bluuebunny/crossref_metadata_2025_split huggingface dataset:
SAT-356 Reversible Suppression of Serum 1,25-Dihydroxyvitamin D in Williams Syndrome
Authors: Torchinsky Michael Yuri , Bugaieski Eric 
Published: May 2020

Abstract
Background: Hypercalcemia has been reported in 5% to 50% of patients with Williams syndrome; however, observations about the role of 1,25-dihydroxyvitamin D in hypercalcemia related to Williams syndrome have been contradictory. Objective: The objective was to investigate the mineral metabolism in an 11-month-old patient with Williams-Beuren syndrome who had hypercalcemia, hypercalciuria and nephrocalcinosis. Methods: We reviewed test results in an 11-month-old infant with Williams syndrome who developed hypercalcemia following excessive dietary calcium intake that was two to three times higher than recommended for age because he continued receiving a premature formula with high calcium content from birth. Results: Our patient presented with feeding difficulties, daily vomiting, weight loss, and constipation. He had serum total calcium 14.8 mg/dL, phosphorus 4.0 mg/dL, PTH &lt;4 pg/mL, 1,25-dihydroxyvitamin D &lt;8 pg/mL, 25-hydroxyvitamin D 29 ng/mL, PTH-related peptide 3.7 pmoL/L (expected &lt;4.2), alkaline phosphatase 118 U/L, beta-crosslaps 1257 pg/mL, and urinary calcium/creatinine ratio 1.19. XR Skeletal Survey revealed increased bone density in the metaphyseal regions, in particular, above the knee, distal radius and ulna that may result from hypercalcemia; no evidence of rickets, osteoporosis or congenital osteodystrophy was seen. Renal ultrasound revealed increased echogenicity of the renal medullary pyramids consistent with medullary nephrocalcinosis. Hypercalcemia and hypercalciuria were initially treated with IV fluids and Furosemide IV, and resolved only after Pamidronate 0.5 mg/kg/dose IV x 2 doses. After serum calcium normalized, the patient’s symptoms resolved, PTH recovered to 18 pg/mL (expected 10 - 65), and 1,25-dihydroxyvitamin D increased to 27 pg/mL (expected 24 - 86). Chromosomal micro-array analysis showed a 1.9 megabase deletion at 7q11.23 that overlapped the Williams syndrome critical region including the ELN gene, consistent with diagnosis of Williams syndrome. Serum calcium remained normal with dietary calcium restriction using a low-calcium formula and complementary foods. Conclusions: PTH-independent hypercalcemia in our patient with Williams syndrome was due to calcium overload resulting from dietary calcium excess in addition to possibly enhanced intestinal calcium absorption. Serum 1,25-dihydroxyvitamin D was undetectable and returned to normal only with resumption of PTH secretion required for its synthesis after hypercalcemia resolved. The balance between bone formation and resorption was shifted to resorption possibly to remove skeletal calcium excess, based on normal alkaline phosphatase (marker of osteoblast activity) and high collagen beta-crosslaps (marker of osteoclast activity). A premature formula with high calcium content should be switched to a full-term formula when risk for rickets of prematurity clears.
