bluuebunny/crossref_metadata_2025_split huggingface dataset:
8768 Initiation of Exogenous Sex Hormones in a Pediatric Gender Clinic
Authors: Roberts Christina M , Knoll Michelle M , Robertson Gail , Egan Anna , Moser Christine 
Published: October 2024

Abstract
Disclosure: C.M. Roberts: None. M.M. Knoll: None. G. Robertson: None. A. Egan: None. C. Moser: None.
Introduction: The demographics of patients seeking care for gender dysphoria have changed significantly since care was started in 1987. The criteria for prescribing sex hormones have also evolved to allow a greater range of patients to receive gender-affirming hormones. We examined factors associated with prescription of gender-affirming hormones to patients presenting for gender-affirming care our clinic. Methods: Secondary analysis of sex hormone initiation as a minor among 14-17-year-old adolescents diagnosed with gender dysphoria, seen in the pediatric gender clinic between 2/19/2018 and 7/20/2022. We extracted demographic variables, appointment dates, and sex hormone prescription data from our electronic medical record. We collected patient reports of gender identity and suicidal behavior, and parent and adolescent reports of general adolescent well-being, months of gender dysphoria and social transition, and parental support for the adolescent’s gender identity from electronic clinic surveys. Results: We identified 273 adolescent-parent dyads with complete data. Most of our sample were assigned female at birth (74.4%), transgender male (59.3%), and white (83.5%) adolescents who lived in urban (86.4%) and lower-middle-income (64.5%) areas. The average age was 15.9+/-1.1 years. A minority of adolescents reported recent suicidal ideation 60/273 (22%), and prior suicide attempts 20/273 (7%). Parents reported a shorter duration of gender dysphoria (-16.7 months (95%CI: -22.0, -11.5)) and social transition (-4.7: -6.5, -3.0), and higher levels of parental support than adolescents. Approximately half, 146/273 (53.5%), of adolescents started sex hormones. The average age at initiation was 16.5 years +/- 0.9. Age at presentation, length of gender dysphoria or social transition, and the presence of suicidal ideation or attempts were not associated with initiation of sex hormones. Assigned males (OR:1.82:95%CI 1.04, 3.19), adolescents with higher parent-reported parental support for the adolescent’s identity (1.03:1.01, 1.04), and adolescents with private insurance rather than Medicaid (Blue Cross- 5.39:2.73, 10.64) were more likely to start sex hormones as a minor. Conclusion: Published criteria for the initiation of sex hormones among adolescents with gender dysphoria, including age, persistence of dysphoria, and the presence of suicidality, did not predict the prescription of sex hormones to minors in our clinic. Parent-reported support for the adolescent’s gender identity played a small role in predicting the prescription of hormones, but male sex assigned at birth and having private health insurance played larger roles. Further exploration of these findings is needed to ensure equity in the treatment of gender dysphoria.
Presentation: 6/3/2024
7269 Interim Analysis of Gonadotropin-Releasing Hormone Agonist (GnRHa) Treatment in Children with Central Precocious Puberty: Focus on FMV Urinary LH Levels and Clinical Outcomes
Authors: Luo Xiaoping , Hou Ling , Li Yanhong , Sun Yan , Jin Xin Kinson 
Published: October 2024

Abstract
Disclosure: X. Luo: Advisory Board Member; Self; Takeda. L. Hou: Advisory Board Member; Self; Takeda. Y. Li: Advisory Board Member; Self; Takeda. Y. Sun: Advisory Board Member; Self; Takeda. X.K. Jin: Employee; Self; Takeda.
