Mixed Gonadal Dysgenesis: A Narrative Literature Review and Clinical Primer for the Urologist.

PURPOSE: Mixed gonadal dysgenesis is a difference of sex development that is often confused with other conditions. Individuals have a 45,X/46,XY karyotype. Gonads are characterized by a streak gonad and a dysgenetic testis at varying levels of descent. Persistent Müllerian structures are typical (eg, hemi-uterus). There is significant phenotypic heterogeneity of the internal and external genitalia that, together with different interpretations of the definition, have contributed to a poor understanding of the condition among pediatric urologists. Mixed gonadal dysgenesis is one manifestation of the 45,X/46,XY karyotype. 45,X/46,XY mosaicism can also be associated with typical female or male external genitalia. This review aims to clarify the mixed gonadal dysgenesis definition and to provide urologists with diagnostic and management considerations for affected individuals. MATERIALS AND METHODS: We searched 3 medical databases for articles related to mixed gonadal dysgenesis. Two hundred eighty-seven full-text abstracts and manuscripts were reviewed for content pertinent to: (1) clarifying the definition of mixed gonadal dysgenesis, and (2) describing the following related to the care of affected individuals: prenatal and neonatal evaluation and management, genital surgery, gonadal malignancy risk and management, fertility, gender dysphoria/incongruence, puberty and long-term outcomes, systemic comorbidities, and transitional care. RESULTS: Fifty articles were included. Key points and implications for each of the above topics were summarized. CONCLUSIONS: Mixed gonadal dysgenesis exists on a wide phenotypic spectrum and management considerations reflect this heterogeneity. Care for individuals with mixed gonadal dysgenesis is complex, and decisions should be made in a multidisciplinary setting with psychological support.

Clinical utility of early rapid genome sequencing in the evaluation of patients with differences of sex development.

Establishing an early and accurate genetic diagnosis among patients with differences of sex development (DSD) is crucial in guiding the complex medical and psychosocial care they require. Genetic testing routinely utilized in clinical practice for this population is predicated upon physical exam findings and biochemical and endocrine profiling. This approach, however, is inefficient and unstandardized. Many patients with DSD, particularly those with 46,XY DSD, never receive a molecular genetic diagnosis. Rapid genome sequencing (rGS) is gaining momentum as a first-tier diagnostic instrument in the evaluation of patients with DSD given its ability to provide greater diagnostic yield and timely results. We present the case of a patient with nonbinary genitalia and systemic findings for whom rGS identified a novel variant of the WT1 gene and resulted in a molecular diagnosis within two weeks of life. This timeframe of diagnosis for syndromic DSD is largely unprecedented at our institution. Rapid GS expedited mobilization of a multidisciplinary medical team; enabled early understanding of clinical trajectory; informed planning of medical and surgical interventions; and guided individualized psychosocial support provided to the family. This case highlights the potential of early rGS in transforming the evaluation and care of patients with DSD.

Preventing Pediatric Medical Traumatic Stress in a Pediatric Urology Outpatient Setting: Application of the Pediatric Psychosocial Preventative Health Model (PPPHM).

OBJECTIVE: The objectives of this topical review are to (1) increase understanding of pediatric medical traumatic stress (PMTS) in pediatric urology populations through literature review, (2) identify a theoretical model to guide prevention of PMTS in this population, and (3) provide clinical care recommendations based on the model identified. Authors introduce a new term "uropsychology" to describe psychological practice that specializes in the treatment of urology patients. METHODS: Pediatric uropsychologists from 5 pediatric medical care centers gathered to discuss their experience with treating PMTS in their settings and to review existing literature related to PMTS in pediatric urology, PMTS in other populations, and established models for prevention. Authors provide recommendations based on literature review for preventing PMTS in a pediatric urology population. RESULTS: Gaps in the evidence base for preventing PMTS in this population are identified. Authors provide a series of clinical care recommendations, utilizing clinical experience, and the Pediatric Psychosocial Preventative Health Model (PPPHM) as a framework. CONCLUSIONS: While there is limited research on PMTS in pediatric urology populations, urologic interventions can be perceived as invasive, painful, distressing, and traumatic. Application of the PPPHM can guide prevention and intervention efforts. Future research is needed to characterize PMTS in this population, evaluate the efficacy of trauma-informed prevention and intervention practices, and develop screening measures that accurately identify at-risk patients. Authors recommend intradisciplinary collaboration among uropsychologists, urology specialists, and patients and families to create formal standards of care, avenues for other future research, and equitable access to uropsychology care.

