Recognition of Hyperinsulinaemic Hypoglycaemia in Infants with Congenital Central Hypoventilation Syndrome.

INTRODUCTION: Congenital central hypoventilation syndrome [CCHS] is a rare autosomal dominant condition due to mutations in the transcription factor PHOX2B. It is characterized by alveolar hypoventilation with symptoms of autonomic nervous system dysfunction. Hyperinsulinaemic hypoglycaemia [HH] due to glucose dysregulation caused by anomalous insulin secretion has been reported as a feature of CCHS. However, HH and glycaemic outcomes in the context of CCHS have not been characterised in longitudinal follow-up. We describe the variable phenotype of glucose dysregulation and glycaemic outcomes in children with CCHS. Case presenatation: : We report 6 children with PHOX2B mutation-positive CCHS diagnosed with HH in a national cohort from two UK Congenital Hyperinsulinism specialist centres. We describe the initial presentation, the challenges in management and glycaemic outcomes in longitudinal follow-up. All patients were term infants diagnosed with CCHS in the neonatal period, due to PHOX2B mutations and required long-term ventilation by tracheostomy. HH was diagnosed at a median age 222 days [range 36-594] with post-prandial hypoglycaemia [4/6 patients] or fasting hypoglycaemia [2/6 patients]. Two patients were treated with diazoxide monotherapy; one with diazoxide and overnight continuous gastrostomy feeds; one with acarbose and two with dietary manifestations and use of continuous glucose monitoring sensor [CGMS]. Three patients who presented earlier in the observation period demonstrated a reduction in the severity of HH over time, leading to hypoglycaemia resolution at a median age of 4.8 years [range 4.45-5.5 years]. CONCLUSION: Patients with CCHS, due to PHOX2B mutations, may experience both fasting and postprandial hypoglycemia, necessitating treatment for HH. Clinicians should screen children with CCHS for hypoglycemia symptoms to quickly identify those affected by HH, initiate prompt treatment, and prevent potential brain injury from severe hypoglycemia. The severity of hypoglycemia due to HH tends to decrease over time, with glycemic resolution potentially being achieved over several years.

Environmental sphagnum-associated fungi target thiol homeostasis to compete with Mycobacterium tuberculosis.

Mycobacterial species in nature are found in abundance in sphagnum peat bogs where they compete for nutrients with a variety of microorganisms including fungi. We screened a collection of fungi isolated from sphagnum bogs by co-culture with Mycobacterium tuberculosis (Mtb) to look for inducible expression of antitubercular agents and identified five fungi that produced cidal antitubercular agents upon exposure to live Mtb. Whole genome sequencing of these fungi followed by fungal RNAseq after Mtb exposure allowed us to identify biosynthetic gene clusters induced by co-culture. Three of these fungi induced expression of patulin, one induced citrinin expression and one induced the production of nidulalin A. The biosynthetic gene clusters for patulin and citrinin have been previously described but the genes involved in nidulalin A production have not been described before. All three of these potent electrophiles react with thiols and treatment of Mtb cells with these agents followed by Mtb RNAseq showed that these natural products all induce profound thiol stress suggesting a rapid depletion of mycothiol. The induction of thiol-reactive mycotoxins through three different systems in response to exposure to Mtb suggests that fungi have identified this as a highly vulnerable target in a similar microenvironment to that of the caseous human lesion.

Paratesticular Fibrous Pseudotumor in 8-Year-Old Male Following Scrotal Trauma.

Fibrous pseudotumors are a rare type of benign paratesticular mass that normally present to patients in their 30s secondary to local trauma. Here, we describe an 8-year-old male patient that presented with several right testicular masses following an injury 2 years prior. The masses were successfully resected in a testicle-sparing operation. This report describes the unique finding of a reactive fibroinflammatory mass in a pediatric patient, and also highlights the uncertainty of pseudotumor diagnostic criteria that need to be recognized in order to avoid unnecessary orchiectomy.

