bluuebunny/crossref_metadata_2025_split huggingface dataset:
SAT325 Recurrent Pericardial Effusion As Initial Presentation Of Primary Adrenal Insufficiency
Authors: Ward Samuel , Champion Amber Laurel , Mrkaic Ana 
Published: October 2023

Abstract
Disclosure: S. Ward: None. A.L. Champion: None. A. Mrkaic: None.
Recurrent Pericardial Effusion as Initial Presentation of Primary Adrenal Insufficiency:
Background: Primary adrenal insufficiency (PAI) is a rare but serious endocrinopathy. High clinical suspicion is important to avoid delay in diagnosis and treatment. We present a rare case of recurrent pericardial effusion as initial presentation. Clinical Case: 63 year old male with hypertension and diabetes presented to ED complaining of SOB and chest pain. Workup revealed pericardial effusion causing cardiac tamponade treated with emergent pericardiocentesis. He was admitted to ICU for hypotension. He was also diagnosed with COVID-19 and acute kidney injury. Labs showed hyperkalemia ranging from 5.3 to 7.2 mmol/l (n= 3.5-5.1 mmol/l) and hyponatremia 132 mmol/L (n= 136-145 mmol/L). He was started on dexamethasone for COVID-19 infection. Etiology of pericardial effusion was unclear; infectious disease workup was unremarkable, cytology showed no malignant cells, autoimmune panel was negative. Initial echo showed EF &gt;55%. After initiation of steroids, symptoms improved with normalization of electrolytes and improvement of hypotension, however was discharged on midodrine 2.5 mg TID. Seven days after discharge patient called 911 complaining of worsening shortness of breath. He was found to be severely hypotensive with BP 67/40. Echo showed moderate to large pericardial effusion causing tamponade. Emergent pericardial window was performed. Labs showed hyponatremia 123 mmol/L and potassium ranging from 4.9-5.4 mmol/L. Patient remained hypotensive requiring pressors despite pericardial effusion drainage. With persistent hypotension, hyperkalemia, and hyponatremia there was strong suspicion for adrenal insufficiency and workup was consistent with PAI. Labs showed 8:00 am cortisol 2.7 ug/dl (n= 3.7-19.4 ug/dl), ACTH 256.0 pg/ml (n=7.2-63.3pg/ml), aldosterone &lt;1.0 ng/dL, renin 4.216 ng/mL/hr (n= 0.167-5.380 ng/mL/hr). Due to his critical illness patient was started on stress dose hydrocortisone 50 mg Q6 hr. With initiation of steroids, hyponatremia, hypokalemia, and hypotension resolved. Patient was weaned to physiological doses of hydrocortisone and started on fludrocortisone at time of discharge. Conclusion: Timely diagnosis of PAI is essential and can be lifesaving, if untreated it can lead to adrenal crisis with high mortality. Having a high clinical suspicion is crucial to a prompt diagnosis. Pericardial effusion should prompt clinical suspicion for PAI as multiple cases have now been reported in the literature. Our patient has multiple potential causes of pericardial effusion including PAI, acute kidney injury, and COVID-19 infection. Although we cannot show definitive causation from PAI, it most certainly contributed and prompt diagnosis and treatment may have prevented recurrence. It is important to raise awareness of PAI as a differential in pericardial effusion.
Presentation: Saturday, June 17, 2023
OR30-01 Waking Salivary 11-hydroxyandrostenedione And Cortisone As A Novel Non-invasive Test For Adrenal Insufficiency
Authors: Lawrence Neil Richard , Keevil Brian George , Elder Charlotte , Fearnside Jane , Caunt Sharon , Dixon Simon , Newell-Price John D C , Ross Richard John M , Debono Miguel 
Published: October 2023

Abstract
Disclosure: N.R. Lawrence: None. B.G. Keevil: None. C. Elder: None. J. Fearnside: None. S. Caunt: None. S. Dixon: None. J.D. Newell-Price: Consulting Fee; Self; Diurnal, Recordati, Crinetics, HRA Pharma. R.J. Ross: Employee; Self; Diurnal. M. Debono: None.