MON-065 Prevention of 6-Mercaptopurine-Induced Hypoglycemia by Levocarnitine Replacement
Authors: Torchinsky Michael Yuri , Miller Mark , Benavides Valeria 
Published: May 2020

Abstract
Background: Symptomatic hypoglycemia has been reported in children less than 6 years of age receiving 6-Mercaptopurine for acute lymphoblastic anemia (ALL); however, the mechanism of 6-Mercaptopurine-induced hypoglycemia has been unclear. Objective: The objective was to investigate the metabolism in a 3-year-old patient with hypoglycemia induced by 6-Mercaptopurine during maintenance therapy for ALL. Methods: We reviewed test results including serum total and free carnitine levels at time of hypoglycemia in a 3-year-old child with ALL who had repeatedly low plasma glucose as a side effect of 6-Mercaptopurine. Hypoglycemia defined as plasma glucose &lt;50 mg/dL was detected using a glucose meter and each time verified in the clinical laboratory. After Levocarnitine was added to 6-Mercaptopurine therapy, the glycemic control was assessed using a glucose meter, the clinical laboratory, and a continuous glucose monitor; serum total and free carnitine levels were repeated when hypoglycemia resolved. Results: Our patient presented with fatigue, tremors and plasma glucose 39 mg/dL to 47 mg/dL, corrected with a sugary beverage. He had plasma ACTH 38 pg/mL, serum cortisol 10 mcg/dL, total carnitine 24 nmol/mL (expected 35 - 84), free carnitine 18 nmol/mL (expected 24 - 63), plasma free fatty acids 1.62 mmol/L (expected &gt;1.5), beta-hydroxybutyrate of 0.2 mmol/L to 0.7 mmol/L (expected &gt;2.0), and urine ketone bodies negative when plasma glucose was between 33 mg/dL and 39 mg/dL. Plasma insulin was undetectable at time of hypoglycemia, and serum glucose increased by less than 30 points in response to Glucagon IV. Serum IGF-1 as measure of growth hormone effect and thyroid function were normal. Hypoglycemia continued to daily recur especially during the night despite bedtime snacks high in complex carbohydrates and was prevented only by Levocarnitine 25 mg/kg/dose every 12 hours PO that raised serum total and free carnitine levels. The patient remained hypoglycemia-free one month after Levocarnitine was added to 6-Mercaptopurine therapy, in particular, he had average glucose 114 mg/dL with standard deviation 30 mg/dL, glucose 70 mg/dL to 140 mg/dL 93% of the time, and glucose &lt;55 mg/dL 0% of the time. Conclusions: Our patient had symptomatic hypoketotic hypoglycemia related to moderately reduced serum total and free carnitine, corrected with Levocarnitine replacement. Increase in plasma free fatty acids without expected increase in plasma and urine ketone bodies may be sign of impaired synthesis of carnitine, which is required for transport of fatty acids into the mitochondria to produce ketone bodies. Measuring serum total and free carnitine in hypoglycemia induced by 6-Mercaptopurine is helpful in identifying children, who may benefit from Levocarnitine replacement.
MON-099 The Association Between C-Reactive Protein, Metabolic Syndrome, and Pre-Diabetes in Korean Children and Adolescents
Authors: Kim Jihyun , Lee Jong Bin , Lim Jung Sub 
Published: May 2020

Abstract
Objective: Cardiovascular disease (CVD) is a leading cause of death worldwide, including in Korea. In adults, the risk for CVD is increased approximately three-fold in subjects with metabolic syndrome (MetS), and the hazard ratio for subjects with MetS has been reported as 1.37 for mortality from CVD after adjustment for other confounders. Furthermore, MetS in childhood predicts adult MetS and T2DM 25 to 30 years later. Because MetS is a state of chronic low-grade inflammation, measurements of the circulating levels of the inflammatory molecules might provide diagnostic and therapeutic approaches to modulate or alter disease progression. High sensitivity C-reactive protein (hsCRP), a biomarker of inflammation, has emerged as an independent predictor of CVD and T2DM development. The aim of this paper is to evaluate the association between hsCRP and MetS and its components in Korean children and adolescents. Methods: We performed a cross-sectional analysis using data from the Korea National Health and Nutrition Examination Survey (KNHANES) 2016–2017. We analyzed the data of 1,247 subjects (633 males, 14.2 ± 2.7 years) from the KNHANES 2016–2017. MetS were defined by the modified NCEP-ATP III criteria. Results: Among the 1,247 subjects (mean age: 14.2 ± 2.7 years), the prevalence of MetS was 5.8% (7.0% in male subjects and 4.6% in female subjects; p = 0.070). The mean hsCRP level was 0.861 ± 1.567 mg/l (median and interquartile range: 0.370 and 0.430mg/l). Subjects with MetS had higher hsCRP levels than subjects without MetS (geometric mean: 1.08 vs. 0.46 mg/l, p &lt; 0.001). The prevalence of MetS in the lowest, second, third, and highest hsCRP quartiles were 1.8%, 2.4%, 3.5%, and 15.2%, respectively. Compared to the lowest quartile, the odds ratio (OR) for having MetS in the highest quartile was 8.414 (3.272–21.638), adjusting for age and sex. The OR for having abdominal obesity and low HDL-C in the highest quartile were 9.657 (4.818–19.355) and 2.408 (1.286–4.510), adjusting for age, sex, and other components of the MetS. Additionally, the OR for having pre-diabetes (HbA1c ≥ 5.7%) in the highest quartile was 2.061 (1.097–3.870). Conclusion: Serum hsCRP level is positively associated with MetS and pre-diabetes in Korean children and adolescents.
