bluuebunny/crossref_metadata_2025_split huggingface dataset:
MON-LB126 A Benign and Favorable Diagnosis: Glycogen Hepatopathy Causing Transient Transaminitis During Diabetic Ketoacidosis in Type 1 Diabetes Mellitus
Authors: Yau Alice , Elayaperumal Pramma , Butala Ashvin , Bahtiyar Gul , Rodriguez Giovanna 
Published: May 2020

Abstract
Background: Transient transaminitis is a rarely discussed complication of uncontrolled diabetes mellitus (DM). Known as glycogenic hepatopathy (GH), it is belived to be caused by build-up of glycogen in hepatocytes. Recognized as benign and reversible, GH is associated with hepatomegaly (&gt;90% cases) and primarily seen in patients with type 1 DM during periods of inadequate hyperglycemic control. Differential diagnoses include glycogen storage diseases, nonalcoholic fatty liver disease, hepatosclerosis, autoimmune hepatitis, hemochromatosis, Wilson disease, and acute viral hepatitis.1
Case Report: A 26-year-old African American female with type 1 DM and sickle cell presented on multiple occasions to the emergency department with abdominal pain associated with nausea, vomiting and diarrhea. Initial labs consistently included glucose levels &gt;600 mg/dL (70-105 mg/dL), elevated anion gap ranging 20-40s mEq/L (5-15 mEq), and severe metabolic acidosis reflective of diabetic ketoacidosis (DKA). Labs were also significant for repeated mild transaminitis despite adequate fluid hydration.
After several admissions, we observed a distinct pattern of mild transaminitis that directly fluctuated with her levels of blood glucose. With some minor lag, the patient’s liver enzymes normalized when her glucose levels normalized and DKA resolved.
Further work-up ruled out more common etiologies of liver injury. Multiple abdominal ultrasounds and CT scans showed a normal sized liver without obvious structural abnormalities. Labs were significant for negative hepatitis B and hepatitis C; several negative anti-smooth muscle, anti-nuclear antibody, centromere antibody, and liver kidney microsomal type 1 antibody; normal levels of ceruloplasmin and alpha 1 anti-trypsin; low iron levels 23 ug/dL (60-180 ug/dL); borderline low IgG 627 mg/dL (700-1600 mg/dL).
We hypothesized that the patient likely had GH by exclusion of other liver pathologies and given the context of transient transaminitis during DKA.
Conclusion: GH is a benign and favorable diagnosis in diabetic patients with elevated transaminases.1 Given the small number of cases of GH reported, there is a need to record and analyze more patients with likely GH in order to better understand the condition. Appropriate clinician awareness of GH can also eliminate the need for time consuming and costly workup.
References:1. Sherigar, Jagannath M et al. “Glycogenic Hepatopathy: A Narrative Review”. World Journal Of Hepatology, vol 10, no. 2, 2018, pp. 172-185. Baishideng Publishing Group Inc., doi:10.4254/wjh.v10.i2.172.
