bluuebunny/crossref_metadata_2025_split huggingface dataset:
SUN-286 Lymphocytic Hypophysitis Mimicking Tolosa Hunt Syndrome
Authors: Del Castillo Ma Dolores , Arkun Knarik , Safain Mina , Lechan Ronald M 
Published: May 2020

Abstract
Introduction:
Lymphocytic hypophysitis often presents with headache, hypopituitarism and visual disturbance, the latter from optic nerve compression. Rarely, it can present with diplopia from cranial nerves III, IV and VI (3.7%) and cavernous sinus involvement (1).
Clinical Case:
A 40 year old woman presented with left eye pain, blurry vision, ptosis and diplopia for 2 days, preceded by headache for 2 weeks. Exam was remarkable for left eye ptosis, mild proptosis, downward and outward gaze and inability to adduct her left eye. Endocrinological exam revealed free T4 0.67 ng/dL (Nl 0.70 - 1.48), TSH 0.67 ng/dL (Nl 0.70–1.48), estradiol &lt;10 pg/mL, LH 1.0 mIU/mL, FSH 6.9 mIU/mL, prolactin 23.3 ng/ml (Nl 5.2–26.5) and IGF-1 95 ng/mL (Nl 52–328). Cortisol was not assessed as patient was already on steroids. Work-up revealed atypical ANCA (1:320) but normal C-ANA (&lt;1:20), P-ANCA (&lt;1:20), and the rest of immune work-up was negative including ACE, ESR, CRP, ANA, serine protease and myeloperoxidase. No systemic manifestations were present concerning for systemic autoimmune disease. CSF exam was unrevealing including a normal ACE level. MRI revealed an enlarged pituitary gland with suprasellar extension containing a focal area of T2 hyperintensity and slight T2 hypointensity at the posterior aspect of the gland. There was a midline, thickened infundibulum, enhancement of both cavernous sinuses and narrowing of right internal carotid artery without occlusion. Endoscopic endonasal transsphenoidal biopsy of pituitary lesion confirmed diagnosis of lymphocytic hypophysitis and did not meet criteria for IgG4 hypophysitis. After 4 weeks of prednisone, she had significant symptomatic improvement and repeat MRI showed decreased pituitary size but persistent abnormal enhancement of the pituitary gland and cavernous sinuses.
Conclusion:
The atypical and variable clinical and radiological findings of lymphocytic hypophysitis can mimic other inflammatory, infiltrative lesions, pituitary tumor with apoplexy and Tolosa Hunt Syndrome. Tolosa Hunt syndrome is an idiopathic granulomatous inflammation of the cavernous sinus involving cranial nerves II to VI and often presenting with painful ophthalmoplegia. Pituitary involvement and carotid artery narrowing have been observed (2). Our case highlights a patient with cranial nerve III palsy and significant cavernous sinus involvement, clinically concerning for Tolosa Hunt syndrome, but confirmed by biopsy to be lymphocytic hypophysitis. There are no specific serum markers to distinguish lymphocytic hypophysitis from other entities and when uncertain, diagnosis is best established by biopsy.
References:
1 Caturegli P, et al. Autoimmune hypophysitis. Endocr Rev 2005, 26: 599–614.
2 A. Kambe et al. A case of Tolosa-Hunt syndrome affecting both cavernous sinuses and hypophysis and associated C3 and C4 aneurysms. Surgical Neurology 65 (2006) 304–307.
SAT-361 Familial Hypocalciuric Hypercalcemia Type 3: AP2S1 Mutation
Authors: Kerut Sarah Elizabeth , Cardozo Licy L Yanes 
Published: May 2020

Abstract
Background: Familial hypocalciuric hypercalcemia (FHH) type 3 can appear similar to primary hyperparathyroidism and make the diagnosis of etiology of hypercalcemia challenging.