Fertility Preservation in Children and Adolescents: Where We Are and Where We Are Going.

PURPOSE OF REVIEW: This review will describe current pediatric and adolescent fertility preservation methodologies and the ethical concerns surrounding these procedures, as well as highlight recent research that may pave the way for the development of new fertility preservation options. RECENT FINDINGS: Research is ongoing to allow prepubertal patients, particularly those with testes, to be able to have biologic children in the future. Studies on sperm in vitro maturation highlight the importance of supporting the spermatogonial stem cell niche for the development of mature sperm. The live birth of a rhesus macaque from in vitro fertilization using prepubertal testicular tissue and in vivo matured sperm gives hope to future human births. For patients with ovaries, prior work has led to successful fertility but further research is underway to refine these techniques and optimize outcomes. Organoid scaffolds have shown promise when being used for in vitro oocyte maturation. For children and adolescents undergoing gonadotoxic treatment, such as chemotherapy, or hormonal treatment, such as gender-affirming hormone therapy, future fertility potential may be negatively impacted. It is recommended that fertility preservation (FP) be offered to these patients and families prior to undergoing treatment. Fertility preservation for postpubertal patients mimics that in adults. For prepubertal children, however, the options are limited and in some cases still experimental. It is essential that this work continues so that we may offer children and adolescents the right to an open future and preserve their fertility potential.

Contemporary disparities in progression to orchiopexy for cryptorchidism as reported in the Pediatric Health Information System (PHIS) database.

INTRODUCTION: When evaluating the timeliness of orchiopexy for cryptorchidism, health disparities are apparent among Hispanic and African American males and those with public insurance. Since the publication of these data, the COVID-19 pandemic has stressed our healthcare system and significantly affected the provision of pediatric urology care. OBJECTIVE: We sought to assess if certain groups were disproportionately affected in progression to orchiopexy after the diagnosis of cryptorchidism during and after the pandemic in US freestanding children's hospitals. STUDY DESIGN: Using the PHIS database, pediatric patients ≤5 years who underwent orchiopexy between January 2018 and December 2022 were retrospectively analyzed. Exclusion criteria included prematurity, retractile testes, and testicular torsion. Primary outcomes were age at orchiopexy and the proportion of individuals undergoing timely orchiopexy for cryptorchidism. RESULTS: Over the study period 3140 patients ≤5 years old underwent orchiopexy for cryptorchidism. Non-Hispanic Blacks and Hispanics were significantly less likely to have timely orchiopexy and underwent orchiopexy 2.13 and 3.60 months later compared to whites (p < 0.01). As compared to pre-COVID-19, during the pandemic the proportion of patients who had timely surgery was higher and the median age was significantly lower (p = 0.01 and p < 0.01, respectively) in white patients only. Over the study period, patients with public insurance were less likely to have timely orchiopexy and underwent orchiopexy 2.94 months later (p < 0.01) than patients with private insurance. Compared to during the pandemic, post-pandemic a significantly lower proportion of publicly insured patients have since undergone timely orchiopexy (p = 0.04). Patients in the West were less likely to have timely orchiopexy and had a higher age at time of orchiopexy (p < 0.01) than other regions. However, in the West during the pandemic, the proportion of children who had timely surgery was higher compared to pre-and post-COVID-19 (p < 0.01). DISCUSSION: Overall, regardless of insurance status, race, or location, a significant proportion of patients did not undergo timely orchiopexy. During the pandemic white patients had a lower median age and an increased proportion underwent timely orchiopexy, despite the number of orchiopexies remaining constant. Disparities in the post-COVID-19 era have been further exacerbated for publicly insured patients, who a significantly lower proportion of have since undergone timely orchiopexy. Specific efforts are required across the United States to increase timely orchiopexy for all boys. CONCLUSIONS: Progression to timely orchiopexy remains low for all boys in the era surrounding COVID-19; certain groups appear to be more adversely affected.

