bluuebunny/crossref_metadata_2025_split huggingface dataset:
MON-660 Hypoglycemia Following OGTT Is More Frequent and Pronounced in CF Compared with Controls
Authors: Harris Clea D , Kilberg Marissa J , Sheikh Saba , Stefanovski Darko , Cuchel Marina , Kubrak Christina , Hadjiliadis Denis , Rubenstein Ronald C , Rickels Michael Roehrhoff , Kelly Andrea 
Published: May 2020

Abstract
Glucose homeostasis is often abnormal in people with pancreatic insufficient cystic fibrosis (PI-CF). This dysfunction is viewed on a continuum from “normal” glucose tolerance to cystic fibrosis-related diabetes (CFRD), and may also include postprandial and oral glucose tolerance test (OGTT)-related hypoglycemia. This study aimed to delineate the mechanism(s) underlying OGTT-related hypoglycemia. We compared extended OGTT with frequent blood sampling of glucose and insulin in adolescents and young adults with PI-CF [CF(+)] to historical data from a healthy cohort [CF(-)]. We hypothesized that the subset of CF(+) with hypoglycemia would demonstrate 1-hour glucose ≥ 155 mg/dL and impaired early phase insulin secretion (insulin secretion within first 30 min of OGTT). Hypoglycemia [hypo(+)] was defined as plasma glucose &lt;65 mg/dL and was used to assign subjects to exposure groups. We restricted analyses to 180 minutes given available control data. Glucose and insulin incremental areas under the curve (Glc-AUC; Ins-AUC) for 30-minute intervals were calculated. One-hour glucose, nadir glucose, Glc-AUC0-30, and Ins-AUC0-30 and were compared between CF(+) and CF(-) subjects using Student’s t-test or Wilcoxon Rank Sum depending upon normality. Participants were 60.5% male, age: 25.4±4.8 years, with BMI-Z: 0.06±0.96kg/m2 [no differences for CF(+) vs CF(-)]. FEV1%-predicted for CF(+) was 83±21. 69.6% of CF(+) participants self-reported prior episodes of hypoglycemia, 68.7% of whom reported confirmation via glucometer. Hypoglycemia occurred by 180 minutes [hypo(+)] in 15/23 (65%) CF(+) and 5/15 (33.3%) CF(-) subjects (p=0.028). For hypo(+), nadir glucose occurred on average at 180 minutes for both CF(+) and (-). Hypo(+) CF(+) had higher mean 1-hour glucose (197±49mg/dL vs 134±66mg/dL, p=0.035), lower mean glucose nadir (48±7mg/dL vs 61±4mg/dL, p&lt;0.01), and lower early-phase insulin secretion (Ins-AUC0-30: 263±168 versus 650±275 µU/mL, p&lt;0.01) than hypo(+) CF(-). There was no difference in Glc-AUC0-30 for hypo(+) CF(+) vs CF(-). Hypoglycemia is frequent in CF, and is associated with early glucose dysregulation (elevated 1-hour glucose) and compromised early-phase insulin secretion compared to controls with presumed non-pathologic reactive hypoglycemia. The mechanism of hypoglycemia in CF appears to be different than that seen in healthy individuals, and its association with progression to CFRD warrants further evaluation.
SAT-430 Quality of Life After Thyroidectomy for Patients with Hashimoto’s Disease and Persistent Symptoms
Authors: Thatipamala Priyanka , Noel Julia , Orloff Lisa 
Published: May 2020

Abstract
Objective: To determine whether thyroidectomy improves quality of life in patients with Hashimoto’s thyroiditis with persistent symptoms despite biochemical euthyroidism.
Study Design: A retrospective cohort study of patients undergoing thyroidectomy for Hashimoto’s thyroiditis.
Setting: Tertiary referral center.
