bluuebunny/crossref_metadata_2025_split huggingface dataset:
PSAT193 Skeletal Fluorosis due to Inhalant Abuse
Authors: Pedraza Lina , Pesantez Maria Jose , Mulukutla Surya 
Published: November 2022

Abstract

Introduction
Skeletal fluorosis (SF) is a rare toxic osteopathy characterized by massive bone fixation of fluoride. It is prevalent in India, China, and Africa due to fluoride water contamination, high consumption of brick tea, or even fluoride air pollution in industrialized areas.
In the United States, it does not constitute a public health issue; however, it has been found recently in individuals who inhale high amounts of recreational fluoride. The mechanism of fluoride toxicity on the bone is attributed to its anabolic effect, and patients usually present with diffuse and chronic bone pain, non-vertebral fractures, and hyperparathyroidism.

Case
We present the case of a 39-year-old woman, who presented with bone pain and joint stiffness leading to impaired gait. She was previously seen by neurology, rheumatology, and oncology due to concern for neuropathy, rheumatoid arthritis, or possible occult malignancy but was referred after an inconclusive workup.
Her past medical history was significant for a motor vehicle accident resulting in chronic back pain. She began using painkillers, but eventually, she turned to inhalants to help with pain management and depressive symptoms. Her inhalant of choice was keyboard cleaner. Slowly, she began noticing diffuse bone pain, muscle weakness, and difficulty walking that required her to use crutches. She continued to abuse inhalants 2-4 cans per day for about 5 years until March 2021. Physical exam was relevant for multiple bony prominences bilaterally on the elbows and thoracic spine. She also had a diminished range of motion of her 4 limbs, being unable to fully extend arms or legs. Lab evaluation showed elevated alkaline phosphatase 971, normal AST/ALT, vitamin-D 52, normal serum calcium and PTH, elevated random urine fluoride 23 mg/L (&lt;3.2), elevated osteocalcin 292ng/ml (&lt;32), and elevated N-telopeptide 2283 BCE/mmol cr (&lt;64). Upper extremity x-rays showed evidence of diffuse mature periosteal reaction involving the proximal humerus, radius, ulna, and metacarpals and prominent medial and lateral epicondylar enthesopathy consistent with fluorosis.

Discussion
Skeletal fluorosis should be suspected in patients at risk for fluoride inhalation. Occupational exposure including huffing was key in this case. Diagnosis was confirmed by the detection of excess fluoride in the blood and urine.
Currently, the only accepted treatment is to stop further ingestion or inhalation of fluoride. The half-life of retained fluoride in the bone is about 7 years. Unfortunately, there is no antidote for SF. One theoretical therapy is teriparatide, which might accelerate bone turnover and release skeletal fluoride. Further research is needed to identify more treatment options for patients diagnosed with this condition as the substance abuse crisis worsens in many developed countries. This case also emphasizes the importance of a thorough history prior to undertaking costly, time-consuming medical workups.
Presentation: Saturday, June 11, 2022 1:00 p.m. - 3:00 p.m.
PSAT198 Atypical Parathyroid Adenoma is an Unusual Cause of Primary Hyperparathyroidism
Authors: Limon Makarem , Ali Mariam Murtaza , Banisaeed Nassim , Shannon Shannon , Sharma Arun , Jakoby Michael 
Published: November 2022

Abstract

Background
Atypical parathyroid adenomas (APA) are considered tumors of uncertain malignant potential and have a biochemical and clinical phenotype that is generally more aggressive than typical parathyroid adenomas (PA). Low incidence as an etiology of primary hyperparathyroidism (PHPT) presents a challenge in understanding the pathological features and natural history of APA. We present a case of PHPT due to an APA and review the published literature for these tumors.

Case
A 37 year-old male was referred for evaluation of hypercalcemia, with serum calcium level 13.6 mg/dL (8.6-10.3). Repeat serum calcium measurement was 12.7 mg/dL, and two measurements of intact PTH were 870 pg/mL and 919 pg/mL (12-88), respectively. The patient's laboratories raised concern for parathyroid carcinoma (PC), but there was no palpable neck mass and no enlarged parathyroid glands were visualized on ultrasonography. Parathyroid scintigraphy was notable for faint but persistent Tc-99 m sestamibi uptake in the left inferior neck. Additional preoperative evaluation revealed significantly reduced bone mineral density in the distal third of the radius (0.638 g/cm2, Z-score -3.2), silent nephrolithiasis of the right kidney, and vitamin D deficiency (25-OH vitamin D 13.4 ng/dL, 30-100). An unequivocally enlarged (175 mg, 2.5 cm in largest diameter) left inferior parathyroid gland was removed, with the remaining glands unremarkable by inspection. Extensive nuclear atypia and cellular pleomorphism indicated an APA, and invasive features of PC were absent. One month after surgery, serum calcium improved to 10.2 mg/dL and intact PTH was 40 pg/mL.

