ResearchPad - pediatric-obesity-thyroid-and-cancer https://www.researchpad.co Default RSS Feed en-us © 2020 Newgen KnowledgeWorks <![CDATA[MON-103 Pattern and Predictors of Thyroid Dysfunction Among Paediatric Endocrine Referrals at Tertiary Care Centre: A Longitudinal Study]]> https://www.researchpad.co/article/elastic_article_8735 Background Post iodisation era has experienced gradual change in pattern of thyroid disorders among paediatric population with autoimmunity taking precedence over iodine deficiency disorders and subclinical hypothyroidism (SCH) now more frequently diagnosed but inappropriately managed. Aims This study was conducted to evaluate pattern of abnormal thyroid function among children referred to our tertiary care centre, to ascertain characteristics that influence treatment decisions and to follow them for various outcome measures. Design It was an observational longitudinal follow up study where all children less than 18 years, referred to our outpatient clinic for suspected thyroid disorder were recruited. Demographic data, personal and family history, clinical features were noted and laboratory tests including TT4, TT3, TSH, anti-thyroid peroxidase(antiTPO) and anti-thyroglobulin(antiTG) antibody were conducted in study subjects. Management was based on the clinical judgment of the attending endocrinologist. Patients were followed at 6 week, 3 months, 6 months and one year with clinical and laboratory work up at each visit. Results A total of 241 subjects aged 18 days to 17 years were included out of which 62.25% were females. Initial evaluation revealed SCH in 40% of refereed subjects, overt hypothyroidism (OH) in 33%, congenital hypothyroidism (CH) in 18% and overt thyrotoxicosis in 5%. Autoimmune thyroiditis constituted the major cause of hypothyroidism in the OH group with significantly higher prevalence of anti-TPO and antiTG antibody in comparison of SCH group (61% vs 31%; 45% vs 21.9%, p<0.05) respectively. All subjects in OH group were treated whereas 76% subjects in SCH group were treated and the mean dose of L thyroxine required to treat OH was significantly higher (2.31+1.1ug/kg/day vs 1.76+1.07ug/kg/day; p<0.001) in comparison of SCH group. A major independent predictor of treatment in SCH was initial TSH which was significantly higher in the treated group (11.65 + 3.80 uIU/ml vs 9.24 + 1.31 uIU/ml; p<0.001). Subjects with congenital hypothyroid presented at a mean age of 6 months (18 days to 2 years) with most common aetiology being thyroid hypoplasia and dyshormonogenesis

(20% each). Graves’ disease was diagnosed in 11 out of 12 subjects with thyrotoxicosis and were treated with antithyroid drugs. Overall 85.5% of refereed subjects were treated and after one-year follow up management was found to be adequate in 81% subjects. Conclusions The evolving trend of diagnosing children having nonspecific symptoms with SCH is a matter of concern as many are subjected to the burden of unwanted prolonged treatment and frequent testing as highlighted in our study. Delayed presentation of CH in our study warrants active surveillance of children at birth for thyroid disorders to avoid long term adverse effects on mental development.

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<![CDATA[MON-101 Costa Rican National Primary Congenital Hypothyroidism Screening Program Evaluation. Retrospective Cohort Trial Between 2015 and 2017]]> https://www.researchpad.co/article/elastic_article_6930 Congenital hypothyroidism (CH) is one of the leading causes of intellectual impairment worldwide in infancy. The newborn screening has been able to prevent this mental disability, by a prompt initiation of therapy. Over the last years the incidence of HC has been increasing, mainly by lowering the screening cut-off level that leads to detection of milder cases. There is conflicting evidence if children with mild CH without treatment may develop neurological impairment in the future.

Costa Rican newborn screening program in divided in three stages, measuring serum TSH concentrations from a heel prick aliquot of capillary blood dried onto a filter paper. Each test has different TSH cut-off values to determine if the newborn needs a clinical evaluation by an endocrinologist, needs another screening test or rules out hypothyroidism.

