Comprehensive transition of care for polycystic ovary syndrome from adolescence to adulthood.

Polycystic ovary syndrome (PCOS) is a lifelong chronic condition that affects one in ten females and can be diagnosed in adolescence. As adolescents with PCOS transition to adulthood, counselling for lifestyle management and mental health concerns often transition from involving the family unit to increasingly individual-focused approaches. PCOS is associated with a large range of comorbidities affecting reproductive, metabolic, dermatological, and psychological health. The diagnosis and comorbidities of PCOS are influenced by pubertal hormones and need to be reassessed continuously to ensure that treatment remains appropriate for age and development. As young patients grow up, personal concerns often change, especially in relation to reproductive management. In this Review, we present prevalence rates, screening tools, and treatment recommendations for PCOS-related conditions, and we consider the diagnostic and clinical elements of optimal transition of care models that ensure continuity of comprehensive care for adolescents moving from the paediatric health-care system to the adult health-care system.

Body Mass Index and Levonorgestrel Device Expulsion in Adolescents and Young Adults.

STUDY OBJECTIVE: To evaluate the relationship between body mass and levonorgestrel intrauterine device (LNG-IUD) expulsion in adolescents and young adults (AYA). DESIGN, SETTING, PARTICIPANTS & INTERVENTIONS: A retrospective chart review was conducted of nulliparous females aged 10-24 years who had a 52-milligram LNG-IUD placed between November 2017 and May 2021 by pediatric and adolescent gynecology providers at a tertiary children's hospital, including those who underwent metabolic and bariatric surgery (MBS). Primary analysis focused on 10-19 year olds as they had comparable anthropometrics (namely BMI percentile [BMIP] as defined by the Centers for Disease Control). Descriptive statistics included means, standard deviations (SD), and ranges for continuous variables counts and percentages for categorical variables. Chi-square or Fisher's exact tests were used to assess associations. Logistic regressions were fit to test the associations between BMIP, MBS, and the odds of expulsion. MAIN OUTCOME MEASURES & RESULTS: A total of 588 patients were included in the primary analysis (10-19 years). Mean age was 15.8 years (±2.0). Using BMIP, 15.5% (n = 91) of the sample was overweight and 22.3% (n = 131) were obese. Within 12 months, 33 patients (5.6%) experienced expulsion. Every one-unit increase in BMIP was associated with a 3% increase in the odds of expulsion (P = .008), and each increase in BMIP category (eg, overweight vs average/underweight) was significantly associated with increased odds of expulsion (OR = 2.77-4.28). Patients who had LNG-IUD placement during MBS (n = 43) had higher odds of expulsion (OR = 3.23; P = .024) than other patients. CONCLUSION: AYA with higher BMIP and/or who undergo MBS are at increased risk of LNG-IUD expulsion within one year of placement.

What Do We C in Children With Scurvy? A Case Series Focused on Musculoskeletal Symptoms.

OBJECTIVES: Vitamin C deficiency in children commonly presents with musculoskeletal symptoms such as gait disturbance, refusal to bear weight, and bone or joint pain. We aimed to identify features that could facilitate early diagnosis of scurvy and estimate the cost of care for patients with musculoskeletal symptoms related to scurvy. METHODS: We conducted a retrospective chart review of patients at a single site with diagnostic codes for vitamin C deficiency, ascorbic acid deficiency, or scurvy. Medical records were reviewed to identify characteristics including presenting symptoms, medical history, and diagnostic workup. The Pediatric Health Information System was used to estimate diagnostic and hospitalization costs for each patient. RESULTS: We identified 47 patients with a diagnosis of scurvy, 49% of whom had a neurodevelopmental disorder. Sixteen of the 47 had musculoskeletal symptoms and were the focus of the cost analysis. Three of the 16 had moderate or severe malnutrition, and 3 had overweight or obesity. Six patients presented to an emergency department for care, 11 were managed inpatient, and 3 required critical care. Diagnostic workups included MRI, computed tomography, echocardiogram, endoscopy, lumbar puncture, and/or EEG. Across all patients evaluated, the cost of emergency department utilization, imaging studies, diagnostic procedures, and hospitalization totaled $470 144 (median $14 137 per patient). CONCLUSIONS: Children across the BMI spectrum, particularly those with neurodevelopmental disorders, can develop vitamin C deficiency. Increased awareness of scurvy and its signs and symptoms, particularly musculoskeletal manifestations, may reduce severe disease, limit adverse effects related to unnecessary tests/treatments, and facilitate high-value care.

