Predictors of Psychosocial Distress in Parents of Young Children with Disorders of Sex Development.

PURPOSE: We evaluated demographic, financial and support predictors of distress for parents of young children with disorders of sex development including atypical genital development, and characterized early parental experiences. This work extends our previous findings to identify those parents at risk for distress. MATERIALS AND METHODS: Participants included mothers (76) and fathers (63) of a child (78) diagnosed with disorders of sex development characterized by moderate to severe genital atypia. Parents completed a demographic questionnaire, measures of anxious and depressive symptoms, quality of life, illness uncertainty and posttraumatic stress symptoms, and rated their satisfaction with the appearance of their child's genitalia. RESULTS: Depressive and posttraumatic stress symptoms of caregivers were comparable to standardized norms while levels of anxious symptoms were below norms. A subset of parents reported clinically elevated symptoms. Overall 26% of parents reported anxious symptoms, 24% reported depressive symptoms and 17% reported posttraumatic stress symptoms. Levels of illness uncertainty were lower than those of parents of children with other chronic illnesses. Differences by parent sex emerged, with mothers reporting greater distress. Lower income, increased medical care and travel expenses, and having no other children were related to increased psychosocial distress. CONCLUSIONS: Early psychosocial screening is recommended for parents of children with disorders of sex development. Clinicians should be aware that financial burden and lack of previous parenting experience are risk factors for distress.

Uncertainty and Posttraumatic Stress: Differences Between Mothers and Fathers of Infants with Disorders of Sex Development.

Parents of children with disorders of sex development (DSD) report significant psychological distress, including posttraumatic stress symptoms (PTSS), with mothers consistently reporting higher rates of psychological distress than fathers. However, psychological factors contributing to PTSS in both parents are not well understood. The present study sought to fill this gap in knowledge by examining PTSS and illness uncertainty, a known predictor of psychological distress, in parents of children recently diagnosed with DSD. Participants were 52 mothers (Mage = 32.55 years, SD = 5.08) and 41 fathers (Mage = 35.53 years, SD = 6.78) of 53 infants (Mage = 9.09 months, SD = 6.19) with DSD and associated atypical genital development. Participants were recruited as part of a larger, multisite study assessing parents' psychosocial response to their child's diagnosis of DSD. Parents completed measures of illness uncertainty and PTSS. Mothers reported significantly greater levels of PTSS, but not illness uncertainty, than fathers, and were more likely than fathers to report clinical levels of PTSS (21.2% compared to 7.3%). Hierarchical regression revealed that parent sex, undiagnosed or unclassified DSD status, and illness uncertainty were each associated with PTSS. The overall model accounted for 23.5% of the variance associated with PTSS. Interventions targeting illness uncertainty may be beneficial for parents of children with newly diagnosed DSD.

Antimicrobial prophylaxis for children with vesicoureteral reflux.

BACKGROUND: Children with febrile urinary tract infection commonly have vesicoureteral reflux. Because trial results have been limited and inconsistent, the use of antimicrobial prophylaxis to prevent recurrences in children with reflux remains controversial. METHODS: In this 2-year, multisite, randomized, placebo-controlled trial involving 607 children with vesicoureteral reflux that was diagnosed after a first or second febrile or symptomatic urinary tract infection, we evaluated the efficacy of trimethoprim-sulfamethoxazole prophylaxis in preventing recurrences (primary outcome). Secondary outcomes were renal scarring, treatment failure (a composite of recurrences and scarring), and antimicrobial resistance. RESULTS: Recurrent urinary tract infection developed in 39 of 302 children who received prophylaxis as compared with 72 of 305 children who received placebo (relative risk, 0.55; 95% confidence interval [CI], 0.38 to 0.78). Prophylaxis reduced the risk of recurrences by 50% (hazard ratio, 0.50; 95% CI, 0.34 to 0.74) and was particularly effective in children whose index infection was febrile (hazard ratio, 0.41; 95% CI, 0.26 to 0.64) and in those with baseline bladder and bowel dysfunction (hazard ratio, 0.21; 95% CI, 0.08 to 0.58). The occurrence of renal scarring did not differ significantly between the prophylaxis and placebo groups (11.9% and 10.2%, respectively). Among 87 children with a first recurrence caused by Escherichia coli, the proportion of isolates that were resistant to trimethoprim-sulfamethoxazole was 63% in the prophylaxis group and 19% in the placebo group. CONCLUSIONS: Among children with vesicoureteral reflux after urinary tract infection, antimicrobial prophylaxis was associated with a substantially reduced risk of recurrence but not of renal scarring. (Funded by the National Institute of Diabetes and Digestive and Kidney Diseases and others; RIVUR ClinicalTrials.gov number, NCT00405704.).

Psychological Adjustment of Parents of Children Born with Atypical Genitalia 1 Year after Genitoplasty.