Subjects and Methods: Included patients underwent thyroidectomy for Hashimoto’s thyroiditis at our institution between 2014 and 2018. The following variables were collected: age, race, body mass index (BMI), pre-operative symptoms, pre-operative thyroid peroxidase antibody (TPO Ab), thyroglobulin antibody (Tg Ab) thyroid stimulating hormone (TSH), free T4, specimen weight, and presence of thyroiditis or malignancy on the pathology report. The primary outcome was general health score on the Short Form-36 Health Survey (SF-36) ranging from 3–35 months postoperatively. Secondary outcomes included an additional questionnaire specifically addressing disease management after surgery as well as any adverse outcomes.
Results: 19 patients were included in the study. 18 of the 19 patients were female with a mean age of 48 years (SD 13.6 years). The majority of patients were Caucasian. There was no significant difference between the general health score of the Hashimoto’s thyroiditis patients post-operatively compared to a healthy control population. (66.9 vs. 74.1; 95% CI [-16.9 + 2.5], p = 0.16). There were also no significant differences between groups within the 7 SF-36 subscores. Elevation in pre-operative TPO Ab correlated with lower reported post-operative energy levels (r = -0.63, p = 0.016) and emotional well-being (r = -.55, p = 0.041). 87.5% (14/16) of respondents reported to be moderately or extremely happy with their decision to proceed with surgery.
Conclusions: Quality of life in patients with Hashimoto’s thyroiditis who undergo thyroidectomy is equivalent to the general population, and the majority of patients are satisfied with the decision to have undergone surgery. Thyroidectomy is a consideration for patients with Hashimoto’s disease and persistent symptoms despite optimization on medical therapy.
SAT-353 Familial Hyperparathyroidism - Due to a Rare Genetic Mutation
Authors: Taiwo Adeyinka , Dillon Joseph Stephen , Stinson Jennifer 
Published: May 2020

Abstract
Introduction: Hyperparathyroidism occurs most commonly in middle age patients, predominantly in women. It can be caused by parathyroid adenoma, hyperplasia or parathyroid carcinoma. Genetic predisposition can be found in about 10% of primary hyperparathyroidism due to certain gene mutations. This case emphasizes the importance of taking a detailed family history when patients present with hyperparathyroidism at a young age, so that familial hyperparathyroidism, if present, can be detected and relatives screened.
Clinical case: A 26 y.o. male presented with symptoms of fatigue and polydipsia for several years.
He was noted to have a serum calcium of 12.4 mg/dL(8.5–10.5), with parathyroid hormone of
213 pg/ml (15–65). He denied any history of kidney stones, fractures and no palpable neck masses. The patient’s family history was significant for his paternal half-sister who had parathyroidectomy for hyperparathyroidism at 20yrs old and paternal grandmother died of parathyroid cancer in her 50s. The patient’s father died of pancreatic cancer at 41yrs old. A neck ultrasound revealed a mass posterior to the left inferior thyroid. A Sestamibi parathyroid scan revealed a parathyroid adenoma at the posteroinferior aspect of the left hemithyroid. Labs for free metanephrines and normetanephrine, prolactin and gastrin levels were all normal.
Due to his young age and the possibility of having familial hyperparathyroidism, he underwent bilateral neck exploration and parathyroidectomy, with removal of his left inferior, right superior, left superior parathyroid glands and left upper thymus. Surgical pathology revealed, hypercellular parathyroid tissue. Post operatively, his calcium and vitamin D remained within the normal range.
Genetic studies revealed a mutation in the parafibromin gene - CDC73 (also called HRPT2), a tumor suppressor gene, which is on chromosome 1q25. The patient currently has 6 children ranging from age 5 months to 6 years. He was advised to have his children tested any time from age 7 years for the gene mutation.
The patient has remained stable 4yrs post operatively, with normal calcium and PTH levels. He does not have any history of jaw tumor. He never had an ultrasound kidney done. He is being monitored with yearly lab tests.
