bluuebunny/crossref_metadata_2025_split huggingface dataset:
SAT239 Deceased Donor Parathyroid Allotransplant For Sever Refractory Iatrogenic Hypoparathyroidism
Authors: Devon Karen M , Zahedi Afshan , Madani Amin , Pasternak Jesse D , Saravana-Bawan Bianka , Humar Atul , Kathryn Tinckam 
Published: October 2023

Abstract
Disclosure: K.M. Devon: None. A. Zahedi: None. A. Madani: None. J.D. Pasternak: None. B. Saravana-Bawan: None. A. Humar: None. T. Kathryn: None.
While Halstead experimented with dog parathyroid transplantation, the first reported human parathyroid allotransplant appeared in the Annals of Surgery in 1911. Efforts of varying success in restoring calcium homeostasis in patients with refractory hypoparathyroidism after thyroidectomy have included parathyroid transplantation(tx), mainly in immunosuppressed kidney transplant recipients, using cryopreserved, cultured, or hypercellular tissues. Recently, cases of living-donor parathyroid allotransplants have been reported. We present a 46 year-old woman who developed hypoparathyroidism following total thyroidectomy 9 years prior. Treatment included two years of continuous teriparatide via CAD pump. Complications of portacath sepsis and pulmonary emboli, required intensive care. At the time of transplantation, our patient was taking elemental calcium 16 grams daily, magnesium 12 grams daily, calcitriol 8 ug daily, hydroochlorothiazide 25 mg twice daily, and nonetheless requiring biweekly intravenous calcium for symptoms and Calcium levels of 1.6 mmol/l (2.2-2.6 mmol/L). PTH ranged between 0.6- 1.2 pmol/l with poor quality of life. A medically suitable neurologically deceased donor was identified through the provincial organ donation organization. Her cumulative cPRA was 98% (Class I-95%/II-81%). Virtual crossmatch only positive for DQ2 specificity with weak positive B-cell flow crossmatch. Four healthy parathyroids were retrieved. The tissue was minced and placed into pockets in the recipient’s right brachioradialis muscle, under local anaesthesia with sedation. Ischemic time was 2.5 hours. She was induced with basiliximab and received tacrolimus, mycophenolate, and prednisone as immunotherapy. Evidence of biochemical parathyroid function was present post-tx day 9, and she was weaned off all calcium homeostatic medications by day 35, remaining asymptomatic at 7 months at the time of writing, with no calcium infusions. Self-reported quality of life is significantly improved. PTH 0.8-2.8 pmol/L and Calcium 2.06-2.37 mmol/l. To our knowledge this is the first successful fresh normal tissue deceased donor parathyroid transplant in a non-transplant recipient in North America. One study suggests that despite immunosuppression risks, 14 percent of patients would entertain parathyroid allotransplantation. We are currently recruiting additional patients with the aim of demonstrating that deceased donor parathyroid allotransplant using fresh tissue and immunosuppression is a novel viable and curative approach to treat permanent severe hypoparathyroidism.
Presentation: Saturday, June 17, 2023
SAT241 Severe Hypercalcemia Secondary To Likely Sarcoidosis
Authors: Nair Nirmal 
Published: October 2023

Abstract
Disclosure: N. Nair: None.