PURPOSE: We examined the psychological adjustment of parents of children born with moderate to severe genital atypia 12 months after their child underwent genitoplasty. MATERIALS AND METHODS: Parents were recruited longitudinally from a multicenter collaboration of 10 pediatric hospitals with specialty care for children with disorders/differences of sex development and/or congenital adrenal hyperplasia. Parents completed measures of depressive and anxious symptoms, illness uncertainty, quality of life, posttraumatic stress and decisional regret. RESULTS: Compared to levels of distress at baseline (before genitoplasty) and 6 months after genitoplasty, data from 25 mothers and 20 fathers indicated significant improvements in all psychological distress variables. However, a subset of parents continued endorsing clinically relevant distress. Some level of decisional regret was endorsed by 28% of parents, although the specific decision that caused regret was not specified. CONCLUSIONS: Overall the majority of parents were coping well 1 year after their child underwent genitoplasty. Level of decisional regret was related to having a bachelor's level of education, increased levels of illness uncertainty preoperatively and persistent illness uncertainty at 12 months after genitoplasty but was unrelated to postoperative complications.

Cryptorchidism: experience and reason.

INTRODUCTION: To characterize our contemporary clinical experience with cryptorchidism. MATERIALS AND METHODS: The records of boys referred for cryptorchidism were reviewed from 2001 to 2011. Data regarding the incidence of retractile testes, testicular ascent, surgical approach and outcomes were tabulated. Follow up was both early (< 12 weeks) and late (> 12 weeks). RESULTS: A total of 1885 patients, or 2593 testes, were identified. Eight hundred and forty-one children (45%) or 1204 testes (46%) were retractile on initial exam-57% bilateral; 187 testes (7%) later 'ascended' on re-examination and underwent surgery--15% bilateral; 1340 (85%) testes were palpable in the inguinal canal and underwent inguinal orchidopexy--98% were successful; 69 (4%) of initially palpable testes were found to be atrophic and removed; 167 (11%) testes were non-palpable and underwent laparoscopy-46 were atrophic and removed; 31 were vanishing; 33 were brought down using an inguinal approach at the same sitting with 97% success; 47 underwent staged Fowler-Stephens orchidopexy (FSO) and 10 underwent non-staged FSO, with 82% and 78% success respectively. All second stages were performed open. CONCLUSIONS: Almost half of children referred for cryptorchidism had retractile testes. Surgery for later ascent was required in 16% of testes judged to be retractile at a median age of 8 years, emphasizing the need for repeat examination. High success rates with inguinal orchidopexy were achieved, even in non-palpable testes. Testes requiring FSO were uncommonly encountered-approximately 5 testes/year or 4% of testes undergoing surgery-and success was achieved in approximately 80%.

Vesicoureteral Reflux and Antibiotic Prophylaxis: Why Cohorts and Methodologies Matter.

PURPOSE: Published cohorts of children with vesicoureteral reflux placed on antibiotic prophylaxis differ in baseline characteristics and methodology. These data have been combined in meta-analyses to derive treatment recommendations. We analyzed these cohorts in an attempt to understand the disparate outcomes reported. MATERIALS AND METHODS: A total of 18 studies were identified from 1987 to 2013. These series retrospectively or prospectively evaluated children with vesicoureteral reflux who were on long-term antibiotic prophylaxis. Presenting demographic data, criteria and methods of evaluation were tabulated. Outcomes were compared, specifically recurrent urinary infections and renal scarring. RESULTS: Significant differences identified in baseline characteristics included gender, circumcision status and reflux grade, and differences in methodology included evaluation of bowel and bladder dysfunction, method of urine collection, definition of urinary infection, measurement of compliance and means of identifying renal scarring. Cohorts with larger numbers of uncircumcised boys had more breakthrough urinary infections. Infection and renal scarring rates were higher in series with higher grades of reflux. Bagged urine specimens were allowed in 6 series, rendering the data suspect. Children with bowel and bladder dysfunction were excluded from 3 cohorts, and bowel and bladder dysfunction was correlated with outcome in only 1 cohort. Compliance was monitored in only 6 studies. CONCLUSIONS: Subpopulations and methodologies vary significantly in published series of children with vesicoureteral reflux on antibiotic prophylaxis. It is inappropriate to combine outcomes data from these series in a meta-analysis, which would serve to blur distinctions between these subpopulations. Broad recommendations or guidelines based on meta-analyses should be viewed with caution.

Characterizing Early Psychosocial Functioning of Parents of Children with Moderate to Severe Genital Ambiguity due to Disorders of Sex Development.

