Risk factors for recurrence in pediatric urinary stone disease.

BACKGROUND: Children's urinary system stones may develop from environmental, metabolic, anatomical, and other causes. Our objective is to determine the recurrence and prognosis, demographic, clinical, and etiological characteristics of children with urolithiasis. METHODS: Medical records of patients were evaluated retrospectively. Patients' demographic data and medical history, serum/urine biochemical and metabolic analysis, blood gas analysis, stone analysis, imaging findings, and medical/surgical treatments were recorded. RESULTS: The study included 364 patients (male 187). Median age at diagnosis was 2.83 (IQR 0.83-8.08) years. The most common complaints were urinary tract infection (23%) and urine discoloration (12%). Sixty-two percent had a family history of stone disease. At least one metabolic disorder was found in 120 (88%) of 137 patients having all metabolic analyses: hypercalciuria was found in 45%, hypocitraturia in 39%, and hyperoxaluria in 37%. Anatomical abnormalities were detected in 18% of patients. Of 58 stones analyzed, 65.5% were calcium and 20.6% were cystine stones. Stone recurrence rate was 15% (55/364). Older age (> 5 years), family history of stone disease, stone size (≥ 5 mm), and urinary system anatomical abnormalities were significantly associated with stone recurrence (p = 0.027, p = 0.031, p < 0.001, and p < 0.001, respectively). In adjusted logistic regression analysis, stone size ≥ 5 mm (OR 4.85, 95% CI 2.53-9.3), presence of urinary system anatomical abnormalities (OR 2.89, 95% CI 1.44-5.78), and family history of stone disease (OR 2.41, 95% CI 1.19-4.86) had increased recurrence rate. CONCLUSIONS: All children with urolithiasis should be evaluated for factors affecting stone recurrence. Children at higher risk of recurrence need to be followed carefully.

Long Term Follow-Up Of Patients With Nonrefluxing Hydronephrosis.

BACKGOUND: The aim of this study is to examine the long-term prognosis of children with ureteropelvic junction obstruction-like hydronephrosis (UPJO-like HN). PATIENTS AND METHODS: The files of children with hydronephrosis (HN) were analyzed retrospectively. Patients with vesicoureteral reflux (VUR) and other genitourinary anomalies were excluded. The final status of the HN, the need for surgery, and urinary tract infection (UTI) frequency were evaluated. RESULTS: The study included 219 patients with 302 renal units (RU) with HN. Surgery rate was higher in RUs with larger kidney size and parenchymal thinning (p:<0.001 for both). Hydronephrosis resolved in 113 (40.2%) RUs, improved in 66 (23.3%), unchanged in 100 (35.5%) and worsened in 4 (1.4%). The frequency of recovery and improvement was found to be less in RUs with severe HN, large kidney size, and thin parenchyma. The UTI frequency was higher in severe HN group (12.2% vs 30.6% p:<0.001). CONCLUSIONS: Children with mild HN had an excellent prognosis. Although the majority of the patients with high-grade HN had also a good prognosis, it seems important to closely follow up patients with severe HN, increased kidney size, and accompanying parenchymal thinning. Clinicians should be aware of the increased frequency of UTIs in children with severe HN.

Could urinary nerve growth factor and bladder wall thickness predict the treatment outcome of children with overactive bladder?

OBJECTIVE: Bladder wall thickness (BWTh) measurements and Nerve Growth Factor (NGF) /creatinine (Cr) values, as noninvasive tools, were found to predict daytime voiding problems in children with overactive bladder (OAB). The goal of this research was to examine if bladder wall thickness together with urine NGF/Cr could be a clinical utility in treatment outcome of OAB in children. PATIENTS AND METHODS: A total of 60 children with OAB, (Group 1; n=40) and healthy normal controls (Group 2; n=20), aged 6-14 years old were involved in this prospective study. Children were evaluated with detailed history and physical examination, including neurologic examination, and were asked to complete a self-reported questionnaire and a 3-day bladder diary with the aid of their parents. Uroflowmetry was performed in all cases. Urinary nerve growth factor levels were measured by the ELISA and BWTh was measured trans-abdominally by one uro-radiologist specialized in pediatric ultrasonography. Urinary NGF levels were normalized by urinary creatinine levels and compared among all subgroups. Children with OAB received urotherapy as first line treatment at least for three months. 18 children refractory to urotherapy received anticholinergic therapy defined as group 3. RESULTS: The median age of the study group was 10 (range 6 to 16). After urotherapy, 22 children had similar BWTh and NGF/Cr values compared to controls. (2.75 ± 1.15; 2.40 ± 1.00 mm; p=0.86 and 1.02 ± 0.10; 0.78 ± 0.15; p=0.12, respectively). After anticholinergic treatment, BWTh levels (2.25 ± 0.90; 2.40 ± 1.00 mm; p=0.94) and NGF/Cr values (0.95 ± 0.10; 0.78 ± 0.15; p=0.42, respectively) had no significantly difference compared to controls (Group 2). In receiver operating characteristic analysis, bladder wall thickness was found to have sensitivity of 85% and specificity of 84.2% (3,20 AUC ,913; 95 %) and NGF/Cr had sensitivity of 90% and specificity of 92.1% (1,595; AUC ,947; 95 %) in predicting treatment outcome in children with OAB. CONCLUSIONS: Bladder wall thickness measurements and NGF/Cr values, as noninvasive tools, could guide outcomes in the treatment of children with overactive bladder.

