bluuebunny/crossref_metadata_2025_split huggingface dataset:
FRI672 Bone Mineral Density In Neurofibromatosis Type 1: A Systematic Review And Meta-analysis
Authors: Charoenngam Nipith , Wattanachayakul Phuuwadith , Jaroenlapnopparat Aunchalee , Ungprasert Patompong , Chenbhanich Jirat 
Published: October 2023

Abstract
Disclosure: N. Charoenngam: None. P. Wattanachayakul: None. A. Jaroenlapnopparat: None. P. Ungprasert: None. J. Chenbhanich: None.
Neurofibromatosis type 1 (NF1) is known to be associated with low bone mineral density (BMD) and fragility fractures. However, evidence supporting specific age of early BMD measurement is limited as data on the degree of bone loss in NF1 are lacking. Using systematic review and meta-analysis technique, we aimed to identify all available data to assess BMD in patients with NF1. Potentially eligible studies were identified from Medline and EMBASE databases from inception to February 2023 using search strategy that comprised of terms for “Bone mineral density” and "Neurofibromatosis type 1”. Eligible study must include adult or pediatric patients with NF1. The study must report mean Z-score with variance of total body, lumbar spine, femoral neck or total hip BMD of the studied patients. Point estimates with standard errors were retrieved from each study and were combined using the generic inverse variance method. A total of 1,165 articles were identified. After systematic review, 19 studies were included. The majority of participants in the included studies were children and younger adults with the mean age of participants lower than 50 years old in all but one study. The meta-analysis revealed that patients with NF1 had negative mean Z-scores for total body BMD (pooled mean Z-score -0.808; 95%CI, -1.025 - -0.591) and BMD at lumbar spine (pooled mean Z-score -1.104; 95%CI, -1.376 - -0.833), femoral neck (pooled mean Z-score -0.726; 95%CI, -0.893 - -0.560) and total hip (pooled mean Z-score -1.126; 95%CI, -2.078 - -0.173). The subgroup meta-analysis in pediatric patients aged &lt;18 years revealed that patients with NF1 had negative mean Z-scores for lumbar spine BMD (pooled mean Z-score -0.938; 95%CI, -1.299 - -0.577) and femoral neck BMD (pooled mean Z-score -0.585; 95%CI, -0.872 - -0.298). Based on the results from the meta-analysis, patients with NF1 had low Z-scores. However, the lower limits of the 95% CIs for the pooled mean Z-score for total body and specific site BMD were all higher than the Z-score cut-off of -2 for “low BMD for chronological age” in children and adults aged &lt;50 years old. Therefore, the degree of low BMD may not be of clinical significance. Our results do not support the role of early BMD screening in children and young adults with NF1.
Presentation: Friday, June 16, 2023
FRI673 REMS Technology For The Evaluation Of BMD In Hypothyroidism
Authors: Lombardi Fiorella Anna , Pisani Paola , Quezada Alba Paola , Muratore Maurizio , Di Paola Marco , Casciaro Ernesto , Conversano Francesco , Casciaro Sergio 
Published: October 2023

Abstract
Disclosure: F.A. Lombardi: None. P. Pisani*: None. A.P. Quezada*: None. M. Muratore*: None. M. Di Paola*: None. E. Casciaro*: None. F. Conversano*: None. S. Casciaro: None. *Equal contributors in alphabetical order
Thyroid hormones play a crucial role in skeletal development, adult bone turnover, and bone maintenance, but they can also have detrimental effects on bone formation when there is thyroid dysfunction. Thyrotoxicosis (hyperthyroidism), including subclinical illness, can affect bone metabolism, resulting in decreased Bone Mineral Density (BMD) and increased fractures risk, and is a significant contributor to secondary osteoporosis, especially in postmenopausal women. On the other hand, little is known about the impact of overt or subclinical hypothyroidism on bone metabolism. However, hypothyroidism lessens bone turnover, reducing both osteoclastic bone resorption and osteoblastic activity. Such alterations in bone metabolism could potentially result in an increase in bone mineralization, although data on BMD and the risk of fracture in hypothyroidism are lacking and conflicting. Radiofrequency Echographic Multi-spectrometry (REMS) is