MON-LB311 Clinical Guidelines for the Research on the Endocrinology of Physical Activity and Sport
Authors: Cadegiani Flavio A , da Silva Pedro Luiz H 
Published: May 2020

Abstract
Background: The importance of physical activity has achieved levels that has been recognized by as a major world public health game changer, which positively engaged a growing number of subjects to become physically active. While effects of exercise on cardiovascular and musculoskeletal systems are well described, little is known regarding hormonal adapted physiology in athletes, as well as how to detect and manage endocrine abnormalities in physically active subjects. Methodological issues and inconsistent findings precluded from a structured understanding of the endocrinology physiology of physical activity and sport. The objective of the present guideline is to uniformize the design and assessment methods of further studies in the field, based on standardized hormonal and metabolic parameters, and dynamic testings. Methods: Guidelines were actively searched within endocrinology, sports medicine, and cardiology societies. Systematic search on PubMed and Cochrane databases for the expressions “(name of the parameter or test)” + “exercise” or “athlete(s)” or “sport(s)” or “validation” or “standardization. Guidelines, consensus, statements, original studies, and reviews that standardized, validated, or proposed parameters and tests that could be potentially employed for the research on physical activity and sport were included.Results: Parameters of the hypothalamic-pituitary-gonadal (HPG), hypothalamic-pituitary-adrenal (HPA), growth hormone-insulin-like growth factor-1 (GH-IGF1), and hypothalamic-pituitary-thyroid (HPT) axes, prolactin, renin-angiotensin-aldosterone system (RAAS), catecholamines and adrenal medulla, bone, water, glucose, and lipid metabolism, and adipose tissue and muscle endocrine profile were analyzed and classified according to the level of standardization, feasibility, and potential roles in physical activity and sport, and recommended accordingly. Recommendations on the characterization of the studied population, including eating, sleeping, social, psychological, and training patterns were depicted. Conclusion: The research on the endocrinology of physical activity and sport requires standardization and uniformization regarding the description of baseline and training characteristics, and which parameters and tests should be employed. These improvements will allow the development of a more structured and comprehensive knowledge on the field, based on comparative joint analyses of further researches, that should employ well-established parameters and adequately controlled for confounding variables.
SAT-347 Diagnosis of Autosomal Dominant Hypocalcemia Type 1 Following the Initiation of Imatinib for Treatment of Chronic Myeloid Leukemia
Authors: Jones-Ryan Madeline L , Desouza Cyrus V 
Published: May 2020

Abstract
Background: Autosomal Dominant Hypocalcemia Type 1 is an underdiagnosed condition due to the vast majority of patients being asymptomatic and having mild hypocalcemia. Imatinib has been associated with hypophosphatemia and hypocalcemia.
Clinical Case: 69-year-old man who had a long history of asymptomatic mild hypocalcemia, calcium level of 7.9 mg/dL (8.4 – 10.2 mg/dL) diagnosed with chronic myeloid leukemia and developed symptoms of hypocalcemia within 2 months of treatment with imatinib. After the initiation of imatinib, his calcium reduced to 6.8 mg/dL (8.4 – 10.2 mg/dL) with a corresponding ionized calcium of 0.98 mmol/L (1.12–1.32 mmol/L) within 2 months. He developed tetany. With the reduced calcium level his PTH was unexpectedly low-normal at 34 pg/mL (16–62 pg/mL). He also had a higher than expected urinary calcium of 342 mg/24 hours. The PTH and 24-hour urinary calcium levels raised concern for an underlying diagnosis of autosomal dominant hypocalcemia type 1. His phosphorous was normal at 3.3 mg/dL (2.6–4.9 mg/dL). He never had hypophosphatemia, which is common with imatinib.
After two doses of IV calcium and initiation of oral calcium replacement, 1000 mg BID, his level continued to be reduced with symptoms. Given his symptoms, laboratory results, and continued hypocalcemia he underwent genetic testing. Results of his genetic testing showed a p.Thr151Met mutation in the CASR gene consistent with autosomal dominant hypocalcemia type 1. With this diagnosis, there is concern for nephrolithiasis with over treatment. His symptoms resolved with treatment with calcium 1000mg TID, calcitriol 0.25 mg BID and vitamin D3 2000 IU daily. He has not developed nephrolithiasis and his urinary calcium increased to 424mg/24 hours. A thiazide diuretic is being considered to decrease urinary calcium excretion.
He continues on imatinib with an excellent response to therapy. It is likely that the trigger for symptomatic severe hypocalcemia was initiation of imatinib superimposed on the hypocalcemia type 1. Possible mechanisms for this are decreased intestinal absorption, increased urinary loss, or decreased dissolution from bone. 1 These mechanisms are also associated with hypophosphatemia, which our patient did not have. Another possible cause could be an immune mediated destruction of the parathyroid gland, a rare finding seen with other tyrosine kinase inhibitors.2
Conclusion: This is the first reported case of imatinib triggering severe symptomatic hypocalcemia leading to the diagnosis of underlying autosomal dominant hypocalcemia type 1.