Clinical practice in vesicoureteral reflux with respect to EAU guidelines: A multicenter study.

PURPOSE: We designed a multicenter, retrospective study to investigate the current trends in initial management of reflux with respect to European Association of Urology (EAU) Guidelines in Urology clinics of our country. MATERIALS AND METHODS: The study group consisted of 1988 renal units (RU) of 1345 patients treated surgically due to vesicoureteral reflux (VUR) (between years 2003 and 2017) in nine different institutions. Patients were divided into two groups according to time of initial treatment and also grouped according to risk factors by "EAU guidelines on VUR." RESULTS: In this series, 1426 RUs were treated initially conservatively and 562 RUs were initially treated with surgery. In initially surgically treated group, success rates of surgery decreased significantly in low and moderate risk groups after 2013 (P = .046, P = .0001, respectively), while success rates were not significantly different in high risk group (P = .46). While 26.6% of patients in low risk group were initially surgically treated before 2013, this rate has increased to 34.6% after 2013, but the difference was not statistically significant (P = .096). However, performing surgery as the initial treatment approach increased significantly in both moderate and high risk groups (P = .000 and P = .0001, respectively) after 2013. Overall success rates of endoscopic treatments and ureteroneocystostomy (UNC) operations were 65% and 92.9% before 2013 and 60% and 78.5% after 2013, respectively. Thus, the overall success rate for surgery was 72.6%. There was significant difference between success rates of UNC operations before and after 2013 (P = .000), while the difference was not significant in the injection group (P = .076). CONCLUSION: Current trends in management of reflux in our country do not yet follow the EAU guidelines on VUR in low and moderate risk groups despite these reliable and accepted guidelines were expected to influence our daily approach.

Transscrotal Near Infrared Spectroscopy as a Diagnostic Test for Testis Torsion in Pediatric Acute Scrotum: A Prospective Comparison to Gold Standard Diagnostic Test Study.

PURPOSE: A rapid test for testicular torsion in children may obviate the delay for testicular ultrasound. In this study we assessed testicular tissue percent oxygen saturation (%StO2) measured by transscrotal near infrared spectroscopy as a diagnostic test for pediatric testicular torsion. MATERIALS AND METHODS: This was a prospective comparison to a gold standard diagnostic test study that evaluated near infrared spectroscopy %StO2 readings to diagnose testicular torsion. The gold standard for torsion diagnosis was standard clinical care. From 2013 to 2015 males with acute scrotum for more than 1 month and who were less than 18 years old were recruited. Near infrared spectroscopy %StO2 readings were obtained for affected and unaffected testes. Near infrared spectroscopy Δ%StO2 was calculated as unaffected minus affected reading. The utility of near infrared spectroscopy Δ%StO2 to diagnose testis torsion was described with ROC curves. RESULTS: Of 154 eligible patients 121 had near infrared spectroscopy readings. Median near infrared spectroscopy Δ%StO2 in the 36 patients with torsion was 2.0 (IQR -4.2 to 9.8) vs -1.7 (IQR -8.7 to 2.0) in the 85 without torsion (p=0.004). AUC for near infrared spectroscopy as a diagnostic test was 0.66 (95% CI 0.55-0.78). Near infrared spectroscopy Δ%StO2 of 20 or greater had a positive predictive value of 100% and a sensitivity of 22.2%. Tanner stage 3-5 cases without scrotal edema or with pain for 12 hours or less had an AUC of 0.91 (95% CI 0.86-1.0) and 0.80 (95% CI 0.62-0.99), respectively. CONCLUSIONS: In all children near infrared spectroscopy readings had limited utility in diagnosing torsion. However, in Tanner 3-5 cases without scrotal edema or with pain 12 hours or less, near infrared spectroscopy discriminated well between torsion and nontorsion.