Background: The ACTH (Cosyntropin) stimulation test (AST) is the reference standard for diagnosis of adrenal insufficiency. We have demonstrated recently that home waking salivary cortisone accurately predicts the ACTH stimulation test outcome and confirms or excludes adrenal insufficiency in 70% of high-risk patients (in press: Home Waking Salivary Cortisone to Screen for Adrenal Insufficiency NEJM Evidence). 11-hydroxyandrostenedione (11-OHA4) is a weak adrenal androgen whose synthesis is dependent on CYP11B1. Since ACTH drives steroidogenesis, and adrenal androgens are low in patients with adrenal insufficiency, we hypothesised that waking 11-OHA4 could also predict adrenal status. Methods: A prospective, diagnostic accuracy study of waking salivary cortisone was performed in 173 patients at high risk of AI. All patients collected a salivary sample on waking, and then attended the endocrine clinic for an AST with the 30-minute cortisol used to diagnose AI. Salivary cortisone and 11-OHA4 was measured in the waking salivary sample. Biomarker discrimination was assessed by calculation of area under the curve (AUC) of the receiver operator characteristics (ROC) plot. Equivocal results were defined to maintain a sensitivity of 95% and specificity of 95%, and percentage of AST saved by calculating the number of AST needed if used only in equivocal cases. Results: The median (IQR) for 11-OHA4 for patients with AI and no AI was 55 (45 to 110) pmol/l vs 508 (255 to 765) pmol/l; p&lt;0.0001. Salivary 11-OHA4 on waking was highly predictive of adrenal insufficiency (R2=0.63, ROC AUC 0.94), similar to waking salivary cortisone alone (R2=0.65, AUC 0.94). Waking 11-OHA4 performed better than either serum cortisol (R2=0.65, AUC 0.88) or salivary cortisone (R2=0.74, AUC 0.93) collected at the baseline of the AST. Combining both waking salivary 11-OHA4 with waking salivary cortisone further improved discrimination (R2=0.70, AUC 0.96), and using this combination would mean that 78% of ACTH-stimulation tests could be avoided. Conclusion: Waking salivary 11-OHA4 can accurately predict AI with similar discriminatory value as waking salivary cortisone. Combining waking 11-OHA4 with waking salivary cortisone improves discrimination for adrenal insufficiency, is a simple non-invasive test that can be used widely in clinical practice in the ambulatory setting, and obviates the need for AST in 78% of cases with greater patient convenience and lower costs.
Presentation: Sunday, June 18, 2023
THU461 Severe Hypercalcemia Associated With Perinatal Hypophosphatasia While Receiving Enzyme Replacement Therapy
Authors: Salama Mostafa , Tebben Peter J 
Published: October 2023

Abstract
Disclosure: M. Salama: None. P.J. Tebben: None.
Introduction: Hypophosphatasia (HPP) is characterized by defective bone mineralization due to reduced function of tissue non-specific alkaline phosphatase (TNSALP) from pathogenic ALPL gene variants. Initial presentation varies according to the clinical phenotype. Hypercalcemia is more common in the perinatal and infantile forms and may be mitigated or prevented with enzyme replacement therapy asfotase alfa (AA). Here, we report a patient who developed severe hypercalcemia while receiving AA and describe our management strategy. Case: A now three-year-old girl with perinatal hypophosphatasia presented with severe hypercalcemia at 9 months of age. Her prenatal ultrasound was suggestive of a skeletal dysplasia. She was diagnosed with HPP shortly after birth and initiated AA on day two of life. She had normal calcium of 10.5 mg/dl at diagnosis. Phosphorus 9.3 mg/dl, total alkaline phosphatase &lt;5 U/L, pyridoxal 5-phosphata (PLP) 974 mcg/L and phosphoethanolamine (PEA) 1084 nmoL/mg. Calcium dropped to 6.8 mg/dL after starting AA for which calcium supplementation was transiently utilized. At nine months of age, she developed severe hypercalcemia (15.8 mg/dl). Her phosphorus was 5.5 mg/dl, PLP 14 mcg/l and 25 hydroxyvitamin D 28 ng/ml. Parathyroid hormone and calcitriol were appropriately suppressed at &lt;6.0 pg/ml and &lt;8.0 pg/mL respectively. She was receiving 2 mg/kg of Asfotase three times weekly. Initial management included intravenous fluids and a reduction in dietary calcium intake by 50%. Within 2 days, calcium came down to upper normal for age at 11 mg/dL. AA dose was increased to 3 mg/kg three times weekly due to hypercalcemia and mildly worsening radiographs. Hypercalcemia recurred and initially responded to IV fluids and further dietary calcium reduction (low calcium formula (2.9 mg/ 5 fl oz)). Despite these measures, she required a third hospital admission when calcitonin 4 IU/kg every 12 hours was added to her restricted dietary calcium intake and AA therapy. On this regimen, her calcium normalized without recurrence of severe hypercalcemia. Over the subsequent eight months, calcium intake was slowly increased, and calcitonin tapered and discontinued with maintenance of calcium within the normal range. Conclusion: Hypercalcemia is common in infants with untreated, severe HPP and is likely mitigated for most when treated with AA. This case highlights the importance of monitoring calcium concentrations and intake in infants with HPP. Although calcitonin is generally not considered a long-term therapy for hypercalcemia, bisphosphonates may worsen the bone disease of HPP and should be avoided. This case demonstrates the utility of calcitonin therapy added to low calcium intake and maximal AA dosing to treat severe, persistent hypercalcemia in an infant with perinatal HPP.
