Trabecular Bone Score (TBS) Varies with Correction for Tissue Thickness Versus Body Mass Index: Implications When Using Pediatric Reference Norms.

Trabecular bone score (TBS) derived from secondary analysis of lumbar spine dual-energy X-ray absorptiometry (DXA) scans improves fracture prediction independent of bone mineral density (BMD) in adults. The utility of TBS to assess fracture risk in younger patients has not been established because pediatric norms have been lacking. Robust TBS reference data from the Bone Mineral Density in Childhood Study (BMDCS) have been published. TBS values for the BMDCS study were derived using an algorithm that accounts for tissue thickness (TBSTH ) rather than the commercially available algorithm that adjusts for body mass index (BMI; TBSBMI ). We examined the magnitude of differences in TBSTH and TBSBMI in a cohort of 189 healthy youth. TBS values using both algorithms increased with age and pubertal development in a similar pattern. However, TBSBMI values were systematically and significantly higher than TBSTH (mean = 0.06, p < 0.0001). The difference between calculated TBSBMI and TBSTH was not uniform. Differences were greater at lower TBS values, in males, in older individuals, in those at later Tanner stages, and in those with a greater BMI Z-score. These systematic differences preclude the development of a simple formula to allow conversion of TBSBMI to TBSTH "equivalents." Because of these systematic differences in these two algorithms, using an individual's TBSBMI to calculate a Z-score using the BMDCS TBSTH reference values results in a falsely higher TBS Z-score (differences mean = 0.73, interquartile range [IQR] = 0.3 to 1.6). Until TBSTH software for Hologic DXA equipment becomes commercially available, BMDCS TBS reference norms should not be used. © 2023 American Society for Bone and Mineral Research (ASBMR).

Treatment Practices and Confidence in the Management of Pediatric Metabolic Bone Disorders.

INTRODUCTION: Pediatric metabolic bone and mineral disorders encompass a wide variety of disorders that can be challenging to diagnose and treat because of inadequate physician training about optimal management. METHODS: As practice variation and confidence levels may impact clinical outcome, we sought to assess physician confidence in managing pediatric metabolic bone and mineral disorders and the spectrum of treatment practices among members of the Pediatric Endocrine Society (PES) and the Canadian Pediatric Endocrine Group (CPEG). Questionnaires were distributed via e-mail to all members of the PES and CPEG and 244 were completed. Responses were summarized using descriptive statistics, and proportions were compared using χ2 or Fisher's exact tests, as appropriate. RESULTS: Variations were observed among the respondents' confidence in the management of bone disorders and in the criteria used to initiate/discontinue intravenous bisphosphonates or prescribe burosumab therapy. Respondents felt confident with the management of 4 out of 20 pediatric bone conditions (confidence was defined as >90% of respondents reporting feeling "somewhat confident" or "very confident"). Physicians working in a bone clinic were more confident in prescribing burosumab for the treatment of X-linked hypophosphatemic rickets compared to those not working in a bone clinic (65% vs. 47%, p = 0.03). Most respondents (52%) reported having received inadequate training in pediatric metabolic bone and mineral disorders. DISCUSSION/CONCLUSION: Dedicated training, knowledge acquisition, and education resources are needed to increase confidence and standardize the use of bone-targeted therapies.

Finding Meaning in Medicine: Pediatric Residents' Perspectives on Humanism.

