commonly presented with lower pole vesicoureteral reflux (67% vs 25%, P = .008). No significant difference in postoperative complications was seen between the two groups. After ureteroureterostomy, one patient developed new onset symptomatic reflux to the upper pole requiring intravesical reimplantation. In the heminephrectomy group, 4 of 11 patients with ureteroceles had ureterocelectomy with concomitant lower pole reimplantation. After heminephrectomy, two additional patients required further interventions: ureterocele excision and transurethral polyp excision. CONCLUSION: For patients with nonfunctional upper poles, lower tract reconstruction is a safe alternative to upper pole heminephrectomy. No significant difference in outcomes was seen. Considering that nearly 1 of 3 of patients with upper pole heminephrectomy required additional lower urinary tract procedures, pursuing upper pole preservation with lower urinary tract reconstruction may be favorable.

Vesicoamniotic Shunting Improves Outcomes in a Subset of Prune Belly Syndrome Patients at a Single Tertiary Center.

Objective: Review outcomes of Prune Belly Syndrome (PBS) with the hypothesis that contemporary management improves mortality. Methods: A retrospective chart review of inpatient and outpatient PBS patients referred between 2000 and 2018 was conducted to assess outcomes at our institution. Data collected included age at diagnosis, concomitant medical conditions, imaging, operative management, length of follow-up, and renal function. Results: Forty-five PBS patients presented during these 18 years. Prenatal diagnoses were made in 17 (39%); 65% of these patients underwent prenatal intervention. The remaining patients were diagnosed in the infant period (20, 44%) or after 1 year of age (8, 18%). Twelve patients died from cardiopulmonary complications in the neonatal period; the neonatal mortality rate was 27%. The mean follow-up among patients surviving the neonatal period was 84 months. Forty-two patients had at least one renal ultrasound (RUS); of the 30 patients with NICU RUSs, 26 (89%) had hydronephrosis and/or ureterectasis. Of the 39 patients who underwent voiding cystourethrogram (VCUG), 28 (62%) demonstrated VUR. Fifty-nine percent had respiratory distress. Nine patients (20%) were oxygen-dependent by completion of follow up. Thirty-eight patients (84%) had other congenital malformations including genitourinary (GU) 67%, gastrointestinal (GI) 52%, and cardiac 48%. Sixteen patients (36%) had chronic kidney disease (CKD) of at least stage 3; three patients (7%) had received renal transplants. Eighty-four percent of patients had at least one surgery (mean 3.4, range 0-6). The most common was orchiopexy (71%). The next most common surgeries were vesicostomy (39%), ureteral reimplants (32%), abdominoplasty (29%), nephrectomy (25%), and appendicovesicostomy (21%). After stratifying patients according to Woodard classification, a trend for 12% improvement in mortality after VAS was noted in the Woodard Classification 1 cohort. Conclusions: PBS patients frequently have multiple congenital anomalies. Pulmonary complications are prevalent in the neonate while CKD (36%) is prevalent during late childhood. The risk of CKD increased significantly with the presence of other congenital anomalies in our cohort. Mortality in childhood is most common in infancy and may be as low as 27%. Contemporary management of PBS, including prenatal interventions, reduced the neonatal mortality rate in a subset of our cohort.

Corrigendum: Vesicoamniotic Shunting Improves Outcomes in a Subset of Prune Belly Syndrome Patients at a Single Tertiary Center.

[This corrects the article DOI: 10.3389/fped.2018.00180.].

Evaluating Natural History and Follow Up Strategies for Non-obstructive Urolithiasis in Pediatric Population.