What happens to asymptomatic lower pole kidney stones smaller than 10 mm in children during watchful waiting?

BACKGROUND: The optimal management of lower pole kidney (LPK) stones in children is controversial. The aim of this study was to determine the outcomes of children with asymptomatic isolated LPK stones smaller than 10 mm during follow-up. METHODS: A total of 242 patients with 284 stones presenting at our institution between June 2004 and December 2014 with an asymptomatic, single LPK stone with a diameter of <10 mm were enrolled in the study. All children were assigned to receive first-line therapy and then categorized according to the need for medical intervention. Age, gender, stone laterality, stone size and type, associated urinary tract problems, and uncontrolled metabolic status were assessed as predictive factors of medical treatment for small (<10 mm) asymptomatic LPK stones. Stone-free rates were compared between interventions. RESULTS: The mean age and mean stone size were 9.4 ± 1.9 years and 7.4 ± 0.6 mm at admission, respectively. Stone progression rate was 61.2%, and the mean time for intervention was 19.2 ± 4.6 months. Flexible ureterorenoscopy (n = 68) or micro-percutaneous nephrolithotomy (n = 4) were performed for 72 stones (25.4%; group 1), and extracorporeal shock wave lithotripsy was performed for 102 stones (35.9%; group 2). The stone-free rates were 81.8 and 79.3% in group 1 and 2, respectively (p > 0.05). The remaining asymptomatic stones (110, 38.8%; group 3) were managed by continued observation, and at the end of the observation time (mean 40.8 ± 20.8 months) the spontaneous passage rate was 9.1% in this group. In the multivariate analysis, stone size of >7 mm, concurrent renal anomalies, and stones composed of magnesium ammonium phosphate (struvite) and cystine were statistically significant predictors of the need for intervention. CONCLUSIONS: Children with stones larger than 7 mm, renal anomalies, or stones composed of metabolically active cystine or struvite are more likely to require intervention, and those with asymptomatic LPK stones smaller than 10 mm can be managed by continued observation.

LRIG2 mutations cause urofacial syndrome.

Urofacial syndrome (UFS) (or Ochoa syndrome) is an autosomal-recessive disease characterized by congenital urinary bladder dysfunction, associated with a significant risk of kidney failure, and an abnormal facial expression upon smiling, laughing, and crying. We report that a subset of UFS-affected individuals have biallelic mutations in LRIG2, encoding leucine-rich repeats and immunoglobulin-like domains 2, a protein implicated in neural cell signaling and tumorigenesis. Importantly, we have demonstrated that rare variants in LRIG2 might be relevant to nonsyndromic bladder disease. We have previously shown that UFS is also caused by mutations in HPSE2, encoding heparanase-2. LRIG2 and heparanase-2 were immunodetected in nerve fascicles growing between muscle bundles within the human fetal bladder, directly implicating both molecules in neural development in the lower urinary tract.

Diagnosing Testicular Torsion before Urological Consultation and Imaging: Validation of the TWIST Score.

PURPOSE: The TWIST (Testicular Workup for Ischemia and Suspected Torsion) score uses urological history and physical examination to assess risk of testis torsion. Parameters include testis swelling (2 points), hard testis (2), absent cremasteric reflex (1), nausea/vomiting (1) and high riding testis (1). While TWIST has been validated when scored by urologists, its diagnostic accuracy among nonurological providers is unknown. We assessed the usefulness of the TWIST score when determined by nonurological nonphysician providers, mirroring emergency room evaluation of acute scrotal pain. MATERIALS AND METHODS: Children with unilateral acute scrotum were prospectively enrolled in a National Institutes of Health clinical trial. After undergoing basic history and physical examination training, emergency medical technicians calculated TWIST score and determined Tanner stage per pictorial diagram. Clinical torsion was confirmed by surgical exploration. All data were captured into REDCap™ and ROC curves were used to evaluate the diagnostic usefulness of TWIST. RESULTS: Of 128 patients (mean age 11.3 years) 44 (13.0 years) had torsion. TWIST score cutoff values of 0 and 6 derived from ROC analysis identified 31 high, 57 intermediate and 40 low risk cases (positive predictive value 93.5%, negative predictive value 100%). CONCLUSIONS: TWIST score assessed by nonurologists, such as emergency medical technicians, is accurate. Low risk patients do not require ultrasound to rule out torsion. High risk patients can proceed directly to surgery, with more than 50% avoiding ultrasound. In the future emergency medical technicians and/or emergency room triage personnel may be able to calculate TWIST score to guide radiological evaluation and immediate surgical intervention at initial assessment long before urological consultation.