Conclusion: CDC73 gene mutation-associated disorders are inherited as an autosomal dominant fashion, with variable penetrance. This gene mutation can be found in conditions such as hyperparathyroidism jaw tumor, familial hyperparathyroidism and parathyroid cancer.
Reference:
1.
CDC73-Related Disorders: Clinical Manifestations and Case Detection in Primary Hyperparathyroidism. J Clin Endocrinol Metab. 2017 Dec 1;102(12):4534–4540.
SAT-461 NIFTP: A Painstaking Diagnosis Through the Pathologist’s Eyes
Authors: Chittimoju Sanjita B , Drake Frederick T , Lee Stephanie L 
Published: May 2020

Abstract
Non-invasive encapsulated follicular variant of papillary thyroid cancer (EFVPTC) was recently reclassified as non-invasive follicular thyroid neoplasm with papillary-like nuclear features (NIFT-P).1 In 2018, revised and stricter criteria were proposed for a lesion to qualify as NIFT-P including no well-formed papilla or BRAF-V600E mutation.2 We are presenting an interesting case to highlight the importance of scrutinizing pathology slides to diagnose NIFTP with these more strict criteria.
35-year-old female from Puerto-Rico was diagnosed with Graves’ disease. After 2 years of methimazole treatment, total thyroidectomy was planned for definitive treatment of Graves’ disease. During the work up, she was noted to have a cystic nodule in isthmus, a 1.1 cm hypoechoic nodule in left mid-lobe and a 1.1 cm isoechoic rounded mass in left level III neck, which was initially thought to be a lateral aberrant thyroid remnant. Her thyroid uptake scan was consistent with a multinodular goiter with no uptake in the extrathyroidal mass. The mass was biopsied and showed Atypia of Undetermined Significance (AUS) Bethesda III with washout positive for thyroglobulin (Tg). Total thyroidectomy with bilateral central and left lateral neck dissection was performed. The pathology showed an intrathyroidal 1.2 cm EFVPTC with predominant follicular features and &lt;1% papillae, without tumor capsular invasion. The initial diagnosis was NIFT-P with a background of chronic thyroiditis. However, on pathology, the level III neck mass was a 2 cm metastatic node with classical PTC. ThyroSeq mutational analysis of tissue blocks for both the thyroid nodule and lymph node were positive for NCOA4-RET (RET-PTC3) gene fusion, a BRAF-V600E-like mutation found in classical PTC. On review of her pathology, the thyroid lesion was noted to have more than one papilla, though &lt;1% papillae and was &gt;30% solid, hence not qualifying as NIFT-P and her histological diagnosis was changed to EFVPTC. She was staged as AJCC 8th edition stage 1 with intermediate ATA risk for which she received adjuvant therapy of 101 mCi 131I.
Although classification into NIFT-P has been shown to reduce overtreatment of low risk encapsulated PTC, pathology slides should be closely scrutinized to ensure fulfillment of all criteria in order for a lesion to qualify as NIFT-P. This will minimize failure to recognize PTCs, that would warrant closer follow up and surveillance for recurrence.