Psychometric properties of the diabetes skills checklist for adolescents with type 1 diabetes and their parents.

OBJECTIVE: Among adolescents with type 1 diabetes, research has found that their perception of their diabetes management is an important predictor of actual diabetes management. There is a need for measures that assess adolescents' perception of their ability to independently complete daily diabetes self-care tasks. The current study examined the psychometric properties of the Diabetes Skills Checklist Teen-Report (DSC-T) and DSC Parent of Teen-Report (DSC-PT), which assess perceived independence in diabetes self-care skills. RESEARCH DESIGN AND METHODS: Data were from 1450 adolescents aged 12 to 18 years and their parents who participated in the Diabetes Camp Matters Study. Families completed the DSC as well as other questionnaires online assessing demographic and diabetes-related information, diabetes strengths, and diabetes-specific emotional distress. RESULTS: Exploratory factor analysis revealed a 14-item DSC-T and 12-item DSC-PT, both with excellent internal consistency and concurrent validity. Both the DSC-T and DSC-PT were found to be positively correlated with diabetes strengths and negatively correlated with HbA1c, and the DSC-PT was significantly correlated with parent-reported diabetes distress. Adolescents who used insulin pumps and continuous glucose monitoring reported higher perceived independence in diabetes self-care skills compared to adolescents who used insulin pens/syringes or blood glucose meters. No differences were found based on demographic characteristics. CONCLUSIONS: The DSC-T and DSC-PT have strong potential to be used during diabetes clinic visits to spark discussion regarding adolescents' self-care, which would allow for a more successful transfer of diabetes care from parent to adolescent, and eventually, the transition from pediatric to adult healthcare.

Patient-Reported and Parent Proxy-Reported Outcomes in Pediatric Medical Specialty Clinical Settings: A Systematic Review of Implementation.

OBJECTIVE: Youth with chronic illness are at higher risk for psychosocial difficulties, leading to a call for screening via patient-reported outcomes (PROs). The purpose of the current review is to summarize PRO implementation in pediatric medical specialty settings. A literature review of PRO implementation in these settings, conceptual issues, value and approach, legal and ethical concerns, as well as a case example of PROA in type 1 diabetes are presented. METHODS: A systematic review was conducted to identify relevant articles published since the most recent Journal of Pediatric Psychology Special Issue on Evidence-Based Assessment in Pediatric Psychology (2008). RESULTS: Thirty-two articles were identified and reviewed. The majority of studies reported that PROA was feasible, did not disrupt clinic flow, identified psychosocial issues warranting intervention, and was acceptable to families and providers. Response to elevated scores and impact on behavioral health referrals varied. CONCLUSION: While many evidenced-based assessment measures are well-validated within pediatric chronic illness groups, the literature regarding implementation of PROs is still emerging. Research findings are promising, with PROs being feasible, acceptable, and leading to increased discussion of psychosocial issues when integrated into pediatric medical settings. Additional research is needed to evaluate the longitudinal impact of PROs and the optimal manner of responding to assessment data, particularly when clinically-elevated. Ultimately, identifying psychosocial issues in pediatric medical settings can promote optimal health and well-being of youth with chronic illness and their families.

Featured Article: Psychosocial and Family Functioning Among Latino Youth With Spina Bifida.

Objective: This study examined differences in psychosocial and family functioning between Latino and non-Latino Caucasian youth with spina bifida (SB), and examined family functioning as a predictor of youth psychosocial functioning as moderated by ethnicity. Methods: Participants were part of a larger, longitudinal study (Devine, Holbein, Psihogios, Amaro, & Holmbeck, 2012) and included 74 non-Latino Caucasian youth with SB and 39 Latino youth with SB (M age = 11.53, 52.2% female). Data were collected at Time 1 and 2 years later, and included questionnaire and observational data of psychosocial and family functioning. Results: Latino youth demonstrated fewer externalizing symptoms, less family conflict, but also less social competence. Family conflict was associated with psychosocial functioning in Latino youth, while family cohesion, conflict, and stress were associated with psychosocial functioning in non-Latino Caucasian youth. Conclusions: Psychosocial and family functioning, and their relationship over time, may be different in Latino versus Caucasian youth with SB.

Milestone achievement in emerging adulthood in spina bifida: a longitudinal investigation of parental expectations.

