Alkaloids from Phaeanthus vietnamensis with inhibitory effect on nitric oxide production lipopolysaccharide-stimulated in RAW264.7 macrophages.

The chemical study of the acidic extract of Phaeanthus vietnamensis leaves led to the isolation of one new alkaloid, vietnamine A (1) and eight known alkaloids (R,S)-2N-norberbamunine (2), grisabine (3), 1S,1'R,O,O'-dimethylgrisabine (4), dauricine (5), neothalibrine (6), vietnamine (7), xylopine (8), and argentinine (9) by NMR and MS and comparing with the data reported in the literature. Compounds 1-9 were evaluated for inhibitory NO production in RAW 264.7 macrophages, LPS-stimulated. Compounds 1-3 significantly inhibited on NO production with the IC50 values of 6.8 ± 0.9, 9.8 ± 1.0, and 7.1 ± 0.4 µg/ml, respectively.

Translating pediatric urodynamics from clinic into collaborative research: Lessons and recommendations from the UMPIRE study group.

Pediatric patients present unique challenges in the performance and interpretation of urodynamic studies. Interpretation of urodynamics to guide clinical management at an institutional level is accepted as reliable. Challenges arise however when multi-site collaborations incorporate urodynamics into study design to determine primary or secondary outcomes or to direct decision-making. Although standardized terminology has been established by ICCS, the application of this shared language to performance and interpretation of pediatric urodynamics to across multiple sites may not be intuitive or reliable. With a primary goal of defining the care necessary to protect future renal function, the UMPIRE protocol (Urologic Management to Preserve Initial REnal function) utilizes a urodynamics-based risk stratification to determine medical management for infants with myelomeningocele. Iterative changes in the protocol are based upon the clinical progress of the enrolled children. Despite a team experienced in subtleties of urodynamics and despite efforts to minimize variability across sites, the UMPIRE study group identified several areas in which the language of urodynamics required additional clarification or creation of more explicit definitions to standardize performance and interpretation across sites. This article reviews the foundations of current urodynamics practice, describes limitations and challenges unique to pediatric studies, and the shares the humble lessons learned by the UMPIRE study group on their journey toward standardized urodynamic language for management of infants and children with myelomeningocele.

Urodynamic characteristics of neurogenic bladder in newborns with myelomeningocele and refinement of the definition of bladder hostility: Findings from the UMPIRE multi-center study.

INTRODUCTION: Infants with myelomeningocele are at risk for chronic kidney disease caused by neurogenic bladder dysfunction. Urodynamic evaluation plays a key role to risk stratify individuals for renal deterioration. OBJECTIVE: To present baseline urodynamic findings from the Urologic Management to Preserve Initial Renal function for young children with spina bifida (UMPIRE) protocol, to present the process that showed inadequacies of our original classification scheme, and to propose a refined definition of bladder hostility and categorization. STUDY DESIGN: The UMPIRE protocol follows a cohort of newborns with myelomeningocele at nine children's hospitals in the United States. Infants are started on clean intermittent catheterization shortly after birth. If residual volumes are low and there is no or mild hydronephrosis, catheterization is discontinued. Baseline urodynamics are obtained at or before 3 months of age to determine further management. Based on protocol-specific definitions, urodynamic studies were reviewed by the clinical site in addition to a central review team; and if necessary, by all site urologists to achieve 100% concurrence. RESULTS: We reviewed 157 newborn urodynamic studies performed between May 2015 and September 2017. Of these 157 infants, 54.8% were boys (86/157). Myelomeningocele closure was performed in-utero in 18.4% (29/157) and postnatally in 81.5% (128/157) of newborns. After primary review, reviewers agreed on overall bladder categorization in 50% (79/157) of studies. Concurrence ultimately reached 100% with further standardization of interpretation. We found that it was not possible to reliably differentiate a bladder contraction due to detrusor overactivity from a volitional voiding contraction in an infant. We revised our categorization system to group the "normal" and "safe" categories together as "low risk". Additionally, diagnosis of detrusor sphincter dyssynergia (DSD) with surface patch electrodes could not be supported by other elements of the urodynamics study. We excluded DSD from our revised high risk category. The final categorizations were high risk in 15% (23/157); intermediate risk in 61% (96/157); and low risk in 24% (38/157). CONCLUSION: We found pitfalls with our original categorization for bladder hostility. Notably, DSD could not be reliably measured with surface patch of electrodes. The effect of this change on future renal outcomes remains to be defined.

