Aberrant behavior checklist in youth with Prader-Willi syndrome: Preliminary study of cross-sectional and longitudinal behavior characterization.

Prader-Willi syndrome (PWS) is a rare genetic disorder caused by the loss of paternal genes on chromosome 15. The Aberrant Behavior Checklist (ABC) is a standardized rating scale for assessing problematic behaviors in persons with developmental disabilities. Our study aims to describe ABC scores in youth with PWS and track their change over time. The analysis included 69 patients. Mean ABC scores were compared in four age groups (5-8, 9-12, 13-16, and 17-22 years). A statistically significant difference was found only in the Irritability subscale, with lower scores in the 5-8 age group compared to the 9-12 age group. For change over time, scores for Irritability, Lethargy, Stereotypic Behavior, Hyperactivity subscales, and Total score were likely to decrease after age 12. Irritability subscale scores of males were predicted to increase more than those of females between ages of 5 and 12 . The Lethargy score in the nondeletion group had a greater reduction than the deletion group in the 12-20 year range. This study highlights the need for systematic collection and characterization of behavioral data given the burden of maladaptive behaviors that often persist for a lifetime.

Vibro-tactile stimulation of the neck reduces pain in people with cervical dystonia: a proof-of-concept study.

BACKGROUND: Pain is a common non-motor symptom in patients with cervical dystonia (CD), severely impacting their quality of life. The pathophysiology of CD is incompletely understood but it involves altered processing of proprioceptive and pain signals. OBJECTIVES: The purpose of this proof-of-concept study was to determine if vibro-tactile stimulation (VTS)-a non-invasive form of neuromodulation targeting the somatosensory system-can modulate neck pain in people with CD. METHODS: In a multi-center study, 44 CD patients received VTS to sternocleidomastoid and/or trapezius muscles for up to 45 min under 9 different stimulation conditions that either targeted a single or a pair of muscles. The primary outcome measure was a perceived pain score (PPS) rated by participants on a 100-point analogue scale. RESULTS: During VTS, 29/44 (66%) of participants experienced a reduction in PPS of at least 10% with 17/44 (39%) reporting a reduction in pain of 50% or higher. After VTS cessation, 57% of participants still reported a 10% or higher reduction in PPS. Effects were significant at the group level and persisted for up to 20 min post-treatment. No distinct optimal stimulation profiles were identified for specific CD phenotypes. Clinical markers of disease severity or duration did not predict the degree of VTS-induced pain reduction. CONCLUSION: This proof-of-concept study demonstrates the potential of VTS as a new non-invasive therapeutic option for treating neck pain associated with CD. Further research needs to delineate optimal dosage and long-term effects.

Diazoxide choline extended-release tablet in people with Prader-Willi syndrome: results from long-term open-label study.

OBJECTIVE: This study assessed the effect of 1-year administration of diazoxide choline extended-release tablet (DCCR) on hyperphagia and other complications of Prader-Willi syndrome (PWS). METHODS: The authors studied 125 participants with PWS, age ≥ 4 years, who were enrolled in the DESTINY PWS Phase 3 study and who received DCCR for up to 52 weeks in DESTINY PWS and/or its open-label extension. The primary efficacy endpoint was Hyperphagia Questionnaire for Clinical Trials (HQ-CT) score. Other endpoints included behavioral assessments, body composition, hormonal measures, and safety. RESULTS: DCCR administration resulted in significant improvements in HQ-CT (mean [SE] -9.9 [0.77], p < 0.0001) and greater improvements in those with more severe baseline hyperphagia (HQ-CT > 22). Improvements were seen in aggression, anxiety, and compulsivity (all p < 0.0001). There were reductions in leptin, insulin, and insulin resistance, as well as a significant increase in adiponectin (all p < 0.004). Lean body mass was increased (p < 0.0001). Disease severity was reduced as assessed by clinician and caregiver (both p < 0.0001). Common treatment-emergent adverse events included hypertrichosis, peripheral edema, and hyperglycemia. Adverse events infrequently resulted in discontinuation (7.2%). CONCLUSIONS: DCCR administration to people with PWS was well tolerated and associated with broad-ranging improvements in the syndrome. Sustained administration of DCCR has the potential to reduce disease severity and the burden of care for families.