AIM: To assess changes over time in parents' expectations of adult milestone achievement (college attendance, full-time job attainment, independent living, marriage, parenthood) for young people with spina bifida, to examine how expectancies relate to actual milestone achievement, and to compare milestone achievement in emerging adults with spina bifida with that of peers with typical development. METHOD: Sixty-eight families of children with spina bifida (mean age 8y 4mo, 37 males, 31 females) and 68 families of children with typical development (mean age 8y 6mo, 37 males, 31 females) participated at Time 1. At all subsequent timepoints, parents of young people with spina bifida were asked to rate their expectations of emerging adulthood milestone achievement. At Time 7, when participants were 22 to 23 years old, milestone achievement was assessed. RESULTS: Parents of young people with spina bifida lowered their expectations over time for most milestones; parents of children with higher cognitive ability reported decreases of lower magnitude. Parent expectancies were optimistic and unrelated to actual milestone achievement. Emerging adults with spina bifida were less likely than individuals with typical development to achieve all milestones. INTERPRETATION: Optimistic parental expectations may be adaptive for children with spina bifida and their families, although it is important for families to set realistic goals. Healthcare providers serve a key role in helping families of young people with spina bifida prepare for emerging adulthood.

Stress, Coping, and Mood Among Latino Adolescents: A Daily Diary Study.

The current study examines whether daily coping moderates the effects of daily stress on same-day mood and next-day mood among 58 Latino adolescents (Mage  = 13.31; 53% male). The daily diary design capitalized on repeated measurements, boosting power to detect effects and allowing for a robust understanding of the day-to-day experiences of Latino adolescents. Hierarchical linear modeling revealed that on days when youth reported higher levels of peer and academic stress, they also reported more negative moods. However, only poverty-related stress predicted mood the following day. Engagement coping buffered the effect of poverty-related stress on next-day negative and positive mood, while disengagement exacerbated the effects of academic and peer stress. The need for interventions promoting balanced coping repertoires is discussed.

Culturally-informed neuropsychological evaluations of patients with spina bifida: Application of the ECLECTIC framework to a complex pediatric medical condition.

Objective: The pediatric patient populations in the United States are becoming increasingly diverse in culture. In addition to medical factors, a variety of sociocultural factors (including educational systems, language, immigration status, etc.) can impact a child's cognitive development, performance on traditional neuropsychological measures, provider interactions, differential diagnosis, and recommendations. A culturally-informed neuropsychological evaluation aims to understand the impact of sociocultural factors and integrate them into the assessment approach. Fujii's ECLECTIC framework (acronym for Education & Literacy, Culture/Acculturation, Language, Economic Issues, Communication Style, Testing Situation, Intelligence Conceptualization, and Context of Immigration) can be used to guide culturally-informed neuropsychological evaluations of children with complex medical conditions. Method: Using the ECLECTIC framework, this paper describes clinical considerations and offers practical solutions for providing culturally-informed pediatric neuropsychology evaluations. Four diverse patients with spina bifida are reviewed. Results: The case presentations highlight the application of the ECLECTIC framework within a medically complex pediatric patient population. Conclusions: Spina bifida is one example of a pediatric medical population in which both medical and sociocultural factors can impact cognitive development and the evaluation process. The ECLECTIC framework defines a variety of sociocultural factors that can influence cognitive development and multiple aspects of a pediatric neuropsychological evaluation. Pediatric neuropsychologists should use the ECLECTIC framework to provide culturally-informed evaluations for our increasingly diverse patient populations.

Learning from the past: How lessons from Hinman syndrome can inform the psychological management of lower urinary tract dysfunction.

INTRODUCTION: Pediatric patients with lower urinary tract dysfunction (LUTD) experience a variety of medical and psychosocial concerns that can negatively impact their clinical management and quality of life. When initially described by Drs Hinman and Baumann, patients with a form of severe LUTD, later dubbed Hinman syndrome, were characterized as having a "general attitude of being failures." While this mention was noteworthy as it incorporated an understanding of the interplay between psychological factors and urologic conditions, there have been delays in implementing psychological intervention as a standard of care in patients with LUTD broadly, and perhaps too keen of a focus on youth diagnosed with Hinman syndrome specifically. METHODS: A non-systematic reviewed of LUTD related to psychological management was performed. Clinical recommendations were developed by a multicenter and multidisciplinary team of care providers with topic expertise. OBJECTIVE: The aim of this paper is to propose a systems thinking paradigm for how to involve psychology, or psychological principles, across the spectrum of patients with LUTD with the hope of improving attention to specific aspects of care that may improve clinical management. DISCUSSION: Children across the spectrum of LUTD are likely to benefit from psychological interventions and would benefit from such involvement early on to mitigate the impact of psychosocial concerns on medical outcomes. Pediatric psychologists are well-suited to identify and provide individualized care to patients in greatest need of intervention, such as through pre-procedural preparedness, addressing non-adherence, and with the use of evidence-based, targeted mental health interventions. Psychologists are also apt at implementing interventions while taking into consideration the severity of LUTD, in the context of the patient's psychological, developmental, cultural, familial, and social determinants of health considerations. Youth with severe forms of LUTD, such as Hinman syndrome, likely exemplify the challenges of LUTD that benefit from multidisciplinary intervention. CONCLUSION: Medical and psychological collaboration are key to ensuring symptom mitigation and emotional support for patients across the entire spectrum of LUTD.

