bluuebunny/crossref_metadata_2025_split huggingface dataset:
MON-LB010 Cyclic Progesterone Therapy for Androgenic Polycystic Ovary Syndrome (PCOS) - A Systematic Review of the Literature
Authors: Shirin Sonia , Goshtasebi Azita , Kalidasan Dharani , Prior Jerilynn C 
Published: May 2020

Abstract
Women living with androgenic PCOS (WLWP) experience unpredictable oligomenorrhea1 and are at increased risk for endometrial cancer2. Oral micronized progesterone (OMP) given cyclically (14 days/cycle or 4 weeks, Cyclic OMP), in luteal phase doses3 (300 mg at bedtime) as a “luteal phase replacement” therapy would be likely to effectively treat both. In addition, evidence suggests PCOS is causally related to rapid pulsing of GnRH and LH 4; OMP normalizes LH pulsatility if androgen levels are not elevated 4. Previous searches did not find progesterone therapy for PCOS 5. Our research question: Does the peer-reviewed literature provide evidence for prescribing cyclic progesterone therapy in PCOS? Literature search methods used Medline (Ovid) and PubMed for published articles. Our search terms were: “polycystic ovary syndrome”, “androgenic PCOS”, and, “micronized progesterone.” We sought publications with eligible women participants having androgenic PCOS, drug exposures (cyclic
OMP, vaginal progesterone, and in varying doses and durations) and specific outcomes (biochemical or patient-reported data or both) in all languages. We excluded reviews and practice guidelines but searched bibliographies for missed citations. Results discovered 18 articles in combined Medline (n=6) and PubMed (12) searches. After excluding duplicates, articles on estradiol (E2) alone E2 with OMP therapy, five eligible articles remained. We read all in full detail.
Progesterone therapy was beneficial for WLWP as, even in sub-therapeutic doses (&lt;300 mg at bedtime) and in cycles of too short durations (&lt;14 days), it decreased luteinizing hormone (LH) 6,7 and total testosterone 7 levels. Vaginal progesterone (200 mg, b.i.d for 2 to 12 weeks) added to letrozole ovulation induction increased the pregnancy rate from 0 to 21% 8. Although present data suggest Cyclic OMP withdrawal predictively causes flow, we found no evidence it improved women’s cycle-related experiences nor decreased acne and hirsutism. Women-reported data on Cyclic OMP for improving androgenic PCOS cycle regularity, daily experiences and risks for endometrial cancer are needed.
Reference: 1Azziz R Nat Rev Dis Primers 2016;2:16057. 2Barry J Hum Reprod Update 2014; 20:748. 3Simon J Fertil Steril 1993;60:26. 4Blank S Hum Reprod Update 2006;12:351. 5Teede H Clin Endocrinol (Oxf) 2018;89:251. 6Livadas S Fertil Steril 2010;94:242. 7Bagis T J Clin Endocr Met 2002;87:4536. 8Montville C Fertil Steril. 2010;94:678.
OR18-05 Thyroid Hormone Use and Survival among Older Adults - Longitudinal Analysis of the Baltimore Longitudinal Study of Aging (BLSA)
Authors: Abbey Enoch Joseph , Simonsick Eleanor M , McGready John , Mammen Jennifer Sophie 
Published: May 2020