Evaluation of torsional strength and torque presentation of coated rectangular stainless steel archwires, an in vitro study.

OBJECTIVES: An in vitro experimental study was performed to evaluate the torsional strength and torque released by esthetic coated archwires. MATERIALS AND METHODS: A total of 52 coated (study group) and 52 stainless steel (control group) rectangular archwires from two manufacturers (brand I: Gestenco International AB, Gothenburg, Sweden and brand II: Ortho Technology, Lutz, FL, USA) in two sizes (0.019â€¯× 0.025 and 0.017â€¯× 0.0250-inch) were evaluated. The straight parts on both ends of each preformed archwire were cut in 30 mm segments. A specially designed experimental device was attached to the universal testing machine (Model Z020, Zwick Roell, Ulm, Germany) to measure torsional strength and to calculate the clinically significant torque interval. The groups were compared based on their brand, presence of coating and size using three-way analysis of variance (ANOVA) test at a significance level of 5%. RESULTS: The results revealed that coating of the wires of brand 1 resulted in a significant reduction of torsional strength in both wire sizes (P-value = 0.0001). For the wires of brand 2, coating of the 0.017â€¯× 0.025-in wire resulted in a significant reduction in torsional strength. However, for the 0.019â€¯× 0.025 in wire size, the presence of coating significantly increased the torsional strength (P-value = 0.0001). Coating did not affect the measured clinical torque interval of the analyzed wires when a torque between 5 and 20 Nmm was applied (P-value = 0.062). CONCLUSIONS: Mechanical behavior of coated archwires depends mainly on the thickness of their inner core alloy. Reduction in the diameter of the inner alloy resulted in reduced torsional strength. Despite lower mean torsional strength, in the settings of this study, coated and conventional wires demonstrated comparable torque angles if loaded in the typical range of activation.

Low Pretreatment Bone Mineral Density in Gender Diverse Youth.

Gender diverse adolescents have low pretreatment bone mineral density (BMD), with variable changes in BMD after initiation of gender-affirming treatment. We aimed to assess factors associated with low BMD in gender diverse youth. Sixty-four patients were included in our analysis (73% assigned male at birth). Subtotal whole-body BMD Z-scores were low in 30% of patients, and total lumbar spine BMD Z-scores low in 14%. There was a positive association with body mass index, and no association with vitamin D level. Male sex assigned at birth was associated with lower pretreatment BMD, with lower average BMD Z-scores compared to previous studies.

Diazoxide Choline Extended-Release Tablet in People With Prader-Willi Syndrome: A Double-Blind, Placebo-Controlled Trial.

CONTEXT: Prader-Willi syndrome (PWS) is a rare neurobehavioral-metabolic disease caused by the lack of paternally expressed genes in the chromosome 15q11-q13 region, characterized by hypotonia, neurocognitive problems, behavioral difficulties, endocrinopathies, and hyperphagia resulting in severe obesity if not controlled. OBJECTIVE: The primary end point was change from baseline in hyperphagia using the Hyperphagia Questionnaire for Clinical Trials (HQ-CT). Other end points included Global Impression Scores, and changes in body composition, behaviors, and hormones. METHODS: In DESTINY PWS, a 13-week, randomized, double-blind, placebo-controlled, phase 3 trial, 127 participants with PWS aged 4 years and older with hyperphagia were randomly assigned 2:1 to diazoxide choline extended-release tablet (DCCR) or placebo. RESULTS: DCCR did not significantly improve hyperphagia (HQ-CT least-square mean (LSmean) [SE] -5.94 [0.879] vs -4.27 [1.145]; P = .198), but did so in participants with severe hyperphagia (LSmean [SE] -9.67 [1.429] vs -4.26 [1.896]; P = .012). Two of 3 secondary end points were improved (Clinical Global Impression of Improvement [CGI-I]; P = .029; fat mass; P = .023). In an analysis of results generated pre-COVID, the primary (HQ-CT; P = .037) and secondary end points were all improved (CGI-I; P = .015; Caregiver Global Impression of Change; P = .031; fat mass; P = .003). In general, DCCR was well tolerated with 83.3% in the DCCR group experiencing a treatment-emergent adverse event and 73.8% in the placebo group (not significant). CONCLUSION: DCCR did not significantly improve hyperphagia in the primary analysis but did in participants with severe baseline hyperphagia and in the pre-COVID analysis. DCCR treatment was associated with significant improvements in body composition and clinician-reported outcomes.

