bluuebunny/crossref_metadata_2025_split huggingface dataset:
SUN-038 Social and Psychological Aspects of Partial Androgen Insensitivity Syndrome, Therapeutic Challenges
Authors: Masood Faryal T , Kiran Zareen 
Published: May 2020

Abstract
Background: Partial Androgen Insensitivity Syndrome (PAIS) is a rare congenital condition with incongruence of chromosomal, gonadal and phenotypic sex and classified as differences of sex development. Distinct from complete Androgen Insensitivity by the presence of ambiguous genitals in a 46, XY individual with normal testis development and partial responsiveness to androgens.
Clinical Case: 18 years phenotypic female presented with primary amenorrhea and ambiguous genitalia with poor secondary sexual characteristics after puberty. Born out of a consanguineous marriage, normal vaginal delivery conducted by midwife at home in a small village, who informed a female with ambiguous genital though. Since childhood she uses to dress up in female attire. She has 5 siblings, two brothers and three sisters, one year back she got engaged to her distant cousin and was about to get married when one of her younger sister now 8 years having similar problem alarmed family to report before the wedlock.Vitals:Weight 55kg,Height 167cm,Physical, biochemical, chromosomal testing and imaging revealed:micropenis 3cm(N=8cm) with hypospadias, a small blind vaginal orifice, hormones within normal male ranges, Karyotype: XY, MRI revealed no female internal organs or prostate gland, left testis seen in partly formed scrotal sac (4.6x2.5cm) right in superficial inguinal region (2.7x1.9cm),normal testes size(4x3cm), bilateral cavernous tissue,respectively. Findings suggested phenotypic female with PAIS. Further investigations could not be carried out due to poor affordability and non-availability of Genetic testing facility. Management: Male gender was preferred (after discussion with urologist and consent of the patient and the family) Assigning the gender, health-related quality of life (QoL), social and psychological well-being, and affective disorders, like fertility and sexual functions in PAIS were discussed. Psychometric data was obtained through psychological questionnaires: Beck Depression Inventory & Hospital Anxiety and Depression Scale revealed moderate depression. An important pre-decision analysis regarding the potential impact of clinical decisions such as the type and timing of genital surgeries on patient’s life is missing due to absence of a multidisciplinary team for counseling and decision making.
Conclusion: After spending 18.yrs as a phenotypic female the patient and her family experienced considerable emotional distress. In our culture and society these types of cases are seldom reported. We as medical professionals need to be sensitive to the social and psychological wellbeing of patients so that they can be settled and acceptable in their part of the world.
SAT-206 A Sweet and Salty Milieu: The Utility of CT in Bilateral Adrenal Hemorrhage
Authors: Tavares Matthew V , Sittol Rani Delraj , Chang Sang-Min , Otuonye Gene , Barnes Tanganyika 
Published: May 2020

Abstract
Introduction: Bilateral adrenal hemorrhage (BAH) is seldomly recognized as a cause of adrenal insufficiency due to nonspecific clinical manifestations within the milieu of major concurrent illnesses. CT abdomen is the investigation of choice to evaluate acute adrenal crisis.
Clinical Case: A 68 year old female with systemic lupus erythematosus, anti-phospholipid antibody syndrome (APS) complicated by multiple strokes, on warfarin, and recent hip fracture presented with confusion, lethargy, fever, and cough. On exam she was febrile, tachycardic, tachypneic, disoriented with right sided hemiparesis, and crepitations on lung auscultation. Lab results were significant for leukocytosis of 14K/uL, hemoglobin (Hb) 9.9g/dL, INR 3.8, azotemia, normal electrolytes, and glucose 67mg/dL. Blood and urine cultures were negative. Anti-dsDNA, C3, and C4 were normal. CT chest showed right lower lobe infiltrates and bronchial debris. She was treated with antibiotics and bronchoscopy with mucus plug removal. Her lethargy and confusion persisted. MRI brain was negative for acute event. Hb fell to 7g/dL; managed conservatively with i.v. iron. CT abdomen showed BAH both measuring 3.5x3.5cm; AM cortisol was 5.5 mcg/dL. Warfarin was reversed with vitamin K; hydrocortisone 50mg q8h was started with improvement in her mental status. ACTH levels were &lt;5pg/ml; ACTH stimulation testing was deferred as steroids were started emergently. Repeat CT showed stable BAH, AM cortisol increased to 19.4mcg/dL, and Hb stable at 8g/dL. She was discharged on hydrocortisone 10mg twice daily.