Turner Syndrome With Y Chromosome and Germ Cells: A Case Report Highlighting the Need to Prioritize Individualized Care.

Turner syndrome (TS) is a genetic condition in phenotypic females in which the individual has 1 intact X chromosome and the second sex chromosome is absent or structurally altered Components of Y chromosome (eg, 45,X/46,XY) have been found in 5%-15% of patients with TS; these patients are often referred to as having "Turner syndrome with Y" (TS+Y). The presence of Y chromosome material increases risk for development of gonadal tumors. Historically, prophylactic gonadectomy has been recommended in this population to prevent malignancy, and patients were presumed infertile due to the presence of streak gonads with no germ cells (GCs). More recently, studies have reported on spontaneous puberty and menarche in TS+Y patients suggesting the presence of viable GC and ovarian function. Our institution offers patients with TS+Y the option of experimental gonadal tissue cryopreservation (GTC) at the time of gonadectomy. We present a unique case of a young girl with TS+Y who had GCs present in her gonads and underwent experimental GTC at the time of gonadectomy.

Young Adults with Type 1 Diabetes.

Young adults experience multiple developmental transitions across social, educational, vocational, residential, and financial life domains. These transitions are potential competing priorities to managing a chronic condition such as type 1 diabetes and can contribute to poor psychosocial and medical outcomes. In this narrative review, we describe population outcomes of young adult populations and the unique considerations associated with managing type 1 diabetes in young adulthood. We provide an overview of the current evidence-based strategies to improve care for young adults with type 1 diabetes and recommendations for future directions in the field.

Workflow for Management of Gonadal Neoplasm in 2 Patients with Differences of Sex Development Enrolled in an Experimental Gonadal Tissue Cryopreservation Protocol.

OBJECTIVE: To outline our experimental gonadal tissue cryopreservation (GTC) protocol that does not disrupt the standard of care in medically-indicated gonadectomy for patients with differences of sex development, including highlighting the multidisciplinary collaborative protocol for when neoplasm is discovered in these cases. METHODS: Two patients with complete gonadal dysgenesis who were undergoing medically-indicated prophylactic bilateral gonadectomy elected to pursue GTC. Both were found to have germ cell neoplasia in situ on initial pathologic analysis, requiring recall of the gonadal tissue, which had been cryopreserved. RESULTS: Cryopreserved gonadal tissue was successfully thawed and transferred to pathology for complete analysis. No germ cells were identified in either patient nor were found to have malignancy, so further treatment beyond gonadectomy was not indicated. Pathologic information was communicated to each family, including that long-term GTC was no longer possible. CONCLUSION: Organizational planning and coordination between the clinical care teams, GTC laboratory, and pathology were key to handling these cases with neoplasia. Processes that anticipated the possibility of discovering neoplasia within tissue sent to pathology and the potential need to recall GTC tissue to complete staging included (1) documenting the orientation and anatomical position of tissue processed for GTC, (2) defining parameters in which tissue will be recalled, (3) efficiently thawing and transferring GTC tissue to pathology, and (4) coordinating release of pathology results with verbal communication from the clinician to provide context. GTC is desired by many families and at the time of gonadectomy and is (1) feasible for patients with DSD, and (2) did not inhibit patient care in 2 patients with GCNIS.

Surgical experiences in adolescents and young adults with differences of sex development: A qualitative examination.

