bluuebunny/crossref_metadata_2025_split huggingface dataset:
SAT323 Management Of Severe Cushing’s Syndrome Due To Cortisol Secreting Adrenal Tumor In A Critically Ill Patient
Authors: Obeid Hiba , Buras Eric Dale , Else Tobias 
Published: October 2023

Abstract
Disclosure: H. Obeid: None. E.D. Buras: None. T. Else: None.
Introduction: Severe Cushing’s syndrome is associated with multiple life threatening complications. Clinical Case: A 69 year-old man with Cushing’s syndrome was admitted to the hospital with septic shock due to bacteremia with necrotic left lower extremity infection. He required above knee amputation. His hospital course was complicated with invasive pulmonary Aspergillosis and ventilator associated pneumonia. He developed acute kidney injury that required dialysis, altered mental status, deep venous thrombosis bilateral pulmonary embolisms, bleeding duodenal ulcer and retroperitoneal hematoma. Prior to admission: Random cortisol was 31.7 mcg/dL (Normal: 4.8-19.5 mcg/dL), AM Cortisol following 1 mg oral dexamethasone was 47 mcg/dL (Normal: &lt;1.8 mcg/dL), DHEA-s was 210 mcg/dL (normal: 33.6-249 mcg/dL) and 24 hour urine free cortisol was 622 mcg/day (Normal: &lt;60 mcg/day). ACTH was undetectable. An FDG-PET scan showed a 6.5 centimeters hypermetabolic left adrenal mass with internal coarse calcifications but no evidence of metastasis. Etomidate drip, Metyrapone and Osilodrostat were tried to lower excess cortisol production. During admission while on Osilodrostat: Cortisol level: 20 mcg/dL (normal: 8-19 mcg/dL). Deoxycorticosterone: 53 ng/dL (Normal: 2-19 ng/dL). 11-Desoxycortisol: 580 ng/dL (Normal: 12-158 ng/dL). 17-alpha-hydroxyprogesterone: 45 ng/dL (Normal: 27-199 ng/dL).
Surgery was considered, but adrenalectomy was deemed high risk and not pursued. Therefore, the patient underwent computed tomography (CT) cryoablation of the left adrenal gland. Labs following cryoablation: Cortisol: 7.7 mcg/dL. Deoxycorticosterone: 10 ng/dL. 11-Desoxycortisol: 34 ng/dL. 17-alpha-hydroxyprogesterone: 25 ng/dL. The procedure was complicated by T11/T12 intercostal artery injury leading to hemothorax. He was continued on physiologic doses of hydrocortisone. On follow up 4 weeks following discharge, he had complete resolution of hypercortisolism symptoms. A repeat whole body PET scan showed persistent FDG-avid neoplasm in left adrenal area, as well as metastasis in the liver and the duodenum. Conclusion: It is critical to control hypercortisolism in patients with Cushing’s syndrome. Several medications can be used. Surgical adrenalectomy remains the gold standard for treatment. CT-guided cryoablation of the adrenal tumor is a reasonable alternative when patient is not a surgical candidate. However, it is not a completely benign procedure as it can be associated with complications. Our treatment approach improved short term outcome for our patient, but did not provide long term benefit since he developed metastasis.
Presentation: Saturday, June 17, 2023
THU433 Spontaneous Vertebral Fractures In The Setting Of Prolonged CRRT
Authors: Akanbi Folake , Kutahyalioglu Merve , Gogineni Preethi , Raja Sobia 
Published: October 2023

Abstract
Disclosure: F. Akanbi: None. M. Kutahyalioglu: None. P. Gogineni: None. S. Raja: None.