Incidence of Urinary Tract Infections in Newborns with Spina Bifida-Is Antibiotic Prophylaxis Necessary?

PURPOSE: Urinary tract infections commonly occur in patients with spina bifida and pose a risk of renal scarring. Routine antibiotic prophylaxis has been utilized in newborns with spina bifida to prevent urinary tract infections. We hypothesized that prophylaxis can safely be withheld in newborns with spina bifida until clinical assessment allows for risk stratification. MATERIALS AND METHODS: Newborns with myelomeningocele at 9 institutions were prospectively enrolled in the UMPIRE study and managed by a standardized protocol with a strict definition of urinary tract infection. Patient data were collected regarding details of reported urinary tract infection, baseline renal ultrasound findings, vesicoureteral reflux, use of clean intermittent catheterization and circumcision status in boys. Risk ratios and corresponding 95% confidence intervals were calculated using log-binomial models. RESULTS: From February 2015 through August 2019 data were available on 299 newborns (50.5% male). During the first 4 months of life, 48 newborns (16.1%) were treated for urinary tract infection with 23 (7.7%) having positive cultures; however, only 12 (4.0%) met the strict definition of urinary tract infection. Infants with grade 3-4 hydronephrosis had an increased risk of urinary tract infection compared to infants with no hydronephrosis (RR=10.1; 95% CI=2.8, 36.3). Infants on clean intermittent catheterization also had an increased risk of urinary tract infection (RR=3.3; 95% CI=1.0, 10.5). CONCLUSIONS: The incidence of a culture positive, symptomatic urinary tract infection among newborns with spina bifida in the first 4 months of life was low. Patients with high grades of hydronephrosis or those on clean intermittent catheterization had a significantly greater incidence of urinary tract infection. Our findings suggest that routine antibiotic prophylaxis may not be necessary for most newborns with spina bifida.

Prevalence of high-risk bladder categorization with prenatal and postnatal myelomeningocele repair types.

INTRODUCTION: Urologic substudies of prenatal myelomeningocele (MMC) closure have focused primarily on continence without significant clinical benefit. Fetoscopic MMC repair (FMR) is a newer form of prenatal intervention and touts added benefits to the mother, but urological outcomes have yet to be analyzed. We set out to focus on bladder safety rather than continence and examined bladder outcomes with different prenatal MMC repairs (FMR and prenatal open [POMR]) and compared bladder-risk-categorization to traditional postnatal repair (PSTNR). METHODS: An IRB-approved retrospective analysis of all patients undergoing all forms of MMC repairs with inclusion and exclusion criteria based on the MOMS trial was performed. Bladder safety assessment required initial urodynamic studies (UDS), renal bladder ultrasound (RBUS), and/or voiding cystourethrogram (VCUG) within the 1st year of life. Follow-up analyses within the cohorts required follow-up studies within 18 months after initial evaluations. Outcomes assessed included bladder-risk-categorization based on the CDC UMPIRE study (high, intermediate, and safe), hydronephrosis (HN), and vesicoureteral reflux (VUR). A single reader evaluated each UDS. RESULTS: Initial UDS in 93 patients showed that the prevalence of high-risk bladders were 35% FMR versus 36% PSTNR and 60% POMR. Follow-up UDS showed only 8% of FMR were high-risk compared to 35% POMR and 36% PSTNR. Change from initial to follow-up bladder-risk-category did not reach significance (p = .0659); however, 10% PSTNR worsened to high-risk on follow-up, compared to none in either prenatal group. Subanalysis of follow-up UDS between the prenatal cohorts also was not significant (p = .055). Only 8% of FMR worsened or stayed high-risk compared to 35% with POMR (p = .1). HN was significantly different at initial and subsequent follow up between the groups with the least in the FMR group. CONCLUSIONS: Early outcome UDS analyses demonstrated lower incidence of high-risk bladders in FMR patients with a trend toward clinically significant improvement compared to POMR in regard to all evaluated metrics. Larger, prospective, confirmatory studies are needed to further evaluate the potential benefits on FMR on bladder safety and health.