Conclusions
APA account for 1-2% of PHPT cases. This patient's case has several features that distinguish it from a PA including degrees of preoperative hypercalcemia and intact PTH elevation, low vitamin D level, significantly reduced bone density in the distal third of the radius, nephrolithiasis, relatively young age, and male gender. Fibrous bands, pseudocapsular invasion, and trabecular growth pattern are the most common histologic features of APA, but nuclear atypia and cellular pleomorphism are well established diagnostic criteria. APA may be biochemically similar to PC but are distinguished from PC by the absence of gross invasion into adjacent anatomic structures, distant metastases, and vascular or perineural invasion. Additionally, CDC73 mutations are present in nearly all cases of PC but not in APA. Over 90% of patients with APA are successfully treated by parathyroidectomy. Careful follow up of APA patients is recommended, though there are no specific guidelines regarding a minimum satisfactory duration of postsurgical monitoring.
Presentation: Saturday, June 11, 2022 1:00 p.m. - 3:00 p.m.
PSAT211 Low Grade Parathyroid Carcinoma: A Challenging Diagnosis in the Evaluation of Primary Hyperparathyroidism
Authors: Samuel Dennis , Augustine Marilyn 
Published: November 2022

Abstract

Background
Parathyroid carcinoma often presents with severe symptomatic hypercalcemia, bone and kidney involvement, and PTH &gt; 4x the upper limit of normal. Diagnosis is challenging and requires classic pathologic findings.

Case Presentation
A 34-year-old woman presented with hypercalcemia. She was found to have primary hyperparathyroidism with elevated total calcium (11.8 mg/dL), elevated PTH (352.8 pg/mL), and elevated 24-hour urine calcium (758 mg/24hr). A 99mTc sestamibi scan showed focal uptake in the right inferior thyroid pole. Intraoperatively, there was a lobulated, fibrotic right parathyroid mass sized 3.5×2.5×2.0 cm. Pathology showed low-grade parathyroid carcinoma with lymphovascular invasion, extension into soft tissue, and focal positivity in resection margin. Post-operative PTH was 19.5 pg/mL. One month later, PTH was elevated to 82.0 pg/mL with calcium of 9.1 mg/dL. PET scan did not show metastases. The patient then underwent a right thyroid lobectomy with central compartment lymph node dissection. Pathology showed residual parathyroid carcinoma within the perithyroidal scar tissue but was negative for lymph node involvement. Postoperatively, her PTH was 52.5 pg/mL. Genetic studies showed no mutations in CDC73, MEN1, RET, RB1, and TP53 genes. She underwent external beam radiation treatment. Following radiation, her total calcium and PTH level remained in the normal range with no recurrence on thyroid ultrasound.

Clinical Lessons
On ultrasound, parathyroid carcinomas are large, have increased heterogeneity and lobulations, as well as suspicious lymph nodes. Intraoperative suspicion is based on large size, capsular or local tissue invasion, and lymph node metastases. Histologic assessment can be challenging as findings of parenchymal mitoses, trabeculated parenchyma, and thick fibrous bands are also seen with parathyroid adenomas. Local tissue invasion (angiolymphatic, perineural or adjacent structures) or metastases such as lymph nodes seen on pathology are diagnostic of parathyroid carcinoma. PET can be used post-operatively to identify recurrence or metastases. Genetic studies especially for mutations in CDC73 can be helpful in diagnosis as it is the most common cause of syndromic and sporadic parathyroid carcinoma.Resection of the tumor and adjacent tissues is the mainstay of treatment. A regional lymph node dissection should be done if there are suspicious lymph nodes. Neither thyroidectomy, chemotherapy nor radiation has been shown to improve overall survival. Radiation has shown to have some benefit for local recurrence and disease-free survival. Lifelong monitoring of calcium and PTH levels is required, and if elevated, should prompt imaging with thyroid ultrasound and PET scan. Prognosis is generally poor but does improve if there is complete surgical resection on initial diagnosis. Almost all patients experience recurrence within 2-23 years after resection
Presentation: Saturday, June 11, 2022 1:00 p.m. - 3:00 p.m.
PSAT213 Intrathyroidal Parathyroid Adenoma Diagnosed on Thyroseq v2
Authors: Tsai Karen , Gofnung Yaroslav , Livhits Masha 
Published: November 2022