OBJECTIVES: To explore pediatric residents' perspectives on humanism and how residency impacts humanism formation. METHODS: We conducted a qualitative study with pediatric resident focus groups at Stanford. Transcripts were analyzed by two investigators using grounded theory. Subsequent literature review led us to adapt Cruess et al. 2015's professional identity formation framework to describe development of a humanism identity in residency. Member check was done to verify themes and the adapted framework. RESULTS: Thirty two pediatric residents participated. Five themes emerged: 1) Empathy, compassion, and respect are foundational elements of humanism. 2) Each resident had a unique view of humanism derived from personal values. 3) Residents felt that the terms excellence and resilience (Gold Foundation IECARES model) did not fit with their own definitions. 4) Residents felt that humanism is a central part of their practice and training. 5) The demands, structure, and culture of residency were often in conflict with promoting humanism in residents. Based on residents' perspectives, we modified the professional identity formation and socialization conceptual framework proposed by Cruess et al. 2015 to reflect humanism identity formation during pediatrics residency. The new framework emphasizes the increased power of the healthcare system and unconscious acquisition on humanism formation in residency as compared to medical school. CONCLUSIONS: Residents believe that humanism is a core part of practicing medicine and should be reinforced during residency training. Cruess' professional identity and socialization framework is a tool for a better understanding of the complexity of humanism development in residency.

Hormonal Contraception and Bone Health in Adolescents.

Hormonal contraception is prescribed commonly to adolescents for myriad indications from pregnancy prevention to treatment for acne, hirsutism or dysmenorrhea. Although use of these hormones generally has no effect or benefits bone health in mature premenopausal women, the same may not be true for adolescents. The teen years are a critical period for acquiring peak bone strength. Sex hormones, growth hormone, and insulin-like growth factors (IGFs) interact to modulate the changes in bone size, geometry, mineral content, and microarchitecture that determine skeletal strength. Combined oral contraceptives (COCs) and intramuscular depo medroxyprogesterone (DMPA) can compromise the expected gains in adolescence by altering estrogen and IGF concentrations. Use of these medications has been associated with slower accrual of bone mineral density (BMD) and increased fracture risk in some studies. Far less is known about the skeletal effects of the newer long acting reversible contraceptives (LARCs). This review takes a critical look at the gaps in current knowledge of the skeletal effects of COCs, DMPA, and LARCs and underscores the need for additional research.

The Golden Ticket Project for peer recognition.

BACKGROUND: A supportive working environment can be protective against burnout in residency training. To help foster a positive culture, we developed the 'Golden Ticket Project' (GTP) and evaluated it as a tool for peer appreciation. METHODS: In October 2013-July 2014, all paediatric residents (n = 83) at a paediatric residency programme were invited to participate in the GTP. Through an electronic form, residents could award a co-resident with a 'Golden Ticket' for any behaviour that they were grateful for. Two authors manually coded and analysed the tickets using content analysis to identify recurring themes. We also distributed an anonymous survey to all residents to assess their attitudes toward the GTP. RESULTS: The majority (61%) of residents either gave or received 'Golden Tickets' (51/83). Reasons for receiving tickets fell into five categories: (i) teamwork; (ii) positive attitude; (iii) patient care; (iv) resident-resident support; and (v) supervisory skills. Across all three classes, teamwork was recognised most frequently, noted in 43% of all tickets. The recognition of other behaviours varied by class. Fifty-five percent (46/83) of residents completed the programme evaluation survey. Seventy-six percent (35/46) of respondents wanted the programme to continue; of those, 49% (17/35) 'agreed' or 'strongly agreed' that they were 'more aware of acts of kindness in the residency program because of the GTP'. A supportive working environment can be protective against burnout in residency training DISCUSSION: The GTP is a feasible and valued peer-support initiative for residents. The GTP characterised behaviours and attitudes that residents were grateful for in their peers. These behaviours could inform the development of wellness interventions for residents that focus on building supportive working environments.

Osteoporosis in Children with Chronic Illnesses: Diagnosis, Monitoring, and Treatment.