We developed an observational, descriptive, retrospective study, based on medical records, to evaluate our newborn screening program performance. The study included the total national population of screened newborns from 2015 to 2017. Descriptive analysis and analytical analysis of variables were done, and test’s sensitivity and specificity were determined.

The study analyzed 204.241 screened newborns, and 145 children referred to the Endocrinology Department of the National Children Hospital. This population represents 97% of births in these 3 years. The recall rate for a first positive test was 0.3%. Congenital hypothyroidism was confirmed in 73% of children referred to the Endocrinology Department because of a positive screening. Incidence was 1/1926 births. Detection rate was independent of birth weight nor gestational age. 45.3% of children diagnosed with CH were detected after the first screening test, 52.8% after the second screening test and 1.9% after the third screening test. Screening test analysis showed high sensitivity and specificity, with diagnostic accuracy above 90%, except for the third screening test. Free thyroxine measurements alone weren’t useful to predict CH diagnosis.

The coverage of the national neonatal screening program extends almost to the entire population. Our test specificity is within international standards. Incidence of CH in Costa Rica is similar to that reported in medical literature. With current detection cut-off level, there is no need of performing a second mandatory evaluation to preterm and low weight newborns. The third screening test has a low performance rate, does not improve detection of children with CH significantly, and delays clinical evaluation by the endocrinologist. Prognosis of treated children with CH is excellent, with no evidence of severe cognitive deficit.

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<![CDATA[MON-092 Clinical Characteristics and Management of Thyroid Cancer in Pediatrics: Results from the Mexican Population Registry Between 2010-2019]]> https://www.researchpad.co/article/elastic_article_6772 METHODS

Study: Analytical and retrospective

Patients files diagnosed with Thyroid Cancer treated during January 2010 to May 2019, who underwent surgical intervention and histopathological study were reviewed. Chi Square test were used as statistical analysis. A level of significance p <0.001 was established.

INTRODUCTION: Cancer thyroid in pediatrics is characterized by advanced presentation, coupled with frequent lymph nodal metastases and often pulmonary metastases. There are few reports on the rate of cancer and hypoparathyroidism in children.

OBJECTIVE: Describe of pediatric thyroid cancer with particular emphasis on the clinical characteristics, risk factors associated and with treatment outcomes

RESULTS

43 patients were included in the study 69.8% were female. Average age of 12 years (range 5 to 17 years). Association of cancer and thyroiditis 25% and Cancer with Graves Disease 2.3%.

Histopathological diagnoses of thyroid cancer: 86% papillary, 2.3% follicular, 11.6% medullary, 69.8% patients presented with metastases, most often lymph node (46.5%), pulmonary (2.3%) and pulmonary plus lymph node (23.3%), 11.6% patients do not present metastasis and 16.3% without data.

Surgical interventions: only thyroidectomy 16.3%, Hemitiroidectomy 4.7%, total thyroidectomy with lymph node emptying 74% and modified radical neck dissection 5%.In 16% of the patients, second surgery was required after the histopathological report. Post-surgical hypoparathyroidism was recorded in 27 patients (62%): transitory in 11 (25%) and 16 (37%) permanent.

Biochemical variables: (N /%/ Hypoparathyroidism /NO hypoparathyroidism) of 18 patients with post-surgical PTH <10 pg/ml (18/42%/17/1) 17 of them presented post-surgical hypoparathyroidism (P <0.001), PTH >10 pg/ml (13/30%/9/4), and without post-surgical measurement of PTH (12/28% / 6/6). Post-surgical ionized calcium <4mg/dl in 29 patients of which 22 had hypoparathyroidism (P <0.001) (29 / 67.4% / 22/7), with calcium >4 mg/dL (14 / 32.6% / 9/5). 21 patients with seric calcium <8mg/dl 20 with hypoparathyroidism. (21/48%/ 20/1) (P <0.001). Seric calcium >8 (16/37%/9/7) without measurement data. Serial calcium in 6 patients (6/15% / 3/3). Postoperative positive thyroglobulin parameter was presented as an risk factor for complication and metastases OR 1.42 (1-1.6)

Regarding iodine treatment, 29 patients received an average dose of 200 (range 100- 720 milicuries mCi).