Abstract

Background
The incidence of intrathyroidal parathyroid adenoma varies from &lt;1% to 6% and can be a diagnostic dilemma. Intrathyroidal parathyroid adenomas can have suspicious sonographic features that prompt fine needle aspiration (FNA) to evaluate for thyroid cancer. Differentiation between thyroid and parathyroid tissue on cytology can be difficult, and these lesions may be diagnosed as indeterminate thyroid nodules. Molecular testing is a useful adjunct that allows for identification of parathyroid adenomas in this setting.

Clinical Case
A 59-year-old female with osteopenia and a recent history of traumatic vertebral fracture presented for workup of asymptomatic hypercalcemia. Labs showed intact parathyroid hormone (iPTH) 168 pg/mL (reference 11-51 pg/mL), serum total calcium 12.2 mg/dL (reference range 8.6-10.4 mg/dL), albumin 4.7 g/dL (reference 4.1-5.3 g/dL), phosphorus 2.1 mg/dL (reference 2.3-4.4 mg/dL), creatinine 0.7 (reference 0.6-1.3 mg/dL), and 25-OH vitamin of 28 ng/mL (reference 20-50 ng/mL). Cervical ultrasound showed a 2.1 cm hypoechoic left thyroid nodule. Subsequent 99mTc-Sesatmibi scan showed increased tracer retention in the left posterior thyroid lobe. FNA of the left thyroid nodule was performed with cytology showing follicular lesion of undetermined significance (FLUS). Molecular testing with Thyroseq v2 showed parathyroid tissue consistent with intrathyroidal parathyroid adenoma. The patient underwent successful left thyroid lobectomy with removal of an intrathyroidal parathyroid gland, with subsequent decrease of iPTH to 44 pg/mL (reference 11-51 pg/mL) and total serum calcium to 9.5 mg/dL (reference 8.6-10.4 mg/dL). Surgical pathology confirmed parathyroid adenoma.

Discussion
Due to the ubiquity of thyroid FNAs to rule out malignancy, there has been a rise in the number of incidental parathyroid gland aspirations. Thyroid lesions with diagnosis of FLUS or oncocytic features are prone to misdiagnosis due to similarities in morphologic overlap and anatomic proximity. Molecular testing such as Thyroseq v2 routinely tests for PTH and can identify parathyroid tissue in lesions initially diagnosed as indeterminate thyroid nodules. Newer molecular test versions including ThyroSeq v3 similarly have high analytical accuracy in detecting thyroid cancer and parathyroid lesions.
Presentation: Saturday, June 11, 2022 1:00 p.m. - 3:00 p.m.
PSAT214 Management of Hyperparathyroidism Associated with Severe Hypercalcemia Presenting During Labour
Authors: Mendez Juan Vasquez , Goulden Peter , Nassar Rawann 
Published: November 2022

Abstract

Background
Hypercalcemia is a rare pathology in pregnancy, and primary hyperparathyroidism (PHPT) has been described as the most common cause. No guidelines exist concerning treatment of PHPT during pregnancy, although most authors favor surgery as the treatment of choice during the second trimester. Nonetheless, management during the third trimester of pregnancy is challenging due to lack of current guidelines.

Clinical case
This 29-year pregnant female presenting at 38-weeks gestation with spontaneous rupture of membranes and admitted for latent labor. She was incidentally found to have hypercalcemia. Endocrinology was consulted for asymptomatic hypercalcemia in the third trimester of pregnancy. A diagnosis of primary hyperparathyroidism (PHPT) was suspected based on elevated serum calcium adjusted for albumin (16 mg/dL, normal range 8.5-10.5 mg/dL) and elevated PTH levels (121 pg/mL, normal range 14-65 pg/mL). In addition, parathyroid hormone related peptide (PTHrP) was less than 2.0 pmol/L (normal range &lt;2.0 pmol/L), 25-hydroxy vitamin D was 35.6 ng/mL (normal range 30-50 ng/mL) and urine calcium/creatinine clearance ratio (CCCR) level was 0.02 (&lt;0.01 familial hypocalciuric hypercalcemia is likely, &gt;0.02 PHPT is likely) further supporting the diagnosis of PHPT. No parathyroid imaging was performed as there was no plan for surgical intervention at that time. Conservative management was initiated with aggressive oral and intravenous fluid rehydration. The patient subsequently had an uneventful vaginal delivery with the arrival of a healthy newborn eight hours after treatment was initiated. With continued IV and oral hydration, corrected calcium fell by a total of 5 mg/dL. Given an uneventful postpartum course as well as improving calcium levels with hydration, the patient was discharged on day three of hospitalization with a plan for outpatient endocrinology follow up in preparation for future parathyroidectomy.

