Unaided Visual Inspection for Assessment of Penile Curvature in the Clinical Setting of Hypospadias Surgery: Survey of Members of Society of Pediatric Urology (India).

Purpose: To compare the accuracy of unaided visual inspection (UVI) to Software App measurement (SAM) of penile curvature (PC) during hypospadias surgery. Methods: Seven clinical pictures of PC (15°-60°) taken during hypospadias repair were shared with 300 members of the Society of Pediatric Urology (India). The respondents were asked to assess the angles by UVI and indicate their preferred correction method of that PC. For each picture, the angles of curvature estimated by UVI were compared with the objective angle measured using an app (SAM), which was considered an accurate estimation. Statistical analysis was done using software; P<0.05 was considered as statistically significant. Results: Ninety-one of 101 (90%) respondents preferred UVI to measure PC during hypospadias surgery. For 6/7 pictures, <40% of participants estimated the angle correctly by UVI (P < 0.001), with the difference in estimation being 3.6°-14.9°. For pictures with PC >30°, the error in UVI estimation was >10°, with no correlation between the accuracy of UVI estimate and surgeon experience. A significant proportion of surgeons chose the incorrect option for PC correction, which was the lowest (69%) for PC 35.8°. Conclusions: Most surgeons preferred UVI to assess PC; UVI is an erroneous technique to measure PC angle, especially in the PC range 30°-60°, where the error was >10°. Most errors were an underestimation of the PC, irrespective of surgeon experience. There was a significant error in the choice of technique for PC correction for a PC of 35°. These results strongly support the objective assessment of PC using SAM during hypospadias repair.

A Pilot Survey of Indian Stakeholders: Parents, Doctors, and Grown-Up Patients of Disorders of Sexual Differentiation on Management Decisions and Associated Gender Dysphoria.

Background and Aims: Of late, there are many legal representations from select quarters to halt all medical interventions in children with differences of sex development (DSD). In this survey on management decisions in DSD, we distil the views of Indian stakeholders: parents, physicians, and grown-up patients with DSD on their management decisions to identify decisional satisfaction or gender dysphoria. Methods: The survey domains included the patient demographics, final diagnosis, decision on the sex of rearing, surgical interventions, opinion of the stakeholders on the preferred age of sex assignment, final sex of rearing, and agreement/disagreement about sex assignment (gender dysphoria). Results: A total of 106 responses were recorded (66% parents, 34% grown-up patients aged 12-50 years). Among parents, 65/70 (95%) preferred the sex to be assigned soon after birth. All grown-up patients preferred sex to be assigned soon after birth. Regarding decisions on surgery, 74% of physicians and 75% of the grown-up patients felt parents should be allowed to decide interventions. Among Indian parents, 90% felt they should have the right to decide surgery in the best interest of their child for a safe social upbringing. Overall, gender dysphoria among Indian DSD patients was <1% (1/103, 0.97%). Conclusions: The predominant preference and opinion of major Indian stakeholders (physicians, parents, and grown-up DSD patients) support the existing approach toward DSD management, including early sex assignment and necessary medical intervention.

Does Age Influence the Functional Recovery after Infant Pyeloplasty?

Purpose: The purpose was to study the correlation between age at surgery and functional recovery after infant pyeloplasty. Materials and Methods: All infants who underwent pyeloplasty were analyzed retrospectively in this multicenter study. Anteroposterior diameter (APD) >2 cm, split renal function (SRF) <40%, and Society of Fetal Urology (SFU) grade 3-4 were surgical indications. Based on the age at pyeloplasty, they were divided into Group 1 (1-3 months) and Group 2 (4-12 months). APD and SRF were compared before and after surgery in both groups. The fractional recoverable function (post-SRF-pre-SRF)/(50-pre-SRF) ×100 was correlated with age. Results: Fifty-one infants underwent pyeloplasty (mean age: 1.6 months - Group 1 and 7.2 months - Group 2). The mean APD decreased from 3 cm to 1.2 cm in Group 1 while 2.8 cm to 2 cm in Group 2 (P = 0.001). The mean SRF increased from 32.28% to 42.81% in Group 1 while 31%-34.18% in Group 2. SRF recovery was significantly higher in Group 1 compared to Group 2 (P = 0.001). Regression analysis showed a weak negative correlation (r = -0.2792) between age at surgery and renal function improvement. Conclusion: Functional recovery after pyeloplasty is better when done earlier (1-3 months), as this gives the growing kidney the best opportunity to recover.