A 42 year old gentleman with no known past medical history initially presented to the Endocrinology office referred from his primary care provider for the evaluation of severe hypercalcemia. He initially presented to his PCP with 8 months of progressive tachycardia, shortness of breath and persistently elevated blood pressures but was noted with a serum calcium of 14.9. On my initial evaluation, patient was well appearing, tachycardic with normal rate and rhythm with otherwise no abnormal findings. His blood pressure was elevated to 150s systolic from a baseline of less than 120. Based on the severity of the initial hypercalcemia, an extensive workup was sent to rule out potential etiologies for his hypercalcemia including Vitamin D, PTH, PTHrp, serum free and light chains, CMP, ionized calcium, and serum protein electrophoresis. The initial workup revealed a calcium improved to 12.1 from the initial 14.9, with significant elevation in ionized calcium. PTH and 25 hydroxyvitamin D were suppressed, ruling out primary hyperparathyroidism. Interestingly, 1,25 dihyroxyvitamin D levels were within the upper limit of normal, raising the possibility of granulomatous disease. PTHrp was elevated. Serum protein electrophoresis had revealed mild elevations in beta immunoglobulins. Based on the findings of the initial workup, concern remained for granulomatous disease and malignancy, and a CT of the chest, abdomen and pelvis was ordered. CT showed bilateral perihilar interstitial nodules, mildly enlarged mediastinal and hilar lymph nodes, and splenomegaly with ill defined splenic lesions. No mass or tumor was identified in this study. Although the potential for monoclonal gammopathies and malignancy remain in the differential diagnosis, his presentation, CT scan, and lab findings may be explained by sarcoidosis. While the normal 1,25 Vitamin D levels in the setting of suppressed PTH and 25 Vitamin D may be more classically associated with granulomatous disease, significant renal impairment, elevations in PTHrp and immunoglobulins are not uncommon, making sarcoidosis a likely explanation for his symptoms short of obtaining a biopsy. He was subsequently referred to pulmonology to evaluate for sarcoidosis and to oncology to evaluate further the elevations in serum immunoglobulins. This patient presentation is unique as it is not common to encounter cases of severe hypercalcemia in the outpatient setting. Here, it is important to note that PTHrp elevations and elevations in light chains may be seen in sarcoidosis as well as malignancy. This case illustrates the importance of a wide differential diagnosis in order to avoid the pitfall of prematurely determining a working diagnosis.
Presentation: Saturday, June 17, 2023
SAT247 CODE STEMI Due To Excessive Ingestion Of TUMS
Authors: Hershkop Eliyakim 
Published: October 2023

Abstract
Disclosure: E. Hershkop: None.
Hypercalcemia is defined as a serum calcium greater than 10.5mg/dL. It is most commonly caused by malignancy or hyperparathyroidism; a less common cause is excess calcium ingestion, referred to as Milk Alkali Syndrome (MAS). Severe hypercalcemia can lead to renal failure, arrhythmias, and coma. A rare EKG finding is ST elevation mimicking an acute ST Elevation Myocardial Infarction (STEMI). We describe a case of severe hypercalcemia caused by excessive ingestion of TUMS antacids that presented as diffuse ST elevations on EKG which were mistaken for an acute MI. To our knowledge, this is the first case in the literature describing hypercalcemia with ST elevations caused by excessive TUMS ingestion. Case: A 74 year old male with a history of diabetes, hypertension, stroke, and acid reflux presented for recurrent falls due to leg weakness. He endorsed constipation with bowel movements every 5-6 days. Upon further questioning, he reported taking 6 to 7 TUMS antacid tablets (each containing 200mg calcium) daily for the past two months to treat ‘heartburn’. He denied having chest pain. His medications included hydrochlorothiazide, losartan, amlodipine, atorvastatin, glimepiride, and famotidine. On physical exam he had reduced strength in both lower extremities - 1/5 left, 3/5 right. EKG on arrival showed ST elevation in leads III, aVF, and V1-V5, with T wave inversions in aVR and aVL, suggestive of a STEMI. A STEMI code was called. Since he denied chest pain, troponins were lateralized at 0.1 ng/mL, and an echocardiogram showed no wall motion abnormalities, cardiology recommended conservative management. Laboratory evaluation revealed calcium of 15.5 mg/dL, albumin of 3.8 g/dL, BUN of 39 mg/dL, creatinine of 2.2 mg/dL, magnesium of 1.4 mg/dL, phosphorus of 3 mg/dL, 25-OH vitamin D of 24 ng/mL, PTH of 9 pg/mL, undetectable 1-25 OH vitamin D and PTHrP, and normal TSH and SPEP. CT head/chest/abdomen/pelvis showed no evidence of malignancy. He was treated with IV hydration and 1 dose of calcitonin. His calcium level, leg strength, and ST elevations began to improve within 12 hours and returned to normal within four days. Conclusion: Our patient had severe hypercalcemia likely secondary to excessive consumption of oral antacid tablets, as other causes were ruled out, and his EKG findings masqueraded as a STEMI-a rare presentation of hypercalcemia. Consumption of supplements may be overlooked when investigating hypercalcemia and should be specifically asked about. In addition, providers should be aware of EKG changes found in hypercalcemia to avoid unnecessary cardiac intervention.