SUN-133 Intratumoral Expression of Steroid Receptors, CD68+ Macrophages and Mdm2 in Different Molecular-Biological Types of Endometrial Cancer
Authors: Berstein Lev M , Ivantsov Alexander , Vasilyev Dmitry , Iyevleva Aglaya 
Published: May 2020

Abstract
Background and aims : In Risk Classifier for Endometrial Cancer/EC (ProMisE) 4 molecular biological types of this tumor are described recently [1,2,3] and need an additional research, including evaluation of hormone-associated characteristics of both tumor tissue and patients [4]. Aim of the work was a study of estrogen (ER) and progesterone (PR) receptors in comparison with CD68+ macrophage infiltration (which promotes the invasion of tumor cells into the myometrium [5]) and expression of MDM2 protein, a negative regulator of p53 [6], in EC types presented in the ProMisE classification. Materials and methods: The tumor tissue of 218 EC patients included in the study (mean age 60.6 years) was assigned according to the data of genetic and immunohistochemical analysis to the types of carcinomas with gene POLE mutations, deficiency of mismatch repair proteins (MMR-D), expression (positive or diffuse) of p53 oncoprotein and to the type without characteristic molecular profile, WCMP. Immunohistochemistry was used also for evaluation of ER (Ventana antibodies, clone SP1) and PR (Ventana antibodies, clone 1E2) according to Allred, MDM2 (antibodies ABCAM, dilution 1:200) and macrophage marker CD68 (DAKO antibodies, clone CD8/144B). Results: According to the averaged data, the highest expression of ER and PR was found in EC types MMR-D and WCMP, and the lowest, respectively, in types POLE and p53+. Most often, positive expression of MDM2 (in 93.2% and 96.9% of studied cases) was detected respectively in MMR-D and p53+ type of EC, indicating, therefore, a positive relationship between MDM2 and the presence of steroid receptors in the first of these types (MMR-D) and negative - in the second of them (p53+). Expression of CD68+ macrophages demonstrated (contrary to the EC types POLE and p53+) a tendency to the lower values in types MMR-D and WCMP (128.0 ± 8.1 and 113.5 ± 6.3 cond.un.), i.e. in tumors with potential sensitivity to estrogen. Conclusions: The results indicate the importance of taking into account of both - the molecular biological type of the EC as well as the role of microenvironment of tumor cells, including the colonization of the neoplasm’ tissue by macrophages (and possibly lymphocytes) and the features of hormonal signal transmission in it. For further analysis, it is desirable to consider also the EC histotype, as one of the factors underlying various prognostic groups of this tumor [7].
References:
1.Talhouk A, McAlpine JN. Gynecol Oncol Res Pract. 2016; 3:14.
