bluuebunny/crossref_metadata_2025_split huggingface dataset:
SAT-556 Use of ACTH-Stimulated Lateralization Indices Underestimates Surgically Curable Primary Aldosteronism
Authors: Yozamp Nicholas , Sacks Barry , Underhill Jonathan , Fudim Tali , Vaidya Anand 
Published: May 2020

Abstract
Introduction: Adrenal venous sampling (AVS) is recommended to assess laterality in primary aldosteronism to determine whether a patient has unilateral, or surgically curable, disease. Institutional practices differ in whether ACTH stimulation is used or not and if so, whether values are obtained after a single injection of ACTH or during an ACTH infusion. Studies have demonstrated appreciable discordance between lateralization based on unstimulated versus stimulated AVS.
Objective: To assess the influence of ACTH-stimulation on lateralization indices.
Methods: We performed a retrospective cross-sectional analysis of 140 patients who underwent AVS between 2012–2019. We then validated these findings in a separate cohort of 233 patients who underwent AVS between 2008–2016. AVS was performed using simultaneous, unstimulated, and triplicate sampling from the inferior vena cava (IVC) and bilateral adrenal veins, followed by repeated sampling in duplicate or triplicate from each site following a bolus of 250 ug of ACTH (cosyntropin). The lateralization index (LI) was defined as the quotient of the aldosterone-to-cortisol ratios from each adrenal vein, and the categorical definition of lateralization was defined as a LI ≥ 2 (unstimulated) and LI ≥ 4 post-ACTH.
Results: The median unstimulated LI was 8.7 compared to 8.9 post-ACTH. Seventy-one of 140 patients (51%) had a decrease in LI following the ACTH bolus. Overall lateralization discordance was 21.4%, with the majority of this discordance (90%) attributed to situations where there was an unstimulated LI ≥ 2 that became a post-ACTH LI &lt; 4, thereby transforming a unilateral interpretation into one of bilateral disease. Comparing the group that had an increase in LI after ACTH to the group that had a decrease in LI, the latter had significantly higher rates of lateralization pre-ACTH (89% vs 74%, p=0.02) and significantly lower rates of lateralization post-ACTH (50% vs 78%, p=0.001). All of these general findings were validated in the separate cohort of 233 patients. The discordance rate between pre-ACTH lateralization and available imaging data was 32%; the same discordance rate was found when comparing post-ACTH lateralization to imaging.
Conclusions: The administration of ACTH during AVS causes an increase in LI in half of patients and a decrease in LI in the other half. Using conventional cut-offs, pre-ACTH and post-ACTH lateralization indices disagree on laterality more than 20% of the time and almost always involve pre-ACTH unilateral disease that is classified as bilateral disease post-ACTH. These findings underscore that while ACTH stimulation may be useful for confirming adrenal vein selectivity, the decrease in post-ACTH LI may result in misclassification of surgically curable primary aldosteronism in a substantial proportion of patients.
SAT-263 A Vicious Cycle of Hypercortisolism - Cyclical Cushing’s Syndrome
Authors: Force Bahar Kapoor , Yoshor Daniel , Sebastian Sherly , Takashima Mas , Samson Susan Leanne 
Published: May 2020

Abstract
Background: Cyclical Cushing’s syndrome (CS) is described as periods of excess cortisol secretion interspersed with low or normal cortisol. The generally acceptable criteria for diagnosis is demonstration of 3 peaks and 2 troughs in cortisol secretion but, in practice, patterns and duration of cycles are unpredictable and can present a significant diagnostic challenge. Here, we present a case series of three patients who did not meet the published criteria but had pathological confirmation of ACTH-dependent CS.