Background: Central precocious puberty (CPP) refers to early activation of the hypothalamic–pituitary–gonadal (HPG) axis in children. There is an urgent need to understand the treatment pattern and clinical outcomes among children suffering from CPP. Studies have reported that first morning voided (FMV) urinary Luteinizing hormone (LH) and Follicle stimulating hormone (FSH) have substantial association with CPP tested in GnRH stimulation test and can be useful in diagnosis and management of CPP in children. Gonadotropin-releasing hormone agonists (GnRHa) such as Leuprolide acetate (LA) are the recommended treatment for children suffering from CPP. The current study aimed to provide the information on level of LH and FSH in FMV urine when CPP diagnosis at baseline and the present results may also revealed trend of FMV-LH and FMV-FSH change during LA intervention in children with CPP. Here we present interim results from this study. Methods: This is an open-label, multicentre, single arm, prospective study that enrolled 31 subjects with CPP. Individuals were administered LA depot 11.25 mg, subcutaneously every 12 weeks. The study comprises a screening period; a 6-month treatment period: 24 weeks; and a post-treatment follow-up period: 12 weeks. The FMV urinary LH and FSH were diagnosed by Clopper-Pearson method with a 95% confidence interval. Results: Due to the data collection time point for the interim analysis, only 8 female subjects reached the 12-week visit time point and had a 12-week visit for FMV urinary Gn testing. Therefore, for this interim analysis, only FMV Gn decline at week 12 of the 8 subjects who had a 12-week visit will be presented. At baseline, the mean value of FMV urinary LH and FSH is 0.58 IU/L and 2.27 IU/L respectively. The FMV Gn urinary LH was demonstrated a decrease in 87.5% (95% CI: 47.35,99.68) of the children after LA intervention. Additionally, urinary FSH value of FMV urinary Gn was found to decrease in 75.00% of the children (95% CI: 34.91,96.81) compared to baseline FSH value. Conclusion: The present study reveals a decreasing trend in FMV Gn urinary LH and FSH levels in CPP children treated with LA during interim analysis. LA was shown to be safe and effective in ameliorating the level of LH and FSH in children suffering from CPP. From the study, it can be concluded that urinary LH and FSH may act as a potential biomarker for diagnosis and management of CPP.
Presentation: 6/3/2024
7039 Isolated Gonadotropin Deficiency Transmitted From Mother to Daughter Conceived Through Assisted Reproduction Due Newly Identified, Autosomal Dominant Mutation Within the SOX10 Gene
Authors: Bakjaji Shadi , Hoffman Robert Paul 
Published: October 2024

Abstract
Disclosure: S. Bakjaji: None. R.P. Hoffman: None.
Title: Isolated gonadotropin deficiency transmitted from mother to daughter conceived through assisted reproduction due newly identified, autosomal dominant mutation within the SOX10 gene. Objectives: We report a case of genetic isolated gonadotropin deficiency occurring in a mother and in her daughter conceived through assisted reproductive technology using mother’s ovum. Methods Case report Results: The daughter was initially seen at 12 years 0 months for evaluation of short stature and concerns she had not grown in the last year. She was otherwise in good health and reported normal sense of smell. At the time of assessment, her height was 132.6 cm (0.61 percentile; Z=-2.5). The mother had been diagnosed with Kallmann’s syndrome and achieved pregnancy through ovarian stimulation. Her height was 152.4 cm, while the father's height was 177.8 cm. Two male siblings were born at the same time and were in normal health. Physical examination of the daughter was unremarkable. She had Tanner stage 1 pubic hair and Tanner stage 1 breasts. The growth chart displayed a notable deceleration in growth Laboratory results showed normal free T4 of (0.9 ng/dL) with slightly elevated TSH (8.348 uIU/mL), positive thyroid antibodies, normal IGFI at 164 ng/mL (normal range for Tanner I: 54 - 301) and IGFBP-3 at 5.8 ug/mL (2.4 - 8.4 ug/mL). The karyotype was 46 XX. Her peak growth hormone using glucagon-arginine stimulation was 12.8 ng/ml. When no pubertal development was seen over the next 6 months, GnRH stimulation testing revealed baseline LH of 0.04 mIU/ml and a peak of 1.78 and baseline FSH of 0.41 mIU/ml with a peak of 2.83. Bone age was 8 years 4 months.Over the next year her growth velocity was 4.8 cm/year and she remained prepubertal. Genetic testing done identified a new heterozygous sequence variant (c.226G&gt;A) in the SOX10 gene which would lead to replacement of valine by isoleucine at position 76 and was thought to be damaging in silico. This variant was subsequently found to be shared with her mother but not her father. Conclusions: Most Kallmann’s syndrome or genetic isolated gonadotropin deficiency cases are due to autosomal dominant but spontaneous new mutations since infertility is usually present in those with the syndrome. This case emphasizes the importance of genetic counselling regarding inheritance risk when assisted reproduction technology using maternal ovum is used.