Discussion: Trauma, corticotropin stimulation during stress, anticoagulation therapy, and APS inducing adrenal venous thrombosis have been identified as risk factors that predispose to BAH. There is a lack of consensus regarding normal cortisol levels in critical illness. Fluctuations in serum cortisol levels are frequent and not necessarily indicative of definite injury to the adrenal gland. CT abdomen, the investigation of choice in the acute setting, has a heterogeneous, high density appearance; the presence of an underlying adrenal mass cannot be excluded. BAH appears as diminished attenuation of the adrenal gland with respect to the adjacent tissues, along with thickening of the ipsilateral crura due to retroperitoneal extension of blood (1). MRI has a greater accuracy for identifying BAH in comparison to CT as it can discern adrenal hematoma from adjacent necrotic tissue and determine the acuity of the hematoma.
Conclusion: The nonspecific clinical manifestations of BAH are frequently inseparable from those of the concurrent major illness. The diagnosis requires a high index of suspicion, initiation of prompt steroid replacement, and confirmation by CT abdomen.
References: (1) Fatima, Z., Tariq, U., Khan, A., et. Al. A Rare Case of Bilateral Adrenal Hemorrhage. Cureus. 2018 Jun; 10(6): e2830. doi: 10.7759/cureus.2830
SAT-127 Localization and Treatment of the Ectopic ACTH Syndrome Using Somatostatin Analogues
Authors: Mago Sheena , Malchoff Carl , Karimeddini David 
Published: May 2020

Abstract
Ectopic ACTH syndrome (EAS) is a rare disorder with a high morbidity and mortality due to the sequelae of severe hypercortisolism and possible underlying carcinoma. It is caused by various tumors, some originating from neuroendocrine cells. Optimal management includes localization and removal of the ectopic ACTH source. About 80% of these tumors have somatostatin (SS) receptors, so that SS analogues may be useful in the localization and treatment. This case demonstrates the use of SS analogues in localization and treatment of EAS.
A 49-year-old female presented with 7 months of worsening Cushingoid features including rounded plethoric face, full supraclavicular fat pads, facial hair growth, multiple bruises, violaceous abdominal striae, kyphoscoliosis, 4+ peripheral edema, weight gain, hypertension (190/110 mmHg) and thoracic compression fractures. AM plasma ACTH concentration was 333 pg/mL (nl = 6 - 58 pg/mL); serum concentration cortisol was 71.4 mcg/dL (nl = 7 - 23 mcg/dL) and serum potassium concentration was 1.2 mmol/L (nl = 3.6 - 5.1 mmol/L). A 24-hour urine cortisol could not accurately be obtained. High dose 8 mg overnight dexamethasone testing demonstrated suppression of serum cortisol concentration from 71.4 to 6.3 mcg/dL, suggesting pituitary Cushing disease. Pituitary MRI scan revealed a 2.5 mm disc shaped cystic focus suggesting a Rathke’s cleft cyst. No central-to-peripheral ACTH gradient was present on bilateral inferior petrosal sinus sampling suggesting an ectopic ACTH source. There was a 1.2 x 1.0 cm nodule in the left lower lung lobe on CT chest. A gallium-68 dotatate PET/CT scan demonstrated enhancement of this same lung lesion and mild uptake in the left inferior hilum, suggesting a neuroendocrine lung tumor with possible metastases to lymph nodes. Biopsy and resection of the lesion was deferred until after control of her hypercortisolism. 30mg of Sandostatin LAR was started every 4 weeks to control the ectopic ACTH secretion and to confirm that the SS analogue positive lung lesion was the cause of the EAS. Sandsostatin LAR therapy over the next 12 weeks resulted in a steady clinical and biochemical improvement with a decrease in serum cortisol to 9.7 mcg/dL, control of hypertension (108/64 mmHg), weight loss of 6 pounds, and resolution of supraclavicular fat pads, hypokalemia, and peripheral edema. This confirmed our impression that the neuroendocrine lung tumor was the ACTH source.
In summary, this patient with EAS had severe clinical manifestations of hypercortisolism and could not safely undergo surgical intervention. This case demonstrates the value of SS analogues (gallium-68 dotatate PET/CT and Sandostatin LAR) in localizing and confirming the source of ectopic ACTH production and markedly improving the clinical and biochemical features of the EAS.