Assessment of Adverse Childhood Experiences and Social Determinants of Health: A Survey of Practices in Pediatric Weight Management Programs.

Introduction: Adverse childhood experiences (ACEs) and social determinants of health (SDoH) are associated with increased incidence of pediatric obesity. Recent literature highlights an imperative need to assess ACEs and SDoH among youth and families with obesity to identify those individuals requiring targeted interventions. The primary objective of the present study was to examine the frequency, methodology, and barriers in evaluation of ACEs and SDoH within pediatric weight management programs (PWMPs). Methods: Invitations were e-mailed to a comprehensive directory of 92 PWMPs in the United States with a link to complete an electronic survey. Results: Forty-one PWMPs from 26 states completed the survey. Assessment of one or more ACEs and SDoH was common and typically took place during the initial patient visit by the psychologist or medical practitioner through unstructured conversations. Reported barriers to assessment included lack of time to assess and to follow-up, lack of clinic protocols, and inadequate referral resources. Programs offering bariatric surgery and those with embedded mental health clinicians reported fewer barriers to ACEs/SDoH referral resources, while family-based and healthy lifestyle-focused programs perceived more barriers related to insufficient support staff and time to follow-up with families. Conclusions: Most PWMPs assess a subset of ACEs and SDoH; however, approaches to assessment vary, are often unstructured, and several barriers remain to optimizing assessment and follow-up. Future research should evaluate standardized ACEs/SDoH assessment protocols, ideal workflow, and their impact on obesity treatment and related health outcomes.

Telehealth Use in a National Pediatric Weight Management Sample During the COVID-19 Pandemic.

Background: This study aimed to assess the implementation and access to telehealth-delivered pediatric weight management (PWM) during the initial phase of the COVID-19 pandemic at six US PWM programs (PWMP) using the RE-AIM (Reach, Effectiveness, Adoption, Implementation, Maintenance) framework. Methods: The COVID-19 period (COVID) was defined in this retrospective, multisite study as the time when each site closed in-person care during 2020. The Pre-COVID period (Pre-COVID) was an equivalent time frame in 2019. Patients were stratified by visit completion status. Patient characteristics for COVID and Pre-COVID were compared to examine potential changes/disparities in access to care. Results: There were 3297 unique patients included across the six sites. On average, telehealth was initiated 4 days after in-person clinic closure. Compared with Pre-COVID, COVID (mean duration: 9 weeks) yielded fewer total completed visits (1300 vs. 2157) and decreased revenue (mean proportion of nonreimbursed visits 33.30% vs. 16.67%). Among the completed visits, COVID included a lower proportion of new visits and fewer patients who were male, non-English speaking, Hispanic, or Asian and more patients who were Black or lived ≥20 miles from the program site (p < 0.05 for all). Among no-show/canceled visits, COVID included more patients who had private insurance, older age, or a longer time since the last follow-up. Conclusion: Rapid implementation of telehealth during COVID facilitated continuity of PWM care. Clinic volume and reimbursement were lower during COVID and differences in the patient population reached by telehealth emerged. Further characterization of barriers to telehealth for PWM is needed.

Caring for Adults With Significant Levels of Intellectual Disability in Outpatient Settings: Results of a National Survey of Physicians.

Between 1% and 2% of the U.S. population has an intellectual disability (ID) and often experience disparities in health care. Communication patterns and sedation use for routine medical procedures are important aspects of care for this population. We explored physicians' communication patterns and sedation use in caring for patients with significant levels of ID through a mailed survey of 1,400 physicians among seven specialties in outpatient settings (response rate = 61.0%). Among physicians who saw at least one patient with significant levels of ID in an average month, 74.8% reported usually/always communicating primarily with someone other than the patient. Among specialists, 85.5% (95% CI: 80.5%-90.5%) reported doing so, compared to 69.9% (95% CI: 64.4%-75.4%) for primary care physicians (p < 0.001). Also, 11.4% reported sedating at least one patient with significant levels of ID for a routine procedure. Three quarters of physicians reported communicating primarily with persons other than the patient usually or always-an approach that, in some instances, may not align with best medical practice. The percentage of physicians who report sedating at least one individual is associated with significant ID and the physician's volume of patients with significant ID.

Case report: Pulmonary hypertensive crisis leading to cardiac arrest during endoscopic evaluation in a 6-year-old boy with autism, severe malnutrition, and undiagnosed scurvy.