Pectin/sodium alginate-based active film integrated with microcrystalline cellulose and geraniol for food packaging applications.

Biopolymer-based packaging films were prepared from pectin (PEC) and sodium alginate (SA), with the incorporation of 10 % MCC and different concentrations of geraniol (GER at 2.5, 5.0, 7.5, and 10.0 %). Rheological properties suggested that film-forming solutions and film-forming emulsions exhibited a shear-thinning or pseudo-plastic non-Newtonian behaviour. The dried films were crosslinked with 2.0 % CaCl2. The addition of MCC into PEC/SA film enhanced the TS but reduced it with the impregnation of GER without influencing the EAB and toughness of the film. The water solubility of the films significantly reduced with the rise in the GER levels but enhanced the water vapor and oxygen barrier attributes. TGA demonstrated that incorporating MCC reduced the film's thermal degradation (44.92 % to 28.81 %), but GER had an insignificant influence on the thermal stability. FTIR spectra revealed that hydrogen bond formation was positively linked with the GER addition in the film formulation. X-ray diffractograms showed that prepared films were predominantly amorphous. Antimicrobial studies showed a complete reduction of Escherichia coli and Bacillus cereus in 24 h. Overall, the composite film displayed excellent physical and active properties and PEC/SA/MCC/5.0 %GER/CaCl2 film was considered the best formulation for food packaging applications.

Utility of ultrasound-based scoring system in post-pyeloplasty recovery.

BACKGROUND/OBJECTIVE: Differentiation of uretero-pelvic junction obstruction (UPJO) from non-obstructive dilatation (NOD) is a major challenge. The aim of this retrospective study is to determine whether pyeloplasty prediction score (PPS) could predict the need for surgery and resolution after surgery. METHODS: Among patients with antenatally diagnosed hydronephrosis, those who were stable during post-natal follow-up were considered NOD. The UPJO group were the ones who worsened and underwent pyeloplasty based on conventional indications. All patients with UPJO underwent laparoscopic dismembered pyeloplasty. PPS was determined based on three ultrasound parameters obtained retrospectively: Society of Fetal Urology (SFU) grade of hydronephrosis, transverse anteroposterior (APD), and the absolute percentage difference of ipsilateral and contralateral renal lengths. RESULTS: Among 137 patients included (R:L = 59:73; M:F 102:35), 96 were conservatively managed (NOD), while 41 patients (29%) needed pyeloplasty (UPJO). Mean PPS was 4.2 (1.2) in the NOD group and it was significantly higher at 10.8 (1.63) in the UPJO group (p = 0.001). All patients with PPS > 8 needed a pyeloplasty, while two patients with PPS of 7 needed pyeloplasty due to drop in renal function. PPS cutoff value of >8 had a sensitivity 95%, specificity 100% and a likelihood ratio of 20. Post-pyeloplasty PPS resolution was proportional to the duration of follow-up. CONCLUSIONS: A PPS cutoff value of 8 or above is associated with the presence of significant UPJO. PPS is also useful in the assessment of hydronephrosis recovery post-pyeloplasty. The limitation of PPS: it can only be applied in the presence of contralateral normal kidney.

Preoperative topical estrogen application in the management of hypospadias: a systematic review.

Local estrogen therapy has been explored as an alternative to conventional testosterone therapy in children requiring urethroplasty for hypospadias. Our objective is to evaluate if preoperative estrogen stimulation reduces post-urethroplasty complications and enhances penile dimensions. A systematic search was conducted on various databases, selecting only randomized controlled trials (RCTs) that tested estrogen on hypospadias patients under 18 years. Articles underwent sorting following PRISMA guidelines and bias risk was assessed using the JBI clinical appraisal tool for RCTs. Out of 607 screened records, 10 underwent full-text review, and 4 randomized controlled trials (RCTs) were selected for analysis. The total patient cohort across studies was 387 with 174 in the estrogen group. All studies utilized topical estrogen, but in different formulations and timings. Prudence is necessary for interpreting results due to variations in formulation, timing, and hypospadias type across studies. Limited by a small number of studies and outcome presentation non-uniformity, the review suggests no change in penile dimensions or postoperative complications with topical estrogen. Further research is needed to explore wound-healing properties of estrogen in hypospadias through animal and human studies.Registration and protocol: Registered in Prospero CRD42024502183.

How effective is nephrectomy in curing hypertension in children with unilateral poorly functioning kidney? A systematic review.