BACKGROUND: Surgical intervention in youth with differences of sex development (DSD) is a controversial topic. Historically, evidence suggests that genital surgery in DSD is associated with mixed patient satisfaction. However, less is known about surgical outcomes under therapeutic advancements in the past several decades. OBJECTIVE: The purpose of the current study is to provide an updated and empirical qualitative examination of the surgical experiences and responses to care of adolescents and young adults (AYA) with DSD in order to fill this gap in the literature and inform patient care. METHODS: Qualitative interviews were conducted with 37 AYA (ages 12-26) with DSD. Interviews were transcribed, coded, and analyzed thematically. RESULTS: Three major themes were identified: 1) knowledge related to surgery; 2) surgical and medical experience; and 3) psychosocial factors related to surgery. Results demonstrated that most AYA were not involved in the decision to pursue surgery and were observed to have varying levels of knowledge regarding their surgeries. Most participants in the current study had received a DSD-related surgery and the majority described having positive surgical experiences and few regrets. Nonetheless, AYA described both medical and psychosocial challenges related to their surgeries and recoveries and offered feedback on ways to improve the surgical process. DISCUSSION: The current study provides a qualitative examination of the surgical experiences of 37 AYA with DSD. Findings highlight the importance of regular and ongoing communication with providers to improve knowledge related to surgery during the decision-making process as well as after surgical intervention. Results underscore the benefits of multidisciplinary teams and the value of patient handouts and decision aids in assisting AYA in the decision to pursue surgical intervention. Other specific recommendations for providers include increased patient privacy during genital exams, greater emphasis on psychoeducation and pain management strategies, and the use of behavioral health services to assist with challenges and social support. CONCLUSIONS: The decision to pursue surgical intervention in youth with DSD remains a complex and controversial issue, and more information regarding patients' perspectives on surgery is needed. The current study provides novel insights into patient experiences of surgical intervention and highlights the need for psychosocial support throughout the shared decision-making process.

Sociodemographic factors and health-related, neuropsychological, and psychosocial functioning in youth with spina bifida.

Objective: Past research suggests that certain sociodemographic factors may put youth with spina bifida (SB) at risk for poor outcomes. The aims of this study were to examine (a) associations between ten sociodemographic factors and health-related, neuropsychological, and psychosocial functioning among youth with SB,(b) cumulative sociodemographic risk as a predictor of youth outcomes as moderated by age, and (c) SB-related family stress as a mediator of longitudinal associations between cumulative sociodemographic risk and youth outcomes. Method: Participants were youth with SB (N = 140 at Time 1; Mage at Time 1 = 11.43, 53.6% female) recruited as part of a larger, longitudinal study. The study included questionnaire (parent-, teacher-, and youth-report), neuropsychological testing, and medical chart data across three time points, spaced 2 years apart. Results: A subset of the sociodemographic factors and their cumulative risk were associated with study outcomes. Specifically, youth characterized by sociodemographic risk had greater pain and lower academic achievement, but also fewer urinary tract infections and fewer attention and executive function problems. Age did not moderate the association between cumulative risk and outcomes. Cumulative risk predicted lower SB-related family stress, which, in turn, predicted several outcomes. Conclusions: Examining a range of sociodemographic factors is warranted. Sociodemographic risk is linked to poorer outcomes for some risk indicators but similar or better outcomes for others. Results have implications for delivering evidence-based, diversity-sensitive clinical care to youth with SB. (PsycInfo Database Record (c) 2021 APA, all rights reserved).

Examining Indirect Effects of Anxiety on Glycated Hemoglobin via Automatic Negative Thinking and Diabetes-Specific Distress in Adolescents With Type 1 Diabetes.

OBJECTIVES: In this study, we examined the indirect effects of anxiety on glycated hemoglobin (A1C) via automatic negative thinking and diabetes distress among adolescents with type 1 diabetes (T1D) during the follow-up interval of a randomized controlled trial of an intervention targeting resilience promotion/depression prevention. METHODS: Adolescents (N=264) participating in the Supporting Teen Problem Solving clinical trial were included and assessed at 8, 12, 16 and 28 months postbaseline. A serial, double-mediation model was used to examine path effects from anxiety to A1C through automatic negative thinking, through diabetes distress and through both automatic negative thinking and diabetes distress. Relevant demographic and clinical covariates were included. RESULTS: Anxiety significantly predicted increases in both automatic negative thinking and diabetes distress. Automatic negative thinking was not found to mediate the association between anxiety and A1C, but diabetes distress did mediate the association. The double-mediation path through automatic negative thinking and diabetes distress together was significant. The indirect effect of anxiety on A1C through diabetes distress was significant and greater than the indirect effect of the double-mediator path. Anxiety did not predict A1C independent of its effects on automatic negative thinking and diabetes distress. Inclusion of demographic covariates did not substantively change the results. CONCLUSIONS: Analyses suggest that automatic negative thinking and diabetes distress mediate the relationship between anxiety and A1C among adolescents with T1D. Diabetes distress appears to be a robust factor linking anxiety to A1C. Diabetes distress should be further examined as a mediator of glycemic variability in anxious youth with T1D.