Pediatric gastroenterologists are often responsible for the evaluation of malnutrition in the setting of selective eating. Endoscopic evaluation for conditions including eosinophilic esophagitis and celiac disease can help to identify and treat mucosal disease contributing to food selectivity. However, undiagnosed micronutrient deficiencies can cause cardiovascular derangements that significantly increase a patient's anesthetic risk. Vitamin C deficiency in particular, alone or in combination with severe malnutrition, is associated with a severe but reversible form of pulmonary arterial hypertension that, while life threatening in the acute phase, may significantly improve within days of starting ascorbic acid replacement therapy. Here we present a case of a 6-year-old boy with autism spectrum disorder (ASD), severe malnutrition, and undiagnosed chronic vitamin C deficiency who developed a pulmonary hypertensive crisis after induction of general anesthesia leading to cardiac arrest during endoscopic evaluation. While the association between food selectivity among youth with neurodevelopmental differences and vitamin C deficiency is well-described, and pulmonary hypertension is a recognized rare complication of scurvy, extant literature has not addressed next steps to improve patient outcomes. Using this case report as a foundation, we discuss specific patient populations to screen and treat for micronutrient deficiencies prior to anesthesia and propose a novel clinical algorithm for pre-anesthesia risk stratification and mitigation in patients specifically at risk for scurvy and associated pulmonary hypertension.

Weight Management in Adolescents with Polycystic Ovary Syndrome.

PURPOSE OF REVIEW: Polycystic ovary syndrome (PCOS) is a common condition that clinically presents during adolescence. PCOS is associated with increased rates of overweight and obesity, as well as higher rates of metabolic disease, especially type 2 diabetes. Weight loss decreases PCOS symptoms and risk for metabolic disease. The goal of this review is to evaluate recent studies describing the hormonal, metabolic, and weight effects of different weight loss strategies: dietary, physical activity, pharmacotherapy, bariatric surgery, mood modification, and sleep. RECENT FINDINGS: Calorie restriction continues to be supported as the primary nutrition intervention to achieve weight loss in individuals with PCOS, and a dietary macronutrient composition with lower compared to higher glycemic carbohydrates may be more effective. There is limited data that vitamins, nutraceuticals, and probiotics may improve hormonal and metabolic outcomes. Most types of physical activity are effective in improving outcomes in PCOS and lowering weight. Whereas there are promising data on anti-obesity medications such as glucagon-like peptide-1 receptor agonists in adults with PCOS and adolescents with obesity, further work is needed to know if these therapies are effective in youth with PCOS. Research is lacking on the effectiveness of other anti-obesity medications in PCOS. Bariatric surgery is especially promising for decreasing weight in adults and youth, and reversing type 2 diabetes in youth, though PCOS data are lacking. Treatment of depression in adolescents with insulin resistance and women with PCOS is associated with improved weight loss. Adolescents with PCOS and obesity may have greater sleep-related risks including circadian misalignment and obstructive sleep apnea, interventions for which have not yet been conducted. Clinical trials on weight loss strategies in adolescents with PCOS remain limited, with most information inferred from studies in women with PCOS or adolescents with obesity. However, there are multiple options to optimize weight loss in dietary, activity, pharmacotherapy, bariatric surgery, mood modification, and sleep domains.

Obstructive sleep apnea and early weight loss among adolescents undergoing bariatric surgery.

BACKGROUND: Little is known regarding obstructive sleep apnea's (OSA's) prevalence or the factors related to OSA remission post-metabolic bariatric surgery (MBS) in adolescents. OBJECTIVES: To identify the baseline OSA prevalence in adolescents with severe obesity and examine factors associated with post-MBS OSA remission. SETTING: Tertiary-care children's hospital. METHODS: We conducted a retrospective chart review of 81 patients pre-MBS with OSA assessments done between June 2017 to September 2020 to collect demographic characteristics; co-morbidities; polysomnography (PSG) results, if indicated; and weight data. Chi-square or Mann-Whitney tests compared baseline characteristics and surgical outcomes by pre-MBS OSA status. McNemar's test or t tests assessed differences in baseline characteristics, stratified by remission versus no remission of OSA. RESULTS: The patients were 71% female, had an average age of 16.9 ± 2.0 years, and had a mean body mass index (BMI) of 47.9 ± 7.3 kg/m2. Half (50%) of the patients were Hispanic and 20% had type 2 diabetes. The OSA prevalence, defined as an Obstructive Apnea Hypopnea Index (OAHI) score ≥5, was 54% pre-MBS (n = 44), with 43% having severe OSA (OAHI > 30). Those with OSA were older (17.3 versus 16.4 yr, respectively; P = .05), more likely to be male (79% versus 42%, respectively; P = .022), and had higher baseline weights (142.0 versus 126.4 kg, respectively; P = .001) than those without OSA. Of the 23 patients with a post-MBS PSG result (average 5 mo post MBS), 15 (66%) had remission of OSA. Patients with OSA remission had a lower average pre-MBS BMI (46.0 versus 57.7 kg/m2, respectively; P < .001) and weight (132.9 versus 172.6 kg, respectively; P = .002) but no significant differences in percentage weight loss through 12 months post MBS versus those with continued OSA. CONCLUSION: The OSA prevalence in an adolescent MBS population was higher than that in the general adolescent population with severe obesity. Remission of OSA was correlated with lower pre-MBS BMI and weight, but not weight loss within the first year post-MBS.