The Antitubercular Activities of Natural Products with Fused-Nitrogen-Containing Heterocycles.

Tuberculosis (TB) is notorious as the leading cause of death worldwide due to a single infectious entity and its causative agent, Mycobacterium tuberculosis (Mtb), has been able to evolve resistance to all existing drugs in the treatment arsenal complicating disease management programs. In drug discovery efforts, natural products are important starting points in generating novel scaffolds that have evolved to specifically bind to vulnerable targets not only in pathogens such as Mtb, but also in mammalian targets associated with human diseases. Structural diversity is one of the most attractive features of natural products. This review provides a summary of fused-nitrogen-containing heterocycles found in the natural products reported in the literature that are known to have antitubercular activities. The structurally targeted natural products discussed in this review could provide a revealing insight into novel chemical aspects with novel biological functions for TB drug discovery efforts.

Pubertal Status at the Time of Fertility Preservation in Transgender Girls.

BACKGROUND: Puberty blockade and gender-affirming hormone therapy can impair fertility. Counseling on fertility preservation is important before initiation of therapy. Our study aimed to assess Tanner staging and the presence of virilizing secondary sex characteristics at the time of sperm collection and correlate the viability of sperm with the Tanner staging and degree of virilization. METHODS: A retrospective chart review of 23 transgender girls referred to pediatric urology at our academic pediatric transgender clinic for fertility preservation counseling was performed. At the initial visit before treatment, pubertal staging along with the assessment of virilizing secondary characteristics was performed. The semen analyses were evaluated for volume, sperm concentration, total sperm count, motility, and total motile count. RESULTS: Of the first 23 transgender girls evaluated and counseled, 8 transgender females successfully produced a sperm sample for cryopreservation. The average testicular volume was 18.5 ml and ranged from 12 ml to 20 ml. The median semen parameters were at or above the 2021 World Health Organization threshold. The 8 transgender females who successfully underwent fertility preservation presented with at least 1 secondary sex characteristic. In 1 subject with Tanner stage 3 pubic hair development and a testicular volume of 12 ml, only voice change was reported. Facial hair and laryngeal prominence were not present on examination. CONCLUSION: More than a third of our patients who accepted a referral to pediatric urology agreed to produce a sperm sample for cryopreservation. All of these patients successfully preserved sperm. With counseling and early referral to pediatric urology, a high percentage of fertility preservation in transgender girls was achieved.

Inter-disciplinary provider development of an online, interactive adolescent varicocele decision aid prototype.

BACKGROUND: Decision-making regarding varicocele management can be a complex process for patients and families. However, to date, no studies have presented ways to mitigate the decisional conflict surrounding varicoceles. OBJECTIVE: To facilitate a discussion among physicians in order to develop a framework of the decision-making process regarding adolescent varicocele management, which will inform the development of the first online, interactive decision aid. MATERIALS AND METHODS: Semi-structured interviews with pediatric urologists and interventional radiologists were conducted to discuss their rationale for varicocele decision-making. Interviews were audio recorded, transcribed, and coded. Key themes were identified, grouped, and then qualitatively analyzed using thematic analysis. Utilizing the common themes identified and the Ottawa Decision Support Framework, a decision aid prototype was developed and transformed into a user-friendly website: varicoceledecisionaid.com. RESULTS: Pediatric urologists (n = 10) and interventional radiologists (n = 2) were interviewed. Key themes identified included: (1) definition/epidemiology; (2) observation as an appropriate management choice; (3) reasons to recommend repair; (4) types of repair; (5) reasons to recommend one repair over another; (6) shared decision-making; and (7) appropriate counseling. With this insight, a varicocele decision aid prototype was developed that engages patients and parents in the decision-making process. DISCUSSION AND CONCLUSIONS: This is the first interactive and easily accessible varicocele decision aid prototype developed by inter-disciplinary physicians for patients. This tool aids in decision-making surrounding varicocele surgery. It can be used before or after consultation to help families understand more about varicoceles and their repair, and why intervention may or may not be offered. It also considers a patient and family's personal values. Future studies will incorporate the patient and family perspective into the decision-making aid as well as implement and test the usability of this decision aid prototype in practice and in the wider urologic community.