Clinical Case: Two women (A: 30 year-old, B: 40 year-old), and one man (C: 30 year-old) presented to the Pituitary Center for second/third opinions regarding CS. All patients had significant central weight gain. Patient A described cycles of rapid weight gain and loss, fluctuating facial and leg swelling, anxiety and darkening of stretch marks or scars punctuated with episodes of fatigue, lightheadedness and nausea. Patients B and C reported cyclical leg swelling, facial redness and acne. These patients had additional clinical signs of Cushingoid facies, dorsocervical and supraclavicular fat pads. Patient C had violaceous striae.
Multiple tests for CS were performed: 1 mg overnight dexamethasone suppression test, two midnight salivary cortisols, and 24h urine free cortisol (UFC). All testing was normal, with a few exceptions. Patient A had an isolated midnight salivary cortisol elevated to 0.426 (&lt;0.112 ug/dL). Patient C had two mildly elevated 24h UFC collections of 1.4 times upper limit of normal corresponding with his cycles of peripheral edema and acne. Patient B had completely negative testing. In all 3 patients, ACTH levels were not suppressed but also not elevated. Random serum DHEA-S levels were elevated in patients A and B.
Patients A and C were found to have possible pituitary microadenomas on MRI with dynamic contrast. Patient B’s MRI was negative but inferior petrosal sinus sampling revealed a markedly elevated tumoral level of ACTH of &gt;62,500 pg/mL with CRH stimulation. All 3 patients underwent endoscopic transsphenoidal resection by an expert pituitary surgeon with no post-operative complications in patients A & B, but development of partial diabetes insipidus in patient C. In patients A & C, pathology revealed corticotroph hyperplasia while patient B was found to have a corticotroph adenoma. Patients A & B persisted to have symptoms of hypercortisolism, opted to undergo bilateral adrenalectomy and pathology showed diffuse adrenocortical hyperplasia for both.
Conclusion: Cyclical CS is likely underdiagnosed because of difficulty in obtaining biochemical confirmation even with strong clinical suspicion of CS. DHEA-S has a long half-life as well as minimal diurnal variation, and could be an additional clue in patients with suspected pituitary source of cyclical hypercortisolism. Ultimately, clinical concern should drive further diagnostic imaging and management.
SUN-698 Management of Hyperglycemia in an Adult Patient with Glycogen Storage Disease Type 1b
Authors: Mikhael Alexandra , Zimmerman Robert S 
Published: May 2020

Abstract
Background: Glycogen storage disease type 1b (GSD1b) is caused by a deficiency of glucose 6 phosphatase leading to glycogen deposition. The hallmark findings of GSD1b are hypoglycemia and lactic acidosis. GSD1b can be associated with hepatomegaly, hypertriglyceridemia, hematologic abnormalities, hypothyroidism, inflammatory bowel disease, proteinuria, and hypoglycemic seizures. Main stay of therapy is to avoid fasting and to ingest frequent feeds high in complex CHO. Raw cornstarch (CS) has been used for the treatment of hypoglycemia in GSD1b since the early 1980s. CS is digested slowly, providing a steady release of glucose allowing for more stable glucose levels over a longer period of time as compared with other sources of CHO. Adults may require more CS to maintain BG &gt;70 mg/dL and lactate &lt;2mmol/L during the night. We report a case of an adult patient with GSD1b admitted with inability to tolerate oral intake and found to have persistent hyperglycemia on admission requiring insulin therapy.