Presentation: Saturday, June 17, 2023
SAT250 A Case Of Hypercalcemia In Pregnancy - A Diagnostic And Management Challenge
Authors: Cheng Wee Mee , Chien Kuan Yueh 
Published: October 2023

Abstract
Disclosure: W. Cheng: None. Y. Kuan: None.
Hypercalcemia in pregnancy is rare and poses significant diagnostic and management challenges especially in a low resource setting. Although primary hyperparathyroidism (PHPT) is the main cause, physiological changes in calcium homeostasis make it difficult to distinguish from familial hypocalciuric hypercalcemia (FHH) unless genetic testing is done. Furthermore uncertainty exists on the safety of pharmacotherapy for hypercalcemia in pregnancy which if untreated, can be detrimental to both mother and child. Described herein a 17 year-old primigravida who presented at 32 weeks of gestation for asthma exacerbation precipitated by COVID-19 infection. She was incidentally found to have PTH-dependent hypercalcemia with albumin-corrected calcium 3.42 mmol/L [2.10-2.55] associated with raised intact parathyroid hormone (iPTH) 15 pmol/L [1.6 - 6.0] and deficient serum 25OH Vitamin D at 19.95 nmol/L [50-125]. She was given IV saline of 3-3.5L/day but to no avail as her serum calcium was persistently above 3 mmol/L with an episode of premature uterine contraction. Calcitonin was given in short courses and withheld once albumin-corrected calcium dropped below 3 mmol/L but rebound within three days. As bisphosphonates is contraindicated in pregnancy, we introduced cinacalcet, up to 75mg/day after discussion with the patient and her family. Meanwhile, investigations were done to ascertain underlying cause. Fractional urine calcium excretion ranged from 0.0068 (spot urine sample) to 0.0568 (24-hour urine sample). Ultrasound neck did not reveal enlarged parathyroid glands or an adenoma. Sestamibi scan was contraindicated in pregnancy. Genetic testing for FHH was too costly but all her first-degree relatives tested to have normocalcemia. Blood pressure remained normal throughout pregnancy. She was discharged with cinacalcet at calcium level of 2.7 mmol/L. However she defaulted follow-up and medications and presented at 36 weeks of gestation for preterm labour. She gave birth to a healthy, 2.46kg baby girl. The baby had neonatal jaundice but normal calcium level. Postpartum, the patient was asymptomatic despite persistent hypercalcemia 3.12 - 3.15nmol/L. As she was non-compliant to cinacalcet it was stopped and serum calcium did not worsen. Sestamibi scan is planned after she stops breastfeeding and a reassessment of the urine calcium once vitamin D is adequately replenished. She refused exploratory parathyroidectomy. She continued to be followed up at the endocrine clinic while her baby at primary care. Contraception is advised untill definitive diagnosis and treatment of hypercalcemia. This case illustrates the challenges in managing hypercalcemia in pregnancy and difficulty in distinguishing PHPT vs FHH without genetic testing.
Presentation: Saturday, June 17, 2023
SAT256 Buschke Loewenstein Tumor Presenting With Hypercalcemia
Authors: Jones Kai , Krutilova Petra , McGill Janet B 
Published: October 2023

Abstract
Disclosure: K. Jones: None. P. Krutilova: None. J.B. McGill: None.