Low-dose diazoxide is safe and effective in infants with transient hyperinsulinism.

OBJECTIVE: Transient hyperinsulinism (THI) is the most common form of recurrent hypoglycaemia in neonates beyond the first week of life. Although self-resolving, treatment can be required. Consensus guidelines recommend the lower end of the diazoxide 5-15 mg/kg/day range in THI to reduce the risk of adverse events. We sought to determine if doses <5 mg/kg/day of diazoxide can be effective in THI. DESIGN, PATIENTS, MEASURMENTS: Infants with THI (duration <6 months) were treated with low-dose diazoxide from October 2015 to February 2021. Dosing was based on weight at diazoxide start: 2 mg/kg/day in infants 1000-2000 g (cohort 1), 3 mg/kg/day in those 2000-3500 g (cohort 2) and 5 mg/kg/day in those >3500 g. RESULTS: A total of 73 infants with THI (77% male, 33% preterm, 52% small-for-gestational age) were commenced on diazoxide at a median age of 11 days (range 3-43) for a median duration of 4 months (0.3-6.8), with no difference between cohorts. The mean effective diazoxide dose was 3 mg/kg/day (range 1.5-10); 35% (26/73) required an increase from their starting dose, including 60% (9/15) of cohort 1. There was no association between perinatal stress risk factors or treatment-related characteristics and dose increase. Adverse events occurred in 13 patients (18%); oedema (12%) and hyponatraemia (5%) were the most common. Two infants developed suspected necrotising enterocolitis (NEC); none had pulmonary hypertension. CONCLUSION: Diazoxide doses <5 mg/kg/day are effective in THI. While the nature of the association between diazoxide and NEC was unclear, other adverse events were mild. We suggest considering starting doses as low as 2-3 mg/kg/day in THI to balance the side effect risk while maintaining euglycaemia.

Does KUB play a role in the diagnosis of bladder bowel dysfunction?

INTRODUCTION: Kidney ureter bladder radiography (KUB) is widely used for the evaluation of constipation in children with bladder and bowel dysfunction (BBD); however, there is varying evidence to support its routine diagnostic use. One drawback to KUB is radiation exposure. The dangers of radiation in children are well-documented, and per As Low As Reasonably Achievable, non-beneficial radiation should be avoided. This risk is especially high in children who undergo repeated imaging in the follow up of constipation treatment. OBJECTIVE: We sought to assess the utility of KUB in diagnosing children with BBD by comparing it to four diagnostic tests and/or validated instruments: the Dysfunctional Voiding Symptom Score (DVSS), Rome IV criteria, rectal diameter on ultrasound (RD), and the Bristol Stool Form Score (BSFS). STUDY DESIGN: We prospectively enrolled a cohort of patients presenting to an academic pediatric urology practice with symptoms of BBD. Severity of stool burden on KUB (mild, moderate, or severe), RD on ultrasound (≥3.4 cm), DVSS, Rome IV, and BSFS were obtained for each patient. All imaging was interpreted by a pediatric radiologist and pediatric urologist. Primary outcomes were the association between the four diagnostic tests and KUB stool burden. Bivariate analysis of all individual variables versus KUB was performed, as well as multivariate regressions to determine if multiple measures were predictive of KUB stool burden when combined. RESULTS: Between October 2020 and May 2022, 50 patients were enrolled. All children were under the age of 18, with a median age of 8 years (IQR 3-13). 38 % were male. Median BMI-for-age-percentile was 80.8 (IQR 50.3-98.3). When comparing individual variables to KUB in bivariate analyses, it was found that RD on ultrasound is predictive of significant stool burden on KUB (p = 0.03). No other individual variables were predictive. In the multivariate analyses, no combination of tests was found to be predictive of KUB. DISCUSSION: We compared the effectiveness of four commonly used diagnostic tests in children with BBD to validate the use of KUB. In conclusion, our results support the use of RD on ultrasound as a non-radiating alternative to KUB to assess stool burden. Data also suggest that KUB for fecal load does not correlate with urinary (DVSS) or bowel (Rome IV, BSFS) symptoms in BBD, and that symptoms scores should still be used independently for diagnosis and monitoring of treatment response. CONCLUSION: In conclusion, KUB has a limited role in the diagnosis of BBD.