Patient and Caregiver Attitudes toward Disorders of Sex Development Nomenclature.

PURPOSE: The medical terminology applied to differences/disorders of sex development has been viewed negatively by some affected individuals. A clinical population of patients with differences/disorders of sex development and their caregivers were surveyed regarding current nomenclature, hypothesizing that those unaffiliated with support groups would have more favorable attitudes. MATERIALS AND METHODS: We recruited English and Spanish speaking patients 13 years old or older with differences/disorders of sex development and their caregivers at 5 national tertiary care clinics from July 2016 to December 2018. No diagnoses were excluded. Participants completed a survey rating terminology commonly applied to differences/disorders of sex development. Responses were compared between subgroups, including members vs nonmembers of a support group. RESULTS: Of 185 potential participants approached 133 completed the survey (72% response rate). Congenital adrenal hyperplasia (33%) was the most common diagnosis. "Variation of sex development" was the most liked term (37%) but was not liked more significantly than "disorders of sex development" (27%, p=0.16). No term was liked by a majority of respondents. "Disorders of sex development" (37%) and "intersex" (53%) were the only terms most frequently viewed unfavorably. Support group members were significantly more likely to dislike the term "intersex" (p=0.02) and to like "variation of sex development" (p=0.02). CONCLUSIONS: A clinical population of patients and their caregivers had generally neutral attitudes toward nomenclature applied to differences/disorders of sex development. Members of a support group had clearer terminology preferences. "Variation of sex development" was the most liked term, and "disorders of sex development" and "intersex" were the most disliked. No term was liked by most respondents, and no clear alternative to the present nomenclature was identified.

Open versus robot-assisted laparoscopic ureteral reimplantation: Hospital charges analysis and outcomes at a single institution.

PURPOSE: Robot-assisted laparoscopic extravesical ureteral reimplantation has previously been described as a viable minimally invasive option to open surgery. However, concerns for robotic surgery have been raised owing to assumed higher costs and heterogeneous clinical outcomes. We hypothesized that similar hospital charges and clinical outcomes occur when comparing open and robotic cases in matched cohorts. MATERIALS AND METHODS: Open and robotic reimplantation cases from 2013 to 2015 for primary vesicoureteral reflux were matched by age using 1:1 nearest neighbor matching. The matched cohorts were analyzed and compared for their direct itemized hospital charges per surgical case, complications, and clinical outcomes. RESULTS: There were 38 patients in each group after age-matching the 135 patients. Operating room charges were higher for the robotic group compared to the open group (p=0.002), whereas pharmacy and laboratory costs were lower for the robotic group. However, there were no significant differences in total overall charges between the open and robotic groups with cystoscopy or without cystoscopy (p=0.345, p=0.533), since the median hospital stay length was shorter for the robotic group (p<0.001). Clinical success rates were identical for the two groups (open 94.8% vs robotic 94.8%). There were also no significant differences in number of complications between the two cohorts. CONCLUSIONS: This is the first age-matched study comparing hospital charges and clinical outcomes of pediatric open and robotic reimplantation. While operating room charges were higher for the robotic cohort, lower hospitalization charges led to comparable overall hospital charges, as well as equivalent clinical outcomes for both cohorts. LEVEL OF STUDY: Level III (Retrospective comparative study) TYPE OF STUDY: Retrospective Study.

Testosterone versus hCG in Hypogonadotropic Hypogonadism - Comparing Clinical Effects and Evaluating Current Practice.