Introduction: Humoral hypercalcemia of malignancy is known to be associated with squamous cell carcinomas (SCC), although rarely seen in the setting of skin cancer. We present an interesting case of a young patient with giant condyloma acuminatum complicated by SCC presenting with severe hypercalcemia. Case Description: A 37-year-old man presented with pain and purulent drainage due to bacterial superinfection of genital warts. He had recurrent and locally advanced condylomas, first occurring more than 10 years ago. Skin lesions in the genital and perianal area never resolved and he required multiple surgical interventions including sigmoid colostomy placement due to Fournier gangrene. His laboratory evaluation this admission was notable for an elevated calcium level (13.0 mg/dL [8.5-10.3], albumin 3.4 g/dL [3.5-5.0]), phosphorus 3.9 mg/dL [2.3-4.5], and normal kidney function. He did not have history of hypercalcemia and was not taking any calcium supplements. Hypercalcemia prompted work-up including: PTH 4 pg/mL [15-65], 25-OH vitamin D 36 ng/mL [30-80], unremarkable SPEP/UPEP, TSH 0.91 mcIUnit/mL [0.3-4.2]. Initial differential diagnosis included hypercalcemia due to granulomatous process and increased production of calcitriol vs. PTH-related protein (PTHrP) mediated hypercalcemia. 1,25-OH vitamin D was normal (33 pg/mL [18-64]) and PTHrP was elevated (6.7 pmol/L [&lt;4.2]). The patient subsequently underwent biopsy of groin lesions which demonstrated SCC in situ. PET/CT only demonstrated hypermetabolic changes in the perineum and groins bilaterally. Inguinal lymph node biopsy was negative for malignancy. His hypercalcemia persisted despite administration of IV fluids and he was treated with IV zolendronic acid. Calcium level subsequently normalized (9.1 mg/dL). He underwent extensive excision of the tumor involving the right gracilis muscle, left testicle, portions of the perineum, and penile shaft skin. Discussion: While cases of hypercalcemia in malignancy are common, this is an unusual case of giant condyloma acuminata (GCA) heralding development of SCC by elevated calcium levels. GCA also known as Buschke-Loewenstein tumor is a rare disease with estimated incidence of 0.1% in the general population and a male-to-female ratio of 2.7:1, typically associated with human papilloma virus subtypes 6 and 11. It is a locally aggressive tumor with a potential for destructive growth and malignant transformation (estimated rate up to 56%), very high rate of recurrence after excision (66%), and overall mortality of approximately 20%. Early recognition and treatment of GCA is essential in order to prevent devastating complications. Purzycka-Bohdan D, Nowicki RJ, Herms F, et al. The Pathogenesis of Giant Condyloma Acuminatum (Buschke-Lowenstein Tumor): An Overview. Int J Mol Sci. 2022;23(9):4547. Published 2022 Apr 20. doi:10.3390/ijms23094547
Presentation: Saturday, June 17, 2023
OR23-06 Long Term Outcomes Of Parathyroid Autografts In Primary Hyperparathyroidism
Authors: Chuki Elias , Graf Akua , Ninan Anisha , Tora Rana , Abijo Tomilowo , Nilubol Naris , Scott Weinstein Lee , Kishore Agarwal Sunita , Simonds William F , Jha Smita 
Published: October 2023

Abstract
Disclosure: E. Chuki: None. A. Graf: None. A. Ninan: None. R. Tora: None. T. Abijo: None. N.M. Center: None. N. Nilubol: None. L.S. Weinstein: None. S.K. Agarwal: None. W.F. Simonds: None. S. Jha: None.