Clinical Case: A 31 year old female with a history of GSD1b complicated by hypoglycemia, Crohn’s disease, chronic pancreatitis and neutropenia was admitted for abdominal pain, emesis and inability to tolerate CS. She was made NPO and was started on a dextrose drip to avoid hypoglycemia and hyperlactatemia. The rate of dextrose infusion was adjusted to maintain lactate levels &lt;2 mmol/L. She developed persistent hyperglycemia with glucose values of 250–350 mg/dL. Laboratory evaluation revealed an HbA1c of 7.6% (reference [ref] 4.3–5.6%), C-peptide of 1.3 ng/mL (ref 0.8–3.9 ng/mL), lactate of 2.3–3.1 mmol/L (ref 0.5–2.2 mmol/L). BHB levels were normal (WNL). Anti-GAD, insulin and islet cell antibodies were negative. The main goal was to avoid hypoglycemia while keeping ketone and lactate levels WNL. The decision was made to start a regular insulin infusion at a constant rate of 1 u/hr to keep BG &lt;180 mg/dL while on the dextrose infusion with close monitoring of lactate and BHB. Insulin and dextrose drip rates were adjusted based on BG. The ultimate goal was to determine total daily insulin requirements while ingesting her home CS doses and to transition to long acting insulin. Short acting insulin boluses were not used given risk of hypoglycemia. The patient’s unpredictable tolerance to CS made determining a fixed insulin dose challenging. She eventually managed to tolerate CS and was transitioned to 10 units of insulin Detemir twice daily. She was discharged with plans to get a CGM and a ketone meter. BG readings at home were between 140–170 mg/dL.
Conclusion: We report a rare case of GSD1b and diabetes. The pathogenesis may be related to effects of chronic pancreatitis or metabolic syndrome. Treatment of hyperglycemia in patients with GSD1b is challenging given the heightened risk of fasting hypoglycemia. Treatment options are limited, and there are no data regarding the safe use of insulin in this patient population.
SUN-409 Subclinical Hypothyroidism in Hospitalized Patients with Chronic Heart-Failure or Chronic Renal-Failure Is Most Probably Not Due to Thyroid Disease
Authors: Bashkin Amir , Saleh Wagde Abu , Ronen Ohad 
Published: May 2020

Abstract
Introduction: Subclinical hypothyroidism is common in chronic diseases such as heart failure and advanced chronic renal failure. It is unclear whether this is a thyroid disease or an isolated TSH elevation. The goal of this study was to investigate the prevalence of worsening thyroid function in these patients with recurrent admissions. Methods: We performed a retrospective review of medical records of hospitalized patients in non-surgical wards from 2013–2016. First, all patients with TSH levels above the normal range (4.95 mIU/L) and up to 12 mIU/L with FT4 levels in the normal range were identified. We then investigated which of these patients were re-hospitalized at least once within at least six months. According to data from the re-hospitalization, an increase in TSH level above 12 mIU/L or initiation of levothyroxine treatment was defined as worsening of thyroid function. Patients treated with a drug affecting thyroid function or with a known thyroid disease prior to first hospitalization were excluded from the study. Chronic heart failure and chronic renal failure were determined according to reported diagnosis and drug treatment. Chronic renal failure patients were included if the glomerular filtration rate (GFR) in the first hospitalization was below 30 ml/min/1.72 square meter. Results: Overall, 90,199 TSH tests were sent from the non-surgical wards, most of them as part of the admissions profile. Of these, 2,116 hospitalizations met the inclusion criteria of the first hospitalization. In the final analysis, 126 inpatients with at least one re-hospitalization were included, of whom 43 (34.1%) had chronic heart failure and 22 (17.5%) had chronic renal failure. According to the most recent re-hospitalization, thyroid function was worse in 11(8.7%), 4 (9.3%) and 2 (9.1%) patients of the total, heart failure and renal failure groups respectively. The TSH level was found to be normal in re-hospitalization in 81.4% of those with heart failure and 86.4% of those with renal-failure. No association between heart failure or renal-failure and thyroid function worsening was found (p = 1.00 for both). Of 34 patients with chronic heart failure re-hospitalized after 1/2-1 year, in 29 (85.3%) the repeated TSH was normal, in 3 (8.8%) it was unchanged and in 2 (5.9%) it was worse. In most re-hospitalization the worsening was due to initiation of Levothyroxin treatment and because of the retrospective nature of the study we cannot be sure whether the initiation was justified; therefore, it is likely that the worsening percentage is even lower. Conclusions: An isolated TSH elevation in hospitalized patients with past medical history of chronic heart-failure or chronic renal failure does not indicate thyroid disease, in most cases.