PURPOSE: Some children with hypertension (HTN) have unilateral poorly functional kidney (PFK). This provides an opportunity for the clinician to cure the HTN by removal of the PFK, thereby avoiding the problems of long-term medication. However, there is sparse data in children regarding the effect of PFK nephrectomy on curing HTN. In this review, we analysed the etiology of PFK causing HTN and the effectiveness of nephrectomy in curing HTN in children. METHODS: We searched the databases to identify papers between January 2000 to December 2020 pertaining to children with PFK and HTN who underwent nephrectomy. Outcome analyzed was the resolution of HTN following nephrectomy. Duplicate publications, review articles and incomplete articles were excluded. Meta-analysis of heterogeneity was reported with I2statistics. Forest plot was constructed to compare the pooled prevalence of HTN resolution. RESULTS: Five articles with 88 patients were included. Majority (43%) of PFK were due to the unilateral atrophic kidney with or without vesicoureteral reflux (VUR); ureteropelvic junction obstruction and multicystic dysplastic kidney together accounted for 35% of cases and renovascular pathology for 22% of cases. With a follow-up of 1.5 to 3.3 years, nephrectomy was effective to cure HTN in 65.9% (95% CI 55-75%) children. CONCLUSIONS: In children with HTN and a unilateral PFK, nephrectomy cured the HTN in two-thirds of children. Unilateral atrophic kidney due to VUR was the most common cause of PFK. An increase in the utilisation of laparoscopy was observed in recent publications, hence laparoscopic nephrectomy may be considered a first choice of treatment in these children.

A Novel Large Deletion in the EVER1 Gene in a Family With Epidermodysplasia Verruciformis From India.

ABSTRACT: Epidermodysplasia verruciformis (EV) is a rare autosomal recessive genodermatosis due to mutations in EVER1 and EVER2 genes. The genetic profile of Indian patients with EV has not been previously studied. This report describes the clinical presentation and molecular analysis of a family with EV. Using genomic DNA from two affected probands and healthy controls (two other siblings), conventional polymerase chain reaction (PCR) was conducted with novel primer sets designed to amplify the coding and splice-site regions in the genes EVER1 and EVER 2 . This revealed no amplification with a primer set for exons 16 to 18 in the EVER1 gene of both the probands. Subsequently, long-range PCR spanning the length of exon 15-20 and next-generation sequencing demonstrated a homozygous deletion of 2078 bp in the EVER1 gene ( EVER1 :c.2072_2278del). Screening the family revealed the same homozygous deletion (similar to index cases) in two other affected siblings. The parents and two asymptomatic siblings were heterozygous carriers for the deletion while one healthy sibling was negative. These results were validated with Sanger sequencing. This deletion in exons 17 and 18 of the EVER1 gene results in a frameshift, followed by a premature termination resulting in a severe phenotype. The identification and validation of this large deletion was detected using stepwise amplicon-based target enrichment and long-range PCR, respectively. In this family, this simple strategy greatly enhanced genetic counseling as well as early genetic diagnosis and screening. However, functional assays and larger studies are required to characterize and validate the genetic diversity among Indians with EV.

Comparison of Urinary Biomarkers in Diagnosis of Ureteropelvic Junction Obstruction and Differentiation from Nonobstructive Dilatation.

Background and Aims: Differentiation of nonobstructive dilatation (NOD) from ureteropelvic junction obstruction (UPJO) is a challenge in children with antenatally detected hydronephrosis. The aim of this study is to compare the utility of urinary biomarkers: carbohydrate antigen (CA 19-9), neutrophil gelatinase-associated lipocalin (NGAL), and kidney injury molecule (KIM) in diagnosing UPJO. Methods: A prospective study was conducted after obtaining ethical clearance between 2021 and 2022. Group 1 - control group (n = 30): children with normal antenatal ultrasound with no urinary symptoms. Group 2 - study group (n = 48): children with unilateral hydronephrosis: Group 2a - NOD (n = 24): children stable on ultrasound and diuretic renogram and Group 2b - UPJO (n = 24): children who worsened to Grade 4 hydronephrosis on ultrasound/worsening of differential renal function (10% drop) on renogram who underwent pyeloplasty. Urinary biomarkers NGAL, KIM-1, and CA 19-9 were measured using the enzyme-linked immune absorbent assay method. Results: The urine CA 19-9 level was 128.05 ± 4.08 U/mL in the UPJO group, and this was significantly higher (P = 0.001) than NOD, 70.29 ± 4.41, and controls, 1.91 ± 1.57. The urine NGAL level was 21.41 ± 4.44 pg/mL in UPJO, and this was significantly higher than controls, 2.669 ± 0.513, but not NOD, 24.55 ± 2.67. The urine KIM level was 817 ± 15.84 pg/mL in the UPJO group, and this was significantly higher than controls, 285 ± 8.10, but not NOD, 768.23 ± 15.12. Receiver operating characteristic analysis of CA 19-9 revealed a urine biomarker cutoff of 95 U/mL for diagnosing UPJO (sensitivity 95%; specificity 96%; and area under the curve 0.99). Conclusions: CA 19-9 is a superior marker compared to NGAL and KIM in differentiating UPJO from NOD. Further studies with larger numbers are warranted.