References:
1.
Vandyke, Kate, et al. “Dysregulation of Bone Remodeling by Imatinib Mesylate.” Blood, vol. 115, no. 4, 2010, pp. 766–774., doi:10.1182/blood-2009-08-237404.
2.
Petrić, Marija, et al. “A Rare Case of Hypocalcemia Induced by Nilotinib.” Endocrine Oncology and Metabolism, 3 Mar. 2017, pp. 32–53., doi:10.21040/eom/2017.3.5.1.
SUN-525 Successful Surgical Management of Graves’ Disease in Pregnancy
Authors: Ali Dalal , Balan Gabriela , Wan Mahmood Wan Aizad , McDermott Enda , Crowley Rachel 
Published: May 2020

Abstract
Background: Total thyroidectomy in pregnancy is not a widely used approach for management of Graves’ disease (GD) but is indicated when thyrotoxicosis persists in spite of efforts to optimise thyroid status.
Clinical case: A 27-year-old lady with history of GD, presented at the 9th week of her second pregnancy. She had been counselled about anti-thyroid medications but was on carbimazole (CBZ) 30 mg tds and propranolol LA 80 mg od at presentation. She complained of palpitations, heat intolerance, irritability, weight loss and difficulty swallowing. On clinical examination, she had a heart rate of &gt; 100/min and diffusely enlarged goiter with a bruit. Thyroid Ultrasound showed a right lobe of 6.5 x 2.8 x 2.7 cm and left lobe 5.3 x 2.6 x 2.4 cm. Free thyroxine (FT4) was 42.3 pmol/L (12–22), free triiodothyronine (FT3) 9.09 nmol/L (1.3–3.1), and TSH &lt; 0.01 mIU/L (0.27–4.2). TRAB titer was &gt;40 IU/L (0.0–1.75). She was advised to switch to propylthiouracil (PTU) and labetalol to minimize fetal adverse outcomes. She reported that she was unable to afford PTU and requested a switch back to CBZ.
During her course of therapy, she had recurrent admissions with thyrotoxicosis, tachycardia, panic attacks and difficulty in swallowing. A decision was made to manage her with total thyroidectomy in the second trimester. She was treated with Lugol’s iodine, beta blockers and CBZ 2 weeks prior to her surgery and there were no immediate post-operative adverse events. Histology was consistent with GD. Her post-op TRAB titer remained &gt;40 IU/L until present.
She delivered at 28 weeks of gestation due to threatened premature labor a baby boy who had neonatal thyrotoxicosis, required admission to the neonatal ICU and therapy with flecanide and CBZ. His TSH was 0.09 mIU/L, (FT4) 68.7 pmol/L and TRAB 19.4 IU/L. He is currently 18 months old, well and not on any medications.
Conclusion: Poor control of thyrotoxicosis is associated with pregnancy loss, prematurity, stillbirth, thyroid storm, and maternal congestive heart failure. Therefore, pre-pregnancy counseling is crucial to establish Euthyroid state for the safety of mother and fetus.
Reference: (1) Davis LE, Lucas MJ, Hankins GD, Roark ML, Cunningham FG. Thyrotoxicosis complicating pregnancy. Am J Obstet Gynecol. 1989;160:63–70. doi: 10.1016/0002-9378(89)90088-4. (2) Vini L, Hyer S, Pratt B, et al. Management of differentiated thyroid cancer diagnosed during pregnancy. Eur J Endocrinol. 1999;140:404–406.
MON-250 Late Diagnosis of ACTH-secreting Pulmonary Neuroendocrine Tumor by Repeated 68Ga Dotatate Pet/ct: Influence of Tumor Size in Abnormal Uptake?