Background. Gonadotropin therapy is not typically used for pubertal induction in hypogonadotropic hypogonadism (HH), however, represents a promising alternative to testosterone. It can potentially lead to the maintenance of future fertility in addition to testicular growth. We compared the pubertal effects of human chorionic gonadotropin (hCG) versus testosterone in adolescent males with HH. We evaluated the current practice, among pediatric endocrinologists, to identify barriers against gonadotropin use. Methods. In this retrospective review, we compared the effect of testosterone versus hCG therapy on mean testicular volume (MTV), penile length, growth velocity, and testosterone levels. We surveyed pediatric endocrinologists at our center, using RedCap. Results. Outcomes were assessed in 52 male patients with HH (hCG, n = 4; T, n = 48) after a mean treatment duration of 13.4 (testosterone) and 13.8 months (hCG; P = .79). Final MTV was higher with hCG (8.25 mL) than testosterone (3.4 mL; P < .001). The groups did not differ in penile length, growth velocity, or testosterone levels. Survey results showed that more than half the providers were aware of the benefits of gonadotropins, however, 91% were uncomfortable prescribing hCG. Commonly reported barriers to prescribing hCG were lack of experience (62%) and insurance coverage concerns (52%). Conclusions. Larger testicular volume predicts faster induction of spermatogenesis. Since hCG promoted better testicular growth, compared to testosterone, it may potentially improve future fertility outcomes in HH patients. Our results identify an opportunity to improve current practice among pediatric endocrinologists worldwide and reduce barriers to prescribing gonadotropins in the adolescent population.

Differences in continence rates in individuals with spina bifida based on ethnicity.

BACKGROUND: Ethnic disparities in continence rates in spina bifida (SB) have been studied regionally but not nationally. National SB Patient Registry (NSBPR) data were analyzed to explore differences in prevalence of bowel and bladder continence and interventions between Hispanics/Latinos and others. METHODS: Participants 5 to 21 years were categorized into Hispanic/Latino and non-Hispanic/non-Latino. Bladder/bowel continence was defined as dry/no involuntary stool leakage during the day or none/⩽ monthly incontinence. Chi-square test, Wilcoxon Two Sample Test, and generalized estimating equation (GEE) were used for statistical analysis. RESULTS: Twenty-five percent of the 4,364 patients were Hispanic/Latino. At their most recent clinic visit, Hispanics/Latinos demonstrated lower rates of urinary continence (38.6% vs. 44.9%; p= 0.0003), bowel continence (43.9% vs. 55.8%, p< 0.0001), private insurance (p< 0.0001), bowel (p< 0.0001) or bladder surgeries (p= 0.0054), and more vesicostomies (p= 0.0427) compared to others. In multiple GEE models, Hispanic/Latino participants demonstrated lower odds of bowel continence as compared to non-Hispanic/non-Latino participants (estimated odds ratio, 0.82, 95% CI, 0.72-0.94, p= 0.0032). CONCLUSIONS: After controlling for covariates, Hispanics/Latinos with SB are less likely to report bowel continence. Clinicians are encouraged to consider the risk of negative health disparities for Hispanic patients with SB and work to mitigate this risk.

Clinical pregnancy following GnRH agonist administration in the luteal phase of fresh or frozen assisted reproductive technology (ART) cycles: Systematic review and meta-analysis.

OBJECTIVES: To study if the GnRH agonist administration in luteal phase improves clinical pregnancy rate of fresh and frozen embryo transfer. Also, this meta-analysis compares the treatment effect of luteal GnRH agonist administration between long agonist and antagonist protocols of fresh cycles, and between two types of treatment: fresh and frozen embryo transfers. STUDY DESIGN: Systematic review and meta-analysis (registration number CRD42017059152). RESULTS: For the overall 20 studies (5497 patients), clinical pregnancy rate significantly increased in group of GnRH agonist administration compared to control group (RR 1.24, 95% CI 1.14-1.34, p < 0.0001). Regarding the treatment effect of luteal GnRH agonist administration between long agonist and antagonist protocol fresh cycles, no significant difference was observed (RR = 1.28, 95% CI 0.98-1.67, p = 0.07). Also, in comparison between fresh and frozen embryo transfer, similar effect of GnRH agonist administration was found (RR = 0.93, 95% CI 0.74-1.16, p = 0.49). CONCLUSIONS: There is evidence that GnRH agonist administration in luteal phase improve clinical pregnancy rate in both fresh and frozen cycles. Within fresh cycles, no significant difference of clinical pregnancy rate is found between two protocols. In frozen cycles, the effect of GnRH agonist administration in enhancing clinical pregnancy rate is similar to fresh cycles.

Hormones and their Structural and Functional Effects on the Brain: How Can We Change our Practice Moving Forward?