MON-620 Ademolus Hypoglycemic Index
Authors: Ademolu Adegbenga Bolanle 
Published: May 2020

Abstract
This article proposes ADEMOLUS HYPOGLYCEMIC INDEX (AHI) which is a mathematical representation of hypoglycemic episode (HE) in a patient with recurrent hypoglycemia over a consecutive three months period. It also apply it to clinical practice using diabetic patients in order to demonstrate and emphasize its relevance in present day medical practice worldwide.
This is a retrospective study of 65 HE occurring in 6 randomly selected diabetes mellitus patients from 86 case files studied who had attended the endocrinology unit. The data was analyzed by using Ademolus Classification of hypoglycemia (ACH) and the 2018 ADA/EASD Classification of hypoglycemia to define hypoglycemia. AHI was calculated from the HE using the proposed mathematical formula. SPSS version 23 was used for data analysis. All six patients had a series of hypoglycemic episodes occurring in three consecutive months. Patient 1 had an AHI of 0.53 using both ACH and 2018 ADA/EASD classification.By using the Pearson correlation statistics, AHI using ACH correlated well with AHI using 2018 ADA/EASD classification of hypoglycemia with a value of 0.993.Similarly the findings of AHI derived using ACH is significant with values derived using ADA/EASD 2018 classification of hypoglycemia with a p-value of 0.000 (correlation is significant at values of 0.01). One of the clinical implication of AHI is that the risk of developing reversible or irreversible neurological damage can be reduced clinically as patient with mild to moderate form of chronic hypoglycemia yet to develop irreversible neurological damage or neurological sequelae can be prevented early enough from progressing since a reduced AHI value out of a series will be a pointer towards progression to neurological damage if it has not occurred! In patient 1 her AHI was not in the severe range. In patient 3, in the last quarter of 2016, her AHI was 0.60, in the first quarter of 2017, her AHI was zero, then between June, July and August 2017 her AHI was 0.64. At this juncture in this patient management, it will be good to evaluate the etiological factors in this chronic kidney disease diabetic patient who once again has started having recurrent hypoglycemia as it was some 6 months earlier. Patient 6 had two consecutive reading of AHI for two consecutive quarters of a year.The result reveals that she is chronically deteriorating gradually and tending towards more severity in her development of HE as her AHI fell from 0.52 in the preceding quarter to 0.46., this connotes worsening chronic hypoglycemic state over time and a poorer prognosis. The lower the AHI, the poorer the prognosis.AHI is relevant for monitoring of chronic or long term recurrent HE in susceptible individuals whether diabetic or not.
References: 1. Fonseca VA, Kirkman MS, Darsow T, Ratner RE (2012) The American Diabetes Association Diabetes Research Perspective. Diabetes Care 35(6): 1380–1387.
SAT-345 Etanercept Induced Hypercalcemia
Authors: Chaker Bayan , Alim Hussam , El-Zein Louna S 
Published: May 2020

Abstract
Non-PTH mediated hypercalcemia has multiple etiologies including medications. There are some case reports showing TNF-alpha inhibitors causing sarcoidosis or sarcoidosis type presentation involving lungs, skin, lymph nodes, and kidneys.