Can the success of structured therapy for giggle incontinence be predicted?

INTRODUCTION: To evaluate possible factors that can guide the clinician to predict potential cases refractoriness to medical treatment for giggle incontinence (GI) and to examine the effectiveness of different treatment modalities. MATERIAL AND METHODS: The data of 48 children referred to pediatric urology outpatient clinic between 2000 and 2013 diagnosed as GI were reviewed. Mean age, follow-up, GI frequency, associated symptoms, medical and family history were noted. Incontinence frequency differed between several per day to less than once weekly. Children were evaluated with uroflowmetry-electromyography and post-void residual urine. Clinical success was characterized as a full or partial response, or nonresponse as defined by the International Children's Continence Society. Univariate analysis was used to find potential factors including age, sex, familial history, GI frequency, treatment modality and dysfunctional voiding to predict children who would possibly not respond to treatment. RESULTS: Mean age of the patients was 8.4 years (range 5 to 16). Mean follow-up time and mean duration of asymptomatic period were noted as 6.7±1.4 years and 14.2±2.3 months respectively. While 12 patients were treated with only behavioral urotherapy (Group-1), 11 patients were treated with alpha-adrenergic blockers and behavioral urotherapy (Group-2) and 18 patients with methylphenidate and behavioral urotherapy (Group-3). Giggle incontinence was refractory to eight children in-group 1; six children in-group 2 and eight children in-group 3. Daily GI frequency and dysfunctional voiding diagnosed on uroflowmetry-EMG were found as outstanding predictive factors for resistance to treatment modalities. CONCLUSIONS: A variety of therapies for GI have more than 50% failure rate and a standard treatment for GI has not been established. The use of medications to treat these patients would not be recommended, as they appear to add no benefit to symptoms and may introduce severe adverse effects.

Is top-down vs bottom-up radiological evaluation after febrile urinary tract infection really less stressful for the child and family? Challenging the dogma.

PURPOSE: We evaluated whether stress levels in children and parents during radiological evaluation after febrile urinary tract infection are really lower using the top-down approach, where (99m)technetium dimercaptosuccinic acid renal scintigraphy is used initially, than the bottom-up approach, where voiding cystourethrography is initially performed and repeated examinations are easier for all. MATERIALS AND METHODS: We prospectively evaluated 120 children 3 to 8 years old. Pain ratings were obtained using the Faces Pain Scale-Revised, and conversation during the procedure was evaluated using the Child-Adult Medical Procedure Interaction Scale-Revised by 2 independent observers. To evaluate parental anxiety, the State-Trait Anxiety Inventory form was also completed. Following a documented febrile urinary tract infection children were randomized to the top-down or bottom-up group. A third group of 44 children undergoing repeat voiding cystourethrography and their parents were also evaluated. RESULTS: Child ratings of pain using the Faces Pain Scale-Revised were not significantly different between the top-down group following (99m)technetium dimercaptosuccinic acid renal scintigraphy (2.99 on a scale of 10) and the bottom-up group following voiding cystourethrography (3.21). Also the Faces Pain Scale-Revised was not significantly different in the repeat voiding cystourethrography group (3.35). On the Child-Adult Medical Procedure Interaction Scale-Revised there was negative correlation between child coping and child distress, as well as rate of child distress and adult coping promoting behavior. Parental state anxiety scores were significantly less in the top-down and repeat voiding cystourethrography groups than in the bottom-up group. CONCLUSIONS: Although the top-down approach and repeat voiding cystourethrography cause less anxiety for caregivers, these values do not correlate to pain scale in children. This finding might be due to lack of appropriate evaluation tools of pediatric pain and anxiety. However, the theory that the top-down approach is less invasive, and thus less stressful, requires further research. The Child-Adult Medical Procedure Interaction Scale-Revised data indicate that influences in adult-child interaction are bidirectional.

Does structured withdrawal of desmopressin improve relapse rates in patients with monosymptomatic enuresis?

