Trends in Presentation and Management of Pediatric Renal Trauma.

Objective: We queried the Pediatric Health Information System (PHIS) to evaluate the presentation, management, and outcomes of renal trauma in children from birth to 18 years from 2007-2018.

Methods: Patients were categorized as infants (0-1 year), toddlers (2-4 years), children (5-9 years), preteen (10-14 years), and teens (15-18 years), and patient demographics, grade of injury, and mechanism of injury including sports-related trauma (SRT) were collected. Each group was then evaluated for the level of management and patient outcome.

Parental Uncertainty Scale Factor Structure in Pediatric DSD With Ambiguous Genitalia.

Objective: Illness uncertainty is a salient experience for caregivers of children with disorders/differences of sex development (DSD) presenting with ambiguous genitalia; however, no validated measure of illness uncertainty exists for this unique population. Thus, the current study aimed to preliminarily identify the factor structure of the Parental Perception of Uncertainty Scale (PPUS) in caregivers of children with DSD presenting with ambiguous genitalia and examine the convergent validity of the PPUS.

Methods: Participants included 115 caregivers (Mage = 32.

Cost comparison of intra-detrusor injection of botulinum toxin versus augmentation cystoplasty for refractory neurogenic detrusor overactivity in children.

Introduction: Treatment options for refractory neurogenic detrusor overactivity (NDO) in children include botulinum toxin type A (BTX-A) and augmentation cystoplasty (AC). Although BTX-A is accepted in contemporary pediatric urologic practice, cost and long-term outcomes data for BTX-A are limited relative to the gold standard, AC. The purpose of this study was to compare the projected 10-year costs of AC versus BTX-A.

Majority of females with a life-long experience of CAH and parents do not consider females with CAH to be intersex.

Introduction: To assess opinions of females with CAH, and parents of females with CAH, about designating this population "intersex," particularly in legislation about genital surgery during childhood.

Methods: We conducted a mixed-methods (quantitative and qualitative) anonymous cross-sectional online survey of females with CAH (46XX, 16+years old) and independently recruited parents of girls with CAH (2019-2020) diagnosed in first year of life from the United States. A multidisciplinary CAH team drafted the survey in collaboration with women with CAH and parents.

Pediatric renal cell carcinoma.

Purpose Of Review: To review the presentation, natural history and treatment of renal cell carcinoma in children and young adults with renal cell carcinoma (RCC).

Recent Findings: Complete resection of lymph nodes at the time of tumor resection can improve clinical outcomes and limit the need for adjuvant chemotherapy. Genetic alterations that lead to translocation tumors are a therapeutic target of receptor tyrosine kinase inhibitors.

Macrophage-secreted TGF-β contributes to fibroblast activation and ureteral stricture after ablation injury.

Iatrogenic injury to the healthy ureter during ureteroscope-guided ablation of malignant or nonmalignant disease can result in ureteral stricture. Transforming growth factor (TGF)-β-mediated scar formation is considered to underlie ureteral stricture, but the cellular sources of this cytokine and the sequelae preceding iatrogenic stricture formation are unknown. Using a swine model of ureteral injury with irreversible electroporation (IRE), we evaluated the cellular sources of TGF-β and scar formation at the site of injury and examined in vitro whether the effects of TGF-β could be attenuated by pirfenidone.

Robotic-Assisted Urologic Surgery in Infants: Positioning, Trocar Placement, and Physiological Considerations.

Pediatric robotic-assisted laparoscopic procedures are becoming increasingly common. They have been shown to be safe in younger patients, including infants. Successful adoption of robotic-assisted surgery in infants requires an understanding of the technical factors unique to this patient population.

Ruxolitinib inhibits cyclosporine-induced proliferation of cutaneous squamous cell carcinoma.

Organ transplant recipients (OTRs) on cyclosporine A (CSA) are prone to catastrophic cutaneous squamous cell carcinoma (SCC). Allograft-sparing, cancer-targeting systemic treatments are unavailable. We have shown increased risk for catastrophic SCC in OTRs via CSA-mediated induction of IL-22.

The Role of Non-invasive Testing in Evaluation and Diagnosis of Pediatric Lower Urinary Tract Dysfunction.

Purpose Of Review: The symptoms of lower urinary tract dysfunction (LUTD) including urinary incontinence, frequency, and urgency are among the most common reasons children are referred to pediatric urologists. Despite this, the workup for LUTD is often time consuming and a source of frustration for patients, parents, and clinicians alike. In the current review, we summarize the important role non-invasive testing plays in the diagnosis and management of children with LUTD and to show how use of these tests can help avoid the need for more invasive testing in the majority of children.

Pressure and stretch differentially affect proliferation of renal proximal tubular cells.

Renal obstruction is frequently found in adults and children. Mechanical stimuli, including pressure and stretch in the obstructed kidney, contribute to damage; animal models of obstruction are characterized by increased cellular proliferation. We were interested in the direct effects of pressure and stretch on renal tubular cell proliferation.

Linking the degree of virilization in females with congenital adrenal hyperplasia to genotype.

Mutations of CYP21A2 variably decrease 21-hydroxylase activity and result in a spectrum of disease expressions in patients with congenital adrenal hyperplasia (CAH). We examined the association between CYP21A2 mutations and virilization (Prader score) in females with CAH. The study population included 187 CAH females with fully characterized CYP21A2 mutations.