Introduction: Bone resorption and hypercalcemia due to prolonged immobilization are known complications in critically ill patients. This is more pronounced in patients with high bone turnover rates such as in secondary hyperparathyroidism. In this case report, the chelation of calcium by citrate masked the immobilization hypercalcemia, resulting in marked bone loss and spontaneous vertebral fractures in the context of normal calcium levels. Case report: 23 year old man with no past medical history was seen on account of concerns for calcium loss from his bones as he was on CRRT, for 4 months and the amount of calcium in the extracorporeal circuit was more than expected. He had presented with new onset systolic heart failure (EF 10%) secondary to fulminant post-COVID-19 myocarditis, and renal failure due to acute tubular necrosis. CRRT with citrate dextrose anticoagulation was initiated at presentation. He had received pulse dose prednisone for presumed post-viral myocarditis. He was awaiting heart and kidney transplantation. Greater than average calcium clearance during CRRT prompted workup for hypercalcemia. He denied personal or family history of hypercalcemia, renal stones, fractures, dental issues or hyperparathyroidism. He reported eating adequate amounts of dairy products. Labs showed corrected calcium 9.3 (8.6-10.3 mg/dl), elevated PTH 189 (10-65 pg/ml), low serum protein and albumin, and low 25OHVD that was 14 (25-100 ng/mL) on presentation and actively being replaced with ergocalciferol. Phosphorus, magnesium, liver and thyroid function were normal. The patient had complained of back pain after being immobile for 3 months. MRI showed subacute vertebral fractures of T12 and L1. Thereafter, spine X-ray showed multiple vertebral fractures of L2-L5. Beta CTX was 4153 (120-946 pg/ml) and DXA scan revealed low bone density for age with Z score -3.7 in the femoral neck, invalid in the spine due to the fractures, felt to be the source of high extracorporeal calcium concentration. A dose of Denosumab was given for treatment with improvement of calcium clearance during CRRT. Discussion: Acute fractures and infrequently, prolonged immobilization cause hypercalcemia with suppression of PTH. In our patient who was critically ill and immobilized for 4 months, the use of CRRT maintained normocalcemic blood levels, and renal failure with vitamin D deficiency caused secondary hyperparathyroidism. Only the declining requirements for calcium replacement during CRRT prompted workup for hypercalcemia that lead to identification of significant vertebral fractures and treatment. Declining need for calcium replacement during CRRT should prompt investigation and treatment of disorders of hypercalcemia and metabolic bone disorders in this patient population.
Presentation: Thursday, June 15, 2023
THU440 A Case Of Osteomalacia In A Young Adult Female With Premature Ovarian Insufficiency
Authors: Kasimoglu Ismail Hakki , Vu Timothy , Dai-Ju Jenny Qian 
Published: October 2023

Abstract
Disclosure: I.H. Kasimoglu: None. T. Vu: None. J.Q. Dai-Ju: None.
Background: Osteomalacia is a condition due to inadequate mineralization during the bone-remodeling process. Common causes of bone demineralization include Vitamin D deficiency and low calcium intake. Consequently, the treatment often involves both Vitamin D and calcium supplementation, corresponding dietary modifications, and adequate exposure to sunlight. Clinical Case: 34-year-old female with past medical history of sickle cell disease s/p allogenic bone marrow transplant in 11/2020, brain toxoplasmosis infection 3/2021, secondary amenorrhea due to premature ovarian insufficiency diagnosed in 12/2021, was referred to endocrinology for management of osteomalacia. Dexa Scan from 11/2021 reported lower bone density than expected compared with the same age/gender group (Z-Scores: Left Hip – 0.3, Neck –1.4, and L1-L4 –3.6). Vitamin D 25-OH level was 8.3 (n&gt;30) ng/mL and serum calcium level as low as 5.8 (n8.6-10.3) mg/dL in 3/2021, PTH-I elevated to 459 (n10-68) pg/mL, alkaline phosphatase elevated to 166 (n34-104) U/L. The patient was encouraged to increase Vitamin D3 to 4000 iu daily and calcium intake to 1200 mg daily. After discussion with patient of benefits and risks of hormone replacement, patient was started on very low dose of estradiol patch (0.025mg/day) for concern of prior history of pulmonary embolism, and progesterone 100 mg oral daily 3/2022. Vitamin D 25-OH, PTH, serum and 24 hr urine calcium have been within normal range since 9/2022. Dexa Scan from 11/2022 showed significant improvement of bone density (32.0% increase of lumbar spine, 11.2% increase of left hip). This case of osteomalacia resulting from Vitamin D deficiency and subsequent secondary hyperparathyroidism demonstrates that it is imperative to recognize and treat Vitamin D deficiency because it restores bone strength and prevents fractures while simultaneously correcting biochemical abnormalities (1). This case is also interesting as the patient still carries an increased risk of bone density loss complicated by underlying premature ovarian insufficiency and hypoestrogenic state. Thus, by providing patients with the appropriate Vitamin D and calcium supplementation recommendations along with hormone replacement therapy in young adults, it is possible to rehabilitate their overall bone health as well as reduce future risks of fractures. Conclusion: Adequate Vitamin D and calcium supplementation is important for the management of osteomalacia. In patients with premature ovarian insufficiency, it is even more imperative to promote supplementations, dietary modifications, and sunlight exposure in conjunction with traditional agents of hormonal therapy to promote overall bone health.