Treatment of bilateral encrusted ureteral stents, large volume renal stones, and ureteral stricture in a complex pediatric patient: a surgical video and case review.

Myotonic dystrophy is a debilitating genetic disease that carries a predilection for a variety of comorbidities. Kidney stone disease in this population can present a variety of unique challenges related to patient age, comorbidities, and social factors. We present a video review case of a 13-year-old girl with myotonic dystrophy who was treated surgically for large bilateral stone burden, bilateral retained ureteral stents with nephrostomy tubes, and right ureteral stricture. The patient had multiple prior urologic procedures and recurrent admissions for infection prior to presentation. Preoperative planning included non-contrast CT imaging, admission to an intensive care unit, and multidisciplinary discussion of treatment and goals. Through combined antegrade and retrograde approaches, the patient's stone burden was cleared, right ureteral stricture was treated, and all tubes were able to be removed in two major procedures and one minor cystoscopy with stent removal under anesthesia. Early referral to tertiary care centers and involvement of multiple specialist teams may help reduce perioperative risk and minimize the number of surgeries. Additionally, patients at high anesthesia risk may benefit from concurrent percutaneous nephrolithotomy with endopyelotomy.

At-home use of parasacral transcutaneous electrical nerve stimulation for pediatric voiding dysfunction: a randomized controlled trial to assess its safety and feasibility.

Introduction: Treating pediatric voiding dysfunction involves behavioral changes that require significant time or medications that are often avoided or discontinued due to side effects. Using parasacral transcutaneous electrical nerve stimulation (PTENS) has shown to have reasonable efficacy, but the safety and feasibility of its off-label use for pediatric voiding dysfunction are not well-established. Concerns have also been raised over treatment adherence. In-home therapy might improve adherence compared with office-based therapy; however, no studies have evaluated in-home feasibility to date. This study aims to assess the safety and feasibility of off-label use of PTENS for pediatric voiding dysfunction. Materials and methods: A single-institution prospective, randomized controlled study was conducted from March 2019 to March 2020. Participants aged 6-18 years diagnosed with voiding dysfunction, overactive bladder, or urinary incontinence were eligible for the study. Those with known neurologic disorders, implanted electrical devices, anatomic lower urinary tract abnormality, and recurrent urinary tract infections and those taking bladder medications were excluded. Children with primary monosymptomatic nocturnal enuresis were also excluded due to previous work suggesting a lack of efficacy. Participants were randomly assigned to receive 12 weeks of urotherapy alone (control) or urotherapy plus at-home PTENS treatment. Families were contacted weekly to assess for adverse events (AEs) and treatment adherence. The primary and secondary outcomes were safety, defined as the absence of AEs and treatment adherence, respectively. Results: A total of 30 eligible participants were divided into two groups, with 15 participants in each arm. The median age was 9.4 years (interquartile range: 7.7-10.6). In total, 60% were male. Baseline demographics and urotherapy compliance were similar between the two groups. With PTENS use, two AEs were reported, including mild pruritus at the pad site and discomfort when removing pads, while no AEs were noted in the control group. In total, 60% of patients completed three 30-min sessions per week, and all participants were able to complete treatment sessions for at least 10 weeks, involving 30 min of PTENS treatment each time. Conclusion: This randomized controlled study confirms that at-home use of PTENS is feasible with reasonable treatment adherence and minimal AEs. Future collaborative, multi-institutional studies may better determine the efficacy of this treatment modality.

