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A Novel CDC73 Gene Mutation in Hyperparathyroidism Jaw Tumor Syndrome Associated With Ectopic-pelvic Kidney.

A 21-year-old woman presented with polyuria, fragility fractures, and a history of recurrent renal calculi, which was also present in her maternal aunt. Examination revealed an oval palpable mass in the neck. Biochemistry revealed a grossly elevated serum calcium, PTH, and serum alkaline phosphatase with low serum phosphorous, suggestive of primary hyperparathyroidism. Ultrasonography of the neck and parathyroid scintigraphy localized a large lesion arising from the right posterior and inferior aspect of the thyroid gland, suggesting a parathyroid tumor. Parathyroid carcinoma was suspected bas…

Recurrent renal secondary hyperparathyroidism caused by supernumerary mediastinal parathyroid gland and parathyromatosis: A case report.

BACKGROUND: Surgical parathyroidectomy (PTX) is necessary for patients with severe and progressive secondary hyperparathyroidism (SHPT) refractory to medical treatment. Recurrence of SHPT after PTX is a serious clinical problem. Both supernumerary mediastinal parathyroid gland and parathyromatosis are the rare causes of recurrent renal SHPT. We report a rare case of recurrent renal SHPT due to supernumerary mediastinal parathyroid gland and parathyromatosis. CASE PRESENTATION: A 53-year-old man underwent total parathyroidectomy with autotransplantation due to the drug-refractory SHPT 17 years…

Case Report: Primary Hyperparathyroidism due to Posterior Mediastinal Parathyroid Adenoma With One-Year Follow-Up.

Ectopic parathyroid adenoma, though rare, is one of the causes of persistent hyperparathyroidism and recurrence of hyperparathyroidism. Ectopic parathyroid glands can be seen in thymus, thyroid, and mediastinum. However, ectopic parathyroid adenoma occurred in the posterior superior mediastinum is extremely rare. Here, we report a case of primary hyperparathyroidism caused by ectopic parathyroid adenoma located in the posterior superior mediastinum. Serum parathyroid hormone, calcium, and vitamin D levels of the patient was followed up for one year.

Etelcalcetide controls secondary hyperparathyroidism and raises sclerostin levels in hemodialysis patients previously uncontrolled with cinacalcet.

INTRODUCTION: There is scarce clinical experience with etelcalcetide in patients with secondary hyperparathyroidism uncontrolled with cinacalcet. The effect of etelcalcetide on serum sclerostin levels remains to be clarified. MATERIALS AND METHODS: Prospective cohort study in prevalent hemodialysis patients with uncontrolled sHPT under cinacalcet for at least 3 months, mean parathyroid hormone (PTH)>800pg/mL and calcium (Ca)>8.3mg/dL. Etelcalcetide 5mg IV/HD was initiated after cinacalcet washout. Levels of PTH, Ca, and phosphorus (Pi) followed monthly for 6 months. Plasma sclerostin levels m…

Primary hyperparathyroidism-induced brown tumors caused by parathyroid carcinoma: a case report and literature review

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… Introduction Brown tumors are rare benign and reactive lesions caused by the abnormal bone metabolism in patients with uncontrolled hyperparathyroidism (HPT). … The incidence of primary hyperparathyroidism (PHPT) is lower (<2%) than that of secondary hyperparathyroidism (SHPT), 1 while this tumor-like lesion that represents the terminal stage of the bone remodeling process in prolonged HPT is much rarer with an overall incidence of between 2% and 3%. 2 Bone …

Baseline renal dysfunction determines mortality following parathyroidectomy in primary hyperparathyroidism: analysis of Indian PHPT registry.

INTRODUCTION: Primary hyperparathyroidism (PHPT) in India is mostly symptomatic with renal and skeletal complications. Evidence on mortality outcomes following parathyroidectomy from India, where the disease is predominantly symptomatic is limited. MATERIAL AND METHODS: This was a prospective study to evaluate mortality outcomes in the Indian PHPT registry over the past 25 years (n = 464). Pre- and postoperative parameters and mortality data were obtained from medical records and/or by verbal autopsy, a method validated by WHO for data collection in settings where several deaths are noninstit…

Outcomes of Subtotal Parathyroidectomy Versus Total Parathyroidectomy With Autotransplantation for Tertiary Hyperparathyroidism: Multi-institutional Study.