Reference: 1) Bhan A., et al. Osteomalacia as a result of vitamin D deficiency. Endocrinol Metab Clin North Am. 2010;39(2):321–331. 10.1016/j.ecl.2010.02.001
Presentation: Thursday, June 15, 2023
THU443 Acute Iridocyclitis Associated With Intravenous Zoledronic Acid
Authors: Ankireddypalli Anvitha Reddy , Sibley Shalamar Dionne 
Published: October 2023

Abstract
Disclosure: A.R. Ankireddypalli: None. S.D. Sibley: None.
Introduction: Bisphosphonates are widely used for osteoporosis. Intravenous (IV) zoledronic acid (ZA) is frequently prescribed in cases of oral bisphosphonate intolerance or noncompliance. Well known immediate ZA side effects include flu-like symptoms such as nausea, myalgias and fever. Here we report a case of a rare side effect of acute anterior uveitis following initial ZA dosing. CASE: A 71-year-old female was diagnosed with osteoporosis; DEXA scan had lowest T score of -2.6 at the lumbar spine. Vitamin D level was 10 ng/mL. She received calcium and vitamin D supplementation. Two months later vitamin D level was 31 ng/mL. ZA was recommended due to difficulty with adherence to oral bisphosphonate. Five days after infusion she presented to our emergency department (ED) with progressive symptoms of bilateral eye itching, redness, excessive tearing, blurry vision, photosensitivity, floaters, pain and headaches that had begun 1 day post infusion. She’d had 2 prior outside ED visits for these symptoms and received polymyxin B and later ofloxacin eye drops which did not relieve them. She denied fever, chills, arthralgias and myalgia. She denied any prior known autoimmune disorder or prior history of eye symptoms. On examination she was afebrile and had bilateral conjunctival injection, excess tearing and watery discharge. She was discharged with olopatadine eye drops and cetirizine, diagnosed with allergic conjunctivitis, and referred to ophthalmology. On ophthalmic exam 7 days after her infusion, visual acuity was 20/60 in the right eye, and 20/60 in the left eye. Intraocular pressure was 12 mmHg in the right eye and 16 mmHg in the left eye. Slit lamp eye exam showed diffuse conjunctival injection of the right eye, mild corneal endothelial folds, and flare with cells in anterior chamber and synechiae posterior to the iris. Right acute iridocyclitis was diagnosed, she received prednisolone eye drops and cyclopentolate eye drops. Symptoms improved in 1 week with almost complete resolution after 3 weeks of therapy. She has not had a recurrence of these symptoms; she has declined future treatment with ZA. Conclusion: ZA-associated ocular inflammation is a rare but serious side effect. This adverse effect has been hypothesized to be associated with increased levels of pro-inflammatory cytokines including Interleukin (IL)-1, IL-6, and tumor necrosis factor (TNF)-alpha. As with other acute phase reactions to ZA, it does not appear that ocular inflammation tends to recur with subsequent ZA. Optimizing vitamin D levels prior to treatment may help to prevent this condition. There is some evidence for higher rates of acute phase reaction in patients with lower levels of vitamin D but this patient’s vitamin D status was adequate at the time of treatment. Clinicians should be aware of the rare occurrence of post-ZA ocular inflammation. Early recognition and prompt treatment are essential.
Presentation: Thursday, June 15, 2023
THU452 Polyostotic Fibrous Dysplasia Complicated By Aneurysmal Bone Cyst In A Patient With McCune-Albright Syndrome
Authors: Milazzo Eliana , Hermayer Kathie Lynn 
Published: October 2023

Abstract
Disclosure: E. Milazzo: None. K.L. Hermayer: None.