CONCLUSIONS

Papillary cancer is the most common type in pediatric patients, evidence of metastases find in 69.8% most frequent lymph node, postoperative thyroglobulin OR 1.42. They should always be considered in the management of these patients. As a post-surgical complication, there is an increased risk to present hypoparathyroidism when PTH less < 10 pg / ml, post-surgical ionized calcium < 4 mg/dl and serum calcium <8 mg/dl.

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<![CDATA[MON-083 Childhood Diabetic Ketoacidosis (DKA) in New-Onset and Established Patients in Central Illinois: Contributing Factors and Risk Stratification]]> https://www.researchpad.co/article/elastic_article_6726 Introduction: DKA is the leading cause of hospitalizations in children with type 1 diabetes mellitus (T1DM). Although most cases are preventable, DKA continues to occur in established patients.

Aim: To identify contributing factors and outcomes of DKA pediatric admissions in a tertiary referral center with a large rural catchment area to assess for actionable items to prevent DKA.

Methods: A retrospective, single-center chart review assessing children ˂19 years old admitted in DKA from October 2014 to May 2018. DKA was defined as a pH of ≤7.3 or bicarbonate of ≤15. Demographic data included gender, age, zip code, insurance type and ethnicity. Admission measures included HbA1c, DKA group (new-onset “NT1” or “ET1” established T1DM diagnosis), DKA severity (severe pH <7.1, CO2 <5mEq/L), contact with clinic, home insulin delivery. Outcomes included length of stay (LOS), total admission costs (TAC) and reimbursements amounts (RA).

Results: 272 patients were included (mean age 11.7 y, range 4.4-16; 60% female, 83% Caucasian, 14% African American). Of these, 33% were NT1 DKA. Compared to NT1 DKA, ET1 DKA patients were older (8.7 vs. 13.1 years, p < 0.001), more likely female (49% vs. 65%, p 0.034) with public insurance (55% vs 63%, p 0.028); 73% didn’t contact the diabetes team prior to admission and 52% used an insulin pump. There were no significant differences in HbA1c or DKA severity.

LOS was similar between NT1 and ET1 DKA (p 0.051). Severe DKA was associated with longer LOS (RR 1.47, p < 0.0001). Public vs. private insurance was associated with 1.28 times longer LOS (p < 0.0001).

While there was no difference in TAC between NT1 and ET1 DKA groups (p 0.877), costs were higher with public vs. private insurance (>$900, p 0.050) and severe DKA (RR 1.92; 95% CI 1.62-2.27; p <0.0001). TAC were different between regions within central Illinois (RR 1.39; 95% CI 1.08-1.80; p 0.002).

Hospital RA was higher for NT1 vs. ET1 group (RR 1.26; 95%CI 1.03-1.54; p 0.0237) and higher DKA severity (RR 1.57; 95% CI 1.26-1.95; p <0.0001); but lower for public vs. private insurance (RR 0.43; 95% CI 0.35-0.52; p <0.0001).

Discussion: Established DKA patients tended to be rural teenage females, poorly controlled and public health insured. Severity of DKA and LOS did not differ between the groups. While TAC were similar among the groups, TAC were higher with public insurance and severe DKA. Lower hospital RA were seen for recurrent cases and public insurance. This study provides valuable information about non-metropolitan at-risk population characteristics to inform targeted preventive interventions. These findings suggest a significant difference in hospitalization RA, providing incentive for health care facilities / providers to invest in early outpatient interventions and QI initiatives.