Assessment of readability and quality of patient education materials specific to nocturnal enuresis.

BACKGROUND: Bedwetting, or nocturnal enuresis (NE), is a common childhood disease. Families increasingly turn to free online resources for health education in order to navigate treatment options. OBJECTIVE: We aim to determine the readability and quality of online health information that families may encounter when searching for information on pediatric NE. STUDY DESIGN: The search term 'bedwetting' was queried in commonly used search engines. Included articles were further categorized as institutional/reference, commercial, non-profit/charitable, or personal. An online readability platform calculated 3 commonly used readability assessments as well as a consensus score. Quality was assessed by two independent pediatric urologists using a validated DISCERN instrument. Differences in readability were further assessed by article category type. RESULTS: 36 websites were reviewed, 3 did not include treatment options and were not included in quality assessment. 55.6% of the articles were categorized as institutional/reference, 27.8% as non-profit/charitable, 11.1% as personal, and 5.6% as commercial. The average, standard deviation, and range of readability level were as follows: overall consensus score = 9.56 ± 2.09, FK Grade Level Formula score = 9.38 ± 2.17 (range 5.8-14.1), SMOG Index score = 8.89 ± 1.79 (range 6.4-12.9), and the GF Index score = 11.86 ± 2.34 (range 8.4-16.9). The overall consensus score, 9.56, correlates to a 9th-grade reading level. 16 articles were considered to be of 'good' quality, 12 of 'fair' quality, and 5 of 'poor' quality; according to the DISCERN scoring. There was no statistically significant difference in readability scores across website categories. Two articles included the outdated treatment option of intranasal DDAVP. DISCUSSION: These findings are concordant with the increasing body of literature demonstrating that patient education materials are too difficult to read. Pediatric urologists should be aware of the readability and quality of available online content for common clinical presentations. They can ensure that institutional articles online are useful to patients by participating in the development and design of these materials. CONCLUSION: We demonstrated that online articles that families encounter via a search engine query for bedwetting are written at a higher level than recommended and the majority are of 'fair' quality. There is much room for improvement for institutions to provide high quality, readable content that supports the needs of families seeking information on NE.

The Assessment of TikTok as a Source of Quality Health Information on Varicoceles.

OBJECTIVE: To evaluate the quality of varicocele information on TikTok, a popular video platform that adolescent patients are now turning to as a source of medical information before visiting a doctor. METHODS: Using the key word "varicocele," we retrieved the top 225 videos listed on TikTok in May 2022. We extracted general video and engagement data. Video information was coded and analyzed for the completeness of different types of content (definition, symptoms, evaluation, management, and outcomes). The quality of consumer health information was rated using the DISCERN instrument. RESULTS: Thirty-six videos met inclusion criteria; 17 were created by general users, 16 by healthcare professionals or systems (2 by urologists), and 3 by scientific communities. Healthcare professionals had more views than non-healthcare (P = .05). The quality of health information using the DISCERN instrument was "poor" from health care professional videos and "very poor" from non-health care professionals. However, the quality of information provided by healthcare professionals was statistically better than that of non-healthcare (P< .05). Video content mainly focused on symptoms: 50% of videos from healthcare providers had some or extensive content and 41% of non-healthcare had some content. 23% of videos had misinformation. CONCLUSION: The overall quality of information for varicoceles on TikTok is not acceptable and does not meet patient needs. It is imperative that urologists create robust, accurate content, and partner with these platforms to connect users with higher quality information. TikTok users should also be mindful that information may not be medically accurate.

Primary Care Counseling of Parents Regarding Childhood Screen Media Use.