We report here a 69-year-old male with a past medical history of prostate cancer status post prostatectomy (in remission), CKD stage 3, and seronegative rheumatoid arthritis who was sent to the ED from clinic due to hypercalcemia. On admission, patient had a calcium level of 14.2 (8.7–10.4 mg/dL), albumin 4.1 (3.4–4.8 g/dL), magnesium 2.0 (1.3–2.7 mg/dL), creatinine 2.5 (0.7–1.3 mg/dL), iPTH &lt;2 (14–88 pg/mL), PSA 0.13 (0–4 ng/mL), 25-OH vitamin D 45.3 (30–100 ng/mL), 1,25-OH vitamin D 144 (19.9–79.3 pg/mL), alkaline phosphatase 46 (46–116 U/L) along with generalized weakness, nausea, vomiting, poor appetite, decreased oral intake, dizziness, and slight epigastric pain. Patient was given IV fluids and a dose of Reclast 3.3 mg IV was given 2 days after admission. At home for at least the past year before admission, patient was on etanercept for seronegative rheumatoid arthritis which was held on admission. CT thorax was done which showed geographic ground glass worst in the bilateral upper lobes where there is interlobular septal thickening and interstitial consolidation highly suspicious for interstitial pneumonitis and reactive appearing mediastinal lymphadenopathy. During the admission PTH-rP was 2.5 (0–2.3 pmol/L) and angiotensin converting enzyme was 36 (9–67 U/L). Based on previous case reports, etanercept was held. Upon discharge 6 days later, calcium had improved to 10.0 mg/dL, albumin 3.3 g/dL, and creatinine to 1.5 mg/dL. Upon follow up, etanercept was continued to be held. One month later, calcium level improved to 9.2 mg/dL, albumin 4.0 g/dL, iPTH 187 pg/mL, PTH-rP 3.0 pmol/L, and 1,25 OH vitamin D 50.7 pg/mL. Patient’s calcium levels remained within normal range for over one year after admission and 1,25-OH vitamin D remained normal. Moreover, patient had nuclear medicine parathyroid scan which showed no evidence parathyroid adenoma. An ultrasound thyroid was done which was negative for any thyroid/parathyroid mass or nodule. Whole body bone scan was done which showed no evidence of osseous metastatic disease. Given up trending PTH-rP levels, patient was evaluated by oncology and was found to be up to date on age recommended cancer screening and no evidence of current malignancy. Repeat CT thorax done over one year later showed stable to mildly improved bilateral ill-defined centrilobular ground glass nodules in both lungs, greater on the left side, may represent improving infectious/inflammatory process. In conclusion, when in doubt, a good medication review is essential in the evaluation of hypercalcemia because TNF-alpha inhibitors like etanercept may cause a sarcoidosis like presentation.
SAT-518 Progression of Graves Disease to Hashimoto’s Thyroiditis Following Alemtuzumab Therapy for Multiple Sclerosis
Authors: Rapier Brandon , Gargasz Frank , Zmeili Omar Suheil 
Published: May 2020

Abstract
Introduction: Alemtuzumab, an anti-CD52 monoclonal antibody used in the treatment of relapsing-remitting multiple sclerosis is most commonly associated with Graves disease, but autoimmune hypothyroidism may also be seen. We present an unusual case where both were present in the same patient and progression from hyperthyroidism to hypothyroidism was seen within only a few months.
Clinical Case: A 33-year-old female referred to Endocrinology clinic for evaluation of hyperthyroidism. She was complaining of palpitations, tremors, increased sweating, heat intolerance, and unintentional weight loss for 3 months. She received 2 cycles of alemtuzumab treatments over the last 21 months for her multiple sclerosis. Last treatment was 8 months before she developed hyperthyroid symptoms. Patient had no prior history of thyroid disorder. Thyroid stimulating hormone (TSH) level was within normal range before alemtuzumab was administered. TSH was monitored periodically and was normal till 8 months after receiving alemtuzumab therapy. Physical exam was remarkable for diffuse enlarged thyroid, not tender, without palpated thyroid nodules but with thyroid bruit. No proptosis was present.
Thyroid function tests obtained by her primary care physician were consistent with hyperthyroidism. Patient found to have suppressed TSH &lt;0.015 IU/mL [0.465 - 4.680IU/mL], elevated total T3 372ng/dL [97-169ng/dL], and elevated total T4 &gt;24.9 ug/dL [5.5 - 11.0 ug/dL].
Further workup revealed elevated Free T3, 10.90 [2.77 - 5.27 pg/mL] and elevated free T4 &gt; 6.99 ng/dL [0.78 - 2.19 ng/dL]. Thyrotropin receptor antibody (TR Ab) was elevated as well at 3.43 IU/L [&lt;1.75 IU/L]. Pregnancy test was negative. Thyroid ultrasound demonstrated goiter with no focal thyroid nodules seen. She was started on methimazole 10 mg daily.