1. Rossi, Esther D, et al. Noninvasive Follicular Thyroid Neoplasm with Papillary-Like Nuclear Features: Update and Diagnostic Considerations—a Review. Endocrine Pathology 30.2 (2019)
2. Nikiforov, Yuri E et al. Change in Diagnostic Criteria for Noninvasive Follicular Thyroid Neoplasm With Papillary-like Nuclear Features. JAMA oncology vol. 4,8 (2018)
MON-067 A Perplexing Case of Hyponatremia and Abdominal Pain
Authors: Yeliosof Olga , Balachandar Sadana 
Published: May 2020

Abstract
Previously healthy 20-year-old female presented with diffuse lower abdominal pain, cramping in nature, multiple episodes of emesis as well as urinary frequency. On day 2 of symptoms, she was treated for a urinary tract infection with antibiotics, as well as NSAIDs and opiates for pain relief. Her serum sodium was 133 mmol/L at this time. On day 3 of symptoms, a CT scan of the abdomen was performed however did not reveal pathology. Her serum sodium was 129 mmol/L at this time. She presented to our ED, on day 5 of symptoms, where serum sodium was down to 122 mmol/L. Despite IV fluids, her sodium continued to decrease to a nadir of 117 mmol/L. Further testing, including a serum osmolality of 242 mOsm/kg, urine osmolality of 540 mOsm/kg, and urine sodium of 207 mmol/L, was consistent with a diagnosis of SIADH. Given persistence of abdominal symptoms along with SIADH further imaging studies, including US abdomen, CT brain and Chest XR, were ordered and returned unremarkable. The constellation of SIADH along with persistent abdominal pain, with negative imaging, lead to consideration of acute intermittent porphyria as a diagnosis. Random urine porphobilinogen was found to be elevated to 147.2 mcmol/L (≤ 2.4) leading to the presumptive diagnosis of acute intermittent porphyria presenting as a neurovisceral attack. Biochemical and genetic testing is being pursued to confirm her diagnosis.
Acute intermittent porphyria is an autosomal dominant hematologic disorder characterized by deficiency in porphobilinogen deaminase, an enzyme in the heme synthesis cascade. Acute attacks are caused by accumulation of porphyrin resulting in autonomic and peripheral neuropathy which can present as abdominal pain, urinary retention, polyneuropathy, dark urine and psychiatric disturbance. Hyponatremia is present in 25–60% of cases which is caused by SIADH or sometimes renal and gastrointestinal sodium loss. Triggers for acute attacks include medications, starvation, infections, hormonal changes and alcohol. Treatment includes avoidance of triggers, IV dextrose and high carbohydrate diet. In severe attacks, IV hemin is used.
Our patient’s urinary tract infection likely triggered her acute symptoms, which was further exacerbated by treatment with NSAIDS and opiates. She developed SIADH which improved with hypertonic saline and fluid restriction.
This case illustrates the need to consider acute intermittent porphyria in the differential diagnosis of SIADH presenting with abdominal pain of unknown etiology.
OR29-07 A Novel Estimate of Creatinine Excretion to Determine Adequacy of 24-Hr Urine Collection
Authors: Kondapalli Ananya V , Li Xilong , Huet Beverly Adams , Maalouf Naim 
Published: May 2020

Abstract
24-hour urine collections are used to assess excretion of various analytes. Although concomitant measurement of creatinine excretion adjusted for body weight (BW) is utilized to determine adequacy of collection, no gold standard exists for determining completeness of a collection. Use of current reference ranges for daily creatinine excretion/BW of 15–20 mg creatinine/kg BW/day in women and 20–25 mg creatinine/kg/d in men established prior to the rising prevalence of obesity has resulted in a large proportion of contemporary individuals appearing to have “incomplete” urine collections.
Our objective was to evaluate the range of creatinine excretion in accurately collected urine specimen from adults with a wide spread of age and BW, and to generate an equation that accurately predicts 24-hour urine creatinine excretion rate from readily available clinical parameters to aid in assessing adequacy of a 24-hour urine collection.
We analyzed data from participants who completed two consecutive 24-hour urine collections while consuming a fixed metabolic diet during inpatient research admissions. Results from participants with 24-hour urine creatinine excretion rate differing by &gt;10% between the two consecutive collections were excluded. In the initial 115 pairs of inpatient 24-hour urine collections (50 female, 65 male) participants, creatinine excretion/BW fell outside the currently accepted reference ranges in &gt;50% of collections. The proportion below the reference range increased with higher BMI. In this derivation dataset, linear regression models were then constructed to predict 24-hr urine creatinine excretion from race, sex, age, weight and height. Reliable prediction of observed 24-hr urine creatinine excretion was confirmed in a validation dataset that included 50 pairs of 24-hour urine samples similarly collected in an inpatient research setting.