Leuprolide Acetate for Puberty Suppression in Transgender and Gender Diverse Youth: A Comparison of Subcutaneous Eligard Versus Intramuscular Lupron.

PURPOSE: To compare the efficacy of intramuscular Lupron and subcutaneous Eligard, two formulations of leuprolide, for puberty suppression in transgender and gender diverse (TGD) youth. METHODS: A retrospective chart review of TGD youth receiving Lupron or Eligard 22.5 mg every 3 months was conducted to determine hormone levels obtained 1 hour after an injection (1hrPost) and patient-reported clinical puberty suppression. RESULTS: Forty eight patients were analyzed: 33% assigned female at birth of which 25% were premenarchal, mean age at first injection 13.7 years, and 50% received concurrent gender affirming hormones. Of these, 13% received Lupron, 52% Eligard, and 35% initially received Lupron then transitioned to Eligard due to drug shortages. There were 55 incidents of 1hrPost levels, 42 after Eligard and 13 after Lupron. Clinical puberty suppression occurred in all patients; however, biochemical suppression occurred in 90% of Eligard and 69% of Lupron (p = .06). DISCUSSION: Eligard and Lupron were both effective in suppressing clinical puberty progression in our population of TGD youth, of which 50% were receiving concurrent gender affirming hormones.

The effect of multiple enamel conditioning on enamel micro-hardness.

Background: White spot formation is one of the common side effects in orthodontic treatments and multiple enamel conditioning might happen even during on session of fixed orthodontic treatments. The aim of the present study was to evaluate the impact of multiple enamel conditioning with different methods on enamel micro-hardness (MH). Materials and Methods: In this In vitro experimental study, the buccal surfaces of 105 extracted premolars were evaluated in seven groups: One control and six experimental groups. The enamel conditioning was performed in three ways: Etching with phosphoric acid 37%, etching with phosphoric acid 37% followed by primer application and conditioning with self-etch primer. The conditioning process in each way was also performed twice consecutively. The specimens were submitted in pH cycling model with demineralization and re-mineralization solutions for 14 days. Afterward Vickers MH test was applied with 0.981N force on the teeth for 10 s indentation time. Data were analyzed using One-Way ANOVA and Tukey HSD (honestly significant difference) test for multiple comparisons. A value of P < 0.05 was considered statistically significant. Results: MH analysis showed statistically significant differences between the control group and the other conditioned groups (P < 0.05). The groups conditioned with acid-etch and primer, particularly twice, showed the lowest amount of MH in comparison to other groups. Self-etch primer had the least effect on MH of the enamel. Single time etching without using primer, made no considerable difference when compared to multiple etching. Conclusion: Etching process and covering the enamel with primer decrease enamel MH. Using self-etch primer is a more conservative method of enamel conditioning.

Use of nano-hydroxyapatite serum and different finishing/polishing techniques to reduce enamel staining of debonding after orthodontic treatment : A randomized clinical trial.

PURPOSE: The aim of this study was to assess the effect of nano-hydroxyapatite serum and different finishing, polishing techniques on color alterations of enamel caused by debonding procedures after comprehensive orthodontic treatment by use of a spectrophotometer. METHODS: This randomized clinical trial recruited 20 participants with previous non-extraction orthodontic treatment and acceptable hygiene to evaluate enamel staining after orthodontic debonding. The usage of a carbide bur alone, as the conventional method, and the combination use of carbide burs and Sof-Lex discs (3M™ ESPE, St. Paul, MN, USA) were compared to each other followed by 10 days application of nano-hydroxyapatite after debonding. Then the enamel staining was evaluated by a reflectance spectrophotometer in three periods: immediately, and 2 and 4 months after debonding. RESULTS: The comparison of the groups showed a significant interaction between Sof-Lex groups and the control side after the first interval of the study (p = 0.042). Application of nano-hydroxyapatite demonstrated no significant difference in color parameters between upper and lower arches of the participants at all intervals of this study (p > 0.05). The mean total color change (ΔE) in all groups and between all intervals had been clinically perceptible (ΔE > 3.3). CONCLUSIONS: The applied concentrations of nano-hydroxyapatite had no significant effect in reducing tooth color changes after debonding in orthodontic treatment. Sof-Lex discs can significantly reduce tooth color changes in a short time.