Clinical Case: A 45-year-old man with hypertension and glaucoma was evaluated in clinic for hypercalcemia. His calcium was 12.3 mg/dL (8.4–10.2), PTH 41.9 pg/mL (15–65), Vitamin D 14.8 ng/mL (6.6–49) and phosphorus 1.7 mg/dL (2.7–4.5). He denied history of thiazide diuretic use, fragility fracture, nephrolithiasis and family history of calcium disorders. Further workup revealed normal kidney function, undetectable PTH related peptide and dual-energy x-ray absorptiometry (DEXA) scan with a T-score of -3.3 at spine L1-L4, -2.7 at femoral neck and -2.1 at distal one-third forearm. A 24-hour urine collection revealed a urinary calcium of 42.4 mg/24-hour (100–300) and calcium: creatinine clearance ratio of 0.003. Diagnosis of primary hyperparathyroidism was made despite low urinary calcium as this was thought to be due to vitamin D deficiency. Sestamibi scintigraphy and four-dimensional computed tomography did not localize a parathyroid adenoma, however, the patient was sent to surgery for four gland parathyroid exploration for primary hyperparathyroidism in setting of high calcium and young age with evidence of end-organ failure of osteoporosis. During surgery, three large abnormal parathyroid glands were identified and one normal parathyroid gland. Patient had a three-gland parathyroidectomy with intraoperative drop in PTH by 26.5%. Pathology returned as benign parathyroid tissues. After surgery, patient had persistently elevated calcium level of 12.6 mg/dL and an inappropriately non-suppressed PTH. He was then started on bisphosphonate and cinacalcet for osteoporosis and hypercalcemia, respectively and sent for genetic testing of FHH. His CASR gene was negative but his AP2S1 gene was positive which confirmed the diagnosis of FHH type 3. His calcium responded well to cinacalcet and repeat DEXA scan showed stability of bone mineral density in spine and hip after two years of treatment with bisphosphonate therapy.
Conclusion: Familial hypocalciuric hypercalcemia type 3 is caused by an inactivating mutation in the AP2S1 gene. This gene encodes the adaptor-related protein complex 2, sigma 1 subunit which is located downstream from calcium-sensing receptor. This genetic mutation can appear similar to primary hyperparathyroidism in that it produces high levels of calcium and PTH and low phosphorus. Hypercalcemia, however, persists despite removal of parathyroid gland. This genetic mutation can be treated with cinacalcet in patients with high levels of calcium (&gt;1 upper limit of normal) or symptoms of hypercalcemia.
SAT-182 Bilateral Adrenal Hemorrhage with Clinically Preserved Adrenal Function Leading to the Diagnosis of Antiphospholipid Syndrome
Authors: Hurtado Carolina , Kim Sarah Sangnim Rhee , Grock Shira 
Published: May 2020

Abstract
Background: Spontaneous bilateral adrenal hemorrhage (BAH) is a rare complication of antiphospholipid syndrome (APS), which is the most common identifiable risk factor for BAH. Although adrenal dysfunction is generally irreversible, adrenal function might be preserved or even recover in rare cases1.
Clinical case: A 48 year-old man with history of hypertension and gout presented with right upper quadrant abdominal pain following trauma to his left leg. He was found to have a left lower extremity deep vein thrombosis and bilateral pulmonary emboli (PE) and was started on anticoagulation therapy. He continued to have abdominal pain and a CT abdomen revealed BAH. Three am cortisol level was 21 mcg/dL (8–25 mcg/dL), ACTH 37 pg/mL (6–59 pg/mL), aldosterone &lt;3 ng/dL (4–31 ng/dL), renin 2.6 ng/mL/hr (0.2–1.6 ng/mL/hr), sodium 130 mmol/L (135–146 mmol/L) and potassium 4.3 mmol/L (3.6–5.3 mmol/L). Patient was hemodynamically stable and did not report symptoms of adrenal insufficiency. Hypercoagulable work-up was consistent with APS and Lupus. Despite normal cortisol levels, he was started on hydrocortisone in the setting of anticoagulation and recent hemorrhage. Given low aldosterone with slightly high renin he was also started on fludrocortisone. Six weeks after discharge, his morning cortisol was 6 mcg/dL and ACTH was elevated at 76 pg/mL which was concerning for adrenal insufficiency. However, 250 mcg IM ACTH stimulation test showed peak cortisol of 17 mcg/dL which is considered adequate. Aldosterone and renin levels normalized so fludrocortisone was discontinued.
