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Abstract Context Normocalcemic hyperparathyroidism (NPHPT) is characterized by persistently normal calcium levels and elevated parathyroid hormone (PTH) values, after excluding other causes of secondary hyperparathyroidism. The prevalence of the disease varies greatly and the data on the natural history of this disease are sparse and inconclusive. Objectives The objectives of this study are to describe the prevalence of NPHPT and its natural history in a referral population and to compare the variability of serum calcium with a group of patients with primary hyperparathyroidism (PHPT). Design A retrospective study was conducted over 5 years. Setting The setting for this study was a metabolic bone referral center. Patients A total of 6280 patients were referred for a bone mineral density measurement (BMD). Main Outcome Measures The prevalence and natural history of NPHPT and variability of calcium were the main outcome measures. Results We identified NPHPT patients using data from the day of the BMD measurement. We excluded patients with low estimated glomerular filtration rate (eGFR) or vitamin D, or with no measurements available. Based on the evaluation of their medical files, we identified 11 patients with NPHPT (prevalence 0.18%). Only 4 patients had consistent normocalcemia throughout their follow-up, with only 2 also having consistently high PTH. None had consistently normal eGFR or vitamin D. Intermittent hypercalcemia was present in 7 of the 11 NPHPT patients. The mean adjusted calcium was found to be significantly lower in the NPHPT group compared with the PHPT group but higher than the control group. PTH was similar for NPHPT and PHPT. These 2 groups had similar variability in serum calcium. Conclusions NPHPT patients often have episodes of hypercalcemia. We believe that NPHPT is a mild form of PHPT.

Daily parathyroid hormone (PTH) (1-34) administrations can reduce the required total daily dose of calcium and calcitriol and restore normocalcemia in refractory hypoparathyroidism. However, most PTH(1-34) trials have been conducted on small cohorts including subjects with hypoparathyroidism of various etiologies, and quality of life (QOL) was not investigated. To investigate the effects of 24-month PTH(1-34) treatment in a homogeneous cohort of adult subjects with postoperative hypoparathyroidism and to evaluate QOL changes. Prospective open-label study. Italian multicenter study. 42 subjects. Twice-daily PTH(1-34) 20 μg subcutaneous injection. Calcium and vitamin D supplementation requirements, serum calcium, phosphate, and urinary calcium excretion (3, 6, 12, 18, 24 months). At baseline and at 6 and 24 months, QOL was evaluated by the RAND 36-Item Short Form (SF-36) Health Survey, covering eight domains of physical and mental health. Mean serum calcium concentration significantly increased from baseline to 3 months (7.6 ± 0.6 vs 8.9 ± 1.1 mg/dL, P < 0.001) and remained stable until the end of the study, despite reductions in calcium and vitamin D supplementation. Phosphate levels gradually decreased from baseline to 6 months (4.3 ± 1.1 vs 3.9 ± 0.6 mg/dL, P < 0.019), remaining stable until 24 months. Serum alkaline phosphatase and calcium excretion gradually increased from baseline to 24 months. Data from SF-36 showed a significant improvement in the mean scores of all eight domains (P < 0.001). This study demonstrates the efficacy and safety of PTH(1-34) to treat adult patients with postsurgical hypoparathyroidism. PTH(1-34) may improve their mental and physical health.

The role of preoperative parathyroid localization for tertiary hyperparathyroidism(tHPT) is unclear. We reviewed our experience regarding the feasibility of parathyroidectomy without preoperative localization. This is a retrospective study of renal allograft recipients who underwent parathyroidectomies (2001–2019). Patients with normal preoperative serum calcium and PTH levels or previous cervical operations were excluded. Patient demographics, laboratory results, radiology reports, pathology reports, and clinical notes were reviewed. Wilcoxon signed rank test, Mann-Whitney U test, Fisher's exact and Pearson chi-square analysis were used to compare patients with and without preoperative localization. Of the 118 patients, 25 patients(21.1%) had preoperative localization. Of the 36 ectopic glands recovered, 31(86.1%) were found without preoperative localization. All 118 patients achieved normocalcemia at the time these data were collected. There were no differences in surgical cure rate, recurrence rate, temporary hoarseness, or postoperative symptomatic hypocalcemia between the two groups. We observed no differences in surgical cure rate, reoperation rate, or postoperative complications without preoperative localization. Under most circumstances, it is appropriate to perform parathyroidectomy for tHPT without localization. •Parathyroidectomy without preoperative localization is feasible for tertiary hyperparathyroidism.•86.1% of ectopic glands were found intraoperatively without preoperative localization.•95.8% of patients achieved surgical cure at 6-month postoperative visit.•No differences in the surgical outcomes when parathyroidectomy was performed without preoperative localization.