Introduction: McCune-Albright Syndrome (MAS) is a rare mosaic disorder with GNAS mutations, encoding the alpha-subunit of the Gs G-coupled protein receptor, activating it, and inappropriately producing cAMP. The hyperfunctioning endocrinopathies associated with MAS are due to signaling through the TSH, LH, FSH, GHRH, and ACTH receptors. MAS clinical presentation occurs with café-au-lait skin macules, precocious puberty, and fibrous dysplasia (FD) of the bone. FD is a benign tumor that leads to increased rates of bone fractures and if it causes facial deformities, can be complicated with orbital neuropathies. An aneurysmal bone cyst (ABC) is a blood-filled cyst that can be a primary or secondary lesion due to co-existing lesions. ABC is a rare complication of FD. We report a rare case of ABC in a MAS patient who presented with acute visual disturbance and enlargement of the forehead and was successfully treated with surgery. Clinical Case: A 20-year-old female diagnosed with MAS at the age of 2 who had precocious puberty by the 3rd year of life and developed polyostotic FD in the right arm, left femur, cranial, and skull bones with multiple long bone fractures by the age of 5-6 and required numerous orthopedic surgeries. She presented with the characteristic café-au-lait skin macules on the back and right arm. In 2018, a slow decline of left eye vision required left cranial decompression of the optic nerve, with pathology showing fibrous tissue compatible with FD. In 2021, she complained of acute right forehead enlargement, compressing her right eye with blurry vision. An MRI showed FD involving the facial and skull bones with a new expansible lesion in the right frontal bone with internal layering fluid, exerting a mild mass effect on the frontal lobe and extending superiorly to the orbit with inferior displacement of the globe. These findings were consistent with an ABC. Right frontal craniectomy and cranioplasty were performed in 12/2021, with pathology showing a fibrous pseudocyst wall with associated lymphoplasmacytic inflammation and evidence of prior hemorrhage with Ki-67 in the cyst wall at 15%. Lab work showed chronic alkaline phosphatase elevation at 567IU/L (42-106IU/L), normal calcium at 9.2mg/dl (8.4-10.3mg/dl), phosphate at 3.3mg/dl (3.3-5.1mg/dl), and PTH at 26pg/ml (15-65mg/dl), with Vitamin D insufficiency at 25.1ng/ml (30-100ng/ml). Endocrinology workup was negative for thyroid, adrenal, gonadotrophic, and GH dysfunction. 5 months post-surgery, the patient demonstrated complete recovery, and MRI showed no recurrence of the ABC. Conclusion: ABC is a benign solitary tumor generally arising from the metaphysis of long bones and rarely involves the cranium. ABC associated with FD has only been reported in a few cases. The treatment is surgical excision, with radiation and cryotherapy used in nonorbital cases.
Presentation: Thursday, June 15, 2023
THU457 Variable Presentations Of Hypophosphatasia In Two Patients In The Outpatient Setting
Authors: Elmaaz Ahmed , Batool Syeda Sabeeka , Dandona Paresh 
Published: October 2023

Abstract
Disclosure: A. Elmaaz: None. S. Batool: None. P. Dandona: None.
Introduction: Hypophosphatasia is a rare disorder characterized by defective mineralization of bone and teeth which is caused by an autosomal inherited mutation in the tissue nonspecific alkaline phosphatase (ALP) encoding genes. Here we present two cases of multiple dental and bony defects secondary to low ALP enzyme activity due to hypophosphatasia. Case 1: A 27-year-old female with history of mitochondrial disease, gastroparesis, malabsorption syndrome, and autonomic dysfunction was referred to Endocrinology clinic because of persistently low serum ALP levels associated with recurrent muscle, joint, and bone pain since the age of 13. She had history of multiple tooth extractions because of dental developmental abnormalities and fractures. She also reported history of fractures of three of her fingers in both hands during her childhood and teenage years. The patient was adopted and was unaware of any biological family history. Physical examination was remarkable for short-statured female, 4'8". Notably, the middle phalanges in little fingers bilaterally were shorter than the other fingers. Workup showed vitamin D level of 21 (30-80) ng/ml, despite being on 50,000 IU twice a week for 6 months with normal PTH and ionized calcium levels. The repeated serum level of ALP was 24 (31-125) U/L with bone and liver isoenzymes levels of 42% and 58 % respectively. Vitamin B6 level was 13.2 (2.1-21.7) ng/ml. Bone mineral density was unremarkable. Case 2: A 53-year-old female with history of congenital left hip dysplasia, hypertension, and dyslipidemia was seen by Endocrinology service for evaluation of decreased ALP levels. She denied any history of falls or fractures. However, she reported chronic bone pain, especially in bilateral hip joints with history of multiple surgeries in her left hip joint. She had required teeth braces and multiple dental surgeries during her childhood due to dental maldevelopment. Her height was 4'8". Laboratory workup was remarkable for low serum ALP level of 30 U/L with bone isoenzyme of 24 %. Vitamin B6 level was 206.7 (2.1-21.7) ng/ml. She had elevated serum phosphorus levels with normal calcium, PTH, and vitamin D levels. Pyridoxal-5- Phosphate level was high. Bone mineral density revealed osteopenia. Conclusion: Hypophosphatasia is a rare disease, hence it could be easily missed due to high variability in clinical presentations and severity of symptoms. Low serum levels of ALP with history of bone pain, recurrent fractures, premature loss of deciduous teeth, or loss of adult dentition should raise the suspicion of hypophosphatasia. Resistance to vitamin D supplementation is another clue to the diagnosis. A multidisciplinary approach is required for appropriate management.