Patient subsequently self-discontinued all medications for 1 month with no symptoms of adrenal insufficiency, and later restarted hydrocortisone on his own. Repeat ACTH stimulation test showed baseline ACTH 57 pg/mL with peak cortisol of 17 mcg/dL. Patient was tapered off hydrocortisone and displayed no subsequent symptoms of adrenal insufficiency.
Conclusion: This case highlights the need to consider APS in patients with spontaneous BAH. Additionally, patients with BAH may have relatively preserved adrenal function. There is limited data to guide when steroid replacement is necessary for patients without clear adrenal insufficiency. It may be reasonable to monitor these patients off hydrocortisone replacement with close monitoring.
1. Ramon I, Mathian A, Bachelot A, et al. Primary adrenal insufficiency due to bilateral adrenal hemorrhage-adrenal infarction in the antiphospholipid syndrome: long-term outcome of 16 patients. J Clin Endocrinol Metab. 2013;98(8):3179–3189.
SUN-626 Survival of Patients with Gastroenteropancreatic Neuroendocrine Tumors and Diabetes Mellitus in a Seer-Medicare Cohort
Authors: Thapi Sahityasri , Baeg Kiwoon , Kim Michelle K , Gallagher Emily Jane 
Published: May 2020

Abstract
Background: The incidence and prevalence of gastroenteropancreatic neuroendocrine tumors (GEP-NET) is increasing globally and has been associated with diabetes mellitus (DM). In this study we aimed to compare tumor characteristics, disease-specific survival (DSS) and overall survival (OS) of GEP-NET patients (pts) with and without DM. Methods: Using the Surveillance, Epidemiology, and End Results registry (SEER) linked to Medicare claims, we identified pts diagnosed with GEP-NET between January 1995 and December 2010, aged ≥65 years at the time of GEP-NET diagnosis. We included patients who were in exclusive Medicare coverage without healthcare management organizations and had Medicare Parts A and B coverage for ≥1year after GEP-NET diagnosis or until death. Within the pts with GEP-NET diagnosis, we identified those without a diagnosis of DM prior to the GEP-NET diagnosis. We compared baseline sociodemographics, co-morbidities, and GEP-NET location, stage, grade and treatment between pts with and without DM using χ 2 analysis. Kaplan Meier (KM) curves were used to compare OS and DSS up to 10 years between the DM and non-DM groups. We used Cox proportional hazards analysis to compare the DSS between the groups, adjusting for confounding variables. Results: We identified a cohort of 1,969 well-characterized GEP-NET patients with accurate tumor stage, grade, comorbidities, and treatment data. 478 (25.7%) had DM and 1,383 (74.3%) did not have DM. There were no statistically significant differences in gender or age at the time of GEP-NET diagnosis in the DM (mean age 74.7±SD 6.6 yrs) and non-DM (74.9±7.4 yrs) groups. Significant differences in race were found in the DM (80.6% white, 13.6% black, 1.3% hispanic) and non-DM (86.8% white, 8.2% black, 1.8% Hispanic) groups (p=0.002). Patients with DM had more gastric (14.7%), duodenal (10.9%) and pancreatic (21.0%), and less jejunal/ ileal (12.8%) NETs compared with the non-DM group (9.7%, 6.4%, 16.9%, 18.2%, respectively, p&lt;0.0001). Patients with DM had earlier stage disease than those without DM (p=0.0012), but no difference in tumor grade or treatment was found. KM curves revealed no differences in OS and DSS in the GEP-NET patients with and without DM across all stages. Multivariate adjusted Cox proportional-hazards model found no significant difference in DSS between those with and without DM (HR=0.97, 95%CI: 0.76–1.24). Compared with pts with pancreatic NETs, pts with colon (HR=1.39, 95%CI: 1.04–1.86) had worse survival, while those with jejunal/ileal (HR = 0.59, 95%CI: 0.42–0.83) NETs had a better survival. Discussion: This is the first study to investigate the effect of DM on survival of pts GEP-NETs. We found a high prevalence of pre-existing DM in pts with GEP-NETs, but no difference in OS or DSS in pts with and without DM. Interestingly, pts with DM had more foregut GEP-NETs which may suggest mechanistic links between DM and GEP-NETs at these sites.
