Clinical and Investigative Endocrinology and Diabetes

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Clinical and Investigative Endocrinology and Diabetes APRIL 2017 VOLUME 23 SUPPLEMENT 3 Clinical and Investigative Endocrinology and Diabetes 2017 AACE ANNUAL SCIENTIFIC & CLINICAL CONGRESS ABSTRACTS ABSTRACT CATEGORIES 1 Adrenal Disorders 121 Obesity 29 Diabetes Mellitus/Prediabetes 131 Other 83 Hypoglycemia 161 Pituitary Disorders/Neuroendocrinology 95 Lipid/Cardiovascular Disorders/Hypertension 227 Reproductive Endocrinology 99 Metabolic Bone Disease 247 Thyroid Disease OFFICIAL JOURNAL OF THE AMERICAN ASSOCIATION OF CLINICAL ENDOCRINOLOGISTS AND THE AMERICAN COLLEGE OF ENDOCRINOLOGY • WWW.AACE.COM ABSTRACTS Table of Contents Adrenal Disorders . 1 Diabetes Mellitus/Prediabetes . 29 Hypoglycemia . 83 Lipid/Cardiovascular Disorders/Hypertension . 95 Metabolic Bone Disease . 99 Obesity . 121 Other . 131 Pituitary Disorders/Neuroendocrinology . .161 Reproductive Endocrinology . 191 Thyroid Disease . 209 Author Index . 263 Notes . 277 ABSTRACTS ADRENAL DISORDERS the presence of gonads. Primary or ACTH-independent bilateral macronodular adrenal hyperplasia (BMAH) is Abstract #100 usually associated with Cushing’s syndrome and suppressed ACTH. In CAH on the other hand, steroidogenesis is ACTH AUTONOMOUS MASSIVE MACRONODULAR mediated and levels are usually elevated >70. Our patient had ADRENALS IN THE SETTING OF CONGENITAL an ACTH in the normal range indicating that the adrenals ADRENAL HYPERPLASIA seemed to have become partially autonomous. The presence of long-term untreated CAH caused the adrenals over time to Joyce George, MD, Betul Hatipoglu, MD, Adi Mehta, MD become autonomous and escape the control of ACTH. Conclusion: A very interesting case depicting the co- Cleveland Clinic Foundation occurrence of BMAH and CAH. BMAH is one situation where one should check a 17-OHP level. Case Presentation: A 74 year old male presented to our Endocrinology clinic for workup of incidentally found Abstract #101 adrenal enlargement and nodules on CT. Both adrenals showed diffuse nodular thickening and measured 7.7 cm A CURIOUS CASE OF SPINAL EPIDURAL on greatest dimension on the left and 6.5 cm on the right. LIPOMATOSIS (SEL): A SIGN INDICATING The left adrenal had a 4.3 x 2.4 cm nodule (38 HU; 75% CUSHING’S SYNDROME washout) and right adrenal had a 2.5 x 1.9 cm nodule (38 HU; 67% washout). On history patient stated that he was born Shilpi Singh, MD, MPH, Abbey Reed, MD, Mohsen Zena, MD with a birth defect (hypospadiasis and undescended testis bilaterally). Also, at age 12 during an appendectomy, a benign Creighton University Medical Center tumor was removed from his pelvis (no records available). Patient claims normal puberty, but he did stop growing early Objective: Recognizing spinal epidural lipomatosis (SEL) as after being the tallest kid in his class briefly. Patient had a one of the signs of glucocorticoid excess. male phenotype with male pattern hair growth, height 4’ 11” SEL is accumulation of excessive fat within the spinal epidural and a normal physical except for micropenis, hypospadiasis space, most commonly in the lumbar region, compressing and absent testis bilaterally. Hormonal workup showed a the thecal sac, resulting in compressive symptoms. The most 17-hydroxyprogesterone (17-OHP) level >50,000 (40-180 common cause being excess glucocorticoids either exogenous ng/dl), DHEAS 150 (10-204 mcg/dl), testosterone 121 (220- or endogenous (Cushing’s syndrome). Also seen in morbid- 1000 ng/dl), FSH 102 (1-10 mU/ml), LH 55 (1-7 mU/ml), obesity, while some are idiopathic. Clinical presentation is Androstenedione 5.3 (0.7-2.2 ng/ml), 11-deoxycortisol 16 usually non-specific, varying from completely asymptomatic (<49 ng/dl), 11-deoxycorticosterone <5, ACTH 38 (8-42 pg/ to pain with radicular symptoms and weakness in lower ml). Patient had normal plasma metanephrines, aldosterone, extremities. renin, salivary cortisol and normal suppression of cortisol with Case Presentation: We report a 74-year old Nepalese women dexamethasone. After administration of 1 mg dexamethasone, who had MRI-Lumbar Spine for evaluation of chronic 17-OHP dropped to 2,471 and androstenedione to 2.2. On back pain which showed epidural lipomatosis. Since spinal ACTH stimulation test for adrenal insufficiency, the patient epidural lipomatosis is seen in glucocorticoid excess, a 1 had a sub-optimal response of 9.8, 9.9, 12.1 (baseline, 30, mg overnight dexamethasone suppression test (ODST) was 60 min). US revealed no testis in the scrotum or inguinal performed. Early morning cortisol level was 16.8 ug/dl and area. Review of CT images showed no gonads in the pelvis. Dexamethasone level was 792 ng/dl (Reference range <50 Estradiol was 27 (10-60 pg/ml), inhibin <10 pg/ml, AMH ng/dl). Cut off value of 1.8 ug/dl for AM cortisol was used <0.003 (2-30 ng/mL). Chromosome