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<![CDATA[MON-102 Serum Concentrations of FT4 and TSH in the First Six Months of L-Thyroxine Treatment in Infants with Congenital Hypothyroidism: Target Attainment Rates Should Be Improved]]> https://www.researchpad.co/article/elastic_article_6498 Levo-Thyroxine (L-T4) is the medication of choice for treating congenital hypothyroidism (CH). Adequate L-T4 treatment is essential for early neurodevelopment in affected patients. Both under- and overtreatment with L-T4 were associated with long-term adverse neurological outcomes. Based on clinical experience, initial L-T4 dosing does not always result in optimal TSH and FT4 concentrations in all CH patients. The purposes of this study were 1) to quantify FT4 and TSH target attainment rates (TAR) in the first six months of L-T4 treatment in infants with CH, 2) to compare characteristics of patients with FT4 concentrations “OUT of” versus “IN” the target range at first time of monitoring.

A multicenter retrospective analysis was conducted in infants born between 1995 and 2018. TSH and FT4 TARs were defined according to the most recent guidelines of the European Society for Paediatric Endocrinology (ESPE), as the percentage of concentrations “in” and “in the upper half” of the corresponding laboratory age-specific reference range for TSH and FT4, respectively.

We analyzed a total of 208 TSH and 186 FT4 serum concentrations from 60 patients during the first 6 months of L-T4 treatment. The pretreatment FT4 and TSH serum concentrations (mean±SD) were 8.3±5.7 pmol/L and 338±248 mU/L, respectively. CH severity according to ESPE guidelines was severe, moderate and mild for 32%, 27% and 32% of the patients. Postnatal age (PNA) (mean±SD) at start of treatment was 10±12 days. Starting dose of L-T4 (mean±SD) for severe, moderate and mild CH were 10±4, 10±3, and 7±4 µg/kg/day, respectively. Over the study period, TSH TARs of 63% did not further improve between the first monitoring (mean at 17 days of treatment) and fourth monitoring (mean at 4 months of treatment), while FT4 TARs increased from 22% to 45% paralleled with a decrease of too high FT4 values from 55% to 21%.

Comparing patients with FT4 concentrations “OUT of” versus “IN” the target range at first time monitoring (16 versus 18 days after starting treatment; p=0.45), they did not differ in pretreatment FT4 concentrations (p=0.2). In contrast, patients who had FT4 concentrations “OUT of” versus “IN” the target range received first dose of L-T4 at an earlier median PNA (7 versus 16 days; p=0.008), had higher pretreatment mean TSH concentrations (364 versus 181 mU/L; p=0.02) and received a higher mean initial L-T4 dose (10.3 versus 7.1 µg/kg/day; p=0.01).

First, our results show that FT4 and TSH target ranges were not reached in all patients in the first six months of treatment. Second, our data suggest that TARs could be improved by individualizing initial L-T4 dosing not only according to pretreatment FT4 but also to pretreatment TSH concentrations. L-T4 dosing optimization is needed in this population.

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<![CDATA[MON-113 The Effect of Body Mass Index on the Peak Growth Hormone Level After Growth Hormone Stimulation Test in Children with Short Stature]]> https://www.researchpad.co/article/elastic_article_6434 Objective: The aim of this study is to evaluate the effect of body mass index (BMI) on peak growth hormone (GH) response after GH stimulation test in children with short stature.

Methods: Data was obtained from retrospective review of medical records who visited the pediatric endocrinology at St. Vincent hospital of catholic university for short stature from January 2010 to June 2019. We studied 115 children (aged 3-17 years old) whose height was less than 3percentile for one’s age and sex and who underwent GH stimulation test {GH deficiency (GHD) = 47, Idiopathic short stature (ISS) = 68)}. Peak GH response was stimulated by dopamine (n=111), clonidine (n=7), glucagon (n=19), insulin (n=56) and arginine (n=32). Birth weight, parental height, chronologic age, bone age, height SDS (standard deviation score), weight SDS, BMI SDS hemoglobin, fT4, T3 TSH, cortisol, ACTH, GH, IGF-1 SDS, IGF-BP3 SDS and peak stimulated GH were analyzed.