MON-442 Tall Cell Variant of Papillary Thyroid Carcinoma Presenting Acutely as a Right Thyroid Mass and Thyroiditis - an Unusual Presentation
Authors: Goyal Itivrita , Bhat Salman Zahoor , Batra Manav , Karunakaran Abhijana 
Published: May 2020

Abstract
INTRODUCTION
Aggressive malignant thyroid tumors (AMTT) can rarely present with signs and symptoms mimicking that of acute suppurative thyroiditis (AST). In its early course, correct diagnosis may be difficult but a prompt recognition is crucial. We report an interesting case of an aggressive papillary thyroid cancer with presenting acute symptoms masquerading as AST.
CLINICAL CASE
A 50-year-old Caucasian male was admitted for 2 day history of sore throat, odynophagia, low grade fever with a tender and enlarging right neck swelling, not improving with oral antibiotics. CT neck showed a 5.8 x 4.5cm mass in right thyroid lobe with shallow retropharyngeal effusion. Labs showed elevated WBC 10.7 x 109/L (4–10.5), ESR 20 mm/hr (0–12), CRP 92.93 mg/L (0.2–10), with negative rapid strep and IgM for viruses EBV and CMV. Thyroid function tests were normal. Ultrasound neck showed an ill-defined 6.5 cm hypoechoic mass with cystic component and increased vascularity and calcification, replacing the right thyroid lobe. Due to concern for thyroid abscess he underwent urgent aspiration. Fluid analysis and cultures were negative for bacterial source. FNA (fine needle aspiration) of one specimen was suspicious for follicular neoplasm with predominant hurtle cells and suspicious for thyroid neoplasm with papillary architecture in the other. Core biopsy was suspicious for papillary carcinoma but limited due to scant amount of tissue. Affirma unavailable due to insufficient RNA yield. Flow cytometry was negative for abnormal lymphocyte population. Repeat CT neck 3 weeks later was concerning for metastatic lymphadenopathy. He underwent total thyroidectomy with lymph node (LN) dissection and pathology showed papillary thyroid cancer, tall cell variant, tumor size 6 cm, predominantly hemorrhagic, extending to perithyroidal strap muscles and involving 5/6 LN in right trachea-esophageal groove. It was a stage I, pT3bN1aMx cancer. Subsequently, he underwent thyrogen stimulated I-131 ablation of residual thyroid carcinoma with pretherapy scan showing increased uptake in thyroid bed and a satellite lesion suspicious for LN metastasis. Post-ablative scan showed no other new lesions. He is now on levothyroxine suppressive therapy.
CONCLUSION
Early course of AMTT can rarely present as AST, both being progressive and fatal diseases. Our case had a typical presentation of AST, causing delay in diagnosis of his very aggressive form of thyroid cancer. Gross pathology showed predominantly hemorrhagic areas in the tumor with central necrosis, which was likely the cause of acute mass with systemic signs suspicious for infection. One should consider an alternative diagnosis in suspected cases of AST in the absence of positive cultures or lack of improvement with parenteral antibiotics. It is imperative to make a diagnosis without any delay as prognosis for either condition depends on prompt recognition and treatment.
SAT-248 Cushing’s Disease with Two ACTH-Producing Pituitary Tumors
Authors: Mathai Christine , Anolik Jonathan Robert 
Published: May 2020

Abstract
Objective: The objective of this case report is to discuss a case of Cushing’s disease with two ACTH-producing pituitary tumors and emphasize consideration of repeat surgery as a treatment modality for unsuccessful initial surgery.