Expression of Programmed Cell Death Ligand in Cutaneous Squamous Cell Carcinoma and Treatment of Locally Advanced Disease With Pembrolizumab.

Importance: Limited therapies are available in patients with inoperable locally advanced cutaneous squamous cell carcinoma (cSCC).

Objective: To determine the efficacy of programmed cell death 1 receptor (PD-1) inhibitors in locally advanced cSCC.

Design, Setting, And Participants: A single patient with locally advanced cSCC who declined surgery and radiotherapy underwent treatment with pembrolizumab, an anti-PD-1 antibody, at an academic dermatologic surgery section and cancer center.

Urinary continence outcomes following vaginoplasty in patients with congenital adrenal hyperplasia.

Background: Feminizing genitoplasty techniques have diversified to encompass the spectrum of atypical genitalia in 46,XX congenital adrenal hyperplasia (CAH) patients. However, long-term outcomes evaluating postoperative continence following complex vaginoplasty remain scarce.

Objective: The aim was to review our surgical experience and assess postoperative urinary continence outcomes in CAH patients following complex, primary vaginoplasty.

Interleukin-22 and Cyclosporine in Aggressive Cutaneous Squamous Cell Carcinoma.

Cutaneous squamous cell carcinomas (SCCs) account for up to 10,000 deaths annually in the United States. Most of the more than 700,000 SCCs diagnosed are cured by excision with clear margins; however, metastasis can occur despite seemingly adequate treatment in some cases. Immune-suppressed organ transplant recipients are 60 to 100 times more likely to develop SCC than immune-competent individuals.

Cyclosporine A immunosuppression drives catastrophic squamous cell carcinoma through IL-22.

Immune-suppressed organ transplant recipients (OTRs) can develop catastrophic squamous cell carcinoma (SCC), characterized by multiple primary tumors, extensive body surface area involvement, or metastases. There are currently no curative systemic therapies available. We previously showed that IL-22 enhances SCC proliferation.

Hydrogel coated mesh decreases tissue reaction resulting from polypropylene mesh implant: implication in hernia repair.

Purpose: Use of polypropylene mesh (PPM) in hernia repair is associated with tissue reactivity. We examined, in a rat model, a novel non-biodegradable hydrogel coated PPM which may allow for decreased inflammation and a decreased foreign body reaction.

Methods: Through a dorsal midline incision, a 2 cm × 2 cm section of PPM (either coated or uncoated) was placed on the fascial surface 1.

Nerve Sparing Clitoroplasty is an Option for Adolescent and Adult Female Patients with Congenital Adrenal Hyperplasia and Clitoral Pain following Prior Clitoral Recession or Incomplete Reduction.

Purpose: In the past many female patients with congenital adrenal hyperplasia and atypical genitalia were surgically treated with clitoral recession or incomplete reduction of erectile bodies. We report the results of repeat clitoral surgery performed for clitoral pain or enlargement using a nerve sparing reduction clitoroplasty technique.

Materials And Methods: We identified 6 female patients with congenital adrenal hyperplasia who had undergone prior clitoral recession or incomplete reduction elsewhere.

CLIC4 regulates apical exocytosis and renal tube luminogenesis through retromer- and actin-mediated endocytic trafficking.

Chloride intracellular channel 4 (CLIC4) is a mammalian homologue of EXC-4 whose mutation is associated with cystic excretory canals in nematodes. Here we show that CLIC4-null mouse embryos exhibit impaired renal tubulogenesis. In both developing and developed kidneys, CLIC4 is specifically enriched in the proximal tubule epithelial cells, in which CLIC4 is important for luminal delivery, microvillus morphogenesis, and endolysosomal biogenesis.

Management of adolescents with congenital adrenal hyperplasia.

The management of congenital adrenal hyperplasia involves suppression of adrenal androgen production, in addition to treatment of adrenal insufficiency. Management of adolescents with congenital adrenal hyperplasia is especially challenging because changes in the hormonal milieu during puberty can lead to inadequate suppression of adrenal androgens, psychosocial issues often affect adherence to medical therapy, and sexual function plays a major part in adolescence and young adulthood. For these reasons, treatment regimen reassessment is indicated during adolescence.

Gene expression profiling of the leading edge of cutaneous squamous cell carcinoma: IL-24-driven MMP-7.

The precise mechanisms governing invasion at the leading edge of squamous cell carcinoma (SCC) and its subsequent metastasis are not fully understood. We aimed to define the cancer-related molecular changes that distinguish noninvasive tumor from invasive SCC. To this end, we combined laser capture microdissection with complementary DNA (cDNA) microarray analysis.

Pressure activates epidermal growth factor receptor leading to the induction of iNOS via NFkappaB and STAT3 in human proximal tubule cells.

Ureteral obstruction leads to increased pressure and inducible nitric oxide synthase (iNOS) expression. This study examined the involvement of epidermal growth factor (EGF) receptor (EGFR), nuclear factor-kappaB (NFkappaB), and signal transducers and activators of transcription 3 (STAT3) in iNOS induction in human proximal tubule (HKC-8) cells in response to pressure or EGF. HKC-8 cells were subjected to 60 mmHg pressure or treated with EGF for 0-36 h.