This new prediction model performed significantly better than the currently used reference ranges in a large outpatient dataset including 1,399 pairs of 24-hour urine collections. In women, actual creatinine excretion fell within the 95% prediction intervals for our derived equation in 90% of cases using the new interval vs 46% using the current reference range. The corresponding values were 90% and 33% in men. In both genders, the superiority of the new prediction over the current reference range was more pronounced at higher BMI.
We therefore propose revision of currently used criteria to define adequacy of 24-hour urine collection to account for the impact of obesity. The proposed equation incorporates readily available demographic parameters to predict urine creatinine excretion. These findings have wide implications on patient care and research studies, and future studies should test this equation in different settings, diets, and populations.
SUN-369 Different Association of Dietary Fat Intake with Femoral Neck Strength According to Gender: A Nationwide Population-Based Study
Authors: Kim Beom-Jun , Kim Hyeonmok 
Published: May 2020

Abstract
Purpose: Despite the general belief that higher fat intake may be harmful for bone health, its impact on bone strength has not been thoroughly studied. Methods: We conducted a population-based cross-sectional study derived from the Korea National Health and Nutrition Examination Surveys, including 2,590 participants. Composite indices of femoral neck strength, such as the compression strength index (CSI), bending strength index (BSI), and impact strength index (ISI), were generated by combining bone mineral density, body mass, and height with the femoral axis length and width. Results: Dietary fat intake (%) was inversely related to CSI and ISI in men, but not in women. Men in the highest three fat intake quintiles had lower CSI, BSI, and/or ISI than those in the lowest quintile (P=0.003–0.024). In women, compared with participants in the third fat intake quintile, those in the other four quintiles had lower CSI, BSI, and/or ISI (P=0.004–0.049). When the participants were allocated to three groups according to the dietary reference intake of fat in Koreans [low (&lt;15%), moderate (15–30%), or high (≥30%)], men with a moderate or high fat intake had significantly lower ISIs than those with a low fat intake (P=0.046 and 0.039, respectively). By contrast, compared with women consuming a moderate amount of fat, those with a high intake had lower CSI, BSI, and ISI (P = 0.025–0.047). Conclusion: These findings suggest that higher fat intake in men and low or high fat intake in women may contribute to deteriorations in bone strength.
SAT-603 Growth Hormone-Releasing Hormone (GHRH) Antagonists Stimulate Feeding in Mice
Authors: Leone Sheila , Recinella, PharmD Lucia , Chiavaroli, PharmD Annalisa , Orlando, PharmD Giustino , Ferrante, PharmD Claudio , Gesmundo Iacopo , Granata Riccarda , Cai Renzhi , Sha Wei , Schally Andrew V , Brunetti Luigi 
Published: May 2020

Abstract
Growth hormone-releasing hormone (GHRH) is a hypothalamic neuropeptide which stimulates the synthesis and secretion of growth hormone (GH) in pituitary gland. GHRH was also found to modulate food intake in mammals. MIA-690 is a synthetic GHRH antagonist of the Miami (MIA) series with potent antitumor effects. To date, its role in hypothalamic feeding modulation has not been evaluated. In the present study, we aimed to investigate the effects of chronic MIA-690 administration on feeding behavior, locomotor activity and hypothalamic dopamine (DA), norepinephrine (NE), serotonin (5-hydroxytriptamine, 5-HT), orexigenic peptides [agouti-related peptide (AgRP) and neuropeptide Y (NPY)] and anorexigenic peptides [cocaine and amphetamine-regulated transcript (CART) and proopiomelanocortin (POMC)] activity.