Authors: Andrade Natália Xavier S , Pereira Ana Julia Garcia , Villares Fragoso Maria Candida Barisson , Bronstein Marcello Delano , Machado Marcio Carlos 
Published: May 2020

Abstract
Background: 68Ga DOTATATE PET/CT (68Ga-PET) has been proposed as a superior method in identifying ectopic ACTH syndrome (EAS). However, recent systematic review suggests its sensitivity is not as high as believed (1). We report a challenging case of EAS whose source was uncovered only after repeated 68Ga-PET. Clinical Case: A 15-year-old male presented with rapid onset of typical features of Cushing’s syndrome (CS) and metabolic impairment. Hormone evaluation confirmed severe ACTH-dependent CS. Pituitary transsphenoidal surgery was performed due to positive responses in desmopressin stimulation and high dose dexamethasone suppression test, in addition to a 4 mm nodule in pituitary MRI. No tumor was found in surgical specimen and no hormonal improvement was observed after surgery. Inferior petrosal sinus sampling demonstrated no central to peripheral ACTH gradient. Neck US, thorax/abdomen/pelvis CT were negative and PET-CT/FDG was inconclusive. OctreoScan® identified anomalous uptake on left mediastinum and led the patient to a thoracic surgery (TS) with nodule resection at left hilum. Pathology confirmed ACTH positive 10 mm neuroendocrine tumor (NET) infiltrating a lymph node. The patient had transient clinical and hormonal improvement, with recurrence 7 months later. Thoracic CT (T-CT) showed a 7 mm nodule on inferior segment of superior left lobe, PET-CT/FDG and OctreoScan® were negative but abnormal uptake was verified by 68Ga-PET in subcarinal area. A sub centimetric lymph node was resected and pathology confirmed ACTH positive NET, although the patient did not achieve remission. Octreotide LAR, cabergoline and ketoconazole did not control hypercortisolism and bilateral adrenalectomy was performed. Then, T-CT showed stable lung nodule and 2nd 68Ga-PET was negative. One year later, T-CT evidenced growth of lung nodule to 15 mm and 3rd 68Ga-PET demonstrated for the first time, abnormal uptake in this area. The patient underwent resection of left superior lung lobe along with ipsilateral hilar lymph nodes, and histopathology study revealed an ACTH-secreting atypical pulmonary carcinoid tumor with Ki67 of 10% and 5 out of 11 lymph nodes affected. ACTH fell from 288 to 64 pg/mL after surgery. Conclusion: Despite the high sensitivity attributed to 68Ga-PET, false negatives have been reported. In the present case, primary tumor was evidenced by 68Ga-PET seven years after the first resection of a metastatic lymph node, in the 3rd 68Ga-PET assessment and after tumor growth. This adds to the evidence that further studies are needed to better assess the accuracy of 68Ga-PET for EAS. Reference: [1] Varlamov et al. Diagnostic utility of Gallium-68-somatostatin receptor PET/CT in ectopic ACTH-secreting tumors: a systematic literature review and single-center clinical experience. Pituitary 2019; 22:445–455
MON-671 Embarrassed to Eat: Two Cases of Gustatory Hyperhidrosis
Authors: Tarar Omer H , Munoz Andres J 
Published: May 2020

Abstract
Introduction
Diabetic Gustatory Hyperhidrosis is characterized by profuse sweating with eating and may be a manifestation of Diabetic autonomic dysfunction. Most patients have evidence of other microvascular complications including nephropathy, retinopathy, peripheral neuropathy and other signs of autonomic neuropathy.
We present 2 cases of gustatory hyperhidrosis associated with longstanding poorly controlled type 1 diabetes.
Case 1: 49 year old Male with past medical history of longstanding type 1 diabetes with poor control, complicated with diabetic retinopathy, polyneuropathy, albuminuria presented to endocrine clinic for management of diabetes. His hemoglobin A1c was 10.8%. He was on basal-bolus Insulin at home. However, he admitted to missing most doses of prandial Insulin. On further questioning, he mentioned having episodes of profuse head and neck sweating while eating any type of food. He attributed these episodes to “low blood sugars” without checking and therefore tried to avoid Insulin. However, he continued having these episodes. He was diagnosed with Diabetic gustatory hyperhidrosis and started on topical Aluminum hexahydrate.