Methods: We present a case of a patient with Cushing’s disease with two ACTH-producing pituitary tumors and a literature review.
Results: A 36 year-old female found to have left supraclavicular fossa swelling was screened for Cushing’s syndrome. Midnight salivary cortisol levels elevated at 0.636 ug/dL and 0.316 ug/dL (&lt;0.010–0.090 ug/dL). 24-hour urine cortisol 162 ug/24 hr (0–50 ug/24 hr). 1-mg dexamethasone suppression test 14.0 ug/dL. Serum morning cortisol 26.4 ug/dL with corresponding ACTH 66.7 pg/mL (7.2–63.3 pg/mL). MRI brain with and without contrast showed a 7-mm relatively hypoenhancing lesion of the anterior pituitary gland. 8-mg dexamethasone suppression test 2.7 ug/dL. She underwent transsphenoidal surgery (TSS) and pathology was consistent with a pituitary adenoma staining positive for ACTH. No residual tumor was seen. Postoperative morning serum cortisol 17.0 ug/dL and ACTH 79 pg/mL (9–46 pg/mL). She had repeat TSS and the area of resection was clean with no residual tumor but a second adenoma was found that was not visualized on MRI and was distinct from the initial lesion. Postoperative morning cortisol 0.7 ug/dL and ACTH &lt;9 pg/mL (9–46 pg/mL). Pathology was consistent with pituitary adenoma staining positive for ACTH. She is now on steroids for central adrenal insufficiency.
Discussion: First-line treatment for Cushing’s disease is surgical resection of the primary lesion (Nieman LK, Biller BMK, et al. Treatment of Cushing’s syndrome: an endocrine society clinical practice guideline. J Clin Endocrinol Metab. 2015; 100(8):2807–2831). Remission rates are 73–76% for selectively resected microadenomas but 43% for macroadenomas (Nieman et al. 2015). For patients who undergo a noncurative surgery, second-line therapies include repeat TSS, radiotherapy, medical therapy, and bilateral adrenalectomy. Repeat TSS is recommended particularly in patients who had evidence of incomplete resection or a pituitary lesion on imaging although this was not the case with our patient. Repeat TSS is cited to be successful in about 50–60% of cases (Patil CG, Veeravagu A, et al. Outcomes after repeat transsphenoidal surgery for recurrent Cushing’s disease. Neurosurgery. 2008;63(2):266–270) but carries an increased risk of hypopituitarism and lower likelihood of remission compared to initial surgery. Remission can be achieved more rapidly compared to other second-line treatments.
Conclusion: In Cushing’s Disease with unsuccessful initial surgery, consideration for repeat TSS may be considered when there is access to an expert pituitary surgeon.
OR32-05 Lower Oxytocin Levels Are Associated with Lower Bone Mineral Density and Less Favorable Hip Geometry in Hypopituitary Men
Authors: Maso Ana Aulinas , Guarda Francisco J , Yu Elaine Wei-Yin , Haines Melanie S , Asanza Elisa , Silva Lisseth , Tritos Nicholas A , Verbalis Joseph G , Miller Karen Klahr , Lawson Elizabeth Austen 
Published: May 2020

Abstract
Introduction: Hypopituitary patients are at risk for bone loss. Oxytocin (OT) and vasopressin (VP) are hypothalamic-posterior pituitary hormones with opposing actions on bone (anabolic and catabolic, respectively). Whether OT and/or VP contribute to impaired bone homeostasis in hypopituitarism is unknown.
Hypothesis: We hypothesized that lower plasma OT and higher VP levels would be associated with lower bone mineral density (BMD) and less favorable hip geometry and estimated strength in men with hypopituitarism.