Results: In the characteristics of subject, weight SDS and BMI SDS in GHD group were increased than ISS group (p<0.000, p=0.000). Free T4 was decreased in GHD group than ISS group (p=0.012). In total group, BMI SDS was associated negatively with peak GH level stimulated by dopamine (r=-0.419, p<0.000), insulin (r=-0.271, p=0.044) and arginine (r=-0.368, p=0.038), but did not showed correlation with peak GH level stimulated by glucagon. In GHD group, BMI SDS showed negative correlation with peak GH level using dopamine (r=-0.356, p=0.015) and arginine (r=-0.509, p=0.022). In ISS group, BMI SDS was correlated negatively with peak GH using dopamine (r=-0.330, p=0.007). In multivariate regression analysis of GHD group, weight SDS and BMI SDS were the only two significant predictors of peak GH response in stimulation test stimulated by dopamine (ß=-0.576, p=0.015) and arginine (ß=-0.097, p=0.022). In ISS group, only mother’s height (ß=0.474, p=0.000) and TSH (ß=-2.251, p<0.000) were demonstrated statistically significant predictors of peak GH stimulated by dopamine in multivariate regression analysis. In case of using insulin as a stimulant in ISS group, there is nothing which has statistical significance as a predictor of peak GH response in multivariate regression analysis.

Conclusion: BMI was associated negatively with peak GH response after GH stimulation test in children with short stature, especially in GHD group.

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<![CDATA[MON-088 Impact of Vertebral Fracture on Auxological Profile and Insulin-Like Growth Factors of Children After Acute Lymphoblastic Leukemia Treatment]]> https://www.researchpad.co/article/elastic_article_6124 Purpose: To investigate the overall prevalence of vertebral fractures (VF) following childhood acute lymphoblastic leukemia (ALL) treatment and examine the association of VF with growth trajectory and insulin-like growth factors. Methods: Children (n=172; 59.3 % male) diagnosed with ALL at age between 2 and 18 years were assessed for VF by screening the lateral thoracolumbar spine radiographs (Genant’s semi-quantitative method) when treatment was completed (baseline). Anthropometric measurements between pre- to post-treatment period were obtained and the association of VF with insulin-like growth factor-1 (IGF-1) and insulin-like growth factor binding protein-3 (IGFBP-3) were examined. Results: Thirty-five children (20.3 %) had vertebral fractures at baseline. Among children with vertebral fractures, 97.1 % had either mild or moderate deformity, and the 5th lumbar vertebrae was the most frequently injured site (20.0 %). Median lumbar spine bone mineral density Z-score was -1.0 (IQR of -1.6 and -0.8) in children with VF. Baseline Z-scores for height and weight were lower in children with VF than without VF (-0.5±1.3 and 0.0±0.9, P=0.01; -0.2±1.6 and 0.3±1.1, P=0.04, respectively). Height Z-score in children with VF had greater height decline than without VF (0.5±0.6 and 0.2±0.8; P=0.02). Children with VF had lower IGF-1 and IGFBP-3 Z-score than without VF at baseline (-1.2±1.0 and 0.0±0.8, P<0.01; -2.3±1.1 and -1.3±1.0, P<0.01). Decrease in IGF-1 level was associated with the presence of VF (OR=0.3(95 % CI of 0.2-0.5), P<0.01). Conclusion: Substantial number of children encounter VF after ALL treatment is completed and the presence of VF might be associated with compromised auxological state, prominent height decline and IGF-1 deficiency.

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<![CDATA[MON-081 Mathematical Modeling of Residual Endogenous FT4 Synthesis and Exogenous L-Thyroxine Administration over the First 2 Years of Life in Infants with Congenital Hypothyroidism]]> https://www.researchpad.co/article/elastic_article_6102 L-Thyroxine (L-T4) is the treatment of choice of congenital hypothyroidism (CH). Longitudinal measurements of free T4 (FT4) serum concentrations were collected over the first two years of life with oral L-T4 treatment in infants with CH. Purpose of this study was to develop an integrated mathematical model to characterize the kinetics of exogenous L-Thyroxine (L-T4) after multiple dosing in infants with CH, and the dynamics of residual endogenous FT4 synthesis under treatment in the context of severe, moderate and mild disease.