PURPOSE: Relapse after cessation of desmopressin is an important problem in treating patients with enuresis. Structured withdrawal of desmopressin tablets has been shown to decrease relapse rates. However, scientific data are lacking on the structured withdrawal of the fast melting oral formulation of desmopressin. We compared relapse rates of structured withdrawal using placebo and direct cessation in a population of patients with enuresis who were desmopressin responders. MATERIALS AND METHODS: Patients diagnosed with enuresis and responding to desmopressin from 13 different centers were involved in the study. Patients were randomized into 4 groups. Two different structured withdrawal strategies were compared to placebo and direct withdrawal. Sample size was estimated as 240 (60 patients in each group), with a power of 0.80 and an effect size of 30%. Randomization was performed using NCSS statistical software (NCSS, Kaysville, Utah) from a single center. The relapse rates of the groups were compared using chi-square testing. Logistic regression analysis was performed to define the independent factors having an effect on relapse rates. RESULTS: Desmopressin treatment was initiated in 421 patients, and 259 patients were eligible for randomization. Relapse rates were 39 (1%) and 42 (4%) for the structured withdrawal groups, which were significantly less than for direct withdrawal (55, 3%) and placebo (53, 1%). Logistic regression analysis revealed that initial effective dose of 240 µcg, greater number of wet nights before treatment and nonstructured withdrawal were associated with higher relapse rates. CONCLUSIONS: We found that structured withdrawal with the fast melting oral formulation of desmopressin results in decreased relapse rates. Application of a structured withdrawal program was also an independent factor associated with reduced relapse rates, together with lower initial effective dose and number of wet nights per week. Relapse after cessation of desmopressin is an important problem, and in this study structured withdrawal was observed to be associated with decreased relapse rates compared to placebo and direct withdrawal.

Can factors affecting complication rates for ureteric re-implantation be predicted? Use of the modified Clavien classification system in a paediatric population.

OBJECTIVE: To determine preoperative predictive factors of postoperative complications of ureteric re-implantation in children by using the modified Clavien classification system (MCCS), which has been widely used for complication rating of surgical procedures. PATIENTS AND METHODS: In all, 383 children who underwent ureteric re-implantation for vesico-ureteric reflux (VUR) and obstructing megaureters between 2002 and 2011 were included in the study. Intravesical and extravesical ureteric re-implantations were performed in 338 and 45 children, respectively. Complications were evaluated according to the MCCS. Univariate and multivariate analyses were used to determine predictive factors affecting complication rates. RESULTS: In all, 247 girls and 136 boys were studied. The mean (sd) age was 46 (25) months and the mean (sd) follow-up was 49.4 (27.8) months. The mean (sd) hospitalisation time was 4.7 (1.6) days. Complications occurred in 76 (19.8%) children; 34 (8.9%) were MCCS grade I, 22 (5.7%) were grade II and 20 (5.2%) were grade III. Society of Fetal Urology (SFU) grade 3-4 hydronephrosis, obstructing megaureters, a tailoring-tapering and folding procedure, refractory voiding dysfunction and a duplex system were statistically significant predictors of complications on univariate analysis. Prior injection history, paraureteric diverticula, stenting, gender, age, operation technique (intra vs extravesical) were not significant predictors of complications. In the multivariate analysis refractory voiding dysfunction, a tailoring-tapering and folding procedure, obstructing megaureters (diameter of >9 mm) and a duplex system were statistically significant predictors of complications. CONCLUSION: Ureteric re-implantation remains a valid option for the treatment of certain patients with VUR. Refractory voiding dysfunction, a tailoring-tapering and folding procedure, obstructing megaureters (diameter of >9 mm) and associated duplex systems were the main predictive factors for postoperative complications. Use of a standardised complication grading system, such as the MCCS, should be encouraged to allow the valid comparison of complication rates between series.

Nocturnal lagophthalmos in children with urofacial syndrome (Ochoa): a novel sign.

The urofacial syndrome is a rare condition that occurs in both genders and characterized by uropathy and facial abnormalities. Early diagnosis is crucial for the management and prognosis of urinary problems. Paradoxical inversion of facial musculature when smiling, giving an appearance of crying associated with severe urinary tract dysfunction is typical in these patients. Although facial signs and symptoms are generally ignored and shadowed by the dominant bladder symptoms, we have recently realized a unique but constant finding in majority of these patients, nocturnal lagophthalmos which is described as inability to close the eyelids during sleep. We report 15 patients with urofacial syndrome (Ochoa) whom mostly had admitted with major urological symptoms and 12 of the cases had nocturnal lagophthalmos. Lagophthalmos may lead to keratitis, corneal abrasion, infection, vascularization, and in extreme cases, ocular perforation, endophthalmitis and loss of the eye. Basic modalities like lubricant drops during the day and ointments at night are usually enough to protect the cornea from exposure keratopathy. In moderate to severe cases, overnight taping of the lid or the use of a moisture chamber might be necessary. Majority of our patients responded to basic therapy. Conclusion Nocturnal lagophthalmos is a novel symptom described in patients with urofacial syndrome. The pediatricians and urologists should be careful about this symptom to prevent eye damage and quality of life problems.