Objective: While small non-obstructive stones in the adult population are usually observed with minimal follow-up, the same guidelines for management in the pediatric population have not been well-studied. We evaluate the clinical outcomes of small non-obstructing kidney stones in the pediatric population to better define the natural history of the disease. Methods: In this IRB-approved retrospective study, patients with a diagnosis of kidney stones from January 2011 to March 2017 were identified using ICD9 and ICD10 codes. Patients with ureteral stones, obstruction, or stones >5 mm in size were excluded. Patients with no follow-up after initial imaging were also excluded. Patients with a history of stones or prior stone interventions were included in our population. Frequency of follow-up ultrasounds while on observation were noted and any ER visits, stone passage episodes, infections, and surgical interventions were documented. Results: Over the 6-year study period, 106 patients with non-obstructing kidney stones were identified. The average age at diagnosis was 12.5 years and the average stone size was 3.6 mm. Average follow-up was 17 months. About half of the patients had spontaneous passage of stones (54/106) at an average time of 13 months after diagnosis. Stone location did not correlate with spontaneous passage rates. Only 6/106 (5.7%) patients required stone surgery with ureteroscopy and/or PCNL at an average time of 12 months after initial diagnosis. The indication for surgery in all 6 cases was pain. 17/106 (16%) patients developed febrile UTIs and a total of 43 ER visits for stone-related issues were noted, but no patients required urgent intervention for an infected obstructing stone. Median interval for follow-up was every 6 months with renal ultrasounds, which then was prolonged to annual follow up in most cases. Conclusions: The observation of pediatric patients with small non-obstructing stones is safe with no episodes of acute obstructive pyelonephritis occurring in these patients. The sole indication for intervention in our patient population was pain, which suggests that routine follow-up ultrasounds may not be necessary for the follow-up of pediatric non-obstructive renal stones ≤5 mm in size.

Urologic Outcomes of Children With Hemorrhagic Cystitis After Bone Marrow Transplant at a Single Institution.

OBJECTIVE: To analyze clinical outcomes and the risk factors associated with genitourinary (GU) morbidity and mortality in children who present with hemorrhagic cystitis (HC) after bone marrow transplant (BMT). METHODS: A retrospective chart review of patients with HC who had undergone BMT at a single pediatric hospital from 2008 to 2015 was conducted. Demographic data, severity of hematuria, HC management, and mortality were analyzed. Bivariate analysis and binary logistic regression were performed to identify risk factors. RESULTS: Out of 43 patients who met inclusion criteria, 67.4% were male with a median age at BMT of 10.2 years (interquartile range 5.8-14.6). Percutaneous nephrostomy catheters were inserted in 5 patients for urinary diversion. All-cause mortality was 32.6% (N = 14). Intravesical retroviral therapy (P <.001), HC grade (P <.001), total Foley time (P <.001), total gross hematuria time (P <.001), total days hospitalized (P = .012), and days to most improved hematuria (P = .032) were associated with significant GU morbidity on bivariate analysis. On multivariable analysis, days to most improved hematuria was associated with significant GU morbidity odds ratio of 1.177 (1.006-1.376) (P = .042). Status of percutaneous nephrostomy was not associated with increased mortality (P = .472); however, in the multivariate model, BK viremia (P = .023), need for renal dialysis (P = .003), and presence of Foley catheter (P = .005) were associated with increased mortality. CONCLUSION: Children with HC after BMT fall in a very high-risk category with high mortality and significant GU morbidity. The presence of a Foley catheter, need for dialysis, and BK viremia are associated with increased mortality.

Posterior urethral valves: Risk factors for progression to renal failure.