Background: Autologous implantation of parathyroid tissue is frequently utilized in patients with heritable forms of primary hyperparathyroidism (PHPT). There is sparse data on long-term functional outcome of these grafts and post-graft recurrence of PHPT. Objective: We aimed to investigate the long-term outcome of parathyroid autografts and assess the frequency of post-graft PHPT recurrence. Methods: Retrospective study of patients with PHPT who underwent parathyroid autografts from 1991 to 2020. Results: We identified 115 patients with PHPT who underwent 135 parathyroid autografts. A significant majority (106 of 115 patients, 92%) had a heritable form of PHPT, with MEN1 being the most common (93 of 115 patients, 81%). Median follow-up duration since graft was 10 [4-20] years. 119 (88%) were to brachioradialis, 11 (8%) to sternocleidomastoid and 5 (4%) to deltoid. There were 86 immediate (64%) and 49 delayed (36%) grafts. Of the 135 grafts, 54 (40%) were fully functional at last follow-up, 13 (10%) partially functional, 44 (32%) non-functional while functional status of 24 (18%) grafts could not be ascertained. Age at graft, thymectomy prior to autograft, graft type (delayed/immediate) or duration of cryopreservation did not predict functional outcome. There were 45 (83%) post-graft recurrences of PHPT among 54 fully functional grafts at a median duration of 8 [4-15] years after grafting. Parathyroidectomy was performed in 42/45 but surgical cure was attained in 18/42 (43%) only. 12 of 18 (67%) recurrences were graft-related while the remaining 6 (33%) had a neck or mediastinal source. Median time to recurrence was 16 [11-25] years in neck or mediastinal source versus 7 [2-13] years in graft-related recurrences. Median PTH gradient was significantly higher at 23 [20-27] in graft-related recurrence versus 1.3 [1.2-2.5] in neck or mediastinal source (P = 0.03). Nineteen patients had multiple parathyroid grafts. Median pre-repeat-graft PTH of 7.5 [6.5-18.5] pg/mL with ionized calcium of 1.15 ± 0.145 (reference:1.09-1.30) mmol/L served as an indicator of the need for a repeat graft. Discussion: Post-graft recurrence occurs frequently within the first decade after grafting. Source of excess PTH in post-graft recurrence of PHPT can be challenging to localize. Time to recurrence after graft is significantly shorter and PTH gradient higher for graft-related recurrence.
Presentation: Saturday, June 17, 2023
OR02-04 Peripheral Blood Steroid Profiling Discriminates Aldosterone-producing Adenoma Genotypes From Bilateral Primary Aldosteronism
Authors: Turcu Adina F , Lee Chaelin , Salman Zara , Liu Haiping , Rabbah Amira , Marie Konzen Sonja , Rainey William E , Giordano Thomas J , Zhao Lili , Udager Aaron 
Published: October 2023

Abstract
Disclosure: A.F. Turcu: None. C. Lee: None. Z. Salman: None. H. Liu: None. A. Rabbah: None. S.M. Konzen: None. W.E. Rainey: None. T.J. Giordano: None. L. Zhao: None. A. Udager: None.
Background: Primary aldosteronism (PA) is dichotomized into 2 major subtypes: unilaterally dominant PA, often caused by aldosterone-producing adenomas (APAs), and bilateral PA (BPA). Adrenal vein sampling (AVS)-guided unilateral adrenalectomy is often curative in patients with APA and offers marked cardiovascular and renal benefits. The diagnosis and subtyping of PA is a complex, multistep process, hindered by expertise and resource limitations. Objective: To develop steroid fingerprints that can distinguish genotype specific APAs from BPA in peripheral blood. Design And Methods: We enrolled 120 patients with PA who underwent AVS in a single tertiary referral center. Liquid-chromatography tandem mass spectrometry was used to simultaneously quantify 17 steroids in adrenal veins (AV) and periphery, both at baseline, and 10-20 min after cosyntropin stimulation. Aldosterone synthase (CYP11B2) immunohistochemistry-guided next generation sequencing was employed to identify aldosterone-driver mutations. Kruskal-Wallis tests were performed for comparison of continuous variables across multiple groups. Receiver operating characteristic (ROC) curves were plotted to discriminate APAs from BPA. Results: The 120 study participants included 75 patients with APA who underwent unilateral adrenalectomy and had documented aldosterone-driver mutations, and 45 patients with BPA. Of patients with APAs, 36 carried somatic mutations in CACNA1D, 24 in ATPase, and 15 in KCNJ5 genes, respectively. Patients with KCNJ5 mutations were younger and most were women. In the dominant AVs, the KCNJ5 group had marked elevations of 18-hydroxycortisol, 18-oxocortisol, aldosterone, and 11-deoxycorticosterone (DOC); conversely, the BB group had the lowest concentrations of mineralocorticoid and androgen precursors, despite comparable cortisol levels across all groups. Similar patterns were observed in peripheral blood. Linear discriminant analysis incorporating all steroids measured in baseline peripheral serum showed areas under the curve (AUC) of 0.98, 0.97, and 0.92 for distinguishing APAs with KCNJ5, ATPase, or CACNA1D mutations, respectively, from BPA. Conclusions: Single-assay steroid profiles measured in peripheral serum can distinguish genotype-specific APAs from BPA. Once validated, such tests might circumvent the need for AVS in large subset of PA patients.