Permanent hypoparathyroidism (pHypoPT) is the most common permanent complication of total thyroidectomy. We aim to describe the incidence and predictors of hypoparathyroidism in a consecutive series of patients treated in a high-volume centre and define strategies to reduce the risk of pHypoPT. 1182 patients who underwent total thyroidectomy between April 2018 and June 2022 were analyzed. Temporary hypoparathyroidism (tHypoPT) was defined as PTH <0.4 ​pmol/L (<4 ​pg/mL) at day one post-operatively, or clinical or biochemical evidence of hypocalcemia. pHypoPT was defined as an ongoing need for calcitriol supplementation to maintain normocalcemia at 12 months. Symptomatic tHypoPT (OR 43.97, p ​< ​0.001) and number of parathyroid glands in the operative specimen (OR 2.31, p ​= ​0.022) were also significantly associated with pHypoPT. Biochemical tHypoPT occurred in 205 (17.4 ​%) patients whilst pHypoPT occurred in 6 (0.5 ​%) patients. On multivariate analysis, parathyroid auto-transplantation (PA) independently reduced the risk of pHypoPT (OR 0.04, p ​= ​0.004). The risk of pHypoPT after total thyroidectomy is 0.5 ​% when performed by high-volume surgeons. PA represents an important technique that reduces the risk of pHypoPT. •pHypoPT risk after total thyroidectomy performed by high-volume surgeons is <1 ​%.•Among patients with pHypoPT, one third had recovery of PTH levels, but an ongoing calcitriol supplementation requirement.•On multivariate analysis, parathyroid auto-transplant independently reduced the risk of pHypoPT.

A 70-year-old woman presented with a large anterior cervical mass. Computed tomography and ultrasonography revealed a 70-mm polycystic structure adjacent to the right lobe of the thyroid gland, extending to its caudal aspect. Serum calcium and TSH levels were normal, whereas the serum intact PTH level was slightly elevated. Fine-needle aspiration showed cytology findings consistent with nodular goiter. After 20 months, the tumor was smaller on ultrasound but was completely solid. Serum PTH levels were markedly elevated, and Tc-99m sestamibi scintigraphy revealed striking hyperactivity of the mass. The patient underwent right hemithyroidectomy with en bloc tumor resection. Pathological analysis revealed capsular, thyroid, and venous invasion. Immunohistochemical staining was positive for PTH and galectin-3, and a high mitotic index was observed. Based on these findings, parathyroid carcinoma was diagnosed. Parafibromin staining was positive, resulting in the exclusion of etiology associated with CDC73 pathogenic variants. After 10 months, the patient was clinically free of recurrent disease. This case illustrates an atypical presentation of parathyroid carcinoma with early normocalcemia, followed by overt hyperparathyroidism, possibly because of tumor transformation.

Abstract Context The ketogenic diet is associated with progressive skeletal demineralization, hypercalciuria, and nephrolithiasis. Acute hypercalcemia has been described as a newly recognized complication of this treatment. Objective To describe the clinical characteristics of acute hypercalcemia in children on the ketogenic diet through analysis of the presentation, response to treatment, and natural history in a large cohort of patients. Design A multicenter case series was performed including children who developed acute hypercalcemia while treated with the ketogenic diet. Information on clinical presentation, treatment, and course of this complication was collated centrally. Results There were 14 patients (median (range) age 6.3 (0.9 to 18) years) who developed hypercalcemia 2.1 (range, 0.2-12) years after starting the ketogenic diet. All had low levels of parathyroid hormone and levels of 1,25-dihydroxyvitamin D were low in all except one. Seven (50%) had impaired renal function at presentation. All except the 2 oldest had low alkaline phosphatase levels for age. Once normocalcemia was achieved, hypercalcemia recurred in only 2 of these patients over observation of up to 9.8 years. One patient discontinued the ketogenic diet prior to achieving normocalcemia while 4 more stopped the diet during follow-up after resolution of hypercalcemia. Conclusions Ketotic hypercalcemia can occur years after starting the ketogenic diet, especially in the setting of renal impairment. The mechanism is unknown but appears to be due to reduced osteoblast activity and impaired bone formation. We recommend close attention to optimizing bone health in these children, and screening for the development of ketotic hypercalcemia.

A 58-year-old woman had congenital absence of vitamin D–binding protein, normocalcemia, mild disruption of bone metabolism, and severe autoimmune disease. A homozygous deletion of the group-specific component ( GC ) gene that encodes the protein was found, which provided new data about vitamin D metabolism.