PURPOSE: We examined the psychosocial characteristics of parents of children with disorders of sex development at early presentation to a disorders of sex development clinic. Parental anxiety, depression, quality of life, illness uncertainty and posttraumatic stress symptoms were assessed. Additionally we evaluated the relationship of assigned child gender to parental outcomes. MATERIALS AND METHODS: A total of 51 parents of children with ambiguous or atypical genitalia were recruited from 7 centers specializing in treatment of disorders of sex development. At initial assessment no child had undergone genitoplasty. Parents completed the Cosmetic Appearance Rating Scale, Beck Anxiety Inventory, Beck Depression Inventory, SF-36, Parent Perception of Uncertainty Scale and Impact of Event Scale-Revised. RESULTS: A large percentage of parents (54.5%) were dissatisfied with the genital appearance of their child, and a small but significant percentage reported symptoms of anxiety, depression, diminished quality of life, uncertainty and posttraumatic stress. Few gender differences emerged. CONCLUSIONS: Although many parents function well, a subset experience significant psychological distress around the time of diagnosis of a disorder of sex development in their child. Early screening to assess the need for psychosocial interventions is warranted.

The RIVUR voiding cystourethrogram pilot study: experience with radiologic reading concordance.

PURPOSE: Two reference radiologists independently review voiding cystourethrograms for the National Institutes of Health sponsored RIVUR (Randomized Intervention for Children with Vesicoureteral Reflux) trial for children with vesicoureteral reflux. A pilot study was required from all clinical centers before enrolling patients. MATERIALS AND METHODS: Digital images were reviewed. Responses were compared and discrepancies adjudicated by teleconference to a final assessment. RESULTS: A total of 75 studies from 19 sites were reviewed. Discrepancies in vesicoureteral reflux grade level were noted on the left and right side in 11 (15%, kappa 0.85) and 12 (16%, kappa 0.83) ureters, respectively. Other areas of disagreement were the presence of paraureteral diverticulum (left 11%, kappa 0.31; right 9%, kappa 0.34), urethral anatomy (15%, kappa 0.33), whether the child voided (8%, kappa 0.21), the presence of ureteral duplication (left 7%, kappa 0.64; right 3%, kappa 0.78) and the presence of bladder trabeculation (5%, kappa 0.32). Of 83 ureters in which reflux was seen there was grade disagreement about 23 (28%). Of 61 ureters initially assessed as grade II or III reflux by both readers, there was disagreement on 9 (15%). Of these 9 discrepancies 7 (78%) were adjudicated to the higher grade (grade III). CONCLUSIONS: Discrepancies in the assessment of intermediate grade vesicoureteral reflux were noteworthy. Recommendations for patients with grade II or III reflux advanced by studies which rely on a single reading, which categorize only grade III or higher reflux as significant, may not be valid.

Antibiotic prophylaxis in pediatric urology: an update.

This article discusses the use of antibiotic prophylaxis in the treatment of three common conditions encountered by the pediatric urologist: 1) children with urinary infection and structurally and neurologically normal urinary tracts; 2) children with vesicoureteral reflux (VUR); and 3) children with prenatally diagnosed ureteropelvic and ureterovesical obstruction. The literature remains unclear and contradictory. Additional significant risk factors for recurrent infection are age, circumcision status in boys, and toileting function. The evidence for the use of prophylaxis is best in those with VUR, although not everyone with VUR must be on medication. In the absence of definitive evidence, the clinician must individualize the treatment of each patient and family.

Laboratory Findings After Urinary Tract Infection and Antimicrobial Prophylaxis in Children With Vesicoureteral Reflux.

It is a common practice to monitor blood tests in patients receiving long-term trimethoprim-sulfamethoxazole (TMP-SMZ) prophylaxis for recurrent urinary tract infections. This multicenter, randomized, placebo-controlled trial enrolled 607 children aged 2 to 71 months with vesicoureteral reflux diagnosed after symptomatic urinary tract infection. Study participants received TMP-SMZ (n = 302) or placebo (n = 305) and were followed for 2 years. Serum electrolytes (n ≥ 370), creatinine (n = 310), and complete blood counts (n ≥ 206) were measured at study entry and at the 24-month study conclusion. We found no significant electrolyte, renal, or hematologic abnormalities when comparing the treatment and placebo groups. We observed changes in several laboratory parameters in both treatment and placebo groups as would normally be expected with physiologic maturation. Changes were within the normal range for age. Long-term use of TMP-SMX had no treatment effect on complete blood count, serum electrolytes, or creatinine. Our findings do not support routine monitoring of these laboratory tests in children receiving long-term TMP-SMZ prophylaxis.

Pediatric pyeloplasty: comparison of literature meta-analysis of laparoscopic and open techniques with open surgery at a single institution.