INTRODUCTION: Posterior urethral valves (PUVs) are the most common etiology for congenital urethral obstruction and congenital bilateral renal obstruction. PUVs produce a spectrum of urologic and renal sequelae. Our aims were to assess outcomes of PUV patients, to determine whether vesicoureteral reflux (VUR) is a risk factor for progression to renal failure, and to identify other risk factors for poor outcomes. MATERIALS AND METHODS: We conducted a retrospective analysis of PUV patients from 2006 to 2014. Data collected included demographics, initial renal ultrasound (RUS) findings, creatinine at presentation and nadir, pre- and postoperative VUR status, presence or absence of recurrent urinary tract infections (UTIs), and surgical intervention(s). Univariate and multivariate analyses were used to determine risk factors for renal failure. RESULTS: Of 104 patients, 42.3% (44/104) were diagnosed prenatally, 31.8% (14/44) of whom underwent prenatal intervention. Postnatally, 90.4% (94/104) initially underwent transurethral resection of PUVs (TUR-PUVs). Vesicostomy was the next most common index surgery (4.8%). Forty-two percent (44/104) required >1 surgery. The predominant second surgery was repeat TUR-PUV in 16 patients. At last follow-up (mean 28.8 months after initial surgery), 20.2% had chronic kidney disease (CKD) of at least stage IIIA, and 8.6% had progressed to end-stage renal disease (ESRD). Antenatal diagnosis, prematurity, abnormal renal cortex, and loss of corticomedullary differentiation (CMD) on initial RUS were associated with CKD and ESRD on univariate analysis, as were elevated creatinine on presentation and at nadir. Presence of pre- or postoperative VUR and recurrent UTIs were associated with the need for multiple surgeries, but not with poor renal outcomes. On multivariate analysis, nadir creatinine was the only independent predictor of final renal function. CONCLUSIONS: Our finding that creatinine is the only independent risk factor for poor renal outcomes in PUV patients is consistent with the literature. The effect of VUR has been controversial, and our finding that VUR is associated with need for multiple surgeries but not with CKD or ESRD is novel. Limitations include biases inherent to retrospective studies and relatively small sample size. The majority of patients with PUVs (56.7%) required one surgery and maintained renal function with CKD II or better (79.8%) up to 2 years after initial surgery. While multiple factors were associated with poor renal outcomes, nadir creatinine was the only independent predictor. VUR and recurrent UTIs were not associated with poor renal outcomes. Longer follow-up is necessary to identify risk factors for delayed progression of renal disease.

Standardized process to improve patient flow from the Emergency Room to the Operating Room for pediatric patients with testicular torsion.

BACKGROUND: Testicular torsion (TT) remains one of the most common urological emergencies. The length of time from onset of symptoms to detorsion and degree of spermatic cord twisting are usually the most important factors for testicular damage. Therefore early presentation, accurate diagnosis, and prompt treatment are important factors for optimizing the testicular salvage rate. While delay in seeking medical attention is a common cause of testicular loss in pediatric patients with testicular torsion, delays in diagnosis and treatment can be preventable causes of testicular loss. OBJECTIVE: In this study, we aimed to develop a standardized process to improve the patient flow from the Emergency Room (ER) to Operating Room (OR) for TT patients in an academic children's hospital. STUDY DESIGN: Thirty consecutive pediatric patients with acute testicular torsion between November 2013 and July 2014 served as the control group. A scrotal pain checklist was implemented in July 2014, and 30 consecutive patients from July 2014 until April 2015 served as the study group. Perioperative parameters including times, ultrasound (US) findings, and surgical results were reviewed. RESULTS: The mean ages of the control group and the study group were similar (12.3 ± 4.9 years and 11.5 ± 5 years, respectively) (p = 0.575). ER arrival to OR time, triage completion to OR time, and scrotal US to OR time were significantly decreased in the study group (p < 0.001) (Table). Although triage time and ER arrival to scrotal US times were decreased in the study group, the differences were not significant (p = 0.071, p = 0.112, respectively). DISCUSSION: Utilizing scoring tools during the triage of patients with scrotal pain can help identify high-risk patients earlier and prevent unnecessary use of resources in an ER serving a large pediatric population. Limitations of this pilot study include the limited number of patients and the potential for the Hawthorne effect (staff awareness of the study). Additionally, we did not examine scrotal pain checklist scores for other acute scrotal diseases. This study focused on a quality improvement process for TT patients, in order to reduce ER to OR times. CONCLUSION: A standardized process with use of a scrotal pain checklist and prompt communication between the ER, Urology, and Radiology teams led to significantly reduced times from the ER to the OR. Standardized processes for pediatric patients with testicular torsion may help to improve testicular survival rates.

Surgical antibiotic practices among pediatric urologists in the United States.