Case 2: 34 year old Female with past medical history of long-standing DM type 1 complicated with poly- neuropathy, autonomic dysfunction, nephropathy, Retinopathy, chronic kidney disease stage III presented for follow up of her diabetes. Her hemoglobin A1c was 9.8%. She was on basal-bolus Insulin at home and reported good compliance. Given her extensive polyneuropathy, she was questioned about hyperhidrosis. She reported having profuse facial and neck sweating with eating all types of food which led to increased embarrassment while eating in public. She was diagnosed with diabetic gustatory hyperhidrosis and started on topical aluminum hexahydrate, with plans for Botox if symptoms persisted.
Discussion
Diabetic Gustatory Hyperhidrosis is an under- recognized condition and may be misdiagnosed as hypoglycemia, anxiety, gastroparesis or other conditions. This gustatory sweating is a source of severe distress and embarrassment for patients and can have serious emotional, social and professional implications. Associated symptoms may also be mistaken for hypoglycemia and in turn lead to nonadherence with Insulin and other diabetic medications causing suboptimal glycemic control.
Topical anti-perspirants like Aluminum Chloride hexahydrate are often used as first line therapy. Second line treatment options include glycopyrrolate, Oxybutynin and Botulinum toxin.
Conclusion
Most patients are reluctant to mention these symptoms to health care providers and diligent history taking with specific questions in high risk patients may help in early identification and management of this condition.
Early identification and management can also help promote overall confidence, quality of life and better glycemic control.
SUN-498 A Case of Thyroid Storm with Systemic Thromboembolism
Authors: Maung Aye Chan , Tan Daphne Gardner Su-Lyn 
Published: May 2020

Abstract
Background
Thyroid storm is a rare but potentially life-threatening complication of hyperthyroidism. Whilst a thyroid storm is known to be a hypercoagulable state, it remains unclear if routine anticoagulation should be initiated, especially in the absence of atrial fibrillation.
Case presentation
A 22-year-old seaman presented to Accident and Emergency Department with a history of severe generalized abdominal pain and vomiting for 9 days. He was previously well with no significant past medical history. On examination, he appeared very anxious and agitated. He had sinus tachycardia (170 beats/min), was normotensive (Blood pressure 116/90 mmHg). He had exophthalmos, lid lag, a diffusely enlarged goiter with bruit and fine tremors on outstretched hands. There was generalized abdominal tenderness with guarding and sluggish bowel sounds. Electrocardiogram confirmed sinus tachycardia. Laboratory results showed primary hyperthyroidism [Free T4 66.2 (0.8–14.4 pmol/L), TSH &lt;0.010 (0.65–3.70 MU/L)]. TSH Receptor Antibody was elevated at 6.23 IU/L (&lt;1.76 IU/L), consistent with Grave’s Disease. He had acute renal impairment [urea 10.8 (2.7–6.9 mmol/l), creatinine 221 (54–101 umol/l)]. Burch & Wartofsky score was 60. Treatment with rectal propylthiouracil (PTU), i.v sodium iodide and i.v hydrocortisone were initiated.
An initial CT Abdomen on Day 1 of admission demonstrated a long segment of jejunitis and marked distension of the duodenum, stomach and oesophagus. 4 days later, fT3 and fT4 levels had improved as did tachycardia and his confusion state, yet he remained febrile. Blood and urine cultures did not reveal any causative organisms. A contrast-enhanced CT revealed extensive thromboses of the portal, superior mesenteric, right external iliac, common femoral veins with left lower lobe pulmonary embolism. Thrombophilia screen was normal. He was given low-molecular-weight heparin and required total parenteral nutrition in view of prolonged bowel ileus from mesenteric ischaemia. Rectal PTU was continued to treat thyrotoxicosis. He made sufficient progress with improvement of the bowel ileus with s.c enoxaparin and was discharged 6 weeks later on oral carbimazole (on discharge: fT4 10pmol/L, TSH&lt;0.010). However, due to extensive thromboses within the mesenteric venous system and consequent ischaemic jejunitis, he required a jejunectomy eventually 2 weeks later. Radioiodine ablation was subsequently given and he is currently hypothyroid requiring thyroxine replacement.