a novel radiation-free technology for assessing BMD and fracture risk. In this context, the goal of this study was to assess BMD using REMS in patients with and without hypothyroidism, in order to look a possible correlation between the disease, BMD loss and fracture risk. Methods: A sample of 51 Caucasian females with hypothyroidism was compared to a reference group of healthy women who were matched for gender, ethnicity, age, and body mass index (BMI). Using REMS technology, the BMD was measurement at the lumbar spine in all participants at the study. To determine whether the two groups’ lumbar spine BMD value varied, a t-test was run. Results: Patients with and without hypothyroidism were matched in order to get similar anthropometric parameters. In patient with and without hypothyroidism mean ± standard deviation (SD) for age and BMI are (64.8 ± 10.2, 65.2 ± 9.8) and (25.1 ± 3.6, 25.0 ± 3.7), respectively. Patients with hypothyroidism were shown to have a significative lumbar spine BMD difference (0.801 g/cm2 ± 0.063 vs 0.831 g/cm2 ± 0.071, p=0.03) respect to the healthy subjects. Conclusions: Although just a small number of hypothyroidism patients are included in this investigation, without any indication of the drug therapy and its effect, it has been shown that, as compared to controls, women with hypothyroidism have a net decline in REMS-measured BMD values. Based on this thought, REMS technology, thanks to its precision and diagnostic accuracy, might improve the efficient care and follow-up programmes of these patients.
Presentation: Friday, June 16, 2023
FRI680 Atypical Femoral Fracture In Patients With Hypophosphatasia: A Systematic Review
Authors: Charoenngam Nipith , Thongpiya Jerapas , Yingchoncharoen Pitchaporn , Ponvilawan Ben , Marangoz Mehmet Sercan , Chenbhanich Jirat , Ungprasert Patompong 
Published: October 2023

Abstract
Disclosure: N. Charoenngam: None. J. Thongpiya: None. P. Yingchoncharoen: None. B. Ponvilawan: None. M.S. Marangoz: None. J. Chenbhanich: None. P. Ungprasert: None.
A growing number of case reports and case series have demonstrated that atypical femoral fracture (AFF) can occur in patients with hypophosphatasia (HPP). Due to the lack of summary of the clinical manifestation, risk factors, associated ALPL variants and treatment, we conducted this systematic review with the goal to summarize the characteristics of all reported patients with HPP who sustained AFF. Additionally, we aimed to identify all available evidence to quantify the rate of co-existence between HPP and AFF. Potentially eligible articles were identified from MEDLINE and EMBASE databases from inception to September 2022 using search strategy consisting of terms related to “Hypophosphatasia” and “Atypical femoral fracture”. Eligible articles must report one of the following information: 1.) individual data of patient(s) diagnosed with HPP and AFF, 2.) prevalence of HPP among patients with AFF or 3.) prevalence of AFF among patients of HPP. Characteristics of patients reported in each study were extracted. A total of 148 potentially eligible articles were identified from the databases. After systematic review, 23 articles met the eligibility criteria. A total of 27 patients with AFF and HPP were identified. The mean ± SD age of the reported patients was 53.8 ± 12.7 years and 21 patients (77.8%) were female. Nine patients (33.3%) received antiresorptive medication (bisphosphonate and/or denosumab) and two patients (7.4%) received teriparatide prior to development of AFF. Seven (25.9%) and 17 (63.0%) patients sustained unilateral and bilateral AFF, respectively. Twelve patients (44.4%) had a history of fractures at other sites. Four (14.8%) and six (21.4%) patients received asfotase alfa and teriparatide after sustaining AFF. Two studies that reported the prevalence of AFF among patients with HPP of approximately 10%. One study reported one HPP patient in a cohort of 72 patients with AFF. In summary, based on the limited evidence, AFF occurred in up to 10% of patients with HPP. Based on the 27 case reports, almost two-thirds did not receive antiresorptive treatment, suggesting the HPP itself could potentially be a risk factor for AFF.