PURPOSE: We hypothesized that there are practice variations in the use of surgical antibiotics by pediatric urologists in the United States. MATERIALS AND METHODS: A 31-question online survey was distributed to members of the Society of Pediatric Urology. The questionnaire examined physician preferences for surgical antibiotic use, including indications, antibiotic selection, timing of administration, and duration. RESULTS: 189 pediatric urologists responded to the survey. >85% of responders give antibiotics before open pyeloplasty, after hypospadias repair (when a urethral catheter is left in place), or perioperative or postoperative antibiotics for open neoureterocystostomy or bladder reconstructive surgery. >90% of responders do not give postoperative antibiotics to children who have undergone circumcisions, simple chordee repairs, herniorrhapies, or hydrocelectomies. For all other open, laparoscopic, and endoscopic operations, use of antibiotics varied significantly. Diverse opinions exist regarding antibiotic use, including the importance of costs, potential adverse reactions, reduction in infection risk, and antibiotic resistance. There are major differences in gentamicin dosing and timing of administration of perioperative antibiotics. CONCLUSIONS: Perioperative and postoperative antibiotics are widely used by pediatric urologists. However, there is significant practice variation in surgical antibiotic administration with regards to most areas of pediatric urology, in particular laparoscopic, endoscopic and hypospadias surgery.

Ex-premature infant boys with hypospadias are similar in size to age-matched, ex-premature infant boys without hypospadias.

OBJECTIVE: Studies have postulated that hypospadias, prematurity, and low birth weight are linked by defects in androgen signaling. To determine whether premature, hypospadiac boys are small and remain so, we compared their size at birth and at hypospadias repair to premature boys who underwent post-neonatal circumcision. METHODS: We identified premature boys admitted to Texas Children's Hospital who underwent either hypospadias repair or circumcision after 4 months of age. Age, weight, and height at birth and surgery were recorded. RESULTS: Fifty-four boys had hypospadias and 34 did not. For hypospadiac boys, the mean birth weight and age, height, and weight at surgery were lower than for boys without hypospadias. More importantly, length-for-age and weight-for-age percentiles were also lower for hypospadiac boys. When subset analysis was performed on boys younger than 2 years at surgery, however, there were no significant differences in height or weight between hypospadiac and non-hypospadiac boys. CONCLUSION: Our series suggests that premature, hypospadiac boys are born smaller than age-matched, non-hypospadiac controls. However, there were no age-corrected size differences between hypospadiac and non-hypospadiac boys at surgery. This implies that hypospadiac boys exhibit post-neonatal 'rebound' growth. Global growth deficits, if any, do not persist in hypospadiac boys.

Stenosing ureteritis in a 7-year-old boy with Henoch-Schönlein purpura nephritis: A case report and review of the literature.

INTRODUCTION: Urinary tract obstruction resulting from Henoch-Schönlein purpura (HSP) is an extremely rare urologic entity. If the genitourinary system is involved, it is primarily in the form of a focal proliferative glomerulonephritis. Stenosing disease has received little attention in the literature and treatment options are limited. Despite early intervention renal loss may be inevitable. CASE REPORT: A 7-year-old African American male presented with renal failure secondary to bilateral sclerosing ureteritis 1 month after initial presentation with HSP. There was significant disease progression and he required multiple procedures, ultimately bilateral ureterocalycostomies and a left nephrectomy. DISCUSSION: HSP is an immune-mediated necrotizing vasculitis. It can affect any organ system; however, in the genitourinary system, focal proliferative glomerulonephritis is a common manifestation, occurring in 20-90% of cases [8]. Extrarenal manifestations are rare. CONCLUSION: Ureteritis and obstruction may be late occurrences, but should be considered in all patients presenting with a history of HSP and new-onset flank pain, acute renal failure, or anuria. Families and patients should be counseled that renal impairment may be a consequence of stenosing ureteritis. Management of these patients can be complicated but surgical correction must be considered in the treatment algorithm once the disease has stabilized.