Conclusion
There is no previous case report describing severe hypercalcemia in the third trimester of pregnancy managed conservatively solely with hydration. Calcitonin has been attempted but use is limited secondary to tachyphylaxis. Additionally, some case reports suggest cinacalcet use but this drug crosses the placenta. In this unique and complicated case of severe hypercalcemia diagnosed in the third trimester of pregnancy, conservative management with intensive oral and intravenous fluid rehydration was safe and effective in lowering serum calcium levels and therefore conducive to a positive pregnancy outcome.
Presentation: Saturday, June 11, 2022 1:00 p.m. - 3:00 p.m.
PSAT217 Estrogen Deficiency as a Cause of Decreased 1- alpha - hydroxylase Activity
Authors: Sviderskaya Tatiana 
Published: November 2022

Abstract

Case description
A 40 year old woman receiving cholecalciferol therapy -10,000 IU, and alfacalcidol 1 mcg per day showed a 25 (OH) D increase to 351. 1 ng/mL (normal 65-100) with a normally high parathormone level of 33.5 pg/mL (normal 15-65). Clinically, symptoms of vitamin D deficiency - decreased immunity, decreased muscle mass and strength, depression, and symptoms of estrogen deficiency (hot flashes, scanty menstruation, hair loss) persisted against a high 25(OH)D background, so estrogen determination was performed in the steroid profile of saliva. Estradiol content was decreased - 3.45 pg/ml (norm 4.17 - 9.28), in connection with which transdermal estrogen therapy in a dose of 3.0 g was prescribed. Against the background of estrogen therapy, parathormone decreased from 33.5 to 22.4 pg/ml.
Determination of 25(OH)D and estradiol in saliva was performed by mass spectrometry, determination of parathormone by ELISA.

Discussion
Vitamin D status depends on several factors, in particular on the activity of the enzyme 1 - alpha-hydroxylase (CYP27B1) in the kidney, which converts 25 (OH) D into the active form (1,25 - (OH)2 - D), which in turn depends on both parathormone and sex steroids (estrogen and androgens), determination of which is not a routine practice in vitamin D deficient patients. Elevated parathormone levels are considered a marker of vitamin D deficiency (secondary hyperparathyroidism). Decreased parathormone levels are considered a laboratory manifestation of vitamin D overdose.
In this patient, there was no decrease in parathormone below the differential level of 25(OH)D in the background of high levels.
Sex steroids increase the synthesis of calcitriol (D hormone, dihydrocholecalciferol 1,25 - (OH)2 - D) by activating the enzyme 1 - alpha - hydroxylase (CYP27B1) in the kidneys. In particular, estradiol deficiency exacerbates vitamin D deficiency.

Conclusion
Determination of 25(OH)D levels in patients with decreased 1 -alpha-hydroxylase activity leads to hypodiagnosis of vitamin D deficiency. In all patients with high 25(OH)D levels, the determination of parathormone as well as estrogens in saliva is necessary.
Presentation: Saturday, June 11, 2022 1:00 p.m. - 3:00 p.m.
PSAT226 Management of a Patient with Primary Hyperparathyroidism by Discontinuation of Hydrochlorothiazide: Preventing the Need for Surgical Management
Authors: Romao Isabela J , Shanik Michael Howard , Velayati Sara 
Published: November 2022

Abstract

Introduction
Recurrent or persistent primary hyperparathyroidism has been reported in 2-5% of patients after the initial operation. Reoperations have been associated with significant complications. The aim of this presentation is to highlight the importance of medical management prior to proceeding to surgery.