Design: We performed a cross-sectional study of 37 men with hypopituitarism ages 20–60 (mean±SEM 45.8±1.9) years: 20 with anterior pituitary deficiencies only (APD) and 17 with central diabetes insipidus (CDI; marker of posterior pituitary dysfunction), of similar age, body mass index and number of adenohypophyseal deficiencies, on stable hormone replacement. Main outcome measures were fasting plasma OT and VP levels, and dual X-ray absorptiometry-derived BMD (lumbar spine, total hip, femoral neck, distal radius and subtotal body) and hip structural analysis (HSA; cortical thickness, section modulus, and buckling ratio at narrow neck, intertrochanteric region and femoral shaft). All analyses were adjusted for multiple comparisons using Holm-Bonferroni correction.
Results: Mean BMD Z-scores were lower at all sites and all HSA parameters at the intertrochanteric region as well as cortical thickness at the femoral shaft were less favorable in those participants who had fasting OT levels below the median than in those with higher levels (P≤0.022). There were no differences in any bone variables at any skeletal site in those with fasting VP levels below vs. above the median (P≥0.232). Lower fasting OT levels were positively associated with (1) lower BMD Z-scores at the lumbar spine, femoral neck, total hip and subtotal body (P≤0.02) and (2) less favorable hip geometry and strength variables (lower cortical thickness, lower section modulus and higher buckling ratio) at the intertrochanteric region in CDI (P≤0.018), but not APD participants (P≥0.458 and P≥0.429, respectively). The associations between OT and bone variables remained significant after adjusting for key determinants of BMD including lean body mass and IGF-1 levels. There were no relationships between plasma VP levels and bone variables in CDI or ADP groups (P≥0.173).
Conclusions: OT, but not VP levels, are positively associated with BMD at multiple sites as well as favorable hip geometry and estimated strength in men with hypopituitarism and CDI. Future studies will be important to determine whether OT could be used therapeutically to optimize bone health in patients with hypopituitarism.
MON-106 Prevalence and Incidence of Obesity in Children and Young Adults in Korea: The Kangwha Study
Authors: Suh Junghwan , Song Kyungchul , Choi Han Saem , Kwon Ah Reum , Chae Hyun-wook , Kim Ho-Seong 
Published: May 2020

Abstract
Childhood obesity is a major global concern, arousing a variety of health problems and high social costs. The prevalence of childhood obesity has increased, but plateaued during the first decade of the 21th century in developed countries. Many previous studies reported cross-sectional data with prevalence of obesity, but researches about incidence of obesity based on longitudinal data is insufficient, especially in Korean population. In this study, we analyzed prevalence and incidence of overweight and obesity in Korean children and young adults from a prospective cohort study. We analyzed data from the Kangwha study, which is a community-based prospective cohort study began in 1986 with 6-year-old, first-grade elementary school students in Kangwha county, South Korea. The Kangwha study is a dynamic cohort, which the number of participants was expanded several times during the follow-up period. The study was started with 482 children, which expanded to total 1,223 participants. The participants were examined annually until 1997, which is 17 years of age. Four adulthood follow-up studies were performed in 1999, 2005, 2010, and 2015. The data includes a 30-year period (1986–2015), with total 16 observations conducted. We used the 2007 Korean National Growth Charts to determine cutoffs for normal weight, overweight, and obesity in children. In adulthood, Asia-Pacific classification of obesity from World Health organization (WHO) recommendation was applied to define overweight and obesity. When the children were in their first-grade of elementary school, prevalence of overweight was 1.04% and obesity was 0.62%. Prevalence of overweight and obesity increased since age 10 (fifth-grade in elementary school) (overweight 4.44%, obesity 1.18%), reaching 7.34% and 3.39% in age 12 (first-grade in middle school), and 9.5% and 5.37% in age 17 (third-grade in high school). Prevalence of overweight and obesity in girls were higher than in boys throughout childhood. Annual incidence of obesity showed small peak at entering middle school (2.1%, age 12), and another peak at third-grade in high school (2.33%, age 17). Also, a positive correlation was found between body mass index (BMI) in younger ages and follow-up BMI. Prevalence of overweight and obesity increased since senior years of elementary school, and more prominent in girls. Detailed study design and larger population would be required for subsequent investigations.