A total of 200 FT4 concentrations from 30 patients were available for analysis. At start of treatment, mean (standard deviation [SD]) postnatal age and weight of the population were 11 (8) days and 3.9 (1.3) kg. Mean (SD) pretreatment FT4 concentration was 11.3 (7.4) pmol/L. Measured FT4 concentrations were modelled as sum of residual endogenous FT4 and exogenously administered FT4 (L-T4). The integrated mathematical model consists of an absorption compartment for the exogenous FT4 administration, and a central compartment for measured FT4 with linear elimination. Hence, for residual endogenous and exogenous FT4 the same elimination rate constant was assumed. For the residual endogenous synthesis, different approaches were tested: a constant production and typical time-dependent production functions. FT4 data were analyzed using nonlinear mixed-effects modeling.

The integrated mathematical model with a time-dependent non-linear Emax function describing a decreasing residual endogenous FT4 synthesis for increasing time provided the best data fit in terms of Akaike value and various goodness-of-fit plots. This is in line with the expected progressive suppression of the thyroid stimulating hormone by the exogenous FT4, and the subsequent decrease of residual FT4 endogenous synthesis. The developed mathematical model allows simulation of FT4 pharmacokinetic profiles for different disease severities and different dosing regimens.

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<![CDATA[MON-099 The Association Between C-Reactive Protein, Metabolic Syndrome, and Pre-Diabetes in Korean Children and Adolescents]]> https://www.researchpad.co/article/elastic_article_6099 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 < 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.