SUN-300 Improvement in Cardiovascular Risk Factors in Long Term Follow up of Hypopituitary Septagenarian and Octagenarian Patients
Authors: Rosa Isabella Naves , Soares Alexandre Anderson S M , Naves Luciana Ansaneli 
Published: May 2020

Abstract
Background: Hypopituitarism in the elderly population is an underdiagnosed condition and may increase co-morbidities in glucose metabolism, dyslipidemia and cardiovascular risk factors. Pituitary macroadenomas are benign tumors, which prevalence is unknown in aged people, and is frequently associated to impairment in pituitary function. Objective: The aim of this study is to identify cardiovascular risk factors in hypopituitary septagenarians and octagenarians by diagnosis and after long term follow up of pituitary dysfunction. Methods: This is a retrospective observational study and the patients were recruited and selected from a service registry in a tertiary medical center. We included patients aged from 70-99 years with the diagnosis of pituitary macroadenomas, evaluated hormonal and biochemical parameters, cardiovascular risk scores were calculated by diagnosis and compared after long term follow up. All patients signed informed consent. Results: Thirty five patients were included, 21 patients aged 70-75 years (72.61 yo), 7 patients 76-80 years (77.28yo), 7 patients 81-99 years (89.28 yo). All tumors were macroadenomas, 40% of them Non Functioning Pituitary Macroadenomas, mean maximal diameter 3.4 cm (2.9-.4.3), 40% of them submitted to surgery, 14% adjuvant radiotherapy, 20% presented previous apoplexy. Co-morbidities were frequent by diagnosis, 85.71% presented Hypertension, 37.14% Diabetes, 62.8% Hypercholesterolemia and 45.71% Hypertriglyceridemia. Hypopituitarism was present in 71.42%, GH deficiency in 37.14%, hypogonadism in 60%, central hypothyroidism in 54.28%, adrenal insufficiency in 31.42%, 51.42 % presented more than two combined deficiencies. Analysis of cardiovascular risk prediction in total cohort showed, 57.14% of patients presented reduction of Framingham Score and 45.71% in Coronary Calcium Score, during mean time follow up of 13.09 years (3-32 years after diagnosis). According to ages, Framingham score and Coronary Calcium Score reduced respectively in 66% and 33.3% (70-75 yo), 57.15% and 85.71% (76-80 yo) and 42.85% and 28.57% (81-99 yo), during long term follow up. Discussion and Conclusion: In this study, most of hypopituitary aged patients presented reduction of cardiovascular risk factors during long term treatment and follow up, despite replacement with corticosteroids and gonadal steroids. Considering the importance of early diagnosis and the lack of data observed in the medical literature, larger scale studies should be performed with the objective to assess of the risk benefit ratio of hormonal replacement in metabolic control in septagenarian and octogenarian patients.
SAT-666 Possible Amelioration of Diabetic State After Intravesical BCG Instillations for Bladder Cancer
Authors: Havranova Jana , Gallagher Thomas , Arastu Mohammad Ishaq 
Published: May 2020

Abstract
Introduction - Current research provides strong evidence that intradermal Bacillus Calmette-Guerin (BCG) vaccination improves glucose metabolism by a mechanism of switching oxidative phosphorylation to aerobic glycolysis inducing a state of high glucose utilization. We present a case that demonstrates improvement in blood sugar levels upon administration of a series of intravesical BCG instillations. There is no research, in current literature, correlating blood sugars lowering after intravesical administration of BCG vaccines.
Case Description - An 81 years old male with a medical history of type 2 diabetes mellitus, coronary artery disease, and bladder cancer underwent a series of BCG instillations, as therapy for his bladder cancer. He underwent BCG treatments according the Southwest oncology protocol for bladder cancer. During his BCG treatments, the patient was on Humalog 75/25, 30 units twice a day. Upon completion of his treatments, the patient experienced an episode of hypoglycemia that required hospitalization. His insulin regimen was decreased from 30 to 25 units BID. His A1C at the time of diagnosis was 8.7% and after insulin adjustments declined to 8.1%. We are reviewing a list of type II diabetic patients with bladder cancer who were treated with BCG and analyzing blood sugars before and after BCG exposure. We hypothesize that BCG vaccination precipitated the improvement in his blood sugars.
Conclusion - Additional studies on BCG vaccinated, diabetic, bladder cancer patients are needed for confirmation of this effect. Our case illustrates a probable reduction in blood sugars attributable to his BCG vaccinations. Research suggests that intradermal BCG switches cellular metabolism from oxidative phosphorylation to early aerobic glycolysis. Another effect of BCG is the increasing levels of tumor necrosis factor (TNF) that leads to elimination of self-reactive white blood cells and inducing regulatory T cells. More research is needed to confirm whether intravesical administration of BCG has the same effect as intradermal BCG vaccination, which would have a potential impact on the clinical management of both type 1 and type 2 diabetic patients.