One month later, TSH was elevated at 31.58 though she only took methimazole for one week and then discontinued due to rash and pruritus. At that time, she reported severe fatigue and 25 lbs weight gain. Repeated labs one month later showed elevated TSH, 60.978 IU/ML, low free T4 0.08 pg/mL and low free T3 0.72 ng/dL. Thyroid peroxidase Antibody (TPO Ab) was obtained and was 5308.8 IU/mL [0.0 - 5.5 IU/mL]. She was started on levothyroxine 100 mcg daily. Two months later, levothyroxine dose was increased to 112 mcg daily due persistent TSH elevated. At subsequent visit, patient was euthyroid with normal TSH 3.191IU/mL and normal free T4 1.48 ug/dL.
Conclusion: This case was unique in that the patient developed both TR Ab and TPO Ab after alemtuzumab therapy which resulted in Grave’s disease followed by Hashimoto’s thyroiditis. The case highlights the importance of continuous monitoring of thyroid function in patients treated with alemtuzumab given the unpredictable autoimmune phenomena which may occur.
OR04-03 Calcitonin Receptor Expressing Neurons in the PVH Regulate Feeding Behavior
Authors: Gonzalez Ian Enrique , Cheng Wenwen , Pan Warren , Lu Chunxia , Ramirez-Matias Julliana , Pei Hongjuan , Adams Jessica , Zhu Allen , Faulkner Latrice Doreen , Myers Martin Grosvenor , Olson David P 
Published: May 2020

Abstract
The paraventricular nucleus of the hypothalamus (PVH) is a brain region crucial for energy homeostasis. Abnormal PVH development or damage leads to hyperphagic obesity and energy expenditure deficits underscoring the importance of PVH neuronal activity in energy balance control. Application of salmon calcitonin (sCT) to the PVH suppresses feeding and calcitonin receptor (CalcR) is highly expressed in the PVH of rodents suggesting that CalcR-expressing PVH neurons contribute to energy homeostasis. In situ hybridization reveals that many CalcRPVH neurons express melanocortin-4 receptor (MC4R), a receptor required for normal feeding behavior. To investigate the physiologic roles of CalcRPVH neurons, we generated CalcR-2a-Cre knock-in mice to manipulate CalcR-expressing cells. Deletion of MC4R from CalcR expressing cells using Cre-loxP technology resulted in profound obesity in both male and female mice by 16 weeks of age. This weight gain was attributable to hyperphagia, as cumulative food intake of the MC4R deleted mice was significantly greater than the controls and energy expenditure measurements acquired through CLAMS analysis were not significantly different. To determine the brain regions engaged by CalcRPVH neurons, we used anterograde Cre-dependent viral tracing reagents injected into the PVH of CalcR-Cre mice, and found that CalcRPVH neurons project to brain regions implicated in energy balance control, including the nucleus of the solitary tract and the parabrachial nucleus. To assess the acute effects of activating CalcRPVH neurons, we used DREADD technology to chemogenetically activate CalcRPVH neurons. CalcRPVH neuron activation suppressed feeding but had no significant effect on energy expenditure. To determine if the activity of CalcRPVH neurons is required for energy homeostasis, we silenced them using Cre-dependent tetanus toxin virus. Male mice with tetanus toxin silenced CalcRPVH neurons were obese 7 weeks following injection in part due to greater cumulative food intake; CLAMS analysis revealed no differences in energy expenditure. Mice with silenced CalcRPVH neurons as well as mice with CalcR deleted from the PVH had normal anorectic responses to sCT, suggesting sCT-induced anorexia does not require CalcRPVH neurons or CalcR expression in the PVH. Taken together, these findings suggest CalcRPVH neurons are an essential component of feeding and energy homeostatic circuitry.