Presentation: 6/2/2024
8517 Low Peak GH in Stimulation Tests Among Obese Children; Is it Clinically Meaningful?
Authors: Strich D , Meirman C , Edri S , Gillis D J 
Published: October 2024

Abstract
Disclosure: D. Strich: None. C. Meirman: None. S. Edri: None. D.J. Gillis: None.
Background: Peak growth hormone (GH) levels in stimulation tests are lower in obese subjects. The clinical significance of this finding is unclear. It has been suggested that results of tests should be adjusted for body mass index (BMI). Aims: A. To evaluate the association between GH peak levels and body mass index (BMI ) in a single center. B. To discuss the biological and clinical significance of the findings and make recommendations based on this study and other data from the medical literature. Methods: Peak GH levels and BMI standard deviation scores (BMI-SDS) for a total of 988 tests (Clonidine - 694, Glucagon - 198, Arginine - 96) performed in one center were analyzed. Studies referring to the biology of growth hormone and to the evaluation of GH deficiency were reviewed. Results: In the testing center studied, mean peak GH levels were significantly lower in arginine (p=0.015) and clonidine tests (p=0.018) starting at BMI standard deviation score over 1 compared to below 1 BMI-SDS. The differences still remained significant for a subgroup with peaks below 7.5 ng/dl (p=0.003 and p=0.042 respectively). The proportion of overweight and obese children in our cohort and in similar cohorts of children undergoing stimulation tests was about tenfold lower than in the corresponding general population. In other studies, short obese patients responded better than non-obese to GH treatment and had greater increases both in height and IGF-1. Obese adult patients with low GH peaks had high carotid intima-media thickness compared with non GH deficient obese. Conclusion: Obese children are generally tall and therefore are not referred for testing. Those who are short, tend to have lower peaks than their lean peers in GH stimulation tests and respond better to GH therapy. These patients have biologically significant GH deficiency. We do not recommend "correcting" GH test results for BMI in the decision algorithm for GH therapy.
Presentation: 6/3/2024
7439 Mercury toxicity presenting as Pheochromocytoma
Authors: Ansar Aisha , McNerney Kyle , Marshall Bess Adkins 
Published: October 2024

Abstract
Disclosure: A. Ansar: None. K. McNerney: None. B.A. Marshall: None.
Background: Mercury intoxication is a rare cause of hypertension in children and can mimic other endocrinopathies such as pheochromocytoma. Mercury affects catecholamine metabolism and can cause elevated catecholamine levels, thereby incorrectly suggesting a catecholamine-secreting tumor. Clinical Case: An 8-year-old girl presented to the ED with progressive night sweats, intractable migratory myalgias, hypertension and tachycardia. Patient was in her usual state of health until 2 weeks before presentation when she started to have worsening intermittent pains in her chest, arms, back, and knees. She also experienced restlessness and inability to sleep. On presentation she was hypertensive, with blood pressure of 146/116 mm Hg while calm, heart rate 125 bpm, afebrile. She was anxious, irritable, and seemed uncomfortable. Her skin was dry and rough with a scaly eczematous rash on the arms and legs. She had persistently elevated blood pressure in upper and lower extremities, unremarkable echocardiogram and electrocardiogram, and a normal result on fundoscopic examination. Her initial electrolytes, creatinine, and urinalysis were all normal and remained so on serial evaluations. Urine drug screen was negative. Thyroid function panel showed normal TSH with slightly elevated Free T4 2.28 ng/dL (0.9-1.7), but a normal Free T3 3.9 pg/mL (2.0-4.4) and Free T4 by equilibrium dialysis 1.8 ng/dL (0.8 - 2.0). CT of the chest, abdomen and pelvis was negative and did not show any lesions. Plasma renin activity was 4.6 ng/mL/hour, supine, (&lt;2.0) and plasma aldosterone level &lt; 4.0 ng/dL (&lt; 40 supine) were normal. 24-hour urinary norepinephrine was 90 μg/24h (13-65), epinephrine 37 μg/24h (0.2-10), urine metanephrines 195 μg/24h (18-144), urine normetanephrines 305 μg/24h (29-145), all elevated. Further history taking revealed that patient was taking Ayurvedic supplements prepared by hand by medicinal personnel in India for 3 months prior to presentation. These were stopped 2 weeks prior to her presentation, when she first became symptomatic. Her blood mercury level was 11 ng/mL (n &lt;9), and urinary mercury excretion 67 μg/24 hours (n&lt;10), both obtained 3 weeks after stopping supplementation. She was started on oral chelation therapy with succimer 10 mg/kg/day. Her hypertension was controlled with doxazosin. One week after initiation of therapy, her symptoms improved, and she became normotensive. Her acrodynia and irritability resolved. Urine metanephrine levels will be repeated at upcoming clinic follow up. Conclusion: Mercury toxicity should be considered in the differential of pheochromocytoma to avoid extensive diagnostic testing and morbidity associated with it. Lead, mercury, and arsenic have been detected in traditionally prepared Ayurvedic supplements. A careful history of intake of supplements can be important in cases with unusual symptomatology.