Preoperative three-dimensional modelling and virtual reality planning aids nephron sparing surgery in a child with bilateral Wilms tumour.

Bilateral Wilms tumour (BWT) is a surgically challenging condition. Virtual reality (VR) reconstruction aids surgeons to foresee the anatomy ahead of Nephron Sparing Surgery (NSS). Three-dimensional (3D) visualisation improves the anatomical orientation of surgeons performing NSS. We herewith report a case of BWT where VR planning and 3D printing were used to aid NSS. Conventional imaging is often found to be inadequate while assessing the tumour-organ-vascular anatomy. Advances like VR and 3D printing help surgeons plan better for complex surgeries like bilateral NSS. Next-generation extended reality tools will likely aid robotic-assisted precision NSS and improve patient outcomes.

Results of Temporary Drainage of Poorly Functioning Kidneys With Ureteropelvic Junction Obstruction: Does the Histology of Persistent Poor Functioning Kidneys Indicate an Increased Risk of Hypertension?

OBJECTIVE: To review our experience with managing poorly functioning kidneys with ureteropelvic junction obstruction (PFK-UPJO) with differential renal function (DRF) <10% by a trial of temporary drainage, as the management of such kidneys is controversial. We also studied the histopathologic changes in the nephrectomy specimens of persistent PFK-UPJO, as tubulointerstitial damage may predispose to hypertension. METHODS: A retrospective review of cases undergoing treatment for unilateral UPJO over 5-year period in 2 centers was conducted. In PFK-UPJO, 4-6 weeks trial of drainage with double J stent or percutaneous nephrostomy was employed. Those kidneys that improved DRF to >10% underwent pyeloplasty, while persistent PFK underwent nephrectomy; the specimens were studied for interstitial fibrosis/tubular atrophy (IF/TA), arterial lesions, and arteriole lesions. RESULTS: Of 402 patients with unilateral UPJO that underwent surgical management, 17 (4.1%) had PFK-UPJO. After 4-6 weeks trial of drainage, 6 kidneys (35.2%) with improved DRF underwent pyeloplasty, while 11 kidneys with persistent PFK underwent nephrectomy; significant IF/TA, arterial, and arteriolar changes were noted in 9 (82%), 9 (82%), and 4 (36%) kidneys, respectively, including 7 kidneys in normotensive children. Two (11.7%) children had hypertension at presentation; 1 child remains hypertensive even after nephrectomy. CONCLUSION: In PFK-UPJO, trial of temporary drainage seems appropriate to decide plan of management; 35% of such kidneys improved function after drainage. Most persistent PFK demonstrated severe and irreversible histologic changes that may predispose to hypertension if they are preserved, and we suggest that such kidneys may be removed. Long-term follow-up of all preserved PFK-UPJO is strongly recommended.

Over Active Bladder Index (OABI): A New Objective Tool Based on Uroflow Parameters for Diagnosis of Overactive Bladder in Children.

AIMS: The aim of this study is to propose an objective uroflow based index for diagnosing over active bladder (OAB). MATERIALS AND METHODS: Thirty patients with OAB and 30 controls were used in the pilot study. Three uroflow parameters: Uroflow index (UI = Qave/Qmax), voided volume ratio (VVR = voided volume/expected bladder capacity) and time to Q max (TQmax) were studied. Linear regression was performed for the three parameters with controls/OAB patients. Further 28 patients & 32 controls were recruited in a second validation study. RESULTS: Mean (s.d) UI was 0.7 (0.1) in control while 0.4 (0.1) in OAB (p = 0.001). Mean (s.d) VVR was 0.7 (0.1) in control while 0.5 (0.1) in OAB (p = 0.01). Mean (s.d) TQmax was 5.2 (1.2) in control while 2.1 (1.8) in OAB (p = 0.001). Good correlation was noted with all parameters UI (r = 0.89); VVR (r = 0.87) and TQmax (r = 0.76); when all three were combined as an index the correlation was better (r = 0.95). Based on the beta coefficients an OAB Index [OABI = 8(UI)+9(VVR)+0.5(TQmax)] was proposed; ROC analysis revealed a cutoff of 12 (AUC 0.96). An OABI of <12 was proposed for diagnosing OAB, 12-15 for equivocal and >15 for normal children. In the validation study OABI had 93 % sensitivity; 100 % specificity; 100 % positive predictive value & 94 % negative predictive value. The overall accuracy was 97 %. CONCLUSION: While current diagnosis of OAB is mainly subjective, the proposed OABI is an objective way of diagnosing OAB using uroflow parameters. OABI may also help to assess treatment response. Further larger studies are warranted.