Presentation: Friday, June 16, 2023
FRI684 So Strong Yet So Fragile: Bilateral Periprosthetic Fractures On Denosumab
Authors: Syeda Asma , Mirza Faryal Sardar 
Published: October 2023

Abstract
Disclosure: A. Syeda: None. F.S. Mirza: None.
Background: Denosumab (DN) is a potent antiresorptive and has been associated rarely with atypical femoral fractures. We hereby report the first case of simultaneous bilateral femoral periprosthetic fractures (PPF) while on DN therapy (DNT). Clinical Case: A 78-year-old female with severe osteoporosis on DNT presented in January 2023 with bilateral femoral PPF after a mechanical fall whereby she tripped and landed on her knees. The patient had initially presented to us in late 2012. Treatment history included alendronate (ALN) for 5 years, on drug holiday since 2010. She underwent bilateral knee replacement surgeries in 2012 and DNT was started in 2013 after initial evaluation. She also had primary biliary cirrhosis, and although bone density (BD) improved on DNT, she exhibited escape from DN mediated suppression of bone resorption possibly related to the liver disease that was previously reported (1). DNT was stopped 5 years later when spine BD improved to osteopenic range and ALN 70 mg weekly was started. 1 year later, bone resorption markers were still elevated and zoledronic acid was substituted for ALN. She fell and had a pubic ramus fracture 11 months later. BD showed a 6% decrease for mean total hip and baseline forearm BD showed T-score of -3.7 on the right. DNT was restarted in December 2020 and continued every 6 months. Her health continued to deteriorate over next two years, with increase in LFTS, new anemia and thrombocytopenia due to a combination of myelodysplastic syndrome and splenomegaly. She also needed transcatheter aortic valve replacement for worsening CHF. She tripped and fell in January 2023. X-ray showed acute supracondylar bilateral PPF with comminution. She was high risk for anesthesia and anticoagulation was withheld due to a history of thrombocytopenia and gastrointestinal bleeding. Prior to the anticipated orthopaedic procedure, the patient developed severe hypotension and a rapid drop in hematocrit due to hemorrhagic shock. The family had requested DNR due to the multitude of medical issues. Conclusion: Occasional PPF have been reported in the setting of DNT, which may reflect the severity of osteoporosis. Altered stress distribution and decreased tensile strength of the bone secondary to screw/pin placement are thought to contribute to PPF, although the occurrence of PPF as an unusual presentation of an AFF cannot be excluded. Contribution of other comorbidities to poor bone quality may also have contributed to the bilateral PPF with poor outcome in our patient.
1. Vedere, T.R. and Mirza, F.S., 2017, April. Escape from Denosumab Mediated Suppression of Bone Resorption in Primary Biliary Cirrhosis. In 99th Annual Meeting of the Endocrine Society. Endocrine Society.
Presentation: Friday, June 16, 2023
FRI687 Clinical Relevance Of 24-hour Urine Calcium Measurement In Patients With Primary Hyperparathyroidism (PHPT)
Authors: Bhan Arti , Simon Rebecca , Jacobsen Gordon , Yaseen Aseel , Navaratnarajah Preyantha , Sweidan Aroob , Rao Sudhaker D 
Published: October 2023

Abstract
Disclosure: A. Bhan: None. R. Simon: None. G. Jacobsen: None. A. Yaseen: None. P. Navaratnarajah: None. A. Sweidan: None. S.D. Rao: None.