The impact of audiovisual information on parental anxiety levels prior to hypospadias surgery: A prospective single center cohort study.

BACKGROUND: Most parents have anxiety before a pediatric surgical procedure. Parental anxiety may impair the parents' ability to cope with new or stressful situations while their children are undergoing surgery. By effectively improving the education of parents regarding the diagnosis, treatment, and potential complications of hypospadias and surgical repair, it might be feasible to reduce their anxiety during this process. OBJECTIVE: To determine whether structured audiovisual information would reduce parents' anxiety levels compared to classic verbal information. MATERIAL AND METHODS: The diagnosis was made and, treatment options were explained, and State-Trait Anxiety Inventory Form - State Anxiety (STAI-I) forms were filled out by parents at the first consultation. In the second consultation, parents were divided into structured audio-visual-information (Group-1) and classic verbal information (Group-2) groups. Following these consultations, all parents in both groups filled out STAI-I again. Parents filled out the forms for the last time on the postoperative-14th-day and the results were compared. RESULTS: A total of 124 (51.2%) parents were informed with structured-audiovisual-informational material and 118 parents (48.8%) were informed with classic verbal information. First STAI-I scores were 57.65 ± 5.17 and 56.91 ± 5.28 for Group-1 and Group-2, respectively, and there was no difference between the groups (p = 0.709). The STAI-I scores after the second consultation were 44.82 ± 5.65 and 49.42 ± 2.81 for Group-1 and Group-2, respectively. Parental anxiety decreased in both groups following the second consultation. Notably, a statistically significant superiority was found between the groups in favor of Group-1 (p = 0.001). DISCUSSION: Parents whose children will undergo hypospadias surgery experience significant anxiety. We observed that informing patients adequately and in appropriate language using a suitable method was associated with less anxiety. Preoperative parental anxiety is influenced by several variables, including parent age, parent gender, child age, lack of knowledge, and concerns over complications or pain. Although we are unable to alter the factors of child age and parent gender, we can impact parents' concerns regarding postoperative pain and anesthesia by enhancing their comprehension of the procedure via providing them of sufficient and accurate information. CONCLUSION: Providing parents with structured audio-visual information about the preoperative and postoperative period before hypospadias surgery is associated with lower parental anxiety levels. Supplying structured audiovisual information regarding the preoperative and postoperative periods can help parents have an improved comprehension of the procedure and minimize their anxiety.

Urinary stone in infants; should vitamin D prophylaxis be stopped?

BACKGROUND: This study investigated the effect of the discontinuation of vitamin D supplementation on kidney stone formation in children under 2 years of age. METHODS: This study involved a retrospective analysis of two patient groups. The first group comprised postoperative patients who were stone-free, while the second group consisted of asymptomatic patients with kidney stones. The patients who discontinued vitamin D supplementation and those who continued were compared in terms of stone formation and stone size progression. The data collected included patient characteristics, stone size measurements, and laboratory results. RESULTS: The findings showed that the discontinuation of vitamin D supplementation was not associated with kidney stone formation or the progression of stone size in either group. For patients who were stone-free on ultrasonography 1 month after the operation, according to the 12-month ultrasonography evaluation, in the group that discontinued vitamin D, 42 (78%) patients had no stones, 6 (11%) patients had stones larger than 3 mm, and 6 (11%) patients had microlithiasis. However, in the group that continued vitamin D, 49 (72%) patients were stone-free, 10 (15%) patients had stones larger than 3 mm, and 9 (13%) patients had microlithiasis. There was no difference between the groups in terms of stone status at 12 months (p = 0.76). For patients with asymptomatic kidney stones, the initial stone sizes were similar between the groups (p = 0.74). During the 6th month of ultrasound, the changes in stone size were 1.76 ± 1.81 mm and 1.79 ± 1.75 mm for the two groups, respectively (p = 0.9). During the 12-month ultrasound measurement, the changes in stone size were 1.98 ± 2.93 mm and 2.60 ± 2.48 mm for the two groups, respectively (p = 0.09). CONCLUSIONS: We believe that more research is needed to make definitive recommendations regarding vitamin D prophylaxis in infants with kidney stones. Although the first objective of our study is not conclusively proven with the current findings, we recommend continued vitamin D prophylaxis in infants with urolithiasis.

Endoscopic dilatation/incision of primary obstructive megaureter. A systematic review. On behalf of the EAU paediatric urology guidelines panel.