PURPOSE OF REVIEW: Osteoporosis is an under-recognized complication of chronic illness in childhood. This review will summarize recent literature addressing the risk factors, evaluation, and treatment for early bone fragility. RECENT FINDINGS: Criteria for the diagnosis of pediatric osteoporosis include the presence of low trauma vertebral fractures alone or the combination of low bone mineral density and several long bone fractures. Monitoring for bone health may include screening for vertebral fractures that are common but often asymptomatic. Pharmacologic agents should be offered to those with fragility fractures especially when spontaneous recovery is unlikely. Controversies persist about the optimal bisphosphonate agent, dose, and duration. Newer osteoporosis drugs have not yet been adequately tested in pediatrics, though clinical trials are underway. The prevalence of osteoporosis is increased in children with chronic illness. To reduce the frequency of fragility fractures requires increased attention to risk factors, early intervention, and additional research to optimize therapy and potentially prevent their occurrence.

A Case Report of Hypoglycemia and Hypogammaglobulinemia: DAVID Syndrome in a Patient With a Novel NFKB2 Mutation.

Context: Deficient anterior pituitary with variable immune deficiency (DAVID) syndrome is a rare disorder in which children present with symptomatic adrenocorticotropic hormone (ACTH) deficiency preceded by hypogammaglobulinemia from B-cell dysfunction with recurrent infections, called common variable immunodeficiency (CVID). Subsequent whole exome sequencing studies have revealed germline heterozygous C-terminal mutations of NFKB2 as a cause of DAVID syndrome or of CVID without clinical hypopituitarism. However, to the best of our knowledge there have been no cases in which the endocrinopathy has presented in the absence of a prior clinical history of CVID. Case Description: A previously healthy 7-year-old boy with no history of clinical immunodeficiency presented with profound hypoglycemia and seizures. He was found to have secondary adrenal insufficiency and was started on glucocorticoid replacement. An evaluation for autoimmune disease, including for antipituitary antibodies, was negative. Evaluation unexpectedly revealed hypogammaglobulinemia [decreased immunoglobulin G (IgG), IgM, and IgA]. He had moderately reduced serotype-specific IgG responses after pneumococcal polysaccharide vaccine. Subsequently, he was found to have growth hormone deficiency. Six years after initial presentation, whole exome sequencing revealed a de novo heterozygous NFKB2 missense mutation c.2596A>C (p.Ser866Arg) in the C-terminal region predicted to abrogate the processing of the p100 NFKB2 protein to its active p52 form. Conclusions: Isolated early-onset ACTH deficiency is rare, and C-terminal region NFKB2 mutations should be considered as an etiology even in the absence of a clinical history of CVID. Early immunologic evaluation is indicated in the diagnosis and management of isolated ACTH deficiency.

Tumor-induced Osteomalacia in a 3-Year-Old With Unresectable Central Giant Cell Lesions.

Tumor-induced osteomalacia (TIO) is a rare cause of hypophosphatemia involving overproduction of fibroblast growth factor 23. TIO has been described largely in adults with small mesenchymal tumors. We report a case of TIO in a child who presented with knee pain and radiographic findings concerning for rickets, and was found to have maxillomandibular giant cell lesions. The patient was treated with oral phosphorus and calcitriol, surgical debulking, and intralesional corticosteroids, which resulted in tumor regression and normalization of serum fibroblast growth factor 23 and phosphorus. This case illustrates the occurrence of this rare paraneoplastic syndrome in children and adds to our knowledge about clinical manifestations and pathologic findings associated with pediatric TIO.

Controlled Pilot Study of High-Impact Low-Frequency Exercise on Bone Loss and Vital-Sign Stabilization in Adolescents With Eating Disorders.

PURPOSE: Adolescents with anorexia nervosa (AN) face an increased lifetime risk of bone fragility. This randomized controlled study examined the efficacy and safety of a high-impact activity program on markers of bone turnover and stabilization of vital signs (VSS). METHODS: Forty-one hospitalized adolescents with AN were randomly assigned to routine care or routine care plus 20 jumps twice daily. Bone markers were measured at baseline days 1-3 (T1), days 4-6 (T2), and days 7-9 (T3). The primary outcome was change in bone-specific alkaline phosphatase (BSAP) at T3 adjusted for BSAP and % median body mass index at T1. Secondary outcomes were serum N-telopeptide (NTX) and osteocalcin at T3. Safety was determined by comparing weight gain, time to VSS and length of stay for each group. RESULTS: BSAP, NTX, or osteocalcin did not differ between groups at baseline or at T3. BSAP and NTX at T3 were not associated with group of enrollment or % median body mass index. VSS was significantly reduced in the intervention group compared with the control group (11.6 ± 5.7 days vs. 17 ± 10.5 days, p = .049). There was no significant difference in weight gain or length of stay between groups. CONCLUSIONS: Twice-daily jumping activity failed to influence markers of bone turnover in adolescents with AN but was well tolerated, shortened time to vital-sign stabilization and did not slow weight gain.