Fertility Discussions: Perspectives of Adolescents and Young Adults With Differences of Sex Development.

Objective: Fertility-related health care and decision-making needs for youth with differences of sex development (DSD) are complex and vary by condition and the values and preferences of each individual and their partner and/or family. Discussing fertility implications can be a challenging aspect of clinician and family communication about a DSD diagnosis. This qualitative study assesses fertility-related communication experiences of adolescents and young adults (AYA) with DSD. Method: Participants included 97 AYA with DSD ages 12-26 years (M = 18.5, SD = 3.9) who completed questionnaires on demographic and medical information and patient-clinician communication. A subsample of 33 AYA also completed semistructured interviews about experiences with fertility discussions. Results: Two major themes, each with subthemes, were identified: (1) understanding of fertility related to (1a) one's own fertility status, (1b) reproductive and parenting options, and (1c) emotional reactions to one's own fertility status and (2) conversations about fertility related to (2a) reflections on conversations, (2b) barriers, and (2c) advice. Conclusions: AYA perspectives in this study provide important information about how youth with DSD learn about their fertility status, the impact their fertility status has on them, and the fertility-related conversations they have with their providers and families. Specific recommendations for providers and parents or caregivers include the following: inform youth of their fertility status as early as possible; be direct but patient in delivering information; begin by giving basic information and provide more detail as the conversation unfolds; revisit the conversation over time to allow for further discussion or information-seeking; offer additional information or resources, including behavioral health resources.

Psychosocial Aspects of Diabetes Technology Use: The Child and Family Perspective.

This article offers a systematic review of the literature on psychosocial aspects of technology use in children and adolescents with type 1 diabetes and their families, searching for relevant articles published the past 5 years. Topics included continuous subcutaneous insulin infusion, continuous glucose monitoring, predictive low-glucose suspend, and artificial pancreas systems. The review indicates there are positive and negative psychosocial aspects to diabetes technology use among youth and their families. Although consistent findings were revealed, contradictions exist. Discussed are recommendations for future research and implications for how health care providers can collaborate with families to discuss and manage diabetes technology.

Socioeconomic Status and Parental Perceived Social Support in Relation to Health-Related Quality of Life in Youth with Spina Bifida.

Spina bifida (SB) is a congenital birth defect causing a wide variance of physical and intellectual disabilities. The first objective of this study was to examine SES and parental perceived support as predictors of HRQoL among youth with SB. It was hypothesized that lower SES would predict lower youth HRQoL, and higher parental perceived support would predict higher youth HRQOL. The second objective of this study was to examine parental perceived support as a moderator of the association between SES and youth HRQoL. Parental perceived support was hypothesized to serve as a buffer of the negative impact that low SES has on HRQoL. Results indicated significant effects of SES on school, physical, and total HRQoL subscales when covariates were not included. In addition, parental perceptions of social support from family members were significantly associated with Emotional HRQoL in youth with SB. There was a significant interaction between SES and parental perceived support from friends predicting youth Social HRQoL. However, post-hoc simple slope analyses were not significant. This study works to expand the understanding of the roles of SES and parental perceived social support on the HRQoL in children with SB, a population susceptible to poor quality of life due to the physical and cognitive challenges commonly associated with this condition.

Cultural Considerations for Autonomy and Medical Adherence in a Young Palestinian American Muslim Female With Spina Bifida: A Longitudinal Case Study in a Research Context.

This case study examines the unique presentation of a young Palestinian-American Muslim female, who is a part of an ongoing longitudinal study examining family and peer relationships, psychological adjustment, and neuropsychological functioning in youth with spina bifida. Throughout ten years of data collection, Palestinian-Arab culture and Islamic faith have consistently emerged as important factors that can be considered when interpreting this participant's general level of autonomy, medical autonomy, medical adherence, and psychological adjustment. This case study examines important aspects of adaptive and independent functioning for youth with spina bifida and how this family's culture interacts with these different domains of functioning. Moreover, it explores potential conflicts between an Arab collectivist family structure and Western biomedical ethics and suggests how clinicians might navigate these conflicts and bolster culturally-rooted strengths of families and patients from non-Western backgrounds.