Presentation: 6/3/2024
7814 Non-fasting and Fasting Mixed Meal Tolerance Tests Equally Capture the Triglyceride Response in Participants With and Without MASLD
Authors: Gonzalez Ramirez Lucia A , He Zhulin , Binongo Jose N , Huneault Helaina E , Ramirez Tovar Ana Maria , Welsh Jean A , Vos Miriam B 
Published: October 2024

Abstract
Disclosure: L.A. Gonzalez Ramirez: None. Z. He: None. J.N. Binongo: None. H.E. Huneault: None. A. Ramirez Tovar: None. J.A. Welsh: None. M.B. Vos: Consulting Fee; Self; Boehringer Ingelheim, Prosciento, Target Real World Solutions, Intercept Pharmaceuticals, INC, Novo Nordisk. Grant Recipient; Self; Target Real World Solutions.
Mixed meal tolerance tests (MMTTs) are functional assessments that may identify metabolic dysregulation earlier than fasting tests during the progression to metabolic dysfunction-associated steatotic liver disease (MASLD). Although standards to conduct MMTTs in adults are readily available, the knowledge gap for children is quite large. Furthermore, conducting this type of study in children is challenging because they are less tolerant of long fasting intervals, long periods of testing, and early morning visits that interrupt school attendance. The objective of this study was to assess if conducting an MMTT in a non-fasting state will equally identify variance in the metabolic response of triglycerides (TG) between healthy children and those living with MASLD when compared to a 12-hour fasting MMTT. We collected blood samples every two hours in our 2-day randomized crossover feeding study. During each visit, 9 children with MASLD and 10 healthy controls aged 10 to 18 y underwent 1 standardized MMTT after a 12-hour overnight fasting (breakfast), followed by 2 standardized MMTTs in a non-fasting state (lunch and dinner). Participants were randomized to receive a glucose or fructose beverage at visit 1 and the other at visit 2. For uniformity, we only used data from the glucose beverage visit. We found that TG levels were statistically higher in the MASLD participants when compared to the non-MASLD group at each time point (p= 0.01). The maximum TG metabolic response peak for each MMTT was higher and more delayed in the MASLD group compared to the non-MASLD group. Within groups, postprandial TG level patterns were not significantly different when comparing breakfast, lunch, and dinner. Although TG levels varied throughout the day, the pattern after every meal was similar. These findings suggest that it is possible to identify differences in the TG metabolic response between children living with MASLD and healthy participants in the postprandial state. The results from our study also support the use of non-fasting MMTTs when examining TG metabolism in children, though further research to validate these findings is needed.
Presentation: 6/1/2024
12626 Non-high-density Lipoprotein Cholesterol For Detecting Dyslipidemia Among Korean Adolescents
Authors: Nam H K , Kang E , Rhie Y J , Lee K H 
Published: October 2024

Abstract
Disclosure: H. Nam: None. E. Kang: None. Y. Rhie: None. K. Lee: None.