Abstract Context Hypercalcemia is a common complication of malignancy that is associated with high morbidity and mortality. Objective To support development of the Endocrine Society Clinical Practice Guideline for the treatment of hypercalcemia of malignancy in adults. Methods We searched multiple databases for studies that addressed 8 clinical questions prioritized by a guideline panel from the Endocrine Society. Quantitative and qualitative synthesis was performed. The Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach was used to assess certainty of evidence. Results We reviewed 1949 citations, from which we included 21 studies. The risk of bias for most of the included studies was moderate. A higher proportion of patients who received bisphosphonate achieved resolution of hypercalcemia when compared to placebo. The incidence rate of adverse events was significantly higher in the bisphosphonate group. Comparing denosumab to bisphosphonate, there was no significant difference in the rate of patients who achieved resolution of hypercalcemia. Two-thirds of patients with refractory/recurrent hypercalcemia of malignancy who received denosumab following bisphosphonate therapy achieved resolution of hypercalcemia. Addition of calcitonin to bisphosphonate therapy did not affect the resolution of hypercalcemia, time to normocalcemia, or hypocalcemia. Only indirect evidence was available to address questions on the management of hypercalcemia in tumors associated with high calcitriol levels, refractory/recurrent hypercalcemia of malignancy following the use of bisphosphonates, and the use of calcimimetics in the treatment of hypercalcemia associated with parathyroid carcinoma. The certainty of the evidence to address all 8 clinical questions was low to very low. Conclusion The evidence summarized in this systematic review addresses the benefits and harms of treatments of hypercalcemia of malignancy. Additional information about patients’ values and preferences, and other important decisional and contextual factors is needed to facilitate the development of clinical recommendations.

Lower corrected calcium (cCa) levels are associated with a better prognosis among incident dialysis patients. However, cCa frequently overestimates ionized calcium (iCa) levels. The prognostic importance of the true calcium status defined by iCa remains to be revealed. We conducted a retrospective cohort study of incident hemodialysis patients. We collected data of iCa levels immediately before the first dialysis. We divided patients into three categories: apparent hypocalcemia (low iCa; <1.15 mmol/L and low cCa; <8.4 mg/dL), hidden hypocalcemia (low iCa despite normal or high cCa), and normocalcemia (normal iCa). The primary outcome was the composite of all-cause death and cardiovascular diseases after hospital discharge. Among the enrolled 332 patients, 75% of the patients showed true hypocalcemia, defined as iCa <1.15 mmol/L, 61% of whom showed hidden hypocalcemia. In multivariate Cox models including other potential risk factors, true hypocalcemia was a significant risk factor (hazard ratio [HR], 2.34; 95% confidence interval [CI], 1.03–5.34), whereas hypocalcemia defined as corrected calcium <8.4 mg/dL was not. Furthermore, hidden hypocalcemia was significantly associated with an increased risk of the outcome compared with normocalcemia (HR, 2.56; 95% CI, 1.11–5.94), while apparent hypocalcemia was not. Patients with hidden hypocalcemia were less likely to receive interventions to correct hypocalcemia, such as increased doses of active vitamin D or administration of calcium carbonate, than patients with apparent hypocalcemia (odds ratio, 0.45; 95% CI, 0.23–0.89). Hidden hypocalcemia was a strong predictor of death and cardiovascular events, suggesting the importance of measuring iCa.

The study objective was to evaluate the intraoperative 50% decrease in PTH level ± PTH normalization for its accuracy and efficiency in predicting cure during parathyroidectomy (PTx) for the treatment of primary hyperparathyroidism (PHP). A retrospective review of patients undergoing PTx was conducted. The timepoints at which the 50% PTH decrease was reached were recorded. The accuracy of intraoperative PTH for predicting cure, defined as normocalcemia at 6 months postoperatively, was evaluated. The study population was made up of 248 PHP patients, with 247 patients achieving normocalcemia at 6 months postoperatively. If a 50% PTH decrease was used to indicate operation conclusion, 1 patient would not be cured. Persistent PTH elevation above normal range at T10 had a PPV of 77%, NPV of 99.5%, sensitivity of 95.2% and specificity of 97.3% for predicting the presence of a contralateral pathological parathyroid gland. For the study cohort, 24.5 h of cumulative operating time would be saved if the 50% PTH decrease triggered operation conclusion. A decrease in the pre-excision PTH level to 50% of the baseline level, or a decrease in the higher of the baseline or pre-excision PTH levels by 50% at 5 or 10 min post pathological parathyroid gland removal, regardless of whether the PTH level normalizes, reliably predicts cure from PHP and should be used to guide the surgeon during parathyroidectomy. •248 primary hyperparathyroid patients undergoing parathyroidectomy made up study population.•A 50% decrease in PTH, but not PTH normalization, accurately predicted cure.•A total 24.5 h of operating time would be saved if a 50% PTH decrease triggered operation conclusion.