Abstract
Abstract: Introduction: Thyrotropin (TSH) levels on average are higher and vary more widely among older adults.1 Large meta-analyses and treatment trials for isolated elevated TSH in older adults did not demonstrate harm from no treatment or benefit from treatment in this population.2 Isolated, elevated TSH can result from adaptations to aging, rather than primary thyroid disease, suggesting that thyroid hormone treatment could actually cause harm.3
Objective:To determine if there is a survival effect from thyroid hormone treatment in adults aged 65+.
Methodology:Thyroid functional status and thyroid hormone exposure were analyzed for 1,258 participants of the BLSA aged 65+ through death or end of follow up. We analyzed exposures by visit and also compared survival between individuals with consistently elevated, euthyroid or low TSH both on and off of therapy.Incident rate ratios (IRR) were calculated using time-dependent Poisson regression models. Covariates included age, sex, race, walking index (measure of physical function), self-rated health (SF-12), body mass index (BMI), smoking and comorbidity score.
Results: Average follow-up was 9 years, with 169 deaths over the study period. The cohort comprised 49.5% women, with average age in the study being 78 years (SD ±8.2). Thyroid hormone use trended towards harm analyzed at each visit with an IRR=1.40 (95% CI 0.93–2.12) after adjusting for other covariates. Among ‘treated-to-target’versus euthyroid individuals, thyroid hormone use was associated with a significantly increased mortality rate with an IRR=1.80 (95% CI 1.09–2.96) in multivariable analysis.
Conclusion: Thyroid hormone replacement among older adults,even when treated-to-target, is associated with a significantly increased mortality risk compared to euthyroid individuals with no history of thyroid hormone exposure. This suggests that treating isolated elevated TSH when changes are aging adaptations rather than primary thyroid disease could adversely affect health by altering key homeostatic adaptation. We recommend clinicians consider the underlying physiology of aging and employ age specific reference ranges when deciding on treatment for elevated TSH in older adults.4References 1. Surks et al., J Clin Endo. Met. 2007;92(12):4575–4582. 2. Stott et al., NEJM. 2017;376(26):2534–2544. 3. Mammen et al., Thyroid. 2017;27(11):1370–1377. 4. Surks et al., J Clin Endo. Met. 2010:95(2):496-502Unless otherwise noted, all abstracts presented at ENDO are embargoed until the date and time of presentation. For oral presentations, the abstracts are embargoed until the session begins. Abstracts presented at a news conference are embargoed until the date and time of the news conference. The Endocrine Society reserves the right to lift the embargo on specific abstracts that are selected for promotion prior to or during ENDO.
MON-296 Craniopharyngiomas Presenting as Incidentalomas: Results of Kraniopharyngeom 2007
Authors: Boekhoff Svenja , Bison Brigitte , Eveslage Maria , Sowithayasakul Panjarat , Muller Hermann Lothar 
Published: May 2020

Abstract
Objective: Childhood-onset craniopharyngiomas (CP) are diagnosed due to clinical symptoms (symCP) or incidentally (incCP). We investigated clinical manifestations and outcome in incCPs and symCPs.
Design and methods: IncCP were discovered in 4 (3m/1f) and symCP in 214 CP (101m/113f) recruited 2007–2014 in KRANIOPHARYNGEOM 2007. Age, sex, height, body mass index (BMI), tumor size, degree of resection, pre- and postsurgical hypothalamic involvement/lesions, pituitary function and outcome were compared between both subgroups.
Results: Reasons for imaging in incCP were cerebral palsy, head trauma, nasal obstruction, and tethered-cord syndrome, whereas headache (44%) visual impairment (25%), and growth retardation (17%) lead to imaging in symCP. Tumor size at diagnosis was smaller in incCP (median tumour size 3.26 cm2; range: 0.56–5.13 cm2) when compared with symCP (median tumour size 12.16 cm2; range: 0.004–79.54 cm2). Age, gender, BMI, height, hydrocephalus, tumor location, and hypothalamic involvement at diagnosis of incCP were within the range of these parameters in symCP. Complete resections were achieved more frequently (3/4 patients) in incCP when compared with symCP (20%). Surgical hypothalamic lesions were distributed similar in incCP and symCP. Irradiation was performed only in symCP (33%). No noticeable differences were observed concerning survival rates, endocrine deficiencies, BMI, height, functional capacity and quality of life of the 4 incCP cases when compared with the symCP cohort.
Conclusions: IncCP are rare (1.8%) and characterized by lack of endocrine deficiencies, resulting in normal height and BMI, no hydrocephalus, and smaller tumor size at diagnosis when compared with symCPs. Outcome of the observed incCP is similar with symCP.
SUN-904 Ga68 Dotatate Detects Ectopic ACTH Secreting Atypical Carcinoid Tumor
Authors: Simons Zachary Brent , Wangsiricharoen Sintawat , Gelwan Elise , Lilja Solnes Bjork , Santhanam Prasanna 
Published: May 2020