Sotos syndrome with a novel mutation in the NSD1 gene associated with congenital hypothyroidism.

Childhood overgrowth syndromes are relatively rare. A generalized overgrowth syndrome should be suspected when tall stature and macrocephaly are present, after ruling out nutritional excess and endocrinopathies. Sotos syndrome is a well-described overgrowth syndrome due to haploinsufficiency of the NSD1 gene. We present a case of an infant with permanent congenital hypothyroidism, who had tall stature and macrocephaly by 7 months of age. He was noted to have typical facial features, mild gross motor and speech delay, and scoliosis by 13 months of age. Gene sequencing revealed a heterozygous novel c6076_6087del12: p.Asn2026_Thr2029del variant in exon 20 of the NSD1 gene, pathogenic for Sotos syndrome. Congenital hypothyroidism with Sotos syndrome has been infrequently reported and may expand the spectrum of disease characteristics. Early diagnosis of overgrowth syndromes is important for developmental follow up and multidisciplinary care coordination.

Behavioral features in Prader-Willi syndrome (PWS): consensus paper from the International PWS Clinical Trial Consortium.

Prader-Willi syndrome (PWS) is a rare neurodevelopmental genetic disorder associated with a characteristic behavioral phenotype that includes severe hyperphagia and a variety of other behavioral challenges such as temper outbursts and anxiety. These behaviors have a significant and dramatic impact on the daily functioning and quality of life for the person with PWS and their families. To date, effective therapies addressing these behavioral challenges have proven elusive, but several potential treatments are on the horizon. However, a limiting factor for treatment studies in PWS is the lack of consensus in the field regarding how to best define and measure the complex and interrelated behavioral features of this syndrome. The International PWS Clinical Trials Consortium (PWS-CTC, www.pwsctc.org ) includes expert PWS scientists, clinicians, and patient advocacy organization representatives focused on facilitating clinical trials in this rare disease. To address the above gap in the field, members of the PWS-CTC "Behavior Outcomes Working Group" sought to develop a unified understanding of the key behavioral features in PWS and build a consensus regarding their definition and description. The primary focus of this paper is to present consensus definitions and descriptions of key phenotypic PWS behaviors including hyperphagia, temper outbursts, anxiety, obsessive-compulsive behaviors, rigidity, and social cognition deficits. Patient vignettes are provided to illustrate the interrelatedness and impact of these behaviors. We also review some available assessment tools as well as new instruments in development which may be useful in measuring these behavioral features in PWS.

Gender Diverse Youth on Fertility and Future Family: A Qualitative Analysis.

PURPOSE: Gender-affirming treatment for transgender and nonbinary adolescents has been shown to decrease anxiety, depression, and suicidality, but treatments have medical consequences. Specifically, hormone replacement and pubertal blocking may impact patients' fertility and childbearing capabilities. We interviewed gender diverse adolescents regarding their thoughts on family and fertility. METHODS: We completed semistructured interviews with 23 gender diverse adolescents recruited from the Seattle Children's Gender Clinic. Interviewees included transfeminine, transmasculine, and nonbinary youth. Interviews were recorded, transcribed, and analyzed using Braun and Clarke's theory of thematic analysis, a flexible framework for qualitative analysis. RESULTS: Gender diverse adolescents have myriad views on fertility, but four main themes were identified: (1) an interest in future family, including ideas regarding adoption and biological children; (2) barriers to fertility, including cost and procedure-related dysphoria; (3) factors unique to the developmental stage of adolescents, including the age discordance of making fertility decisions as a teenager and parental influence on decision-making; and (4) suggestions for clinicians approaching fertility counseling with adolescents considering hormone therapy. CONCLUSIONS: Many gender diverse youth asserted an interest in building families, although the process of fertility preservation remains fraught. Relative to other studies, our participants were hopeful, imaginative, and interested in having children. Participants wanted to receive specific counseling on fertility, to receive help navigating the logistics of fertility preservation, and to be listened to when their hopes for children (or no children) were stated. Further research is needed to create care paradigms that address fertility of transgender youth in an affirming, developmentally appropriate manner.