Presentation: Thursday, June 15, 2023
SAT232 Patients With Chronic Hypoparathyroidism Have Higher Risk Of Vertebral Fracture And Lower Risk Of Femoral Fracture Compared To Controls
Authors: Bjornsdottir Sigridur , Kamal Wafa , Lomholt Langdahl Bente , Spelman Tim , Lyman Clarke Bart , Kampe Olle 
Published: October 2023

Abstract
Disclosure: S. Bjornsdottir: None. W. Kamal: None. B.L. Langdahl: None. T. Spelman: None. B.L. Clarke: None. O. Kampe: None.
Context: Previous studies have shown that patients with hypoparathyroidism (hypoPT) have reduced bone remodeling, with consequent increases in bone density, and abnormalities in microarchitecture and bone strength. It is still not clear whether there is an increased risk of fractures in patients with hypoPT. Objective: The aim of this study was to evaluate the risk of fractures in patients with hypoPT in Sweden. Patients: The Swedish National Patient Register, the Swedish Prescribed Drug Register and the Total Population Register were used to identify 1982 patients with chronic hypoPT (duration ≥ 12 months), and 19820 controls matched by age, sex and county of residency between 1997-2018. The 10th revision of the International Classification of Diseases (ICD) was used to identify fractures. Results: Patients with hypoPT did not have higher risk of any fracture compared to controls (HR 0.96; 95% CI 0.86-1.08). However, patients with hypoPT had a slightly higher risk of vertebral fractures (HR 1.33; 95% CI 1.01-1.75), p= 0.047 and a lower risk of femoral fractures (HR 0.60; 95% CI 0.45-0.82), p= 0.001. There was no significant difference in the risk of fractures of the forearm, p= 0.177, proximal humerus, p= 0.096 or pelvis, p= 0.446. There was no difference in the risk of any fracture between females and males with hypoPT (p for interaction= 0.511). Patients with surgical hypoPT had a significantly higher risk of any fracture compared with patients with non-surgical hypoPT (p for interaction= 0.006). Conclusion: The risk of any fracture was not increased in patients with hypoPT. However, the risk of vertebral fracture was increased, and the risk of femoral fracture was lower than for matched controls. No difference was found in fracture risk between females and males. Patients with surgical hypoPT had a higher fracture risk compared to patients with non-surgical hypoPT.
Presentation: Saturday, June 17, 2023
SAT240 Recurrent Hypercalcemia Due To 1,25 Dihydroxy Vitamin D Elevation Of Unclear Etiology
Authors: Javvaji Abhiram , Anastasopoulou Catherine 
Published: October 2023

Abstract
Disclosure: A. Javvaji: None. C. Anastasopoulou: None.