Development and application of an ethical framework for pediatric metabolic and bariatric surgery evaluation.

BACKGROUND: As severe obesity continues to rise among youth, metabolic and bariatric surgery (MBS) will increasingly be used as a treatment of choice for durable weight loss and improvement of obesity-related complications. MBS for youth with intellectual and developmental disabilities (IDD) and for preadolescents has raised ethical questions. OBJECTIVES: The purpose of this article is to present the creation and application of an ethical framework that supports why MBS should be considered in pediatrics based on the principle of justice without automatic exclusions. This framework also provides a guide for how to conduct a robust, ethically grounded evaluation of pediatric patients presenting for MBS in general, and among subpopulations including youth with IDD and preadolescents. SETTING: Academic medical center, United States. METHODS: An ethical framework was developed and applied through a collaboration between an MBS center at a children's hospital and the institution's ethics consult service. RESULTS: Application of the ethical framework to address 4 core ethical questions is illustrated using 2 hypothetical cases: 1 that highlights an adolescent with IDD and 1 that highlights a preadolescent. CONCLUSIONS: We have demonstrated the application of a novel, overarching framework to conduct the ethical evaluation of youth presenting for MBS. This framework resulted from a collaboration between MBS and ethics consult teams and has the potential to be used as a prototype for other youth-focused MBS programs. Next steps include prospective data collection to test the framework and determine its validity in the target population.

Influence of Weight Loss on Obesity-Associated Complications After Metabolic and Bariatric Surgery in Adolescents.

OBJECTIVE: Following metabolic and bariatric surgery (MBS), most adolescents experience weight loss and improvement of many obesity-associated complications (OACs). The relationship between weight loss and remission of OACs after MBS in adolescents has not been well described. METHODS: The Teen Longitudinal Assessment of Bariatric Surgery (Teen-LABS) is a multi-institutional prospective observational study of adolescents who underwent MBS between 2007 and 2012. Lower-weight-loss responders (LWLRs) were defined as having <20% total body weight loss (TBWL) and higher-weight-loss responders (HWLRs) were defined as having ≥20% TBWL at 5 years after MBS. The prevalence of OACs was compared at baseline and 5 years after MBS. RESULTS: Both LWLRs (n = 114) and HWLRs (n = 78) lost significant weight within the first year following MBS; however, the TBWL at 5 years for the LWLRs was 8.6% ± 9.5% compared with 33.8% ± 9.2% for the HWLRs. Those in the HWLR group were more likely to experience durable remission of composite dyslipidemia at 5 years, whereas there were no differences between groups in remission rates of all other OACs. CONCLUSIONS: Greater weight loss after MBS in adolescents was associated with greater remission of composite dyslipidemia; however, remission of other OACs was not dependent on major sustained weight loss.

Lifestyle and pharmacologic management before and after bariatric surgery.

As metabolic and bariatric surgery (MBS) increasingly becomes a treatment of choice for adolescents with severe obesity, there is a need to understand how to deliver pre- and postoperative care in ways that maximize long-term safety and efficacy. This article describes major pre- and postoperative goals, lifestyle modification targets, and, when necessary, pharmacologic management strategies for adolescents undergoing MBS. Three categories of evidence were used-studies of pre- and postoperative interventions and factors influencing MBS outcomes in adolescents, studies of pre- and postoperative associations and interventions in adults, and studies of non-surgical weight management applicable to adolescents pursuing MBS. Finally, priority areas for future research within this topic are identified.

The role of ethics consultation in decision making for bariatric surgery in pediatrics.