analysis revealed XX. which indicated cortisol excess. Three consecutive midnight Discussion: Untreated CAH patients tend to have adrenal salivary cortisol, and Abdomen/pelvis CT with adrenal masses but enlargement to the extent depicted here is unusual. protocol were ordered to confirm the diagnosis and asses the It is also interesting to note that this patient’s adrenals were adrenal glands. able to generate sufficient androgens for the patient to undergo Discussion: The mechanism of epidural lipomatosis caused a precocious puberty and maintain a male phenotype without by glucocorticoid-excess is not exactly known. Epidural - 1 - ABSTRACTS – Adrenal Disorders lipomatosis should raise the suspicion for hypercortisolism elevated. Finally, patient underwent uneventful resection and patients with hypercortisolism who have back pain of PGL with fast recovery. Follow up biochemical testing should be evaluated for epidural lipomatosis. Careful was normal. MIBG scan showed no residual uptake and neurologic examination of patients with Cushing’s genetic testing was negative. syndrome should be performed and should be evaluated Discussion: Manipulation of PGL, biopsy or resection, with spinal MRI if neurological signs and symptoms are presents a unique challenge due to inherent risks present. In most cases, no specific treatment is required, associated with excess catecholamine release and variable although causes of endogenous steroid excess and anatomic presentation. RT is often used for malignant exogenous steroid use should be assessed. In patients with or metastatic lesions. Current guidelines do not support severe symptoms including neurologic deficits, operative RT for treatment of benign PGL. RT results in decreased decompression is recommended. catecholamine production and reduced tumor burden Conclusion: Spinal Epidural Lipomatosis (SEL) seen on without substantial radiological changes. Typical dose used MRI-Spine should raise the suspicion for glucocorticoid is 45 Gy. Despite these effects, RT alone does not suffice excess and prompt further evaluation. in terms of functional control. Our patient had adrenergic crisis in spite of adequate preoperative preparation. RT Abstract #102 dose used in this benign PGL was lower than suggested in literature, yet RT led to tumor necrosis while tumor size EXTERNAL BEAM RADIATION THERAPY AS AN was unchanged. Subsequent surgical resection resulted in ADJUNCTIVE FOR BENIGN PARAGANGLIOMA a favorable outcome. Conclusion: This case highlights the importance of Mohini Bollineni, MBBS, Barbara Mols-Kowalczewski, multidisciplinary approach and use of multiple modalities MD, Gennady Bratslavsky, MD, Ruban Dhaliwal, MD for successful treatment of even benign PGL. SUNY Upstate Abstract #103 Objective: Paragangliomas (PGL) are catecholamine- ADRENAL INSUFFICIENCY AS A HARBINGER secreting extra-adrenal tumors. Management of even OF WORSENING POEMS SYNDROME nonfunctional PGL needs meticulous preparation and a multidisciplinary approach. Radiotherapy (RT) is reserved Meghan Gaule, MD, Whitney Goldner, MD for unresectable, malignant or metastatic lesions. We present a case of benign aortocaval PGL that was clinically University of Nebraska Medical Center silent, yet the definitive treatment proved quite challenging. Case Presentation: 71-year-old male with uncontrolled Objective: POEMS syndrome is a rare paraneoplastic hypertension presented with lumbar and leg pain. CT disorder characterized by polyneuropathy, organomegaly, abdomen revealed a 6.6 cm aortocaval mass. Biopsy endocrinopathy, M protein, and skin changes. was consistent with extra-adrenal pheochromocytoma. Endocrinopathy is a minor criteria for diagnosis of The procedure was uneventful. Biochemical testing POEMS. POEMS syndrome itself is quite rare, and done only after the biopsy showed elevated plasma adrenal insufficiency as an endocrinopathy is also metanephrines 715 (0-62pg/ml), normetanephrines 1615 uncommon. We report a case of simultaneous presentation (0-145 pg/ml), and norepinephrine 1335 (0-874 pg/ml). of adrenal insufficiency and primary hypothyroidism in a MIBG scan showed avid uptake in the right suprarenal patient with Castleman’s Disease and POEMS years after fossa and no distant metastasis. In addition to PGL, a development of other endocrinopathies. We propose that 1.5 cm lung lesion was identified on PET-CT. Biopsy of adrenal insufficiency and primary hypothyroidism were this lesion revealed primary adenocarcinoma. Despite the markers of worsening of POEMS syndrome. preoperative medical optimization over 3 weeks with Case Presentation: The patient was 48 years old when prazosin, bisoprolol and metyrosine, intraoperatively he was diagnosed with Castleman’s and POEMS. patient had extremely labile blood pressure necessitating He was treated with cyclophosphamide,
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