Bone Densitometry in Children and Adolescents.

Concerns about bone health and potential fragility in children and adolescents have led to a high interest in bone densitometry. Pediatric patients with genetic and acquired chronic diseases, immobility, and inadequate nutrition may fail to achieve expected gains in bone size, mass, and strength, leaving them vulnerable to fracture. In older adults, bone densitometry has been shown to predict fracture risk and reflect response to therapy. The role of densitometry in the management of children at risk of bone fragility is less clear. This clinical report summarizes current knowledge about bone densitometry in the pediatric population, including indications for its use, interpretation of results, and risks and costs. The report emphasizes updated consensus statements generated at the 2013 Pediatric Position Development Conference of the International Society of Clinical Densitometry by an international panel of bone experts. Some of these recommendations are evidence-based, whereas others reflect expert opinion, because data are sparse on many topics. The statements from this and other expert panels provide general guidance to the pediatrician, but decisions about ordering and interpreting bone densitometry still require clinical judgment. The interpretation of bone densitometry results in children differs from that in older adults. The terms "osteopenia" and "osteoporosis" based on bone densitometry findings alone should not be used in younger patients; instead, bone mineral content or density that falls >2 SDs below expected is labeled "low for age." Pediatric osteoporosis is defined by the Pediatric Position Development Conference by using 1 of the following criteria: ≥1 vertebral fractures occurring in the absence of local disease or high-energy trauma (without or with densitometry measurements) or low bone density for age and a significant fracture history (defined as ≥2 long bone fractures before 10 years of age or ≥3 long bone fractures before 19 years of age). Ongoing research will help define the indications and best methods for assessing bone strength in children and the clinical factors that contribute to fracture risk. The Pediatric Endocrine Society affirms the educational value of this publication.

A Multifaceted Mentoring Program for Junior Faculty in Academic Pediatrics.

PROBLEM: The departure of physician-scientists from education and research into clinical practice is a growing challenge for the future of academic medicine. Junior faculty face competing demands for clinical productivity, teaching, research, and work-life integration, which can undermine confidence in the value of an academic career. Mentorship is important to foster career development and satisfaction in junior faculty. INTERVENTION: The goals of this academic pediatrics department were to develop, implement, and evaluate a multifaceted pediatric mentoring program to promote retention and satisfaction of junior faculty. Program elements included one-on-one mentor-mentee meetings, didactic workshops, grant review assistance, and facilitated peer-group mentoring. Program effectiveness was assessed using annual surveys of mentees and structured mentee exit interviews, as well as retention data for assistant professors. CONTEXT: The mentees were instructors and assistant professors in the department of pediatrics. OUTCOME: Seventy-nine mentees participated in the program from 2007 through 2014. The response rate from seven annual surveys was 84%. Sixty-nine percent of mentees felt more prepared to advance their careers, 81% had a better understanding of the criteria for advancement, 84% were satisfied with the program, and 95% found mentors accessible. Mentees who exited the program reported they most valued the one-on-one mentoring and viewed the experience positively regardless of promotion. Retention of assistant professors improved after initiation of the program; four of 13 hired from 2002 to 2006 left the institution, whereas 18 of 18 hired from 2007 to 2014 were retained. LESSONS LEARNED: This multifaceted mentoring program appeared to bolster satisfaction and enhance retention of junior pediatric faculty. Mentees reported increased understanding of the criteria for promotion and viewed the program as a positive experience regardless of career path. Individual mentor-mentee meetings were needed at least twice yearly to establish the mentoring relationship. Identifying "next steps" at the end of individual meetings was helpful to hold both parties accountable for progress. Mentees most valued workshops fostering development of tangible skills (such as scientific writing) and those clarifying the criteria for promotion more transparent. Facilitated peer-group mentoring for mentees at the instructor rank provided valuable peer support.