Introduction: PHPT is the third most common endocrine disease and the most common cause of hypercalcemia in outpatient setting, presents with mild to moderate hypercalcemia with unsuppressed or elevated serum PTH levels, and most patients are asymptomatic. A 24-hour urine Ca measurement to exclude both hypo- and hypercalciuria is used to recommend parathyroidectomy. The purpose of our study is to evaluate the clinical relevance of urine Ca measurement in evaluating patients with PHPT. Methods: A retrospective electronic medical record review was performed on all adult patients with PHPT in whom a 24-hour urine Ca was measured between 2015-2019. Patients with secondary HPT, those without a 24-hour urine Ca measurement, and patients with serum Cr &gt;1.5 mg/dl were excluded. Results: 393 patients met inclusion criteria for subsequent analyses. For the entire cohort, mean serum Ca was 10.9 ± 0.6 mg/dl, Cr 0.9 ± 0.2 mg/dl and PTH 111± 65 pg/ml with no significant difference between groups by gender, race, or stone history. Mean urine Ca for the entire cohort was 254 ± 170 mg/day. 46% and 35% had urine Ca &gt;250 or &gt;300 mg/d respectively. Multiple linear regression results indicated that higher serum Ca (p=0.004), lower serum Cr (p&lt;0.001), white race (p&lt;0.001), and higher urine sodium (p&lt;0.001) were all significantly predictive of higher UCa. However, the mean urine Ca did not differ between patients with and without nephrolithiasis (276 ± 182 Vs. 280 ± 247 mg/d; p=0.174). Discussion: The most recent guidelines for the management of asymptomatic PHPT recommend measurement of 24-hour urine Ca in all PHPT patients. However, the validity, sensitivity and specificity of such an approach has never been formally evaluated, considering wide variability of urine Ca excretion in the population and lack of difference in urine Ca between patients with and without nephrolithiasis. Previous guidelines discussed this issue and determined that measurement of a 24-hour urine collection is cumbersome and adds very little to patient management. Our study questions the clinical relevance of re-including 24-hour urine Ca in the latest guidelines. Conclusions: Our study suggests that measurement of 24-hour urine Ca in the routine evaluation of patients with PHPT is unhelpful.
Presentation: Friday, June 16, 2023
FRI693 Pthrp Mediated Hypercalcemia In A Patient With Recurrent Hepatocellular Carcinoma
Authors: Bondarenko Darya , Ahmad Mobeen , Mack Margaret , Ucciferro Peter 
Published: October 2023

Abstract
Disclosure: D. Bondarenko: None. M. Ahmad: None. M. Mack: None. P. Ucciferro: None.
Background: Humoral hypercalcemia is seen in 30 percent of cancer patients, most commonly driven by parathyroid hormone related peptide (PTHrP) production, osteolytic cytokine production, and 1,25-OH vitamin D production. The most common malignancies associated with PTHrP mediated hypercalcemia are squamous cell carcinomas and breast cancer. Less often hepatocellular cancer presents with hypercalcemia. We describe the case of a patient presenting with hepatocellular carcinoma presenting with recurrent episodes of severe hypercalcemia due to excessive PTHrP production. Clinical Case: The patient was a 68-year-old male with a past medical history of recurrent hepatocellular carcinoma with liver transplant in the past and type 2 diabetes mellitus who presented to the emergency department (ED) for one day of altered mental status. The patient was noted to be extremely agitated and confused by his home health aide and EMS was called. In the ED the patient’s vital signs were as follows: blood pressure of 196/80 mmHg, heart rate of 82 beats per minute, respiratory rate of 18 breaths per minute and temperature of 36.6 degrees Celsius. Initial lab work showed: calcium 13.5 mg/dL, albumin 4.2 g/dL, ionized calcium of 1.63 mmol/L, creatinine 0.99 mg/dL (baseline 0.5 mg/dL), glucose of 438 mg/dL, bicarbonate 23 mmol/L, anion gap 19, and beta hydroxybutyrate 51 mg/dL. Hypercalcemia workup revealed a suppressed PTH of 5 pg/mL (normal 11-67 pg), normal 25-OH vitamin D 43.6 ng/ml (normal 30-100 ng/ml), normal 1,25-OH vitamin D 38 pg/ml (normal 18-72 pg/ml), and elevated PTHrp 36 pg/ml (normal 11-20 