OBJECTIVES: Due to the paucity of data and controversy regarding the preferred surgical approach for managing tertiary HPT, we sought to investigate the outcomes of different surgical approaches in managing this challenging disease. METHODS: We performed a multi-center retrospective study to include patients with tertiary HPT who underwent STPX or total parathyroidectomy with autotransplantation (TPX-A). RESULTS: One hundred five patients had kidney transplant, and 43 were on dialysis. In the kidney transplant group, 61 patients underwent STPX, and 44 for TPX-A. Patients' demographics were n

Quality of life assessment after parathyroidectomy in symptomatic primary hyperparathyroidism using the SF-36 questionnaire.

OBJECTIVES: Primary hyperparathyroidism (PHPT) is a systemic disease which, along with bone and stone disease, also causes several subjective symptoms which impairs the quality of life (QoL). However, NIH guidelines do not include non-specific physical and neuropsychological symptoms as an indication of parathyroidectomy. SF-36 is one of the most commonly used tools for assessing QoL; it measures both physical health (PH) and mental health (MH). MATERIAL AND METHODS: This is a prospective observational study including 50 patients of symptomatic PHPT. Patients were categorized into normocalcem…

Predictive markers for severe hypocalcemia in dialysis patients with secondary hyperparathyroidism after near-total parathyroidectomy.

BACKGROUND: Secondary hyperparathyroidism (SHPT) is common in dialysis patients with end-stage renal disease (ESRD). Parathyroidectomy (PTX) is an effective treatment for SHPT. Postoperative severe hypocalcemia (SH) is a common and severe complication after PTX. This study aimed to investigate the potential predictive markers of SH in dialysis ESRD patients with SHPT after near-total PTX (near-tPTX) without autotransplantation (AT). METHODS: A retrospective analysis involving 131 dialysis patients with SHPT who were treated with near-tPTX without AT between January and August 2018 was perform…

Secondary hyperparathyroidism, weight loss, and longer term mortality in haemodialysis patients: results from the DOPPS.

BACKGROUND: Wasting is a common complication of kidney failure that leads to weight loss and poor outcomes. Recent experimental data identified parathyroid hormone (PTH) as a driver of adipose tissue browning and wasting, but little is known about the relations among secondary hyperparathyroidism, weight loss, and risk of mortality in dialysis patients. METHODS: We included 42,319 chronic in-centre haemodialysis patients from the Dialysis Outcomes and Practice Patterns Study phases 2-6 (2002-2018). Linear mixed models were used to estimate the association between baseline PTH and percent weig…

How should we define cure after parathyroidectomy for normocalcemic primary hyperparathyroidism? A retrospective cohort study.

Cure after surgery for normocalcemic primary hyperparathyroidism (NHPT) is defined as parathyroid hormone (PTH) normalization. However, an increase of PTH is frequently observed in cured patients with hypercalcemic primary hyperparathyroidism (HHPT). Therefore, this criterion must be redefined. A single-center retrospective study was performed including all patients who underwent surgery for Primary Hyperparathyroidism from 2013 to 2019. Cure rates of different types of hyperparathyroidism were analyzed. PTH reduction was studied as a possible criterion to define cure in patients with NHPT. On…

Influence of common clinical variables on intraoperative parathyroid hormone monitoring during surgery for primary hyperparathyroidism.

BACKGROUND: Intraoperative monitoring of parathyroid hormone (IOPTH) is a reliable method of predicting the cure of primary hyperparathyroidism (PHPT). The aim of this study is to assess whether common clinical variables (CCV) frequently encountered in patients with PHPT may affect the magnitude of PTH drop or the likelihood of patients meeting the intraoperative cure criterion. DESIGN: Patients who were surgically cured from PHPT caused by single gland disease (SGD) and had full IOPTH protocol (4 measurements) were stratified according to age, gland weight, renal function, vitamin D status a…

Hypocalcemia after ultrasound-guided microwave ablation and total parathyroidectomy for secondary hyperparathyroidism: a retrospective study.

OBJECTIVE: To compare the postoperative hypocalcemia between ultrasound-guided microwave ablation (MWA) and total parathyroidectomy (TPTX) for secondary hyperparathyroidism (SHPT). METHODS: The retrospective study reviewed 286 SHPT patients (171 received MWA and 115 underwent TPTX) between March 2018 and May 2019. Propensity-score matching was used to assemble a cohort of patients with similar baseline characteristics. A total of 184 SHPT patients (92 in each group) were finally enrolled, the occurrence and prognosis of postoperative hypocalcemia were compared. And the risk factors associated…

Effect of etelcalcetide on parathyroid hormone secretion by primary hyperparathyroidism patient-derived primary parathyroid cells.