Effect of Maxillary Posterior Space Discrepancy on the Molars and Overbite in Class II Malocclusions with Different Vertical Patterns.

AIM: The purpose of this cross-sectional study was to investigate the influence of maxillary posterior space discrepancy (MPDD) on angulation and vertical position of molars in patients with skeletal class II malocclusions, presenting long, short, and normal vertical growth patterns. MATERIALS AND METHODS: In total, 120 lateral cephalograms of patients (mean age: 23 years) with skeletal class II malocclusion were evaluated. Patients were divided into six groups based on their vertical growth pattern (normal, long, and short faces) and the presence or absence of maxillary posterior discrepancy. Maxillary molars' sagittal angulation and vertical position were measured on cephalograms via Dolphin™ three-dimensional (3D) software (version 11.5). The comparison between groups with the same vertical dimension and different status of MPDD was done with independent t test. The analysis of analysis (ANOVA) was used to make pairwise comparison between all six groups. Finally, a multiple regression analysis was performed to evaluate the influence of molar position and MPDD on the anterior overbite. RESULTS: The results showed that the angulation of the maxillary first molar was not statistically different between groups with the same vertical dimension but varied in terms of MPDD. The maxillary second molar was more distally inclined in patients with MPDD than those without MPDD with short and normal facial types (p value = 0.016 and p value = 0.001, respectively). The second molar had significantly more distal angulation in long face patients than short and normal face participants, without considering the status of MPDD. The upper first and second molars were erupted more in patients with long than short or normal faces, without any influence of MPDD. CONCLUSION: The presence of MPDD caused more distal inclination of the maxillary second molar, in skeletal class II patients with short and normal vertical growth patterns. CLINICAL SIGNIFICANCE: The status of MPDD and its effect on the maxillary second molar teeth should be considered in skeletal class II patients with short and normal vertical growth patterns.

Effect of mini-screw-facilitated micro-osteoperforation on the rate of orthodontic tooth movement: a single-center, split-mouth, randomized, controlled trial.

OBJECTIVE: The present study aimed to evaluate the effect of MOP over a 3-month period and to determine the influence of the number of perforations on the rate of canine retraction. In addition, the amount of pain and discomfort caused by the MOP method was evaluated. TRIAL DESIGN: A single-center, split-mouth, triple-blind, randomized, controlled trial was conducted. METHODS: The clinical trial was conducted from December 2018 to July 2019 in the Orthodontic Clinic, Shiraz Dental School. Twenty-eight patients (range from16.3 to 35.2 years) who need fixed orthodontic treatment were recruited and randomly assigned to MOP1 and MOP2 groups. In each patient one side of the mouth worked as a control side which received no MOPs. Four months after first premolars extraction, patients in MOP1 group received 3 MOPs on the buccal surface of alveolar bone in the experimental side to accelerate canine retraction whereas patients in MOP2 group received 3 buccal MOPs and 3 palatal MOPs in the experimental side. The amount of canine retraction was measured every 28 days at three intervals on both sides of the mouth. Pain perception was also measured on the day of MOP procedure and subsequently at 24 h. Randomization was performed using online software RANDOM.ORG; the recruited patients were divided into two parallel groups with a 1:1 allocation ratio then the side of MOPs intervention in each subject was randomly determined with coin tossing. Triple blinding design was employed. RESULTS: The result of the intra-examiner reliability using ICC was 0.97 (P <  0.001), indicating excellent repeatability and reliability of the measurements. The baseline characteristics between the groups were similar (P > 0.05). There was a significant difference in the rate of canine retraction between the MOP groups and the contralateral control sides, as well as between the MOP1 and MOP2 groups (P <  0.05). CONCLUSION: The MOP procedure was effective in accelerating orthodontic tooth movement, although the amount of acceleration was not clinically significant in the case of canine retraction. An increase in the number of MOPs resulted in a significant acceleration of the canine retraction. TRIAL REGISTRATION: The trial was registered 30 November 2018 at the Iranian Registry of Clinical Trials (IRCT20181121041713N1).