PURPOSE: We performed a meta-analysis of published series of laparoscopic and open pyeloplasty. We compared these data to open pyeloplasty at our institution using diuretic renography as the indicator for obstruction and postoperative success. MATERIALS AND METHODS: Laparoscopic studies included 19 series published between 1995 and 2007 comprising 346 kidneys. Open pyeloplasty studies included 9 series published between 1998 and 2007 comprising 486 kidneys. Data from our institution included records for 213 patients (224 kidneys). We defined reoperative intervention as unplanned placement of a nephrostomy tube or ureteral stent, balloon dilation or redo pyeloplasty. RESULTS: In the laparoscopic studies 171 cases (49%) were evaluated by preoperative and 137 (40%) by postoperative diuretic renography. Of patients undergoing postoperative diuretic renography 10 (7%) underwent reoperative intervention, including redo pyeloplasty (6, 4%), nephrostomy (3, 2%) and balloon dilation (1, 1%). In the open pyeloplasty studies performed elsewhere 165 cases (34%) were evaluated by preoperative and 230 (47%) by postoperative diuretic renography. Of patients undergoing postoperative diuretic renography redo pyeloplasty was required in 3 (1%). Of 224 cases managed by open pyeloplasty at our institution 215 (96%) had preoperative and postoperative diuretic renography data available. Of these patients 7 (3%) underwent reoperative intervention, including redo pyeloplasty (4, 2%), ureteral stent (2, 1%) and nephrostomy (1, 0.4%). CONCLUSIONS: Most publications do not confirm preoperative obstruction or, following laparoscopic or open pyeloplasty, postoperative success via diuretic renography. While not statistically significant, in the minority of studies with postoperative diuretic renography results the reoperative intervention rate and redo pyeloplasty rate following laparoscopy are approximately double those of open pyeloplasty.

Cost-Utility of Antimicrobial Prophylaxis for Treatment of Children With Vesicoureteral Reflux.

Objective: Antimicrobial prophylaxis for children with vesicoureteral reflux (VUR) reduces recurrences of urinary tract infection (UTI) but requires daily antimicrobials for extended periods. We used a cost-utility model to evaluate whether the benefits of antimicrobial prophylaxis outweigh its risks and, if so, to investigate whether the benefits and risks vary according to grade of VUR. Methods: We compared the cost per quality-adjusted life-year (QALY) gained in four treatment strategies in children aged <6 years diagnosed with VUR after a first UTI, considering these treatment strategies: (1) prophylaxis for all children with VUR, (2) prophylaxis for children with Grade III or Grade IV VUR, (3) prophylaxis for children with Grade IV VUR, and (4) no prophylaxis. Costs and effectiveness were estimated over the patient's lifetime. We used $100,000/QALY gained as the threshold for considering a treatment strategy cost effective. Results: Based on current data and plausible ranges to account for data uncertainty, prophylaxis of children with Grades IV VUR costs $37,903 per QALY gained. Treating children with Grade III and IV VUR costs an additional $302,024 per QALY gained. Treating children with all grades of VUR costs an additional $339,740 per QALY gained. Conclusions: Treating children with Grades I, II, and III VUR with long-term antimicrobial prophylaxis costs substantially more than interventions typically considered economically reasonable. Prophylaxis in children with Grade IV VUR is cost effective.

Baseline Characteristics of Infants With Atypical Genital Development: Phenotypes, Diagnoses, and Sex of Rearing.

PURPOSE: Little is known about the phenotypes, diagnoses, and sex of rearing of infants with atypical genital development in the United States. As part of a multicenter study of these infants, we have provided a baseline report from US difference/disorder of sex development clinics describing the diagnoses, anatomic features, and sex of rearing. We also determined whether consensus guidelines are followed for sex designation in the United States. METHODS: Eligible participants had moderate-to-severe genital atypia, were aged <3 years, and had not undergone previous genitoplasty. Karyotype, genetic diagnosis, difference/disorder of sex development etiology, family history, and sex of rearing were collected. Standardized examinations were performed. RESULTS: Of 92 subjects, the karyotypes were 46,XX for 57%, 46,XY for 34%, and sex chromosome abnormality for 9%. The median age at the baseline evaluation was 8.8 months. Most 46,XX subjects (91%) had congenital adrenal hyperplasia (CAH) and most 46,XY subjects (65%) did not have a known diagnosis. Two individuals with CAH underwent a change in sex of rearing from male to female within 2 weeks of birth. The presence of a uterus and shorter phallic length were associated with female sex of rearing. The most common karyotype and diagnosis was 46,XX with CAH, followed by 46,XY with an unknown diagnosis. Phenotypically, atypical genitalia have been most commonly characterized by abnormal labioscrotal tissue, phallic length, and urethral meatus location. CONCLUSIONS: An increased phallic length was positively associated with rearing male. Among the US centers studied, sex designation followed the Consensus Statement recommendations. Further study is needed to determine whether this results in patient satisfaction.