Abstract
Title Ga68 DOTATATE detects Ectopic ACTH secreting Atypical Carcinoid tumor Introduction Ga-68 DOTATATE PET/CT has been shown to have a significant impact in localizing Ectopic ACTH secreting tumors especially those that are not identified on conventional imaging (CT, MRI). We present a person with a tumor that was reported benign (on biopsy) elsewhere but was found to be the source of the Cushings syndrome and was localized on Ga-68 DOTATATE PET/CT, pathology confirming atypical carcinoid tumor after surgical excision. Discussion She was a 63-year-old who presented with a severe proximal myopathy, high urinary free cortisol- 911.6 (µg/day) (&lt;45), elevated ACTH 104(&lt;63), and negative Inferior Petrosal Sinus Sampling. The CT scan demonstrated a small nodule at the right lung base. Axial Ga-68 DOTATATE PET images demonstrated radiotracer activity in the lesion, SUV max of 7.15. Fused Coronal Images showed a 1.9 cm radiotracer avid nodule, that was detected in the medial segment of the right middle lobe. The patient underwent a pulmonary wedge resection. H and E stain of the tumor -Immunohistochemical stains demonstrated that the tumor was strongly positive for synaptophysin and INSM-1 (markers of neuroendocrine differentiation) and the mitotic count was up to 2–3 mitoses per 10 high-power fields, supporting diagnosis as an atypical carcinoid tumor. The patient had significant improvement of symptoms post excision. Somatostatin Receptor (SSR) based tracer Ga-68 DOTATATE PET/CT has a reported sensitivity for detection of 50 % in occult neuroendocrine tumors (NET), with an overall sensitivity of 64 % in a systematic review. It has been shown in retrospective reviews to help with clinical management both at initial diagnosis as well as follow up of Ectopic Cushings in approximately 65% of the cases. SSR analogue tracers like DOTATATE have been noted to be slightly better than FDG PET/CT at localizing ACTH producing bronchial carcinoids while small-cell lung cancers and other aggressive tumors are better visualized on FDG PET. The favorable aspect of Ga-68 DOTATATE includes great tumor to background ratio and the half-life of Gallium 68 that is suitable for transport and delivery. Metastatic NET in conjunction with ectopic Cushing syndrome can be also be treated with peptide receptor radionuclide therapy using either 90Y-DOTATOC and 177Lu-DOTATATE (both SSR based) resulting in improved clinical outcomes.
MON-291 Postoperative Quality of Life in Children and Adolescents with Craniopharyngioma - Results of the Prospective Multicenter Trial Kraniopharyngeom 2007
Authors: Eveslage Maria , Calaminus Gabriele , Warmuth-Metz Monika , Kortmann Rolf-Dieter , Pohl Fabian , Timmermann Beate , Schuhmann Martin U , Flitsch Joerg , Faldum Andreas , Muller Hermann Lothar 
Published: May 2020

Abstract
Background: Craniopharyngioma is an embryonic tumor of low-grade malignancy. Children and adolescents with this diagnosis are analyzed concerning quality of life (QoL) and (progression-free) survival within the project KRANIOPHARYNGEOM 2007.
Methods: The prospective, multi-center project consists of a randomized, unblinded substudy with adaptive design and an observational study. The randomized substudy for incompletely resected patients compares direct postsurgical radiation with radiation at progression. Endpoint is self-assessment of QoL measured by PEDQOL. In explorative analyses, the influence of additional factors was analyzed using linear mixed models.
Results: In the interim analysis of the randomized substudy according to the intention-to-treat approach only marginal differences concerning QoL between the two treatment groups were observed (n=24). The explorative analyses (n=131) show that ant-/ and posterior preoperative hypothalamic involvement and postoperative hypothalamic lesions are associated with decreased QoL. After complete resection, the QoL is lower than with incomplete resection. Radiation, which is often performed due to progression after incomplete resection, is associated with reduced quality of life.
Conclusion: In order to achieve best QoL for children and adolescents with craniopharyngioma, hypothalamus-sparing therapeutic approaches are recommended. Based on the current data, it is not possible to recommend the optimal time for radiotherapy after incomplete resection with regard to QoL.
SAT-362 A Bone to Pick with FGF23
Authors: Soliman Peter , Kamel Dina , Lopresti Jonathan S 
Published: May 2020