Parental beliefs and motivation are instrumental in improving childhood digital media use (DMU). Parents (n = 611) completed questionnaires about childhood DMU assessing knowledge, interest in counseling, motivation to change, self-efficacy, and beliefs. Less than a third correctly recognized screen time limits. Twenty-seven percent received childhood DMU information from a doctor, while 46% stated they would like such information. Only 2% had a doctor-recommended DMU plan. Interest in DMU topics, motivation to improve, and management self-efficacy were moderate. Top negative beliefs were addiction to DMU (52%), sleep problems (39%), obesity (33%), social skills (33%), and inappropriate content (32%). Differences between age categories existed for social (48%, P = .01) and language (14%, P = .01) concerns (highest for toddlers), attention concerns (27%, P = .02; highest in preschoolers), and depression (13%, P < .001) and low self-esteem (8%, P = .04; highest in teens). Findings support further development of approaches to address DMU, tailored by age-specific common parental views.

Renal Outcomes in Neonates and Infants with Transposition Physiology Undergoing Arterial Switch Procedure.

Acute kidney injury (AKI) in children with Transposition of Great arteries (TGA) undergoing Arterial Switch operation (ASO) is an important complication in the post-operative period associated with worse outcomes. AKI in children post open cardiac surgery has been well studied, with lesser data in literature pertaining to TGA and its sub-types specifically. This was a prospective, observational study enrolling infants with TGA undergoing ASO at a single center over a span of a decade from January 2010 to December 2020. The infants were followed during the duration of ICU and hospital stay, with documentation of baseline and intraoperative parameters as well as post-operative course. Out of 145 infants enrolled in the study, 83.1% developed AKI with majority (83.9%) having stage 1 AKI. Higher odds of AKI were seen in infants requiring Norepinephrine [odds ratio - 16.76 (95% CI 2.19-128.2), p < 0.001] and those who developed gram-negative infections [2.81 (1.04-7.56), p - 0.036]. Infants with AKI had significantly higher vasoactive-inotropic support at day 1 than those without AKI [16 (12.5-21.50 vs 13 (10.25-15.75), p - 0.014]. Seventeen infants in the AKI group (14%) died as opposed to none in the non-AKI group (p = 0.076). Median hours of ventilator support required were significantly higher in those with AKI than those who did not develop AKI (48 vs 45.5 p = 0.015). The infants with ASO + ASD + PDA (53% of neonates who died) were younger, had less weight at admission, more gram-negative sepsis and need for dopamine, as compared to ASO + VSD + ASD (23.5% of mortality) and ASO + ASD + VSD + aortic arch repair (23.5% of mortality). AKI in infants with TGA undergoing ASO is common and associated with poorer outcomes. In this subpopulation, AKI development is associated most commonly with hemodynamic instability and infections. This is the first study, looking at outcomes of TGA depending on the sub-types of ASO surgeries done in the infants [ASO with ASD + PDA or ASD + VSD or ASD + VSD + Arch Repair].

Bilateral Ureterovesical Junction Obstruction Resulting in Anuria in a 3-Week-Old Infant.

Bilateral megaureter with ureterovesical junction obstruction is exceedingly rare. We present a case of a 3-week-old boy who presented in acute renal failure and found to have bilateral megaureter. He was initially decompressed with bilateral percutaneous nephrostomy tubes and once stable underwent bilateral ureterostomy. At 6 months old, renal ultrasound showed stable mild right sided hydronephrosis.

Esophageal Squamous Papilloma in the Pediatric Population.

Esophageal squamous papillomas (ESP) are rare benign tumors of the esophagus, which occur mostly in the adult population. Few cases have been reported in children and due to the low incidence, the pathogenesis of ESP is not entirely understood and the management is not standardized. It is thought that mucosal irritation from underlying inflammation, perhaps from GERD, trauma or human papilloma viruses can play a role in the formation of ESP. This report describes 4 cases of pediatric ESP from a single center and discusses the management of these lesions, including the use of antacids and the human papilloma viruses vaccine as treatment modalities. Given the limited data on ESP in the pediatric population, this report aims to describe the management of this condition in 1 center.