INTRODUCTION: Historically, ureteral reimplantation (UR) has been the gold standard for treatment of primary obstructive megaureter (POM) with declining renal function, worsening obstruction, or recurrent urinary tract infections. In infants, open surgery with reimplantation of a grossly dilated ureter into a small bladder, can be technically challenging with significant morbidity. Therefore, less invasive endoscopic management such as dilatation or incision of the ureter-vesical junction, has emerged as an alternative to reimplantation during the last decades. OBJECTIVE: To systematically evaluate the effectivity, safety, and potential benefits of endoscopic treatment (dilatation with or without balloon or incision) of POM in comparison to UR. STUDY DESIGN: A systematic review was conducted. Randomized controlled trials (RCTs), nonrandomized comparative studies (NRSs), and single-arm case series including a minimum of 20 participants and a mean follow-up more than 12 months were eligible for inclusion. RESULTS: Of 504 articles identified, 8 articles including 338 patients were eligible for inclusion (0 RCTs, 1 NRSs, and 7 case series). Age at time of surgery was minimum 15 days to a maximum of 192 months. Indications for endoscopic treatment (ET) included patients with loss of split renal function (>10%) and worsening of hydroureteronephrosis. The studies analysed reported a success rate ranging from 35% to 97%. Success was defined as stabilization of differential renal function without further procedures. A post-operative complication rate of 23-60% was reported (mostly transient haematuria, urinary tract infections and stent migration or intolerance). In 14% of the cases salvage UR following initial ET, was performed due to relapse of symptomatic POM. CONCLUSION: Endoscopic treatment for persistent or progressive POM in children is a minimally invasive alternative to UR with a long-term modest success rate. Additionally, it can be performed within a wide age span, with equal success rate and complication rates.

European Association of Urology/European Society for Paediatric Urology Guidelines on Paediatric Urology: Summary of the 2024 Updates.

BACKGROUND AND OBJECTIVE: We present an overview of the 2024 updates for the European Association of Urology (EAU)/European Society for Paediatric Urology (ESPU) guidelines on paediatric urology to offer evidence-based standards for perioperative management, minimally invasive surgery (MIS), hydrocele, congenital lower urinary tract obstruction (CLUTO), trauma/emergencies, and fertility preservation. METHODS: A broad literature search was performed for each condition. Recommendations were developed and rated as strong or weak on the basis of the quality of the evidence, the benefit/harm ratio, and potential patient preferences. KEY FINDINGS AND LIMITATIONS: Recommendations for perioperative management include points related to fasting, premedication, antibiotic prophylaxis, pain control, and thromboprophylaxis in patients requiring general anaesthesia. MIS use is increasing in paediatric urology, with no major differences observed among different MIS approaches. For hydrocele, observation is the initial approach recommended. For persistent cases, treatment varies according to the type of hydrocele. CLUTO cases should be managed in tertiary centres with multidisciplinary expertise in prenatal and postnatal management. Neonatal valve ablation remains the mainstay of treatment, but associated bladder dysfunction requires continuous treatment. Among urological traumas and emergencies, renal trauma is still an important cause of morbidity and mortality. Conservative management has become the standard approach in haemodynamically stable children. Ischaemic priapism is a medical emergency and requires stepwise management. Initial management of nonischaemic priapism is conservative. Fertility preservation in prepubertal children and adolescents has become an increasingly relevant issue owing to the ever-increasing number of cancer survivors receiving gonadotoxic therapies. A major limitation is the scarcity of relevant literature. CONCLUSIONS AND CLINICAL IMPLICATIONS: This summary of the 2024 EAU/ESPU guidelines provides updated guidance for evidence-based management of some paediatric urological conditions. PATIENT SUMMARY: We provide a summary of the updated European Association of Urology/European Society for Paediatric Urology guidelines on paediatric urology. There are recommendations on steps to take before and immediately after surgery, management of hydrocele, congenital lower urinary tract obstruction, and urological trauma/emergencies, as well as preservation of fertility. Recommendations are based on a comprehensive review of recent studies.

Update and Summary of the European Association of Urology/European Society of Paediatric Urology Paediatric Guidelines on Vesicoureteral Reflux in Children.