Abstract
A 48 yo M with a past medical history of bilateral femoral neck stress fractures at age 44 status post closed reduction percutaneous pinning (CRPP) presented to the emergency room with bilateral lower extremity pain after a ground level fall. Plain films were notable for a right intertrochanteric fracture and left hip nonunion. The patient underwent removal of right CRPP hardware and revision fixation with a short TFN-Advanced Femoral Nailing System (TFNA) and fixation of the left femoral neck nonunion. Given his injuries following a ground level fall, endocrinology was consulted for recommendations. Labs during his hospital course were notable for hypophosphatemia as well as normal levels of alkaline phosphatase, serum calcium, and PTH. Serum protein electrophoresis and urine protein electrophoresis were within normal limits. A 24-hour urine calcium and phosphorous were done and the patient was found to have a low calculated renal phosphate threshold (TmP04/GFR), concerning for hypophosphatemic osteomalacia. An FGF23 level was drawn and was elevated, suggestive of an FGF23-induced hypophosphatemic osteomalacia. A low vitamin D 1,25-OH was also found consistent with this process, given interference of alpha-hydroxylation of vitamin D 25-OH from FGF23. The patient therefore underwent a PET scan to rule out a mesenchymal tumor-induced osteomalacia but his PET scan was largely unremarkable. Given that there were no issues with bone development or fractures during childhood, the suspicion for X-linked hypophosphatemia or autosomal recessive hypophosphatemia was low. The patient was believed to have either a small mesenchymal tumor not seen on PET scan or an autosomal dominant hypophosphatemia, which can manifest clinically during adulthood, and so was treated with daily Calcitriol and phosphate with subsequent correction of his serum phosphorous. Such a delayed presentation of hereditary hypophosphatemic osteomalacia, however, has been reported only in women either soon after puberty, during pregnancy, or after delivery (1). This case thus represents a possible autosomal dominant hypophosphatemia in a male patient or a rare case of tumor-induced hypophosphatemic osteomalacia.
Reference: (1) Drezner MK, Whyte MP. Heritable renal phosphate wasting disorders. Genetics of bone biology and skeletal disease, 2nd ed. 2018; 761–782.
SUN-503 Thyroid Storm Followed by ATD Induced Agranulocytosis in Late Pregnancy: A Management Dilemma
Authors: Aljehani Faisal , Mushref Malek , Sora Nicoleta Dorinela , Javid Mahsa , Karanchi Harsha 
Published: May 2020

Abstract
Background:
Management of Graves’ disease (GD) in pregnancy presents challenges. Thionamide Antithyroid drugs are the treatment for GD hyperthyroidism with goal of achieving mild but persistent hyperthyroidism and avoiding over-treatment in pregnancy. ATD Induced Agranulocytosis is a rare but serous side effect and presents management dilemmas.
Clinical Case:
A 37-year-old woman with history of Graves’ disease was admitted to our hospital at gestational age of 34 weeks with fever, palpitations and diarrhea. Burch-Wartofsky Point Scale was 35 concerning for impending thyroid storm. She had been diagnosed with hyperthyroidism 6 weeks into her pregnancy, initially treated with PTU which was then changed to methimazole in 2nd trimester. A work up for infection and PE was negative. Non-compliance was suspected, methimazole was resumed, and hydrocortisone and propranolol were added. After 2 days, her vital signs and free hormone levels normalized. Her methimazole dose was decreased and she was discharged home in a stable condition.
Five days after her discharge, she presented with sore throat, fever and chills. She had an absolute neutrophil count (ANC) of 0 and a positive rapid strep test. ATD Induced Agranulocytosis was suspected. Her labs showed elevated fT3 of 4.5(nl 1.7–3.7), normal fT4 and suppressed TSH with &lt;0.01(nl 0.3–4.9). A CT scan of the neck showed no evidence of retropharyngeal or thyroid abscess. Methimazole was stopped and she was started on glucocorticoids (initially betamethasone for fetal lung maturity, then switched to prednisone) and cholestyramine. She was also started on Cefepime and G-CSF for her neutropenia. A thyroid ultrasound showed enlarged and hypervascular gland. TSI was 157%(nl. &lt;122%), and thyroglobulin 155 ng/ml (nl. &lt;33 ng/ml). After 4 days, her ANC started to recover. Simultaneously, she started to show worsening thyrotoxicosis but remined hemodynamically stable. A decision to induce labor was then made and was successfully done on the 6th day of her admission. Post-delivery, PTU was started at low dose along with SSKI to prepare her for total thyroidectomy which was done on day 3 post-delivery. Post-thyroidectomy, she had an uncomplicated course and was discharged on levothyroxine. Her child did well with no evidence of thyroid disease.
Conclusion:
We present a unique case of thyrotoxicosis in late pregnancy complicated by ATD Induced Agranulocytosis. Given the high risk of thyroid surgery during pregnancy, our multi-disciplinary team approach opted for labor induction, followed by preparation for thyroidectomy and subsequent surgery. Individualization of management approach using a multi-disciplinary team with emphasis on maternal and fetal well-being is of paramount importance with such challenging presentations.
SAT-078 Griscelli Syndrome and Late Endocrine Effects After Stem Cell Transplant
Authors: Mencher Shana , Patel Anisha D , Tamborlane William V 
Published: May 2020