Introduction: Recurrent Hypercalcemia is common with primary hyperparathyroidism and malignancy accounting for 90% of cases. Extra renal activation of 1α-hydroxylase with subsequent elevation of 1,25 dihydroxy vitamin D is a relatively uncommon cause of hypercalcemia. Herein, we report an unusual case of recurrent hypercalcemia due to elevated 1,25 dihydroxy vitamin D of unclear etiology, resistant to therapy with denosumab or bisphosphonate that responded to glucocorticoid therapy. Case Presentation: A 68 year old female was admitted to the hospital for worsening calcium with symptoms of polyuria and increased thirst. Review of systems and physical examination were otherwise unremarkable. Initial work-up demonstrated calcium level of 11.7mg/dL (8.7-10.3), creatinine 2.58 mg/dL (0.57-1), PTH 8pg/mL (15-65), Kappa/Lamba ratio 1.82 (0.26-1.6), PTHrP 11pg/mL (11-20), 25 (OH) Vitamin D 14ng/mL (30-100), 24,25 Dihydroxy Vitamin D ratio 25.81 (&lt;25). Bone marrow biopsy, kidney biopsy, and myeloma work up were all unremarkable. Imaging studies including whole body CT and PET CT were also unremarkable. In the acute phase patient was treated with zoledronic acid and later denosumab with only temporary decrease in calcium levels. Ultimately, she responded well to prednisone therapy. Further work-up for finding the underlying cause of elevated 1,25 dihydroxy vitamin D remains ongoing. Discussion: Elevated 1,25 dihydroxy vitamin D level in granulomatous, neoplastic, and certain unusual diseases is well recognized as a cause of hypercalcemia. These levels may be exacerbated by increased intake of calcitriol, or by extra-renal production in granulomatous and lymphoproliferative diseases. Other rare causes of elevated 1,25 dihydroxy vitamin D include granulomatosis with polyangiitis, cat scratch fever, talc granulomatosis, Crohn’s disease, silicone granulomatosis. Patients with all these diseases respond well to steroids and that was another sign that further work-up to find the source of the elevated 1,25 dihydroxy vitamin D in our case is essential. Conclusion: In patients with recurrent hypercalcemia and elevated 1,25 dihydroxy vitamin D levels, a complete work-up for underlying malignancy, granulomatous or other unusual diseases is indicated. Glucocorticoid therapy is the most effective way to control the hypercalcemia related to increased 1,25 dihydroxy vitamin D even in the long-term phase, especially until the main cause of the problem is identified and treated appropriately.
Presentation: Saturday, June 17, 2023
SAT248 A Case Report Of Hypercalcemia Secondary To Calcium Sulfate Antibiotic Beads
Authors: Habibi Roshanak , Vahdani Golnaz , Ebrahimi Neda 
Published: October 2023

Abstract
Disclosure: R. Habibi: None. G. Vahdani: None. N. Ebrahimi: None.
Introduction: Periprosthetic joint infections are a rare and uncommon complication in arthroplasties of the hip or knee, happening in ∼0.7% of these procedures. Placement of antibiotic eluting beads, sometimes referred to as calcium sulfate beads (CSB), which are bio-compatible hydrophilic crystals used to deliver antibiotics locally, are one solution to prevent such complications. Hypercalcemia post-CSB placement is a very uncommon, rare and unusual complication that has only been presented in the literature a handful of times. Below, we report on a such a patient, illustrating the importance of surgical history in investigating the cause of hypercalcemia. Presentation: 63yo F with PMH of HTN, HLD, Crohn’s, T2DM, CKD3 who had a recent hospital admission 2 weeks prior for new arthritis secondary to pseudogout crystals, s/p bilateral synovectomy and CSB placement by orthopedics. She was subsequently discharged to nursing facility and re-presented to hospital for increasing AMS and worsening knee pain. Labs showed evidence of AKI with creatinine 1.9, severe hypercalcemia at 17.5 mg/dl with concomitant hypophosphatemia at 1.1 mg/dl. PTHrP WNL & PTH low at 7pg/ml and histo antigen neg, suggesting this was not related to PTH. Malignancy ruled out based on past imaging that was done in 2 months prior. MM ruled out since normal SPEP, UPEP, and kappa:lambda. Unlikely that this was milk alkali as patient did not have alkalosis. Vitamin 1,25 low at 10pg/ml ruling out sarcoidosis. Vitamin 25 WNL. Urine Ca 24hr WNL suggesting no FHH. Ca levels returned to normal after pamidronate, IV fluids and calcitonin. Chart review suggested that CSB implanted in her knees as the likely cause of her hypercalcemia. Discussion: It is important to bring awareness that one of the causes of severe hypercalcemia is post CSB placement. Vigilant monitoring of calcium levels pre and post operation after CSB placement is indicated, particularly in patients with a previous history of CKD. It has been hypothesized that there is a dose-dependent relationship between CSB volume and hypercalcemia, with suggestions that limiting CSB to less than 40ml per operation may be beneficial. A 10ml pack of CSB contains 5.73 grams of elemental calcium which is subsequently released over a 30-60-day interval. There is limited information on the mechanism of hypercalcemia secondary to CSB use, so further studies need to be implemented. Ultimately, it is crucial for physicians to have a high suspicion for CSB induced hypercalcemia post-arthroplasty as CSB use expands.
Presentation: Saturday, June 17, 2023
THU007 Improving Completeness Of Endocrinology Referrals Using Structured Templates
Authors: Arao Kevin , Wong Denise , Wormwood Jolie , Vimalananda Varsha 
Published: October 2023

Abstract
Disclosure: K. Arao: None. V. Vimalananda: None.