INTRODUCTION: Etelcalcetide (Parsabiv®, AMG 416/ONO-5163) is a novel allosteric modulator for the calcium-sensing receptor approved for hemodialysis patients with secondary hyperparathyroidism of uremia. Etelcalcetide reduced parathyroid hormone levels in hemodialysis patients with secondary hyperparathyroidism of uremia in clinical studies. However, its direct effect on parathyroid hormone secretion in human parathyroid cells remains unknown. This study aimed to determine if etelcalcetide suppresses parathyroid hormone secretion by human parathyroid cells in vitro. MATERIALS AND METHODS: We …

Brown tumor due to primary hyperparathyroidism resulting in acute paraparesis: Case report and literature review.

BACKGROUND: Brown tumor (Osteoclastoma) is a rare benign, focal, lytic bone lesion most commonly attributed to a parathyroid adenoma; it occurs in approximately 5% of patients with primary hyperparathyroidism, and 13% of patients with secondary hyperparathyroidism. Most tumors are located in the mandible, pelvis, ribs, and large bones; only rarely is it found in the axial spine. CASE DESCRIPTION: A 37-year-old male with primary hyperparathyroidism presented with an MR-documented T4 and T5 brown tumor (Osteitis Fibrosa Cystica) resulting in an acute paraparesis. The patient successfully underw…

miR-129 Blocks Secondary Hyperparathyroidism-Inducing Fgf23/αKlotho Signaling in Mice with Chronic Kidney Disease.

BACKGROUND: Secondary hyperparathyroidism, a condition of excess parathyroid hormone (PTH, Pth) production, is often seen in chronic kidney disease (CKD) patients with elevated fibroblast growth factor 23 (FGF23, Fgf23). Elevated FGF23 levels stimulate secondary hyperparathyroidism-associated parathyroid αKlotho signaling. As overexpression of rationally selected microRNAs can suppress target gene activation, we hypothesized that microRNA-based suppression of parathyroid FGF23/αKlotho axis activity may be a potential strategy to combat secondary hyperparathyroidism. METHODS: In vitro lucifera…

Cervical re-explorations and proxy survival following parathyroidectomy for primary hyperparathyroidism using Australian administrative data.

BACKGROUND: Administrative data may have utility in the impartial assessment of surgical outcomes and rare events. We have used a publicly available sample of the Australian pharmaceutical and health service provision (medical benefits scheme) databases to assess outcomes following parathyroidectomy for primary hyperparathyroidism (PHP). METHODS: A cohort study using linked pharmaceutical and medical benefits schemes data was performed covering the period 1993-2014. Procedure codes identified participants undergoing parathyroidectomy for PHP and subsequent cervical re-exploration surgery (CRX…

Intraoperative verification of parathyroid glands in primary and secondary hyperparathyroidism using near-infrared autofluorescence (IOPA).

Intraoperative verification of parathyroid glands relies on visual identification by the surgeon and, with some time delay, on serum parathormon measurements and frozen section. Fluorescence imaging, however, is an instant on-table method for direct visualization of parathyroid tissue which is known to exhibit increased autofluorescence intensity when exposed to near-infrared light. In this retrospective observational study, we evaluate the clinical use of this method in a series of patients with primary and secondary hyperparathyroidism. A total of 66 adenomatous and hyperplastic parathyroid …

US-guided microwave ablation for secondary hyperparathyroidism in patients after renal transplantation: a pilot study.

OBJECTIVE: To assess the safety and efficacy of ultrasound-guided microwave ablation (MWA) in the treatment of patients who develop secondary hyperparathyroidism (SHPT) after renal transplantation (RT). METHODS: In total, nine patients, each with symptomatic SHPT caused by RT and at least one enlarged parathyroid gland, underwent MWA via hydrodissection. Intact parathyroid hormone (i-PTH), serum calcium, serum phosphorus, creatinine and blood urea nitrogen concentrations, before and after MWA, were assessed and compared. RESULTS: Complete ablation was achieved in all patients for a total of …

Bone metabolism markers and hungry bone syndrome after parathyroidectomy in dialysis patients with secondary hyperparathyroidism.

OBJECTIVE: To explore the short-term variation in bone metabolic markers and the characteristics of hungry bone syndrome (HBS) after parathyroidectomy (PTX) with forearm autotransplantation in uremic patients with secondary hyperparathyroidism (SHPT) and to provide a basis for the pathogenesis, diagnosis and treatment of metabolic bone disease in SHPT. METHODS: A total of 115 patients with SHPT receiving PTX from July 2015 to December 2017, hospitalized at the First Affiliated Hospital of Nanjing Medical University, were enrolled in our study. We retrospectively analyzed the baseline clinical…