SUN-027 Testicular Regression Syndrome, an Underdiagnosed Cause of Hypergonadotropic Hypogonadism
Authors: Canales-Ramos Nicolle M , Burgos-Ortega Nydia I , Grana-Santini Alberto J , Colon-Castellano Janet M , Gracia-Ramis Alvaro , Nieves-Rivera Francisco , Alvarado Milliette , Gonzalez-Rodriguez Loida A , Ramirez-Vick Margarita 
Published: May 2020

Abstract
Background: Testicular regression syndrome (TRS), also known as vanishing testes syndrome, is a condition of 46, XY males presenting with male phenotypic genitalia and testicular absence. Normal testes are thought to have once existed in fetal life and subsequently atrophied following a catastrophic event. It may present as partial or complete absence of testicular tissue. Fibrotic remnants are commonly found upon surgical exploration.
Case: This is the case of a 20-year-old male with a birth-diagnosis of primary hypogonadism. The patient was born prematurely at 5 months following a motor vehicle accident that resulted in his mother’s death. Neonatal evaluation for cryptorchidism revealed no radiological evidence of intra-abdominal testicular mass. Referral to a pediatric-endocrinologist was done at early childhood. Testosterone replacement was started at 12 years of age and pubarche was adequate. After surgical exploration, a testicular remnant was surgically removed. Pathology report revealed fibrotic tissue, yet no histological testicular tissue was found. Due to lack of testes in the scrotum, testicular prosthesis was implanted at age 14, but the etiology of the hypogonadism was never elucidated. The patient was initially seen in our adult-endocrinology clinics at age 21. Upon evaluation, secondary sex characteristics were adequate for age. Penile length was of 3.5 inches. Bilateral testicular prostheses were palpable, and no gynecomastia was present. Despite testosterone replacement, total testosterone was 3.38 ng/ml (n; 2.41-8.27 ng/ml), FSH was 106 mIU/ml (n; 1.4-18.1 mIU/ml) and LH was 34.7 mIU/ml ml (n; 1.5-9.3 miu/ml). Based on history, laboratory workup and surgical pathology, TRS was presumed as the etiology of this patient’s hypogonadism. Testosterone therapy was adjusted to reach physiological expected levels.
Conclusion: TRS is an underdiagnosed cause of hypergonadotropic hypogonadism. It is supposed that the initial embryologic development was normal, followed by a vascular accident. There is no clear recommended approach for evaluating a patient once the diagnosis of TRS is suspected. Diagnostic methods may include hormonal and karyotype testing, imaging, and surgical exploration. Furthermore, although clinical recommendations have been published for other disorders of sex development, TRS management has been understudied. Testosterone replacement therapy at the typical time of puberty ensures the development of secondary sexual characteristics, bone health and pubertal growth. Unlike Klinefelter syndrome and cryptorchidism, TRS has not been associated with increased risk of cancer. Thus, it is important to establish the etiology to avoid invasive and unnecessary workup, which will result in psychological distress and excessive healthcare costs.