How Are Transgender and Gender Nonconforming Youth Affected by the News? A Qualitative Study.

PURPOSE: News stories about transgender and gender nonconforming (TGNC) people have become more common in recent years and TGNC youth may be disparately affected by this information compared with their cisgender peers. The aim of this study was to understand how TGNC youth react to news stories about TGNC and other gender and sexual minority (GSM) identifying persons. METHODS: As part of a larger study, 23 TGNC youth were recruited from the Seattle Children's Gender Clinic to participate in a semistructured interview. Interviews were audio recorded, transcribed, and thematically analyzed. RESULTS: Participating youth ranged in age from 13 to 19 years (mean = 16.9 years). Of the 23 participants, 43% identified as having a transfeminine gender identity, 39% transmasculine, and 17% nonbinary/gender fluid. Four main themes were identified: (1) news coverage of current political climate affects gender transition; (2) negative news coverage of GSM people contributes to concerns about mental well-being and safety; (3) geographical location affects perception of news; and (4) positive news coverage of TGNC people increases visibility and hope. CONCLUSIONS: Exposure to negative news about GSM people may contribute to increased levels of stress among TGNC youth. With increased visibility, participants described frustration associated with inaccurate portrayals of the TGNC community in the news; however, with increased visibility, there is also a growing sense of shared community and opportunity for acceptance of TGNC people. Given the scope of responses to negative and positive news on GSM people, TGNC youth may benefit from increased support to promote resilience when interpreting the news.

Gender Affirming Hormone Replacement for the Adolescent and Young Adult Cancer Survivor with Hypogonadism.

Hypogonadism is a known late effect of cancer treatment. Hypogonadism requires replacement of sex steroids to ensure appropriate development of secondary sex characteristics, growth, and other beneficial health effects. We present a cancer survivor with hypogonadotropic hypogonadism and gender dysphoria. The patient received gender affirming care in our gender clinic with a multidisciplinary team that included an endocrinologist. This is not an isolated case at our institution. Survivorship oncologists must include a discussion about gender concurrently with conversations about survivors' development of puberty. Conversations should start early to ensure appropriate referrals and gender affirming hormone replacement.

Efficacy and Safety of Real-Life Usage of MiniMed 670G Automode in Children with Type 1 Diabetes Less than 7 Years Old.

The Medtronic MiniMed 670G system with SmartGuard™ (Medtronic, Northridge, CA) is a commercial hybrid closed-loop (HCL) system approved for use in 2018 for children >7 years. Studies of this HCL system in subjects >7 years old show improvement in glycemic control, but no study has described its use in younger children. This is a retrospective analysis of patients with type 1 diabetes (T1D) <7 years of age who used the 670G HCL system at Seattle Children's Hospital for 3 months. We compared 2-week data from Carelink™ while in manual mode (MM) with suspend before low active with those in auto mode (AM). We used two tailed t-test to compare variables related to glycemic control. Sixteen children were reviewed [age of AM start: average 4.3 years (range 2-6); 10 male]. The average time in AM was 6.3 ± 2.9 months (range 3-12). There was a statistically significant change for A1c [MM 7.9% (62.8 mmol/mol), AM 7.4% (57.4 mmol/mol); P-value <0.001], percentage time in range (MM 42.8%, AM 56.2%; P-value <0.001), percentage hypoglycemia (MM 1.3%, AM 2.4%; P-value 0.04), and average sensor glucose [MM 200 mg/dL (11.1 mmol/L), AM 176 mg/dL (9.8 mmol/L); P-value <0.001]. No serious adverse reports noted. This case series showed improvement in glycemic control in very young children using the 670G HCL. We did note more hypoglycemia although no serious adverse events, such as hypoglycemic seizure, were reported. A HCL system can be used in young children with T1D safely and effectively and should be an option for children <7 years.