2. Talhouk et al., Cancer. 2017; 123(5):802–813.
3. Kommoss FK et al., Br J Cancer. 2018;119(4):480–486.
4. Berstein et al. Future Oncol. 2019; 15(12):1335–1346.
5. Jing X. et al. Immunol Cell Biol. 2019; 97(6):563–576.
6. Zou X. et al. Medicine (Baltimore).2018;97(49):e13273.
7. Bosse T. et al. Am J Surg Pathol. 2018 May;42(5):561–568.
SAT-285 TSH/TSHR Signaling Deficiency Impairs Spatial Learning and Memory
Authors: Luan Sisi , Zhao He , Zhao Jiajun 
Published: May 2020

Abstract
Background: Subclinical hyperthyroidism is associated with cognitive impairment, but the mechanism has remained unclear. As subclinical hyperthyroidism is characterized by significantly decreased TSH levels, this study aimed to investigate whether TSH regulates cognitive function. Methods: The correlation between TSH and cognitive impairment was investigated in a cross-sectional population study. The role of TSH/TSH receptor (TSHR) signaling in spatial learning and memory was further examined by behavior tests in Tshr-/- mice. Dendritic spine, synaptic density and structure of hippocampal CA1 pyramidal neurons were detected by Golgi’s method and electron microscopy. The mRNA and protein expression levels of learning and memory-related genes were assessed by RNA sequencing, real-time PCR, immunoblotting and immunofluorescence approaches. Results: Serum TSH level correlated negatively with cognitive impairment in the current population. Consistently, Tshr deletion in mice led to significantly compromised performance in hippocampus-dependent tasks, reduced dendritic spine density and excitatory synaptic density as well as altered synaptic structure in CA1 subfield of the hippocampus. Furthermore, the mRNA levels of learning and memory-related genes were altered, and protein levels of CREB-regulated genes were downregulated in the hippocampus of Tshr-/- mice. Conclusions: These findings reveal that TSH/TSHR signaling ablation impairs spatial learning and memory, indicating a decline in TSH level might contribute to the increased prevalence of cognitive impairment in subclinical hyperthyroidism patients.
MON-079 Prepubertal Gynecomastia Secondary to Excessive Soy Consumption
Authors: Sea Jessica , Abramyan Mikayel , Chiu Harvey Kenn 
Published: May 2020

Abstract
Enlargement of breast tissue in males, or gynecomastia, is a rare condition in prepubescent boys. While the majority of cases are idiopathic, we describe an 8-year-old patient who developed unilateral gynecomastia secondary to marked dietary soy consumption. Soy products, particularly those consumed by our patient, contain high levels of phytoestrogens which have been documented in limited case studies to contribute to abnormal development of breast tissue in adolescent and adult males. To our knowledge, this is the first documented case of gynecomastia occurring in a prepubescent patient resulting from excessive intake of dietary soy. Importantly, we also report a complete resolution of gynecomastia upon exclusion of dietary products containing significant amounts of soy. While soybeans and soy-derived products can be an important source of nutrition for some, those with abnormal sensitivity to phytoestrogens may benefit from limiting dietary soy consumption to avoid potential adverse effects, including gynecomastia.
SUN-018 No Difference in Breastfeeding Rates in Women with Polycystic Ovary Syndrome
Authors: Bui Leeann , Cooney Laura , Birstler Jen 
Published: May 2020

Abstract
No Difference in Breastfeeding Rates in Women with Polycystic Ovary Syndrome
Objective: Women with PCOS have increased rates of obesity and gestational weight gain compared to women without PCOS, factors which are associated with decreased breastfeeding (BF). Thus, our objective was to evaluate if women with PCOS were less likely to initiate BF.
Design: Cross-sectional analysis of participants in the PRAMS (Pregnancy Risk Assessment Monitoring System) dataset, a national questionnaire from the Centers for Disease Control (CDC) sent to postpartum mothers 2–9 months after delivery. PCOS status and BF were by self-report. Logistic regression was used to assess odds of ever BF. Length of BF was assessed using Cox proportional hazards with right censoring for women who were still BF at the time of follow-up. PRAMS complex survey design was accounted for.