MON-028 Chronic Resveratrol Exposure Improves Glucose Homeostasis and Cardiac Function in a Rat Model of Polycystic Ovarian Syndrome
Authors: Molina Rogelio Enrique , Shao Tiffany , Randhawa Harmanprit , Hoang Vanessa , Vyas Arpita Kalla 
Published: May 2020

Abstract
Polycystic ovary syndrome (PCOS) is the commonest endocrinopathy in women of reproductive age, with a prevalence of 5-8%. Long-term complications seen in PCOS include cardiovascular disease and type 2 Diabetes Mellitus. Current therapies do not completely address the cardiometabolic perturbations seen in women with PCOS. Resveratrol (RSV), a natural polyphenol, is shown to have beneficial cardio-metabolic effects in various pathological conditions including that on insulin sensitivity, cardiovascular function. In-vitro studies suggest it’s beneficial effects on ovarian function as well. Therefore, we hypothesized that chronic exposure to RSV would improve both cardiovascular and metabolic phenotypes in PCOS. To test this hypothesis we used an established rat model of PCOS that develops metabolic derangement and irregular cycles. A 7.5 mg (90-day release) dihydrotestosterone (DHT) pellet providing a daily dose of 83 mcg was implanted in 5-week-old female rats. Studies were also conducted on littermate matched controls (C) with no DHT implant. A subgroup of the control and DHT treated rats (n=6 per group) received a 0.84 g/kg dose of resveratrol (RSV) in their chow starting at age 5 weeks. At 8 weeks, animals were weighed weekly (n=6 per group). Oral glucose tolerance test (OGTT n=6 per group) and cardiac echocardiogram (C n=12, C+RSV n=6, DHT n=10, DHT+RSV n=6) were conducted at 16-weeks of age. Body weight increased significantly in DHT treated rats compared to C between 8 and 16 weeks (40 vs 22 grams, p &lt;0.001). RSV treatment did not mitigate the effects of DHT on body weight (34 vs 40 grams, p&gt;0.5). There was significantly higher glucose excursion at 30 minutes post glucose load in both DHT (148± 7.4 mg/dl) and DHT+RSV (139± 7.4 mg/dl) compared to C group (121± 13 mg/dl, p&lt;0.001, p=0.03 respectively). However, by 60 and 90 minutes only DHT group had a significantly higher glucose excursion compared to both DHT+RSV and C groups (131± 4.1,124± 5.7,110 ± 5.9 mg/dl, p=0.015,p=0.21 respectively); 90min (118±5.8,110±4.7,96±4.2 mg/dl, p&lt;0.01,p=0.09 respectively). By 120 minutes, no significant difference in glucose levels existed between groups. Cardiac echocardiogram showed significantly lower mitral valve E/A ratio (and increased MV isovolumic relaxation time (IVRT) in DHT group compared to C. RSV treatment reversed these changes. In conclusion, RSV improved glucose homeostasis and diastolic dysfunction in the DHT induced rodent model of PCOS and may serve as a novel treatment option targeting the cardiometabolic derangement seen in PCOS. Further studies elucidating the mechanisms underlying the beneficial effects of RSV on cardio-metabolic phenotype in this PCOS rodent model is warranted.
MON-652 The Prevalence and Clinical Characteristics of Adult Patients Presenting with Sodium-Glucose Co-Transporter-2 Inhibitor Associated Euglycemic Diabetic Ketoacidosis
Authors: Clark Alexa , Mohammed Arifuddin Saad , Raut Amol , Moore Sarah , Awad Sara 
Published: May 2020

Abstract
Background: Sodium-Glucose Co-Transporter-2 Inhibitors (SGLT-2i) have been associated with euglycemic diabetic ketoacidosis (EuDKA). The pathophysiology is related to decrease insulin secretion given glucosuria and lower insulin to glucagon ratio, resulting in enhanced lipolysis and ketogenesis at a lower glucose level. SGLT-2i may unmask underlying undiagnosed type-1 diabetes/Latent Autoimmune Diabetes of the Adult (LADA).
Objective: (1)To describe the frequency and clinical characteristics of SGLT-2i associated EuDKA at a Canadian tertiary care centre. (2)To identify the most common underlying diabetes-type associated with EuDKA.
Methods: A chart review identified patients with SGLT-2i-associated EuDKA from June 2015 to May 2019 who presented to a Canadian tertiary care centre. Clinical characteristics include age, gender, diabetes type, SGLT-2i drug prescribed, laboratory results at the time of EuDKA and possible precipitants were reviewed. Pancreatic autoantibodies (anti-GAD-65, anti-islet cell, IA-2, and insulin antibodies) were measured to screen for the possibility of undiagnosed LADA.
Results: Between June 2015 to May 2019, there were 647 DKA events of which 43 were related to SGLT-2i (Prevalence 6.64%). Canagliflozin was the most common SGLT-2i (53.5%) followed by Empagliflozin (34.9%). The most common precipitant was infection (16.3%), followed by surgery (14%). At presentation, average blood glucose was 14.95±12.51 mmol/L, pH 7.23±0.16, HCO3 12.86 ± 5.51 mmol/L, potassium 4.40±0.86 mmol/L and anion-gap 22.05±5.51 mmol/L. Average HbA1c was 9.2%±2.10 and BMI was 29.34±4.49. Twenty patients had pancreatic autoantibodies testing and seven were positive (35%), most commonly anti-GAD-65 (71.4%). As a result, seven patients were diagnosed with LADA who were previously diagnosed with type-2 diabetes. Out of the 7 patients with LADA, 2 had a positive family history of type-1 diabetes.
Conclusion: SGLT-2i associated EuDKA could unmask underlying LADA. Further studies are warranted to determine if routine pancreatic antibodies should be drawn for diabetes typing prior to prescribing or at presentation of SGLT-2i associated EuDKA.