pg/ml). The patient was started on aggressive IV fluid resuscitation with normal saline, administered a one-time dose of zoledronic acid and administered a three-day course of calcitonin. For his significant hyperglycemia the patient was started on an insulin infusion and was eventually transitioned to his home basal bolus insulin regimen. On discharge, the patient was scheduled with his oncologist to begin denosumab as an outpatient; however, he chose a palliative approach due to the terminal nature of his disease and recurrent hospitalizations for hypercalcemia. Conclusion: Hypercalcemia of malignancy is common in many different carcinomas with squamous cell cancer, renal cell cancer, breast cancer and multiple myeloma being commonly associated with this phenomenon. Reports of hepatocellular carcinoma associated with hypercalcemia are less common and occur in only 4-8 % of cases. With this case report, we hope to highlight PTHrP as a potential mechanism of humoral hypercalcemia in patients with hepatocellular carcinoma and propose denosumab as a possible treatment for patients with refractory hypercalcemia of malignancy.
Presentation: Friday, June 16, 2023
FRI697 Severe Hypocalcemia Due To Idiopathic Hypoparathyroidism In An Elderly Female
Authors: Chopra Karishma A. 
Published: October 2023

Abstract
Disclosure: K.A. Chopra: None.
Most cases of acquired hypoparathyroidism in adulthood occur after neck surgery or radiation. However, idiopathic cases, although rare, do occur in elderly patients and should be a diagnosis of exclusion after thorough investigation. 76-year-old female with a history of chronic kidney disease, anemia of chronic disease, and hypertension presented with fatigue. Labs showed calcium (Ca) 5.7(RR 8.4-10.2 mg/dL), albumin 3.5(RR 3.5-5 g/dL), magnesium 2(RR 1.6-2.5 mg/dL), phosphorus 7.8(RR 2.5-4.4 mg/dL), parathyroid hormone 9(RR 15-75 pg/mL), 25-hydroxyvitamin D 32(RR 12-99 ng/mL). Patient denied history of neck surgery or radiation. Patient denied nephrolithiasis, perioral numbness, paresthesias, family history of hypoparathyroidism. Physical exam notable for positive Chvostek sign. Neck ultrasound unremarkable, no evidence of infiltrative disease. Urine Ca was low at 6 mg/24 hour(RR 100-300 mg/24hr). ACE level 10 nmol/ml/min(RR&lt;40 nmol/ml/min) and 1,25 dihydroxyvitamin D level 12(RR 18-78 pg/mL) making sarcoidosis or granulomatous disease unlikely. HIV screen negative and interferon gamma response to mycobacterium tuberculosis antigens was not detected. Ferritin elevated at 300(RR 12-150 ng/mL) but transferrin saturation was 15%(RR 15-55%), not consistent with hemochromatosis. Antiparathyroid antibodies were negative. Patient denied mucocutaneous candidiasis, however testing for underlying autoimmune polyglandular syndrome type 1 (APS type 1) and adrenal insufficiency was completed with cosyntropin stimulation test. Patient had normal response to cosyntropin with baseline cortisol 20.1 (RR 6-18.4 ug/dL) and ACTH 37.7(RR&lt;52 pg/mL), 30 minute cortisol 27(RR 6-18.4 ug/dL) and 60 minute cortisol 37(RR 6-18.4 ug/dL). Patient was treated with Ca gluconate infusion (100 ml of 10% Ca gluconate in 1000 mL of normal saline) at 30 ml/hr and Ca level gradually improved. Once Ca level was greater than 8.0(RR 8.4-10.2 mg/dL), Ca drip was discontinued. Due to patient’s lack of prior neck surgery history, lack of evidence of infiltrative conditions, negative antiparathyroid antibodies, normal adrenal function (unlikely APS type 1), lack of family history, and low urine Ca (unlikely activating mutation of Ca sensing receptor), patient’s hypoparathyroidism was considered idiopathic. Patient was discharged on oral Ca carbonate, Ca acetate, and calcitriol tablets. On follow up, patient had Ca 8.5(RR 8.4-10.2 mg/dL) with symptom improvement. Although hypocalcemia is commonly encountered, it is rare to see severe hypocalcemia in an elderly patient due to hypoparathyroidism without a history of neck surgery or radiation. Therefore, it is vitally important to conduct a thorough investigation to rule out underlying causes before diagnosing idiopathic hypoparathyroidism as highlighted in our case to guide future treatment and optimize patient health.