Is hormone treatment an invasive procedure? In this paper, we discuss aspects related to the choice of treating disorders of sex development (DSD) using hormones. Specifically, we focus on some of the challenging issues related to this treatment and the need to establish a standard of care for the use of hormone therapy in this patient population. The objectives of this paper are to: 1) Enhance understanding of the uncertainties in the decision-making process regarding hormonal interventions to treat patients with DSD. 2) Recognize that the effects of hormonal interventions might require a consent process similar to that applied for surgical procedures. 3) Emphasize the need to establish treatment algorithms that could form the basis of a standard of care for this patient population.

Evaluating Clinical Outcomes of an Advanced Practice Provider-Led Newborn Circumcision Clinic.

OBJECTIVE: To determine the safety and efficacy of advanced practice provider (APP)-performed newborn circumcisions (NBCs), we reviewed outcomes of NBCs performed by pediatric urologists and APPs. We hypothesize comparable clinical outcomes between the groups. METHODS: All urology performed NBCs during a 5-year period were reviewed, including time surrounding implementation of the APP-led clinic. Return to emergency department (ED) rates, return to operating room (OR) rates, and intraprocedure bleeding requiring intervention were reviewed. Fisher exact and Mann-Whitney testing were utilized. RESULTS: There were no statistically significant differences in rates of intraprocedure bleeding, return to ED in 30days, return to OR for revision or other related penile surgery, or the overall number of patients with complications between the groups. Thirteen patients had complications in the APP cohort, compared to 8 in the urologist cohort. There was a difference in age and weight, with urologists performing NBCs on older and heavier patients. There was no difference in clinical outcomes between children over and under 10 pounds (4.5 kg). There was a significant difference in the need for revision circumcision when comparing children older vs younger than 30days (1.9% vs 0%, P = 0.034). CONCLUSION: An APP-led NBC clinic is both safe and feasible. The widely used age and weight cutoffs for NBC need to be further evaluated, as there was no significant difference in clinical outcomes. This practice design provides pediatric urologists more time to focus on the most complex patients, both in the clinic and OR.

Baseline Urinary Tract Imaging in Infants Enrolled in the UMPIRE Protocol for Children with Spina Bifida.

PURPOSE: The lifetime risk of renal damage in children with spina bifida is high but only limited baseline imaging data are available for this population. We evaluated a large prospective cohort of infants with spina bifida to define their baseline imaging characteristics. MATERIALS AND METHODS: The UMPIRE Protocol for Young Children with Spina Bifida is an iterative quality improvement protocol that follows a cohort of newborns at 9 United States centers. Using descriptive statistics, we report the initial baseline imaging characteristics, specifically regarding renal bladder ultrasound, cystogram and dimercaptosuccinic acid nuclear medicine scan. RESULTS: Data on 193 infants from 2015 to 2018 were analyzed. Renal-bladder ultrasound was normal in 55.9% of infants, while 40.4% had Society for Fetal Urology grade 1 to 2 hydronephrosis in at least 1 kidney, 3.7% had grade 3 to 4 hydronephrosis in either kidney and 21.8% had grade 1 or higher bilateral hydronephrosis. There was no vesicoureteral reflux in 84.6% of infants. A third of enrolled infants underwent dimercaptosuccinic acid nuclear medicine renal scan, of whom 92.4% had no renal defects and 93.9% had a difference in differential function of less than 15%. CONCLUSIONS: The majority of infants born with spina bifida have normal baseline imaging characteristics and normal urinary tract anatomy at birth. This proactive protocol offers careful scheduled surveillance of the urinary tract with the goal of lifelong maintenance of normal renal function and healthy genitourinary development.

Complex Robotic Lower Urinary Tract Surgery in Patients with History of Open Surgery.