Influence of Age on Perception of Best Esthetical Profile.

STATEMENT OF THE PROBLEM: As an important determinant of social acceptability, facial attractiveness can be influenced by decisions of orthodontists through treatment. The perception of facial attractiveness is influenced by several factors. PURPOSE: The purpose of this study was to determine the difference in perception of facial attractiveness among male and female raters in different age groups through comparison of silhouettes. MATERIALS AND METHOD: 208 subjects (99 male, 109 female) aged between 18 to 70 years were assigned to three groups of young adults, middle-aged and senior and rated pre-designed silhouettes on a 7-level rating scale (1= the least esthetic, 7=the most esthetic). Two series of 7 silhouettes were prepared for men and women in which the mandible was protruded or retruded in 2-mm increments from the average. The evaluators were asked to grade the profiles separately for male and female. T-test and one-way ANOVA were used for the statistical analysis with α = 0.05. RESULTS: The first and third age groups, favored female profile 2 among class II profiles. However, the second age group preferred the average profile. Male class II profiles were rated exactly as female ones among the three age groups. The average female class III silhouette was preferred in all age groups. The first and second age group preferred the average male profile in male class III silhouettes. The oldest group however, preferred profile 2. For both sexes, the least acceptable profile in each set of silhouettes was the most protruded or retruded. CONCLUSION: There were few differences in perception of facial attractiveness between different age groups. In all groups, even small mandibular protrusion was unacceptable and mild retrusion was considered attractive.

Youth and Parent Experiences in a Multidisciplinary Gender Clinic.

Purpose: To assess youth and parent/caregiver satisfaction with care at a pediatric multidisciplinary gender clinic. Methods: Transgender/gender nonconforming youth (n=33) and their parent/caregiver (n=29) completed self-report questionnaires and individual interviews (n=20) about experiences and satisfaction with care. Results: Quantitatively, participants reported being extremely satisfied with care experiences (parents 97%; youth 94%). Qualitatively, main themes included (1) affirmation due to use of preferred name/pronouns, (2) access barriers due to scheduling and readiness assessments, and (3) positive interactions with Care Navigator. Conclusion: Youth and parents/caregivers are highly satisfied with multidisciplinary, coordinated health care for transgender/gender nonconforming youth; however, some challenges remain.

A multidisciplinary approach to the clinical management of Prader-Willi syndrome.

BACKGROUND: Prader-Willi syndrome (PWS) is a complex neuroendocrine disorder affecting approximately 1/15,000-1/30,000 people. Unmet medical needs of individuals with PWS make it a rare disease that models the importance of multidisciplinary approaches to care with collaboration between academic centers, medical homes, industry, and parent organizations. Multidisciplinary clinics support comprehensive, patient-centered care for individuals with complex genetic disorders and their families. Value comes from improved communication and focuses on quality family-centered care. METHODS: Interviews with medical professionals, scientists, managed care experts, parents, and individuals with PWS were conducted from July 1 to December 1, 2016. Review of the literature was used to provide support. RESULTS: Data are presented based on consensus from these interviews by specialty focusing on unique aspects of care, research, and management. We have also defined the Center of Excellence beyond the multidisciplinary clinic. CONCLUSION: Establishment of clinics motivates collaboration to provide evidence-based new standards of care, increases the knowledge base including through randomized controlled trials, and offers an additional resource for the community. They have a role in global telemedicine, including to rural areas with few resources, and create opportunities for clinical work to inform basic and translational research. As a care team, we are currently charged with understanding the molecular basis of PWS beyond the known genetic cause; developing appropriate clinical outcome measures and biomarkers; bringing new therapies to change the natural history of disease; improving daily patient struggles, access to care, and caregiver burden; and decreasing healthcare load. Based on experience to date with a PWS multidisciplinary clinic, we propose a design for this approach and emphasize the development of "Centers of Excellence." We highlight the dearth of evidence for management approaches creating huge gaps in care practices as a means to illustrate the importance of the collaborative environment and translational approaches.