Presentation: Thursday, June 15, 2023
THU585 Non-alcoholic Fatty Liver Disease (NAFLD), A Silent But Emerging Problem In Type 1 Diabetes
Authors: Taiwo Adeyinka , Merill Ronald , Wendt Linder , Pape Daniel , Thakker Himani , Pothireddy Nikitha , Carlson Bianca , Sanchez Antonio , Ten Eyck Patrick , Jalal Diana , Dokun Ayotunde , Taylor Eric , Sivitz William 
Published: October 2023

Abstract
Disclosure: A. Taiwo: None. R. Merill: None. L. Wendt: None. D. Pape: None. H. Thakker: None. N. Pothireddy: None. B. Carlson: None. A. Sanchez: None. P. Ten Eyck: None. D. Jalal: None. A. Dokun: None. E. Taylor: None. W. Sivitz: None.
Background: Non-alcoholic liver disease (NAFLD) is the hepatic manifestation of the metabolic syndrome. Risk factors include type 2 diabetes, insulin resistance and obesity. Although NAFLD has been widely studied in persons with type 2 diabetes, far less in known about the pathogenesis and severity of NAFLD in type 1 diabetes. Objective: The goal of this study was to understand the metabolic perturbations that contribute to the pathogenesis of NAFLD in type 1 diabetes. Study Design: We conducted a cross-sectional study of 30 participants with type1 diabetes recruited from our institutional diabetes clinic. To assess liver fat content and stiffness, a FibroScan was done. Based on the results, participants were stratified into a case group (evidence of NAFLD) or control group (no evidence of NAFLD). All participants had fasting plasma drawn to determine metabolites. Gas-Chromatography Mass Spectrometry was used for metabolomic analyses and Liquid Chromatography Mass Spectrometry for lipidomic and acylcarnitines analyses. Results: Sixteen of 30 participants were classified as cases and 14 as controls. Cases had higher BMI (P=0.001) and were taking higher daily insulin doses than controls (P=0.008). Metabolomic analyses revealed that cases had elevated levels of glutamate (P=0.001), alanine (P=&lt;0.001), pyruvate (P=&lt;0.001), phenylalanine (P = 0.005), lactate (P=0.019) and anthranilic acid (P=0.029). Lipidomics revealed that, cases had elevated ceramides (P= 0.02), diacylglycerol (P=0.0003) and triacylglycerol (P=0.0003). The acylcarnitine, isovalerylcarnitine (CAR.5.0) a metabolite of isoleucine catabolism, was elevated in the cases (P = 0.010). Pathway enrichment analysis revealed, phenylalanine and tyrosine metabolism, tryptophan metabolism, glucose-alanine cycle, glutamate metabolism and glutathione metabolism were significantly enriched in the cases. Conclusion: We observed evidence of elevated metabolite levels involved in gluconeogenesis, glucose-alanine metabolism, energy metabolism, insulin resistance, and dysfunctional fat synthesis in the cases compared to controls. Our cases had some features similar to Type 2 diabetes with NAFLD, with evidence of elevated levels of aromatic amino acids, ceramides, diacylglycerol and triacylglycerol. However unlike Type 2 diabetes, there was no elevation of branched-chain amino acids levels.
Presentation: Thursday, June 15, 2023