Clinical, Radiological and Histological Features and Their Association with Extranodal Extension in Buccoalveolar Complex Squamous Cell Carcinoma.

Jeyashanth RijuObjectives The study was aimed to (1) evaluate the effectiveness of clinical examination, intraoperative finding, and contrast-enhanced computed tomography (CECT) to detect extranodal extension (ENE) in buccoalveolar complex squamous cell carcinoma (BAOSCC), (2) to know various factors influencing ENE, and (3) to evaluate survival outcome in patients with ENE. Materials and Methods This was a retrospective cohort study, which included 137 patients with BAOSCC who underwent curative treatment between May 2019 and April 2021. Collaborative findings suggestive of ENE were noted during preoperative clinical examination, CECT, and intraoperatively, and their efficacy was compared with postoperative histopathology. Also, the various factors associated with ENE were evaluated and compared. Statistical Analysis Univariate and multivariate analysis of parameters was done using multiple logistic regression analysis and significant correlation was determined using chi-square test between ENE positive and negative categories. Analysis of prognosis and survival was done by Kaplan-Meier curve plotting using regression analysis and its significance was compared. Results The overall prevalence of ENE was 18.98% and that of lymph node involvement was 40.88%. CECT (73.1%) was found to be more sensitive in detecting ENE compared to intraoperative examination (46.2%) and clinical examination (34.6%).In comparison with clinical examination (91.9%) or CECT (78.38%), intraoperative examination (93.7%) showed the highest specificity in detecting ENE. Clinical nodal size ≥ 3 cm ( p ≤ 0.001), fixity ( p ≤ 0.001), and clinical number of nodes ( p ≤ 0.001) had significant association with ENE. The presence of thick nodal walls on CECT increased the probability of predicting ENE 15 times ( p = 0.180, confidence interval: 0.3-765.4). After a mean follow-up of 18 months, subjects without nodal positivity had a survival advantage over patients with positive lymph nodes (86.4% vs. 53.3%) and those with ENE (86.4% vs. 23.2%), respectively. Conclusion The results demonstrated that clinical examination can be used as an adjuvant to radiological imaging for prediction of ENE preoperatively. Clinical finding suggesting size of node ≥ 3 cm and ≥ 2 nodes are strong predictor of ENE, in addition to other known predictors. Patients with ENE had an unfavorable prognosis when compared with subjects with metastatic nodes without ENE. Presence of ENE remains one of the strongest factors predicting recurrence and thus poor prognosis.

A Cost-effectiveness Model Comparing Urinary Biomarkers with Diuretic Renogram in Diagnosing Ureteropelvic Junction Obstruction in Children.

Background and Aims: In this cost-effectiveness model, we compared the cost-effectiveness of commonly used urinary biomarkers with conventional diuretic renogram (DR) in diagnosing ureteropelvic junction obstruction (UPJO). We hypothesized that urinary biomarkers are as effective as DR. Methods: We used incremental cost-effectiveness ratio (ICER) as a tool for our cost-effectiveness analysis model. The cost of biomarker assay and renogram were sourced from the same center while the accuracy data of DR and urinary biomarkers from the relevant pubications. Results: As the accuracy of individual biomarker increased, the ICER also got better. As a panel of biomarker was introduced, the ICER went to the negative range suggesting cost saving as well. ICER of most urine biomarkers is currently less expensive and less effective. When a biomarker panel was applied ICER became more expensive and effective. With higher samples, test running cost is likely to go down in future and thus biomarkers are likely become less expensive and more effective. Conclusions: Individual urine biomarkers are currently less expensive and less effective compared to DR in predicting UPJO. In future, biomarker panel is likely to be more cost-effective and reduce the need for invasive renogram thus reducing the radiation exposure.