Results: PCOS status was available for 14 states. Median response time was 3.7 months postpartum. Data from 16,036 participants were included which represents 855,302 women due to sample weights. 6.6% of women reported having PCOS and 83.8% reported ever BF. Compared to women with a normal BMI, women who were overweight or obese had decreased odds of BF (OR: 0.7, 95% CI: 0.6–0.9, P=0.01; OR: 0.6, 95% CI: 0.5–0.7, P&lt;0.001 respectively); however, PCOS was not associated with BF (OR: 1.1, 95% CI: 0.9–1.3, P=0.6). In multivariate analysis, women with PCOS still were at no decreased odds of BF after adjusting for age, BMI, race, ethnicity, infertility treatment, and delivery factors (ORadj:1.1; 95% CI: 0.8–1.4; P=0.6). Variables associated with decreased odds of BF included: overweight/obesity, age ≤ 19 yrs (vs. 25–29), Black race, smoking, undesired pregnancy intent, gestational age ≤27 wks, and prior live birth. Variables associated with increased BF included: age 30–39 yrs, hospital stay 1–2 days (vs. 3–5), Hispanic ethnicity, and ≥ 3 life stressors. In multivariable Cox models, women with PCOS did not have a shorter length of BF (HRadj: 0.9, 95% CI: 0.8–1.1, P=0.3).
Conclusion: Given the rise of the national rates of obesity and clear maternal and neonatal benefits to breastfeeding, understanding the predictors of BF success is paramount. In this national survey, women with PCOS were at no decreased odds of BF, despite confirming the association between overweight/obesity and decreased BF. However, our data still supports the clinical relevance of carefully targeting women with PCOS for BF education due to the association of PCOS with increased BMI. Additional prospective studies are needed to fully understand the association between PCOS and BF.
OR32-04 Dynamic Interactions Between Luteinizing Hormone and Testosterone in Healthy Community-Dwelling Men: Impact by Age and Body Composition
Authors: Roelfsema Ferdinand , Liu Peter Y , Takahashi Paul , Yang Rebecca , Veldhuis Johannes D 
Published: May 2020

Abstract
Context. Aging is associated with diminished testosterone (Te) secretion, which could be attributed to Leydig cell dysfunction, decreased pituitary stimulation and altered Te feedback. Objective. The goal was to quantify all three regulatory nodes of the GnRH-LH-Leydig cell- axis in the same cohort of healthy men, by measuring (1) indirectly the strength of the endogenous GnRH signal on the gonadotrope, (2) the strength of Te feedback on LH by ketoconazole (KTCZ), and (3) the effect of LH infusions on Te secretion, in relation to age and body composition.
Design. This was a placebo-controlled, blinded, prospectively randomized cross-over study in 40 men, age 19–73 yr, BMI 20–34.3 kg/m2. A submaximal dose of ganirelix (GnRH antagonist) was used to assess outflow of GnRH, by calculating the difference between LH output during the control and ganirelix arm. Ketoconazole (steroidogenic inhibitor) was used to estimate feedback, by the difference in LH output during ketoconazole and control arm. High-dose ganirelix and repeated 6-min LH (18.75 IU) infusions were used to measure testicular responsivity. Blood sampling was at 10-min intervals. The 4 sessions were concluded with, a single submaximally GnRH stimulus to assess the responsiveness of the gonadotrope during ganirelix inhibition.
Setting. The study was performed in a Clinical Translational Research Unit.
Interventions. In 3 of the 4 experiments subjects underwent 5 h of blood sampling at 10-min intervals, starting at 0800 h. At 1100 h GnRH was injected and sampling was continued for another 2 h. Admission was at 1700 h the day before. At 2000 h they received KTCZ, dexamethasone or ganirelix and/or placebo. KTCZ and dexamethasone (or placebo) were administered again at 0700 when the IV catheter was placed. High-dose ganirelix was used to test the testicular responsiveness, and 7 LH pulses (90 min intervals) were given., with blood sampling from 1500 till 1300 h next day.