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<![CDATA[MON-LB017 The Effectiveness of Computed Assessment Using GP and TW3 Hybrid System]]> https://www.researchpad.co/article/N3b23d3fe-3289-42d9-a97f-9f4141662b92 <![CDATA[MON-106 Prevalence and Incidence of Obesity in Children and Young Adults in Korea: The Kangwha Study]]> https://www.researchpad.co/article/N77b2026d-6615-45d3-b4f5-5e340749fa0d <![CDATA[MON-111 Plasma Insulin Measured with a Sensitive Immunoassay May Establish the Diagnosis of Congenital Hyperinsulinism Without Further Testing]]> https://www.researchpad.co/article/N5f411900-3e8c-4ba4-868e-56869eec6b3e 1.5 mmol/L during hypoglycaemia) were used as controls.P-insulin was measured by the high-sensitive assay (Cobas e411 immunoassay analyzer); lower detection limit 1.4 pmol/L (0.2 mU/L); normal range 18-173 pmol/L (2.57-24.7 mU/L). Concentrations <18 pmol/L were considered suppressed; ≥18 pmol/L un-suppressed.Receiver operating characteristics (ROC) curves with determination of area under the curve (AUC) values were performed for the diagnostic performance of p-insulin in the diagnosis of CHI. Results: In the 61 samples from CHI patients, the median (range) p-insulin was un-suppressed in all diagnostic samples [90; 20-758 pmol/L (12.9; 2.9-109.1 mU/L)], while p-insulin was suppressed in all 15 samples from IKH patients [1.5; 1.5-9 pmol/L (0.21; 0.21-1.3 mU/L)]. The ROC AUC was 1.0 (95%CI. 1.0-1.0) for the diagnosis of CHI defined both by the clinic and by gold standard. The optimal p-insulin cut-off was 14.5 pmol/L (2.1 mU/L) or 12.5 pmol/L (1.8 mU/L), for CHI patients by use of a simultaneous p-glucose cut-off of <3.2 mmol/L (57.5 mg/dL; n=61), or 3.0 mmol/L (55 mg/dL; n=49), respectively. Conclusions: The sensitive insulin assay performed excellent in diagnosing CHI with a ROC AUC of 1.0. The use of a p-insulin cut-off of 13 pmol/L (1.86 mU/L) during a diagnostic hypoketotic hypoglycaemia test may establish the diagnosis of CHI without further diagnostic testing. ]]> <![CDATA[MON-084 Hyperosmolar Hyperglycemic State as Initial Presentation of Type 1 Diabetes in Children]]> https://www.researchpad.co/article/N553493c7-7440-4d38-b355-557e601106b6 <![CDATA[MON-LB016 Tyrosine Kinase Inhibitor Induced Hypothyroidism in Pediatric and Young Adult Population: An Institutional Review]]> https://www.researchpad.co/article/Nefcf8cf3-8570-411b-8c62-3870d6752e3b 5mcIU/mL during TKI therapy. Results:A total of 152 patients who were treated with TKIs for malignancy were identified. The mean age was 12.4 years (SD 6.5). About 20% of patients had therapy with multiple TKI drugs. A total of 24 patients were noted to have TSH elevation >5mcIU/ml of which 19 had a TSH >10 mcIU/mL or low free T4. Fourteen patients were started on levothyroxine. Average duration of TKI therapy prior to development of thyroid dysfunction was 6.7 months but over half developed hypothyroidism within 3 months of initiation of TKI therapy. Cabozantinib and pazopanib were responsible for 70% of TKI associated cases of thyroid dysfunction.Conclusion:This is the first report of incidence of primary hypothyroidism in pediatric and young adult patients treated with TKIs. Thyroid dysfunction can develop in the first few months of therapy and often is clinically significant. Early recognition and treatment of this complication will be important for patient care especially as use of these class of drugs increase. ]]> <![CDATA[MON-082 The Neonatal Screen That Cried Wolff]]> https://www.researchpad.co/article/N54d56f36-195e-48c7-94b8-05c38dec89cc 8ug/dL). No family history of thyroid disease; mother was healthy during pregnancy and was not on medications that could affect baby’s thyroid function. Subsequent serum laboratory testing confirmed a TSH of 74.3mIu/mL and Free T4 of 0.6ng/dL. Patient was diagnosed with Wolff-Chaikoff effect, which is the phenomenon of transient hypothyroidism caused by exposure to high doses of iodine (iodine containing contrast agents or topical antiseptics). Pediatric Endocrinology was consulted at 2 weeks of life and she was started on 25mcg of levothyroxine PO daily. Levothyroxine dose decreased at 16 month of age to 12.5mcg due to stable thyroid function tests. The patient was last seen at 30 months of age by Pediatric Endocrinology. She is still on the low dose of levothyroxine and her thyroid labs have been within normal limits for an infant. She will likely not require lifelong thyroid supplementation. Conclusion: Risk of hypothyroidism among neonates must be considered seriously after large iodine exposure and monitoring for transient hypothyroidism should be performed. It is thus recommended that attempts should be made to reduce the amount of iodine used during procedures and to carefully monitor thyroid function in all neonates exposed to an excess of iodine.Reference:Markou, K., et al. “Iodine-Induced