Adult C57/BL6 mice were treated daily for 4 weeks by subcutaneous administration of (5 µg) MIA-690 or vehicle solution. Food intake and body weight were recorded every 4 days throughout the study. Immediately after the last injection, locomotor activity in the home cage was recorded, and thereafter animals were sacrificed. Visceral, subcutaneous and brown fat depots were quickly excised and weighed. Hypothalamus was also dissected for evaluating gene expression of AgRP, NPY, CART and POMC by real-time reverse transcription polymerase chain reaction. In addition, hypothalamic DA, NE and 5-HT levels were measured by high performance liquid chromatography (HPLC) coupled to electrochemical detection.
Our findings show that administration of MIA-690 increased food intake and body weight, without affecting locomotor activity. No difference was observed in visceral, subcutaneous and brown fat mass in animals treated with MIA-690 or vehicle. As for neuromodulatory effects, a significant increase of AgRP gene expression and NE levels, along with a reduction of 5-HT levels were found after MIA-690 treatment. On the other hand, we did not observe any alteration in NPY, POMC and CART gene expression, as well as DA levels, following MIA-690 administration.
In conclusion, chronic peripheral administration of MIA-690 could play an orexigenic role paralleled by increased body weight. The stimulation of feeding could be mediated, at least in part, by increased AgRP gene expression and NE levels and decreased 5-HT levels, in the hypothalamus.
SAT-092 Positive Effect of an Early Start of Growth Hormone Therapy on Auxology and Metabolic Parameters in Children with Prader Willi Syndrome
Authors: Gohlke Bettina , Magill Lucy , Laemmer Constante , Woelfle Joachim 
Published: May 2020

Abstract
Introduction: Prader-Willi-Syndrom (PWS) is a rare multisystem genetic disorder characterized by hypothalamic-pituitary dysfunction. Various characteristic clinical features and several endocrinological problems such as hypogonadism, hypothyroidism, Growth hormone (GH) and adrenal deficiency have been described. Since GH-Therapy (GHT) was approved the physical benefits of the treatment have been established in many studies. It is now recommended to start treatment as soon as possible. The aim of this study is to shed light on possible differences in height, carbohydrate and lipid metabolism between children with PWS in whom GHT was initiated either during or after their first year of life. Patients and Methods: This retrospective longitudinal study included 62 children (31 males) with genetically confirmed PWS in whom fasting morning blood samples and auxological parameters were obtained before start of therapy and semi-annually thereafter. The early treatment cohort A consisted of 21 (11 males) infants who were recruited at 0.3–0.99 yrs (mean 0.72 yrs) for GHT. The later treatment cohort B entailed 41 individuals (20 males) in whom GHT was initiated at 1.02–2.54 yrs (mean 1.42 yr). Results: Auxology: Mean length/height-SDSPWS differed significantly throughout the entire observation period between the groups: 1 yr: A: 0.37 (±0.83) vs B: 0.05 (±0.56); 5 yrs: A: 0.81 (±0.67) vs. B: 0.54 (±0.64); p=0.012). No significant differences were found in BMI, lean body mass or percent body fat. Endocrinological Parameters: Mean IGF-I SDS in group A did not differ significantly from group B and mean IGF-I SDS were mostly below 0 SDS (within normal range) in both groups.Lipid Metabolism: Low-density lipoprotein (LDL) was statistically significantly lower in Group A than in Group B during the entire course of the study (LDL: 1 yr: A: 79 (±20) mg/dl vs. B: 90 (±19) mg/dl; 5 yrs: A: 91(±18) mg/dl vs. 104 (±26) mg/dl; p=0.024).Carbohydrate Metabolism: Significant differences in mean fasting insulin levels and HOMA-IR between the two groups were found (fasting insulin p=0.012; HOMA-IR p=0.006). Significant differences in HbA1c and blood glucose levels were also determined between the two groups (HbA1c: p&lt;0.001; blood glucose: p=0.022). Conclusion:Our analysis shows that early GHT had a statistically significant favorable effect on height-SDS, LDL, HOMA-IR and fasting insulin. The two groups did not significantly differ in BMI-SDS, body composition or IGF-I SDS.