Abstract
BACKGROUND: This a unique case of late-onset endocrinopathies after stem cell transplant in a girl with Griscelli syndrome. Griscelli syndrome is a rare disorder characterized by partial albinism, silver hair and immune failure with alteration in genes necessary for melanin transport, which is curative by stem cell transplant. Although late endocrinopathies are quite common in other disorders after stem cell transplant, these complications have not been reported in Griscelli syndrome.
CLINICAL CASE: A 7-year old female who received a stem cell transplant as a toddler and subsequently developed graft-versus-host-disease (GvHD) at 2 years of age presented for evaluation of growth failure. Patient had severe short stature along with mild hyperthyroxinemia with subsequent diagnosis of Graves’ disease which was treated with methimazole. Although hypothyroidism is more commonly seen after stem cell transplant, rare cases of hyperthyroidism have been reported. Despite normal GH and IGF-1 levels, GH therapy was commenced due to persistent growth failure. She showed a robust increase in growth parameter from -6 to -2 SD below the mean. She started spontaneous puberty, however, biochemical evaluation showed hypergonadotropic hypogonadism with undetectable anti-mullerian hormone (AMH) which is consistent with low ovarian reserve most likely related to total body irradiation prior to stem cell transplant.
CONCLUSION: Our patient demonstrates that growth failure, thyroid disease and ovarian dysfunction can be complications of stem cell transplants in young children with Griscelli syndrome. This can be a result of the underlying disease leading to transplant, conditioning regimen prior to transplant or complications thereafter. GvHD may also be a risk factor for future autoimmune endocrine complications in this syndrome and in other syndromes treated with stem cell transplant.
REFERENCES: (1) Griscelli C, Prunieras M. Pigment dilution and immunodeficiency: a new syndrome. Int J Dermatol. 1978;10:788–91.
(2) Sağ E, Gönç N, Alikaşifoğlu A, et al. Hyperthyroidism after allogeneic hematopoietic stem cell transplantation: A Report of Four Cases. J Clin Res Pediatr Endocrinol. 2015;4:349–54.
SUN-921 Indolent Presentation of Medullary Thyroid Cancer in a Patient with MEN Type 2B Due to a Germline RET M918T Mutationo
Authors: Kim Stephanie , Hsiao Victoria C , Seib Carolyn , Gosnell Jessica Erin , Liu Chienying 
Published: May 2020

Abstract
Background: MEN type 2B is rare and most commonly due to a germline methionine-to-threonine substitution at codon 918 (M918T) of the RET proto-oncogene. Medullary thyroid cancer (MTC) occurs in 100% of the patients affected with the mutation. This mutation is considered the highest risk and is typically associated with aggressive disease and worse overall survival. We describe a case of a late diagnosis of MEN 2B in a patient and his son, both with a relatively indolent presentation of MTC. Clinical Case: A 39-year-old man presented to an outside institution with difficult to control hypertension, headaches and anxiety and was found to have bilateral pheochromocytomas (left, 5.8 x 5.5 x 3.8 cm and right, 9 x 5.2 x 7.3 cm). Upon presentation to our institution, he was noted to have classic phenotypic features of MEN 2B with a marfanoid habitus and multiple mucosal neuromas. Genetic testing confirmed RET M918T mutation. His family history was negative for similar features in his parents and siblings. However, one of his three children, age 12, had similar phenotypic features and was found to have the same mutation. The patient subsequently underwent a successful bilateral adrenalectomy and pathology confirmed pheochromocytomas. Thyroid ultrasound showed multiple nodules with calcifications but no lateral nodal metastases. Calcitonin and carcinoembryonic antigen (CEA) levels were elevated (170 pg/mL, normal ≤10, and 180.4 ug/L, normal &lt;3.8, respectively). He underwent a total thyroidectomy and bilateral central node dissection, with pathology confirming bilateral MTC (2.7 cm and 1.0 cm), metastatic in 4 of 10 positive lymph nodes (largest focus 2 mm). Whole body PET/CT post-operatively did not show metastatic disease. The patient’s son also had multiple thyroid nodules on ultrasound without lateral nodal metastases and elevated calcitonin and CEA levels (3015 pg/ml, normal ≤10, and 433 ng/mL, normal &lt;2.5, respectively). MRI of the abdomen and pelvis was negative for pheochromocytomas. He underwent total thyroidectomy and bilateral central neck dissection, with pathology showing bilateral MTC (2.7 cm and 1.0 cm) with 0 of 14 positive lymph nodes. For both the patient and his son, calcitonin and CEA levels normalized following thyroidectomy and surveillance over a year later reveals no evidence of disease. Conclusion: Early diagnosis of MEN type 2B is important as MTC develops early in life and is the leading cause of death in these patients. When diagnosed early, prophylactic thyroidectomy in childhood is indicated and can improve long-term survival. There are salient phenotypic features associated with this disease which were unfortunately not recognized early in this patient and his son. Fortunately, their MTC presentations appear to be relatively indolent despite their late diagnoses, and they will continue to be closely monitored for recurrent disease.