Objectives: Non-high-density lipoprotein (HDL) cholesterol is an alternative method to assess dyslipidemia. We aimed to assess the validity of non-HDL cholesterol for detecting dyslipidemia among Korean adolescents. Methods: Data for 6,989 adolescents aged 10 to 19 years (3,684 boys and 3,305 girls), were obtained from the Korean National Health and Nutrition Examination Surveys. Dyslipidemia was defined by having one of the followings; total cholesterol ≥200 mg/dL, triglyceride ≥130 mg/dL, low-density lipoprotein (LDL) cholesterol ≥130 mg/dL, or HDL cholesterol &lt;40 mg/dL. Non-HDL cholesterol ≥145 mg/dL was used to detect dyslipidemia based on National Heart, Lung, and Blood Institute 2011. Results: The overall prevalence of dyslipidemia were 27% and 37% among Korean adolescents based on calculated and measured LDL cholesterol level, respectively. The prevalence of non-HDL cholesterol level ≥145 mg/dL were 7.1% and 8.5% in teenage boys and girls, respectively. Dyslipidemia was found in 97.1% and 97.7% of boys and girls with high non-HDL level, respectively. High non-HDL cholesterol level especially detected high measured LDL-cholesterol level with a sensitivity of 96.3%, and a specificity of 96.3%, and a sensitivity of 95.2%, and a specificity of 97.1%, in teenage boys and girls. Using non-HDL cholesterol was more detected high LDL-cholesterol level compared to using total cholesterol alone in teenage boys and girls. Conclusions: Screening dyslipidemia using non-HDL cholesterol level was more useful than using total cholesterol level alone, especially in detecting high LDL cholesterol level. Non-HDL cholesterol was not required fasting and was appeared to be a reliable screening test also in adolescents.
Presentation: 6/1/2024
7685 Outcomes of Low-Risk Differentiated Thyroid Carcinomain a Predominantly Hispanic Pediatric Population
Authors: Delgado E A , Martinez A , Solano N , Baboun D , Alkhoury F , Carrillo-Iregui A , Bustamante Escobar C L 
Published: October 2024

Abstract
Disclosure: E.A. Delgado: None. A. Martinez: None. N. Solano: None. D. Baboun: None. F. Alkhoury: None. A. Carrillo-Iregui: None. C.L. Bustamante Escobar: None.
Background: The American Thyroid Association (ATA) guidelines for the management of low-risk differentiated thyroid carcinoma (DTC) in children, recommend total thyroidectomy and central neck dissection without iodine ablation. Surveillance for recurrence in these patients includes serial TSH-suppressed thyroglobulin levels and thyroid ultrasounds. Iodine ablation is reserved for patients classified as ATA intermediate and high-risk given their risk of persistent cervical disease and distant metastasis, respectively. There is ongoing concern whether withholding Iodine-131 will result in higher rates of disease recurrence among ATA low-risk pediatric patients. To our knowledge this is the first study addressing a predominantly Hispanic pediatric population. Objective: Evaluate the recurrence rate of differentiated thyroid cancer (DTC) in ATA low risk patients since implementing the 2015 ATA pediatric guidelines in a predominantly Hispanic pediatric population. Methods: After obtaining Institutional Review Board approval, we performed a retrospective chart review of patients 19 years and younger, between 2012 and 2023, for DTC. We analyzed demographics, histopathological diagnoses, risk stratification, and post-diagnosis follow up. Results: From a cohort of 63 pediatric patients diagnosed with DTC, a significant majority (n=55, 87%) comprised Hispanic individuals, while non-Hispanic cases represented the minority (n= 8, 13%). The mean age at diagnosis was 14.7 ± 2.6 years. Papillary thyroid carcinoma emerged as the prevailing type in 58 (92%), while 5 (8%) were follicular carcinoma. Among the patients, 12 (20%) were categorized as low risk level and underwent surgery alone. Over a median follow-up period of 2.6 years (IQR 2.97, range 0.94-5.18), 2 out of the 12 (18%) in this low-risk group experienced disease recurrence, with an average time to recurrence of 1.6 ± 0.9 years. Notably, one of the patients had a history of autoimmune thyroiditis. Subsequently, these 2 patients underwent further evaluation and treatment with iodine ablation. Conclusion: In a free-standing hospital with a predominantly Hispanic population, we found a recurrence rate of 18% in patients with ATA low-risk DTC not treated with radioactive iodine. Our rates align closely with those observed in earlier studies, which featured a non-Hispanic population and reported a 24% recurrence rate during the surveillance period. This underscores the crucial role of surveillance in low-risk patients with DTC in a Hispanic pediatric population.
Presentation: 6/1/2024