Hypothyroidism.” Thyroid, vol. 11, no. 5, 2001, pp. 501–510., doi:10.1089/105072501300176462.Linder N, Sela B, German B, et al. Iodine and hypothyroidism in neonates with congenital heart disease. Archives of Disease in Childhood - Fetal and Neonatal Edition 1997;77:F239-F240.Kovacikova, Lubica, et al. “Thyroid Function and Ioduria in Infants after Cardiac Surgery: Comparison of Patients with Primary and Delayed Sternal Closure.” Pediatric Critical Care Medicine, vol. 6, no. 2, 1 Mar. 2005, pp. 154–159., doi:10.1097/01.pcc.0000154960.59452.06. ]]> <![CDATA[MON-109 Modulator of Gut Barrier, Zonulin Was Associated with Waist to Height Ratio in Adolescents]]> https://www.researchpad.co/article/N432b5335-16e3-4cad-a12c-3b97a13a635c <![CDATA[MON-087 Natural History and Neurodevelopmental Outcomes in Perinatal Stress Induced Hyperinsulinism]]> https://www.researchpad.co/article/Nb97eb52c-185e-41b0-81cf-811e29f1df0a <![CDATA[MON-110 Utilization of GluCEST, a Novel Neuroimaging Technique, to Characterize the Brain Phenotype in Hyperinsulinism/Hyperammonemia Syndrome]]> https://www.researchpad.co/article/N5cf8082b-eae4-4149-b155-c2cc32bed4e2 60% have atypical absence seizures (Bahi-Buisson, 2008). These neurologic symptoms are not fully explained by hypoglycemia and are hypothesized to result from central nervous system (CNS) glutamate imbalance due to CNS GDH overactivity. Newer magnetic resonance imaging (MRI) techniques have allowed for sensitive estimation of CNS glutamate using Glutamate Chemical Exchange Saturation Transfer (GluCEST). We aimed to comprehensively characterize the biochemical and clinical neurologic phenotype of HI/HA leveraging GluCEST MRI.Methods: Subjects with confirmed HI/HA diagnosis and without contraindication to MRI had electroencephalogram (EEG), serum ammonia, and the following validated neurodevelopmental assessments: ABAS-3, BRIEF, and ASEBA CBCL (if <18 years) or ASR (if >18 years) completed. GluCEST MRI axial hippocampal and midsagittal slices were acquired on a 7.0T Siemens scanner and reported as GluCEST % contrast. Healthy control GluCEST % contrast data were obtained from a separate study using the same neuroimaging protocol.Results: 8 HI/HA subjects (4 female; mean age 28 years [range 16-56] years) participated to date. Median serum ammonia was 58 umol/L (IQR 39-89). 50% self-reported learning impairments and 37.5% self-reported prior ADHD diagnosis. Marked unilateral increase in hippocampal GluCEST % contrast was observed in 3/6 subjects (2 L>R; 1 R>L). Overall, median peak GluCEST % contrast level was significantly higher in HI/HA subjects than controls (10.3% [IQR 8.9-11.3] v. 8.0% [IQR 7.8-8.4], p=0.0013, n=6).Conclusions: This is the first study to evaluate CNS glutamate via GluCEST in HI/HA. Hippocampal glutamate, measured by GluCEST % contrast, was significantly higher in HI/HA subjects than healthy controls. Laterality in hippocampal glutamate was observed in half of subjects. These findings are remarkable given the known role of abnormal glutamate signaling in the development of epilepsy and neurocognitive impairment. Next steps are to complete midsagittal GluCEST image processing, EEG and neurodevelopmental assessment interpretations to explore correlations between CNS phenotype and brain glutamate pattern. GluCEST holds promise for elucidating the pathophysiology of CNS manifestations in HI/HA syndrome. ]]> <![CDATA[MON-093 Pleuropulmonary Blastoma and Multinodular Goiter in a 22 Yr Old Male with DICER1 Syndrome]]> https://www.researchpad.co/article/Nf88358fd-3d60-4325-a8d9-a165299cf764 1 cm. Biopsy of the right nodule was negative for malignancy. Over the course of 2 years he developed new right thyroid isoechoic nodule in the lower pole 2.1 x 2.5 x 1.9 cm and new left thyroid isoechoic nodule in the upper pole 1.0 x 0.5 x 0.5 cm. Biopsy was negative for malignancy.Due to his PPB, MNG and family history of lung cancer he was evaluated at our genetic cancer clinic and tested positive for germline DICER1 pathogenic variant c.4605_4606del (p.Cys1535Trpfs*3)Conclusion:Our 22 year old male presented with pleuropulmonary blastoma and over the course of few years developed MNG. Genetic testing was positive for germline DICER1 pathogenic variant c.4605_4606del (p.Cys1535Trpfs*3). Our case illustrates the importance of consideration of: 1) Testing children with PPB for DICER1 Syndrome as there are screening recommendations including regular thyroid ultrasound and examinations to look for MNG or other features concerning for thyroid gland neoplasia. 2) MNG is uncommon in children and detection of this should raise suspicion for consideration of testing for DICER1 Syndrome. ]]> <![CDATA[MON-100 Prevalence of Goitre and Thyroid Disorders in Healthy School Children of Kashmir Valley. A Multistage Cross-Sectional Survey]]> https://www.researchpad.co/article/Nbc842b21-04d4-40cb-835d-30937ca3b5f7