BACKGROUND AND OBJECTIVE: The prescriptive literature on vesicoureteral reflux (VUR) is still limited and thus the level of evidence is generally low. The aim of these guidelines is to provide a practical approach to the treatment of VUR that is based on risk analysis and selective indications for both diagnostic tests and interventions. We provide a 2023 update on the chapter on VUR in children from the European Association of Urology (EAU) and European Society for Paediatric Urology (ESPU) guidelines. METHODS: A structured literature review was performed for all relevant publications published from the last update up to March 2022. KEY FINDINGS AND LIMITATIONS: The most important updates are as follows. Bladder and bowel dysfunction (BBD) is common in toilet-trained children presenting with urinary tract infection (UTI) with or without primary VUR and increases the risk of febrile UTI and focal uptake defects on a radionuclide scan. Continuous antibiotic prophylaxis (CAP) may not be required in every VUR patient. Although the literature does not provide any reliable information on CAP duration in VUR patients, a practical approach would be to consider CAP until there is no further BBD. Recommendations for children with febrile UTI and high-grade VUR include initial medical treatment, with surgical care reserved for CAP noncompliance, breakthrough febrile UTIs despite CAP, and symptomatic VUR that persists during long-term follow-up. Comparison of laparoscopic extravesical versus transvesicoscopic ureteral reimplantation demonstrated that both are good option in terms of resolution and complication rates. Extravesical surgery is the most common approach used for robotic reimplantation, with a wide range of variations and success rates. CONCLUSIONS AND CLINICAL IMPLICATIONS: This summary of the updated 2023 EAU/ESPU guidelines provides practical considerations for the management and diagnostic evaluation of VUR in children. ADVANCING PRACTICE: For children with VUR, it is important to treat BBD if present. A practical approach regarding the duration of CAP is to consider administration until BBD resolution. PATIENT SUMMARY: We provide a summary and update of guidelines on the diagnosis and management of urinary reflux (where urine flows back up through the urinary tract) in children. Treatment of bladder and bowel dysfunction is critical, as this is common in toilet-trained children presenting with urinary tract infection.

Mutations in HPSE2 cause urofacial syndrome.

Urinary voiding dysfunction in childhood, manifesting as incontinence, dysuria, and urinary frequency, is a common condition. Urofacial syndrome (UFS) is a rare autosomal recessive disease characterized by facial grimacing when attempting to smile and failure of the urinary bladder to void completely despite a lack of anatomical bladder outflow obstruction or overt neurological damage. UFS individuals often have reflux of infected urine from the bladder to the upper renal tract, with a risk of kidney damage and renal failure. Whole-genome SNP mapping in one affected individual defined an autozygous region of 16 Mb on chromosome 10q23-q24, within which a 10 kb deletion encompassing exons 8 and 9 of HPSE2 was identified. Homozygous exonic deletions, nonsense mutations, and frameshift mutations in five further unrelated families confirmed HPSE2 as the causative gene for UFS. Mutations were not identified in four additional UFS patients, indicating genetic heterogeneity. We show that HPSE2 is expressed in the fetal and adult central nervous system, where it might be implicated in controlling facial expression and urinary voiding, and also in bladder smooth muscle, consistent with a role in renal tract morphology and function. Our findings have broader implications for understanding the genetic basis of lower renal tract malformations and voiding dysfunction.

Pilot feasibility study of transscrotal near infrared spectroscopy in the evaluation of adult acute scrotum.

PURPOSE: We investigated the usefulness of transscrotal near infrared spectroscopy that measures testicular oxygen saturation (StO2%) in the diagnosis of testicular torsion vs other causes of the acute scrotum in adults. MATERIALS AND METHODS: Between January and May 2009, 16 adult males presenting with acute scrotum underwent StO2% measurement of the affected and unaffected testis using near infrared spectroscopy followed by color Doppler ultrasound. If surgically explored, near infrared spectroscopy was performed at surgery and on postoperative day 1. Data were presented as means ± SD and/or normalized to the internal control. Cutoff value was generated for the StO2% difference between the affected testis and control side. RESULTS: Sixteen males with acute scrotum of mean duration 12 hours were enrolled. Of 11 patients with abnormal Doppler undergoing surgical exploration, mean StO2% of the affected testis at presentation and at surgery decreased from 40.09 ± 2.66 (range 36 to 44) to 37.18 ± 3.31 (range 32 to 42), respectively, while StO2% of the unaffected testis remained 63.09 (range 55 to 69) both at presentation (SD 4.72) and at surgery (SD 4.98). Of the 11 surgically explored, 4 had orchiectomy and 7 patients underwent surgical detorsion. At presentation and at surgical exploration of the detorsed group, the mean normalized difference between the StO2% values of the affected and unaffected sides was -16.12 and -25.9, respectively. A cutoff value of -11.5 was measured for the StO2% difference between the affected testis and control side at presentation in patients who underwent surgery. CONCLUSIONS: Near infrared spectroscopy identified all surgically confirmed cases of testicular torsion when affected testis StO2% was more than 11.5 units lower than the contralateral testis. In pilot testing, near infrared spectroscopy is a rapid, noninvasive, easy and safe method for the differential diagnosis of adult human testicular torsion.