Primary ovarian insufficiency in adolescents: a case series.

BACKGROUND: Primary ovarian insufficiency (POI) is characterized by 4 to 6 months of amenorrhea and elevated serum FSH and LH in females less than 40 years. Ovarian insufficiency is uncommon in pediatrics and typically results from a chromosomal abnormality or treatment for malignancy. Idiopathic POI in which no apparent precipitant is identified is even rarer. After encountering three teens with idiopathic POI in recent months, we utilized an informatics-enabled search of the electronic medical records from our hospital to identify all cases of idiopathic POI presenting from 1998-2013. CASES PRESENTATION: 15 girls (ages 14.4 to 17.9 years) met criteria for idiopathic POI. At diagnosis, breast development ranged from Tanner stage 1 to 5; 6 of 15 patients had secondary amenorrhea. All patients presented in the past 11 years and 13 of 15 in the past 5 years. CONCLUSIONS: In this first case series of POI from the United States, we observed a clustering at our institution in recent years. If an increased incidence of idiopathic POI is identified at other institutions, further investigation into potential environmental and genetic precipitants is warranted.

Large Doses of Vitamin D Fail to Increase 25-Hydroxyvitamin D Levels or to Alter Cardiovascular Risk Factors in Obese Adolescents: A Pilot Study.

PURPOSE: Vitamin D deficiency and cardiometabolic risk factors are common in obese adolescents. Observational studies demonstrate an inverse relationship among serum 25-hydroxyvitamin D (25OHD) and obesity, insulin resistance, and inflammatory cytokines. This pilot study explores if vitamin D supplementation could reduce serum concentrations of inflammatory cytokines (interleukin [IL] 6, IL-10, tumor necrosis factor α), adiponectin, lipids, hemoglobin A1C, and high-sensitivity C-reactive protein (hs-CRP). A secondary aim was to determine the associations between baseline serum 25OHD concentrations and body mass index (BMI), hs-CRP, inflammatory cytokines, and lipids. METHODS: Overweight and obese adolescents enrolled in this 24-week, randomized, double-blind study were given 150,000 IU ergocalciferol or placebo at baseline and 12 weeks. Outcome measurements included serum 25OHD, inflammatory cytokines, adiponectin, hs-CRP, lipids, hemoglobin A1C, and BMI at baseline, 12, and 24 weeks. RESULTS: Of 40 participants, 31 (78%) completed the study. Mean ± standard error 25OHD levels were similar in vitamin D and placebo groups at baseline (19.6 ± 5.3 vs. 25.8 ± 10.8 ng/mL) and 24 weeks (20.1 ± 3.4 vs. 24.6 ± 8.4 ng/mL). Inflammatory and cardiovascular markers were not significantly different between groups at 24 weeks. Serum 25OHD at baseline was associated with BMI (r = -.44 [95% confidence interval, -.66 to -.15]) but not with other outcome measures. CONCLUSIONS: Supplementation with vitamin D at 150,000 IU every 3 months failed to increase serum 25OHD or alter inflammatory markers and lipids in overweight and obese youth. Further studies are needed to establish the dose of vitamin D required to increase 25OHD and determine potential effects on metabolic risk factors in obese teens.

Diagnosis and treatment of pediatric osteoporosis.