The decision to pursue metabolic and bariatric surgery (MBS) for pediatric patients has become increasingly accepted by patients and their families and by health care professionals. The advancement of pre- and post-operative MBS guidelines, based on accumulating evidence for safety, efficacy, and cost-effectiveness help to map the clinical pathway for MBS consideration. Ethical issues remain possible for each case, however, and consultation with ethical experts can provide clarity in the consideration of MBS. Specifically, ethical issues related to principles of autonomy, justice, beneficence, and non-maleficence may need to be resolved based on patient characteristics, including preadolescent patients and those who present with intellectual disabilities. Institutions that offer MBS for pediatric patients will benefit from collaborating with ethics consultants to develop a structured approach that helps ensure that ethical principles have been adequately addressed for patients presenting for MBS.

Trajectories of hemoglobin A1c and body mass index z-score over four decades among 2 to 18 year olds with type 1 diabetes.

OBJECTIVES: To determine trajectories of glycemic control and body mass index (BMI) z-score in a large pediatric sample with type 1 diabetes (T1D) over a 38-year period, and to evaluate sex differences and temporal changes in the prevalence of these trajectories. METHODS: We conducted a longitudinal, retrospective study of 7002 2 to 18 year olds with T1D followed between 1978 and 2016 at a single center. Group-based modeling was used to identify trajectories for hemoglobin A1c (HbA1c) and BMI z-score. Multinomial logistic regression identified predictors of membership to less favorable glycemic trajectories. RESULTS: Group-based modeling yielded 5 HbA1c trajectories. A total of 86% of the sample fell within 3 trajectories that were largely stable across childhood and adolescence, and 14% fell within 2 trajectories characterized by marked deterioration beginning in pre-adolescence. Girls were more likely to be in the HbA1c trajectory with the highest starting HbA1c and significant deterioration during adolescence, and in the highest two BMI z-score trajectories. Patients with non-white race had the highest odds of belonging to a less favorable HbA1c trajectory. Prevalence of the high stable HbA1c trajectory decreased and prevalence of the low stable HbA1c trajectory increased over the study period. CONCLUSIONS: A minority of youth with T1D experienced deterioration of glycemic control during adolescence. Girls were more likely to belong to the worst HbA1c trajectory and to BMI z-score trajectories in the overweight/obese range, which may increase cardiometabolic risk. Addressing racial/ethnic disparities in glycemic control should remain a priority. Advances in T1D management correlated with favorable shifts in HbA1c trajectory prevalence.

The cysteine-rich interdomain region from the highly variable plasmodium falciparum erythrocyte membrane protein-1 exhibits a conserved structure.

Plasmodium falciparum malaria parasites, living in red blood cells, express proteins of the erythrocyte membrane protein-1 (PfEMP1) family on the red blood cell surface. The binding of PfEMP1 molecules to human cell surface receptors mediates the adherence of infected red blood cells to human tissues. The sequences of the 60 PfEMP1 genes in each parasite genome vary greatly from parasite to parasite, yet the variant PfEMP1 proteins maintain receptor binding. Almost all parasites isolated directly from patients bind the human CD36 receptor. Of the several kinds of highly polymorphic cysteine-rich interdomain region (CIDR) domains classified by sequence, only the CIDR1alpha domains bind CD36. Here we describe the CD36-binding portion of a CIDR1alpha domain, MC179, as a bundle of three alpha-helices that are connected by a loop and three additional helices. The MC179 structure, containing seven conserved cysteines and 10 conserved hydrophobic residues, predicts similar structures for the hundreds of CIDR sequences from the many genome sequences now known. Comparison of MC179 with the CIDR domains in the genome of the P. falciparum 3D7 strain provides insights into CIDR domain structure. The CIDR1alpha three-helix bundle exhibits less than 20% sequence identity with the three-helix bundles of Duffy-binding like (DBL) domains, but the two kinds of bundles are almost identical. Despite the enormous diversity of PfEMP1 sequences, the CIDR1alpha and DBL protein structures, taken together, predict that a PfEMP1 molecule is a polymer of three-helix bundles elaborated by a variety of connecting helices and loops. From the structures also comes the insight that DBL1alpha domains are approximately 100 residues larger and that CIDR1alpha domains are approximately 100 residues smaller than sequence alignments predict. This new understanding of PfEMP1 structure will allow the use of better-defined PfEMP1 domains for functional studies, for the design of candidate vaccines, and for understanding the molecular basis of cytoadherence.