Presentation: Friday, June 16, 2023
FRI698 Hypoparathyroidism Secondary To Covid-19
Authors: Hussain Mariam , Reddy Tina K , Mohiuddin Arianna , Lovre Dragana , Galagan Robert 
Published: October 2023

Abstract
Disclosure: M. Hussain: None. T.K. Reddy: None. A. Mohiuddin: None. D. Lovre: None. R. Galagan: None.
Introduction: New onset adult hypoparathyroidism is most often caused by thyroid surgery or autoimmune illness. To date, little is known about the effect of the novel coronavirus (SARS-CoV-2) on parathyroid glands. Most recent hypotheses are that Covid-19 might affect the parathyroid glands directly through invasion of parathyroid gland tissues by SARS-CoV-2 virus or indirectly secondary to respiratory failure and chronic respiratory alkalosis.
Case Report: We report the case of a 32-year-old male with post-viral hypoparathyroidism secondary to COVID-19.Only a few cases of COVID-19 induced hypoparathyroidism have been reported in the literature. Our patient was admitted to the hospital for severe and symptomatic hypocalcemia three weeks after a viral-like upper respiratory tract infection and diarrhea. He complained of paresthesias, muscle cramps and muscle weakness. He had no autoimmune or neck surgical history. Initial lab test results revealed a corrected calcium of 4.5 mg/dL (8.4-10.3 mg/dL), ionized calcium 2.4 mg/dL (4.5-5.3 mg/dL), PTH &lt; 1.0 pg/mL (12-65 pg/mL), phosphorus 6.2 mg/dL (2.5-4.7 mg/dL), magnesium 1.6 mg/dL (1.5-2.6 mg/dL), Vit D, 25-hydroxy 17.3 ng/mL (30-100 ng/mL), and creatinine 1.13 mg/dL (0.7-1.4 mg/dL), consistent with hypoparathyroidism. An ECG showed QTc prolongation to 506 msec. SARS-coV-2/flu/RSV cepheid multiplex was negative, SARS-CoV-2 Spike Ab was positive. He had never received a COVID vaccine. The patient was admitted to ICU for IV calcium drip, oral calcium and calcitriol supplementation. He was transitioned to a medical ward when his severe hypocalcemia was corrected and he was ultimately discharged on oral calcitriol 1 mcg BID and calcium carbonate 1000 mg po TID. A thorough workup for a cause of the hypoparathyroidism was performed, including anti-PTH antibody, 21-hydroxylase antibody, TSI, and TPO antibody, all of which were negative. Genetic testing was performed, including CASR gene sequencing analysis and AIRE DNA sequencing, both of which were negative. After other causes of hypoparathyroidism were excluded, COVID-19 was reasoned to be the etiology of this patient’s hypoparathyroidism. Our case highlights a post-viral hypoparathyroidism secondary to COVID-19. Further studies are needed to elucidate the mechanistic relationship between COVID-19 and hypoparathyroidism.
Presentation: Friday, June 16, 2023