Presentation: Thursday, June 15, 2023
THU458 Demineralization Zebras: A Rare Case Of Hypophosphatasia In An Adult Presenting With Osteopenia
Authors: Ramchandani Bhanvi Prakash , Khan Aysha , Aftab Hassaan 
Published: October 2023

Abstract
Disclosure: B.P. Ramchandani: None. A. Khan: None. H. Aftab: None.
Introduction: Hypophosphatasia (HPP) is a rare genetic bone disease with only 500-600 cases reported in the United States. It is characterized by mutations in the tissue non-specific alkaline phosphatase (TNSALP) gene, leading to impaired bone mineralization. HPP presenting in the perinatal period is fatal. During infancy, rachitic deformities develop by age 6 months. Children present with early loss of deciduous teeth and delayed milestones while in adulthood, HPP is characterized by recurrent fractures, osteoporosis, and muscle weakness. We describe a case of HPP diagnosed in a patient after low ALP was discovered on laboratory testing. Case: A 69-year-old female presented for the evaluation of osteopenia. She had a history of fracture of the right knee as well as fracture of the base of the right 5th metatarsal and lateral aspect cuboid, which healed well with conservative management. Dental history was significant for 5 implants over the last 15 years. She was on vitamin D supplements and had also taken risedronate for osteopenia for 14 years which was discontinued at her first visit with us. Family history was significant for osteoporosis in her mother. Initial lab studies were significant for an ALP of 29 U/L (ref range 39-113 U/L), phosphate of 4.9 mg/d (ref range 2.4-4.8 mg/dl), pyridoxal 5’-phosphate level (vitamin B6) of 308 nmol/L (ref range 20-125 nmol/l), a vitamin D 25-OH level of 20 ng/mL (ref range &gt;30 ng/ml) and PTH of 49 pg/dl (ref range 15-88 pg/ml). Repeat lab testing done 6 months after stopping risedronate showed consistent similar results with an ALP of 32 U/L, phosphate level of 5.1 mg/dl, vitamin B6 level of 349 nmol/L, and vitamin D 25OH level of 26 ng/mL. Bone density scan revealed a lumbar spine BMD of 1.503 g/cm2 (T-score 2.5) and a mean total hip BMD of -0.3 consistent with osteoporosis of spine and osteopenia of hip. Genetic testing and counseling were offered to the patient, however she declined. Discussion: Our patient presented with recurrent fractures and loss of dentition requiring multiple dental implants in the background of osteopenia. Given persistently low serum ALP and elevated serum vitamin B6 levels her presentation suggested a diagnosis of HPP. Management includes supportive treatment with prompt treatment of fractures, and routine dental care. Although there is no approved medication for adults, enzyme therapy replacement with asfotase-alfa (a recombinant human ALP) is FDA-approved for pediatric-onset HPP which improves bone mineralization and muscle strength, however discontinuation of therapy leads to reappearance of bone hypomineralization. Once the diagnosis is made, genetic testing and counseling should be offered to prevent complications in future pregnancies.
Presentation: Thursday, June 15, 2023