PURPOSE: We describe our experience with robot-assisted complex lower urinary tract reconstruction in patients with a history of open abdominal surgery. MATERIALS AND METHODS: Patients with any previous open abdominal surgery undergoing robot-assisted complex lower urinary tract reconstruction were included. Complex lower urinary tract reconstruction was defined as bladder neck reconstruction or continent catheterizable conduits or both, redo surgery at the bladder neck for persistent incontinence or any of these procedures with creation of a Malone antegrade continence enema. Ureteral and renal surgeries were excluded. Patient demographics, surgery performed, operative techniques, operative times and outcomes were assessed. RESULTS: A total of 36 patients met inclusion criteria, of whom 21 had undergone multiple laparotomies for ventriculoperitoneal shunt revision, 14 had undergone laparotomy with other adjunct procedures and 1 had undergone laparotomy with colostomy. No access injury occurred and there were 5 conversions. Mean operative time was 8.2 hours (range 4 to 12) and mean length of hospital stay was 74.9 hours (23 to 216). The first 18 cases took longer than the last 18 cases (mean 9.1 vs 7.5 hours, p = 0.002). Patients with multiple ventriculoperitoneal shunt revisions had higher conversion rates (p = 0.01) and longer mean operative times (p = 0.002). Patients with a history of multiple ventriculoperitoneal shunt revisions also had longer hospital stays (p = 0.02). CONCLUSIONS: Robot-assisted complex lower urinary tract reconstruction in patients with previous open abdominal surgery is safe and feasible. Longer operative times should be expected early in the experience of a surgeon. Patients with multiple prior ventriculoperitoneal shunt revisions had higher conversion rates and longer operative times compared to those with other indications for prior surgery.

Surgical interventions and anesthesia in the 1st year of life for lower urinary tract obstruction.

BACKGROUND: Patients with a prenatal diagnosis of lower urinary tract obstruction (LUTO) may undergo prenatal interventions, such as vesicoamniotic shunt (VAS) placement, as a temporary solution for relieving urinary tract obstruction. A recent FDA communication has raised awareness of the potential neurocognitive adverse effects of anesthesia in children. We hypothesized as to whether a prenatal LUTO staging system was predictive of the number of anesthesia events for prenatally diagnosed LUTO patients. METHODS: We retrospectively reviewed the prenatal and postnatal clinical records for patients with prenatally diagnosed LUTO from 2012 to 2015. Patients were stratified by prenatal VAS status and by LUTO disease severity according to Ruano et al. (Ultrasound Obstet Gynecol. 2016). RESULTS: 31 patients were identified with a prenatal LUTO diagnosis, and postnatal records were available for 21 patients (seven patients in each stage). When combining prenatal and postnatal anesthesia, there was a significant difference in the number of anesthesia encounters by stage (1.6, 3.7, and 6.7 for Stage I, II, and III respectively, p = .034). Upon univariate analysis, higher gestational age (GA) at birth was associated with a decreased number of anesthesia events in the first year (p = .031). CONCLUSIONS: The majority of infants with prenatally diagnosed LUTO will undergo postnatal procedures with general anesthesia exposure in the first year of life. Patients with higher prenatal LUTO severity experienced a higher number of both prenatal and postnatal anesthesia encounters. In addition, higher GA at birth was associated with fewer anesthesia encounters in the first year. LEVEL OF EVIDENCE: This is a prognostic study with Level IV evidence.

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

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

The National Spina Bifida Patient Registry: A Decade's journey.

BACKGROUND: The National Spina Bifida Patient Registry (NSBPR) was established in 2008, as a partnership between the CDC and spina bifida (SB) clinics throughout the United States. The purpose of this study is to explore the initial work of this project through a literature review of published studies from the NSBPR and provide a description of how this body of literature has developed overtime. METHODS: We included studies indexed in MEDLINE by means of PubMed from January 2009 through April 2018. Included articles were appraised to identify key themes relevant to SB demographics, interventions, and outcomes. Additionally, information regarding objectives, hypotheses, and results of each study was summarized. RESULTS: We identified 13 articles meeting inclusion criteria. These publications described findings or explored associations using NSBPR variables. They were grouped into four categories: general characteristics (4 studies), mobility and skin injury (2 studies), bowel continence (3 studies), and bladder continence (5 studies). CONCLUSIONS: The NSBPR represents one of the largest described clinical samples of individuals living with SB. The first decade of studies have focused primarily on descriptive analyses and on identifying predictors of clinical outcomes. These initial results may help develop interventions (including culturally appropriate initiatives), be a resource for developing international evidence-based standards of care and best-practices, and lead to improved outcomes for individuals living with SB globally. Additionally, the results underscore the strengths of the NSBPR's design and highlight the potential breadth of research topics that could be addressed in the future.

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

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