Objective: Endocrinologists rely on information in the referral to guide the focus and scope of their evaluations. However, endocrinology referrals frequently lack important clinical information, which may increase the risk of inefficiency, errors, and adverse outcomes. This quality improvement project aimed to improve the completeness of endocrinology referrals by utilizing structured referral templates for common endocrine conditions including diabetes, hypogonadism, osteoporosis, thyroid dysfunction (hyperthyroidism and hypothyroidism), and thyroid nodule. Methods: The project was implemented at a large Veterans Affairs Medical Center (VAMC) in New England. Structured referral templates were designed based on existing templates at other VA health systems, the American College of Physicians High Value Care Coordination Toolkit (developed in partnership with The Endocrine Society), and input from endocrinologists and primary care providers at the VAMC. Templates included fields for essential elements: a consult question, relevant clinical history and past medical history, type of visit preference, pertinent over-the-counter medications, and dates of relevant laboratory and imaging studies. We used functions including auto-population of data, checkboxes, and drop-down menus to ease usability. The templates were embedded into the electronic referral system. We conducted a retrospective chart review to compare referrals for a cohort of patients referred between 1/1/2021-9/1/2021 (pre-intervention) vs. a cohort referred between 10/1/2021-9/30/2022 (post-intervention). Each referral was rated using a scoring system derived from the criteria in the data abstraction tool formulated by the investigators. Results: On average, pre-intervention referrals included 52% of the essential elements and post-intervention referrals included 92%. Post-intervention referrals for diabetes, hypogonadism, osteoporosis, thyroid dysfunction (hyperthyroidism and hypothyroidism), and thyroid nodule improved from baseline by 44%, 45%, 39%, 39%, and 36% respectively. The greatest improvement was for the element “type of visit requested (one-time consultation, shared care, transfer of care, and consultant’s preference)”. A separate analysis excluding “type of visit” showed an average improvement from 64% of essential elements included pre-intervention to 92% included post-intervention. Conclusion: Structured referral templates embedded into an electronic referral system improves availability of essential information for the endocrine consultant. Our next steps are to modify the templates with the ongoing input of both endocrinologists and primary care providers. Future work should examine the effect of referral templates on specialist and primary care provider user experience, patient experience of care, efficiency, and clinical outcomes.
Presentation: Thursday, June 15, 2023
THU263 Intestinal Production of H2S And Its Regulation Of GLP-2
Authors: Hammond Joel , Yang Guangdong , Gagnon Jeffrey 
Published: October 2023

Abstract
Disclosure: J. Hammond: None. G. Yang: None. J. Gagnon: None.
Background: Hydrogen sulfide (H2S) is an endogenously produced gasotransmitter that regulates a variety of physiological processes including hormone regulation. In individuals with Crohn’s disease or colitis, H2S is elevated. Whether this H2S plays a role in gut disease development or protection is not clear. Glucagon-Like Peptide-2 (GLP-2) is a gut health-promoting peptide hormone produced in the enteroendocrine L-cells located primarily in the distal GI tract. In the gut, GLP-2 increases cell proliferation, enhances barrier function, and decreases inflammation. Furthermore, GLP-2 administration has been shown to be protective in both Crohn’s and Colitis. Finally, our previous work has shown a role for H2S in the regulation of glucagon-like peptides. Hypothesis: We hypothesize that local H2S production in the distal GI tract and in enteroendocrine cells can regulate GLP-2 secretion and downstream gut physiology.Methods: GLP-2 secretion and gut physiology were examined in mice receiving exogenous H2S injections or lacking the H2S-producing enzyme cystathionine gamma-lyase (CSE). Additionally, H2S production and GLP-2 secretion were examined from mouse enteroendocrine L-cells (GLUTag). Results: CSE KO mice had significantly reduced intestinal H2S production. A slight increase in circulating GLP-2 was found in CSE KO mice. H2S was also produced directly from enteroendocrine cells which was partially blocked using CSE enzyme inhibitors. When enteroendocrine L-cells were treated with H2S donors (NaHS and GYY 4137), GLP-2 secretion was significantly reduced (with no effect on cell viability). Ongoing work will explore the direct effects of H2S administration in mice on circulating GLP-2, and any changes that occur in the gut epithelium as a result. Discussion/Conclusions: Our work indicates that the enteroendocrine cell can produce the gasotransmitter H2S, and that H2S donors can suppress GLP-2 secretion. These findings suggest that GLP-2 may play a role in the interplay between H2S and gastric health. These results could provide support for the potential use of H2S drugs in the treatment of GI diseases.
Presentation: Thursday, June 15, 2023