PURPOSE OF REVIEW: Progress toward identifying and treating disorders of bone fragility in pediatric patients has been considerable in recent years. This article will summarize several key advances in the management of osteoporosis in children and adolescents. RECENT FINDINGS: Recommendations from the 2013 pediatric Position Development Conference provide expert guidance for evaluating bone health in younger patients. The diagnosis of pediatric osteoporosis can be made in a child with low-trauma vertebral fractures or a combination of low bone mass and long bone fractures. Management of bone fragility includes optimizing nutrition, activity, and treatment of the underlying disease. Pharmacologic agents can be considered if these measures fail to prevent further bone loss or fractures. Although the efficacy and safety of several intravenous and oral bisphosphonates have been examined, there is still no consensus on the optimal drug, dose, or duration of treatment. Observational studies of children with secondary osteoporosis provide insight into risk factors for fracture or the potential for recovery. SUMMARY: Despite advances in the diagnosis and treatment of pediatric osteoporosis, more research is needed. Randomized controlled trials of pharmacologic agents should be defined to target those identified at the highest risk by observational studies. VIDEO ABSTRACT: http://links.lww.com/COE/A9

Dual-energy X-ray absorptiometry interpretation and reporting in children and adolescents: the revised 2013 ISCD Pediatric Official Positions.

The International Society for Clinical Densitometry Official Revised Positions on reporting of densitometry results in children represent current expert recommendations to assist health care providers determine which skeletal sites should be measured, which, if any, adjustments should be made, reference databases to be used, and the elements to include in a dual-energy X-ray absorptiometry report. The recommended scanning sites remain the total body less head and the posterior-anterior spine. Other sites such as the proximal femur, lateral distal femur, lateral vertebral assessment, and forearm are discussed but are only recommended for specific pediatric populations. Different methods of interpreting bone density scans in children with short stature or growth delay are presented. The use of bone mineral apparent density and height-adjusted Z-scores are recommended as suitable size adjustment techniques. The validity of appropriate reference databases and technical considerations to consider when upgrading software and hardware remain unchanged. Updated reference data sets for all contemporary bone densitometers are listed. The inclusion of relevant demographic and health information, technical details of the scan, Z-scores, and the wording "low bone mass or bone density" for Z-scores less than or equal to -2.0 standard deviation are still recommended for clinical practice. The rationale and evidence for the development of the Official Positions are provided. Changes in the grading of quality of evidence, strength of recommendation, and worldwide applicability represent a change in current evidence and/or differences in opinion of the expert panelists used to validate the position statements for the 2013 Position Development Conference.

Burnout in pediatric residents over a 2-year period: a longitudinal study.

OBJECTIVE: Burnout is a work-related syndrome characterized by emotional exhaustion (EE), depersonalization (DP), and lack of personal accomplishment (PA). We hypothesized that the transition into an environment of high physical, intellectual, and emotional demands of the medical profession would lead to an increase in the prevalence of burnout in pediatric residents, which would remain high throughout residency. METHODS: The Maslach Burnout Inventory (MBI) was administered to pediatric residents at Lucile Packard Children's Hospital 6 times between February 2010 and February 2012. These times corresponded to the start of residency, mid-intern year, end-intern year, mid-junior year, end-junior year, and mid-senior year. RESULTS: Mean values of burnout components changed significantly between the start of residency and mid-intern year. EE increased from 15.8 to 24.5 (P < .001), DP increased from 4.5 to 9.2 (P < .001), and PA decreased from 40.2 to 38.3 (P = .04). Similarly, the prevalence of burnout increased from 17% to 46% (P = .012), or 2% to 24% (P = .002) using more restrictive criteria, between the start of residency and mid-intern year. Significant changes in mean scores or prevalence of burnout were not found between any other consecutive times throughout residency. CONCLUSIONS: This longitudinal study documented a significant increase in the components of burnout among pediatric residents between the start of residency and mid-intern year, which persisted through the PGY2 and PGY3 years. Further studies are warranted to identify correlates of resident burnout and to develop preventative strategies to reduce its occurrence.