Case presentation
A 72-year-old man with a past medical history of coronary artery disease and asthma was found to have hypercalcemia and osteoporosis and was diagnosed with primary hyperparathyroidism in 2009. He had no family history of hypercalcemia. Three parathyroid glands were removed in 2010 and his parathyroid hormone normalized immediately after the surgery. About 10 years after the surgery, he developed hypertension and calcium-type kidney stones. He was treated with losartan 50 mg daily and hydrochlorothiazide 12.5 mg daily. One year later his calcium level was 11.1 mg/dL, parathyroid hormone (PTH) 70 pg/mL, 25-OH vitamin D level 44 ng/mL and creatinine was 0.8 mg/dL. A CT scan demonstrated a lesion measuring 1.3×1.1×1.6 cm which was suspicious for an ectopic parathyroid adenoma in the left ascending aorta. He was scheduled for surgical exploration and removal of the parathyroid adenoma for recurrence of primary hyperparathyroidism. Prior to the surgery, the patient was evaluated in our office for a second opinion. Physical exam and review of systems were unremarkable. Thyroid ultrasound showed a heterogeneous thyroid with no thyroid nodules or parathyroid adenoma. Adequate hydration was recommended and since his blood pressure was well-controlled, hydrochlorothiazide was discontinued. Surgery was canceled to confirm that the hypercalcemia was due to hydrochlorothiazide rather than hyperparathyroidism. After 4 months of follow-up, his blood pressure remained controlled on monotherapy with Losartan, and the repeat calcium level was 9.6 mg/dL with a PTH level of 44 pg/mL.

Conclusion
Post-surgical patients with a history of primary hyperparathyroidism, when presenting with hypercalcemia can get misdiagnosed as recurrent or persistent primary hyperparathyroidism; therefore, other causes of hypercalcemia including medications that may affect the calcium level and its metabolism should be considered and excluded prior to surgical management.
Presentation: Saturday, June 11, 2022 1:00 p.m. - 3:00 p.m.
PSUN76 Massive Four-Gland Parathyroid Hyperplasia: The Case of the Missing Glands
Authors: Nicolich-Henkin Sophie , Goldstein Michael , Rothberger Gary 
Published: November 2022

Abstract

Introduction
Parathyroid adenoma (PA) and parathyroid hyperplasia (PH) are common causes of primary hyperparathyroidism (PHPT) for which the only definitive treatment is surgical removal. Surgery is often preceded by imaging for localization, including by ultrasound (US), Sestamibi scan (MIBI), positron emission tomography/computer tomography (PET/CT), and four-dimensional computed tomography (4D-CT). While it is common for parathyroid pathology to not be visualized on imaging, non-visualization is more typical of adenomas and hyperplasia of smaller size.

Clinical Case
A 65-year-old male without history of kidney dysfunction or family history of parathyroid disease was referred to surgery for parathyroidectomy to manage longstanding PHPT. Serology showed a parathyroid hormone (PTH) level of 581 pg/mL (reference range: 12-65 pg/mL) and calcium of 11.8 mg/dL (reference range: 8.6-10.5 mg/dL). Preoperative parathyroid localization US revealed a large well-defined 1.5 cm PA. The patient underwent parathyroidectomy, which detected a large right inferior parathyroid gland, confirmed with pathology to be an atypical parathyroid adenoma weighing 1800 mg. Postoperative PTH level was 550 pg/mL and calcium was 12.6 mg/dL. MIBI did not display any definite parathyroid adenoma or ectopic parathyroid tissue. Further evaluation with PET/CT did not demonstrate any evidence of metabolically active residual or ectopic parathyroid adenoma or metastatic disease, and repeat US showed did not reveal evidence of any abnormal parathyroid tissue. Ultimately, 4D-CT detected a multiglandular parathyroid with a 1.8×1.4×3.0 cm right superior adenoma weighing approximately 3931 mg, 1.2×0.8×2.1cm left superior adenoma weighing approximately 1048 mg, and a 0.3×0.4×0.4cm left superior adenoma weighing approximately 25 mg. The patient underwent re-exploration parathyroidectomy with removal of the right superior adenoma weighing 3105 mg and left superior adenoma weighing 1100 mg. The remaining left inferior gland was biopsied and the majority of it was implanted into the arm. Repeat serology one week post-operatively showed improvement of PTH level to 5 pg/mL and of calcium to 8.9 mg/dL and serology one year post-operatively showed a PTH level of 82 pg/mL and a calcium of 9.4 mg/dL.

Conclusion
We present a patient with massive four-gland PH who initially presented with findings more typical of PA, but ultimately had additional hyperplasic glands that were identified after removal of the adenomatous gland. These glands were unusually large to be seen in PH and yet still be undetected on multiple imaging modalities; no previously reported non-ectopic hyperplastic glands of this size have been documented that were not detected on US or MIBI.
Presentation: Sunday, June 12, 2022 12:30 p.m. - 2:30 p.m.