Outcome measures. Mean concentrations of LH and (bio)Te, deconvolution analysis, endogenous dose-response LH-bioTe relation, and approximate entropy. Abdominal visceral fat (AVF) was calculated from single slice CT.
Results. There were age-, but not body composition-related decreases in estimated endogenous GnRH secretion, Te’s feedback strength on LH, and Leydig cell responsivity to LH, accompanied by changes in approximate entropy. Bioavailable Te levels were negatively related to both age and AVF, without interaction between these variables. The LH response to a submaximal dose of GnRH was independent of age and AVF.
Conclusion. Advancing age is associated with 1) attenuated bioavailable Te secretion caused by diminished GnRH outflow and not by decreased GnRH responsivity of the gonadotrope, 2) diminished testicular responsivity to infused LH pulses, and 3) partial compensation by diminished Te feedback on central gonadotropic regulation.
MON-612 Vitamin D and Its Relation to Type II Diabetes Control and Complications
Authors: Alswat Khaled Abdullah 
Published: May 2020

Abstract
Vitamin D and its Relation to Type II Diabetes Control and Complications
Background:
Type II diabetes (T2D) prevalence in Saudi Arabia is among the highest in the MENA region according to the recent International Diabetes Federation published statistics. Recent study suggested that around 54% of the screened T2D patients have vitamin D deficiency. Our aim is to assess the prevalence of vitamin D deficiency and its relation to glycemic control and T2D related complications.
Methods:
We conducted a cross-sectional study at the Diabetes Center, Taif, Saudi Arabia for T2D patients whom age &gt; 18 years and were seen in the clinic between Aug2015-Jan2017 and agreed to participate. We excluded those with gestational diabetes and type I diabetes. Baseline characteristics and measurement were obtained by the participated physician. Laboratory data was collected from the patient’s EMR. We considered those whom have vitamin D level below 30 (ng/ml) to be deficient.
Result:
A total of 228 patients with a mean age of 59.1 + 12.5 years, diabetes duration of 10.6 + 8.8 years, BMI of 33.4 + 6.2 kg/m2, heart rate 81.6 + 12.5, systolic BP 130.8 + 19.6, diastolic BP 75.4 + 9.9, fasting glucose 9.4 + 9.5 mmol/l, HbA1c 8.0 + 2.1%, vitamin D 23.2 + 12.3 ng/ml, serum creatinine 73.8 + 22.2, total cholesterol 4.5 + 1.1, LDL 2.7 + 0.9, triglyceride 1.6 + 0.8.
76.3% of the screened patients had vitamin D deficiency. Compared to those with vitamin D deficiency, those with normal vitamin D level less likely to have hypertension (P 0.521), to be male (P 0.028), to have microalbuminuria (P 0.331), and to be diagnosed with neuropathy and retinopathy (P 0.431 and 0.185 respectively). Also those with normal vitamin D were older (P 0.537),has shorter T2D duration (P 0.231), higher BMI (P 0.097), lower pulse rate (P 0.127), lower SBP (P 0.228), higher DBP (P 0.275), lower HbA1c (P 0.027), lower FBG (P 0.093), lower serum creatinine (P 0.039), and lower total cholesterol and LDL (P 0.497 and 0.404 respectively) when compared to those with vitamin D deficiency.
Adjusting for age, gender, diabetes duration, BMI, SBP, DBP, vitamin D supplements and dosage, there was non-significant correlation between HbA1c and vitamin D level.
Conclusion:
Vitamin D deficiency is highly prevalent among our sample of T2D patients. In the non-adjusted modules, vitamin D deficiency were non-significantly associated with more microvascular complications and worse measured cardiovascular markers. Also, T2D patients with vitamin D deficiency had significantly higher HbA1c which were non-significant when adjusted for related variables.