Conclusion
Extensive systemic thromboembolism may occur in the setting of a thyroid storm. Routine prophylactic anticoagulation should be considered, even in the absence of atrial fibrillation.
References
1. Lin HC et al. Journal of Thrombosis and Haemostasis 2010, 8: 2176–2181
2.Kootte et al. Thromb Haemost 2012; 107: 417–422
3. Franchini et al. Clinical and Applied Thrombosis 2010, 17(4) 387–392
SUN-173 A Gigantic Uterine Leiomyoma and Big Bilateral Adrenal Myelolipomas as a Result of Untreated Congenital Adrenal Hyperplasia
Authors: La Betty , Nguyen Ha , Tung Celestine , Choi Eugene 
Published: May 2020

Abstract
Introduction
Patients with untreated congenital adrenal hyperplasia (CAH) can present early with severe symptoms of salt wasting, adrenal insufficiency and hyperandrogenism. Late consequences as a result of long term untreated CAH are rarely seen nowadays. We present a patient who presented with a massive uterine leiomyoma and bilateral adrenal myelolipomas due to longstanding treatment noncompliance.
Clinical Case
A female was born with ambiguous genitalia and diagnosed with CAH at birth. She was raised as a female and received steroids until age 29 when she stopped taking steroids on her own with the intention of identifying as a male. At age 37, he presented with abdominal distension, vomiting, and hypotension. Physical exam was notable for hypotension, significantly distended abdomen, hirsutism, gynecomastia and clitoromegaly. Labs revealed sodium 126 meq/L (136–145) cortisol 78.5 ug/dL (3.7–19.4), ACTH 166 pg/mL (6–50), 17-hydroxyprogesterone 4356 ng/dL (≤285), androstenedione 7188 ng/dL (35–250), total testosterone 737 ng/dL (2–45), estradiol 142 pg/mL (48–440), aldosterone &lt;1 ng/dL (3–16), renin 0.45 ng/mL/hr (0.25–5.82), metanephrines 56 pg/mL (≤205), normetanephrines 56 pg/mL (≤148). CT abdomen and pelvis revealed a large 31 x 35 x 31 cm pelvic mass, a 5.9 x 2.4 cm right adrenal mass and an 11.8 x 8.8 cm left adrenal mass. The patient underwent total abdominal hysterectomy and bilateral adrenalectomy. Pathology of the pelvic mass was consistent with uterine leiomyoma (gross tumor was 12.4 kg) and pathology of the bilateral adrenal masses were consistent with bilateral adrenal myelolipomas.
Discussion
Glucocorticoids and mineralocorticoids are the mainstays of treatment in CAH, with the goal of providing adequate replacement while reducing levels of ACTH and adrenal androgens. Persistently elevated levels of ACTH and androgens can lead to many serious sequela, even outside of adrenal insufficiency and virilization. Due to the conversion of androgens to estrogens, untreated females with CAH have significantly elevated levels of both hormones. These high levels of androgens and estrogen can then stimulate growth of estrogen-dependent organs as exemplified by our patient. Chronic ACTH stimulation can cause adrenal hyperplasia, but has also been associated with the development of other adrenal masses including adrenal myelolipomas. Adrenal myelolipomas can become hormonally functional or cause mass effect, hemorrhage, necrosis when reaching a large enough size.
Conclusion
This case demonstrates the importance of CAH treatment compliance as there are many serious sequela outside of the expected adrenal insufficiency and virilization. Even when the desired effect is virilization with physical male features, other means of hormonal therapy should be considered as there remains the risks of abnormal growth of certain organs sensitive to the excessive hormones.