Surgical options in treating patients with primary hyperparathyroidism.
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ABSTRACT:
SUBMITTER: Majcen M
PROVIDER: S-EPMC7087427 | biostudies-literature | 2020 Feb
REPOSITORIES: biostudies-literature
Publications
Surgical options in treating patients with primary hyperparathyroidism.
Radiology and oncology 20200229 1
Background Primary hyperparathyroidism is the third most common endocrine disorder for which surgical procedure called parathyroidectomy is the most effective treatment. Since the early 20th century, parathyroid surgery has improved extensively. With the advances in preoperative imaging and with understanding the causes of disease, new and minimally invasive surgical approaches overrode the standard bilateral exploratory operations. Directed parathyroidectomy is currently the standard technique ...[more]
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Project description:Primary hyperparathyroidism (PHPT) is a common disorder in which parathyroid hormone (PTH) is excessively secreted from one or more of the four parathyroid glands. A single benign parathyroid adenoma is the cause in most people. However, multiglandular disease is not rare and is typically seen in familial PHPT syndromes. The genetics of PHPT is usually monoclonal when a single gland is involved and polyclonal when multiglandular disease is present. The genes that have been implicated in PHPT include proto-oncogenes and tumour-suppressor genes. Hypercalcaemia is the biochemical hallmark of PHPT. Usually, the concentration of PTH is frankly increased but can remain within the normal range, which is abnormal in the setting of hypercalcaemia. Normocalcaemic PHPT, a variant in which the serum calcium level is persistently normal but PTH levels are increased in the absence of an obvious inciting stimulus, is now recognized. The clinical presentation of PHPT varies from asymptomatic disease (seen in countries where biochemical screening is routine) to classic symptomatic disease in which renal and/or skeletal complications are observed. Management guidelines have recently been revised to help the clinician to decide on the merits of a parathyroidectomy or a non-surgical course. This Primer covers these areas with particular attention to the epidemiology, clinical presentations, genetics, evaluation and guidelines for the management of PHPT.
| S-EPMC5385896 | biostudies-literature
Project description:OBJECTIVE:Primary hyperparathyroidism (PHPT) is a common reason for referral to endocrinology but the evidence base guiding assessment is limited. We evaluated the clinical presentation, assessment and subsequent management in PHPT. DESIGN:Retrospective cohort study. PATIENTS:PHPT assessed between 2006 - 2014 (n = 611) in a university hospital. MEASUREMENTS:Symptoms, clinical features, biochemistry, neck radiology and surgical outcomes. RESULTS:Fatigue (23.8%), polyuria (15.6%) and polydipsia (14.9%) were associated with PHPT biochemistry. Bone fracture was present in 16.4% but was not associated with biochemistry. A history of nephrolithiasis (10.0%) was associated only with younger age (P = 0.006) and male gender (P = 0.037). Thiazide diuretic discontinuation was not associated with any subsequent change in calcium (P = 0.514). Urine calcium creatinine clearance ratio (CCCR) was <0.01 in 18.2% of patients with confirmed PHPT. Older age (P < 0.001) and lower PTH (P = 0.043) were associated with failure to locate an adenoma on ultrasound (44.0% of scans). When an adenoma was identified on ultrasound the lateralization was correct in 94.5%. Non-curative surgery occurred in 8.2% and was greater in those requiring more than one neck imaging modality (OR 2.42, P = 0.035). CONCLUSIONS:Clinical features associated with PHPT are not strongly related to biochemistry. Thiazide cessation does not appear to attenuate hypercalcaemia. PHPT remains the likeliest diagnosis in the presence of low CCCR. Ultrasound is highly discriminant when an adenoma is identified but surgical failure is more likely when more than one imaging modality is required.
| S-EPMC6198194 | biostudies-literature
Project description: Not available
| S-EPMC8407481 | biostudies-literature
Project description:BackgroundMultiple studies have reported the increased incidence of thyroid cancer in patients with primary hyperparathyroidism (PHPT). However, the underlying risk factors of concomitant thyroid cancer in patients with PHPT remain unknown. The primary aim of this study was to examine the records of patients with PHPT to identify characteristics that correlated with the presence of coexisting thyroid nodules, and which may have an implication for the prediction of thyroid cancer.MethodsMedical records of consecutive patients with PHPT (n = 318) were reviewed from January 2010 to September 2020 in two tertiary medical centers in China. Patient clinicopathological and biological data were collected and analyzed.ResultsOf a total of 318 patients with PHPT, 105 (33.0%) patients had thyroid nodules and 26 (8.2%) patients were concomitant with thyroid cancer. A total of 38 thyroid nodules taken from 26 patients were pathologically assessed to be well-differentiated papillary thyroid carcinoma (PTC), with 81% being papillary thyroid microcarcinoma (PTMC). In 79% (30/38) of these cancers, thyroid nodules were considered suspicious following preoperative ultrasound. Multinomial logistic regression analysis revealed that female gender was associated with increased risk of thyroid nodules (OR = 2.13, 95% CI: 1.13-3.99, P = 0.019), while lower log-transformed parathyroid hormone levels were an independent predictor of thyroid cancer in patients with PHPT (OR = 0.50, 95% CI: 0.26-0.93, P = 0.028).ConclusionIn conclusion, we observed a relatively high prevalence of thyroid cancer in our cohort of Chinese patients with PHPT. Evaluation of thyroid nodules by preoperative ultrasound may be advisable in patients with PHPT, particularly for females and patients with modestly elevated serum parathyroid hormone levels.
| S-EPMC8346191 | biostudies-literature
Project description:BACKGROUND:Primary hyperparathyroidism (PHPT) is a relatively rare disorder among children, adolescents and young adults. Its development at an early age is suspicious for hereditary causes, though the need for routine genetic testing remains controversial. OBJECTIVE:To identify and describe hereditary forms of PHPT in patients with manifestation of the disease under 40 years of age. DESIGN:We enrolled 65 patients with PHPT diagnosed before 40 years of age. Ten of them had MEN1 mutation, and PHPT in them was the first manifestation of multiple endocrine neoplasia type 1 syndrome. METHODS:The other fifty-five patients underwent next-generation sequencing (NGS) of a custom-designed panel of genes, associated with PHPT (MEN1, CASR, CDC73, CDKN1A, CDKN1B, CDKN1C, CDKN2A, CDKN2C, CDKN2D). In cases suspicious for gross CDC73 deletions multiplex ligation-dependent probe amplification was performed. RESULTS:NGS revealed six pathogenic or likely pathogenic germline sequence variants: four in CDC73 c.271C>T (p.Arg91*), c.496C>T (p.Gln166*), c.685A>T (p.Arg229*) and c.787C>T (p.Arg263Cys); one in CASR c.3145G>T (p.Glu1049*) and one in MEN1 c.784-9G>A. In two patients, MLPA confirmed gross CDC73 deletions. In total, 44 sporadic and 21 hereditary PHPT cases were identified. Parathyroid carcinomas and atypical parathyroid adenomas were present in 8/65 of young patients, in whom CDC73 mutations were found in 5/8. CONCLUSIONS:Hereditary forms of PHPT can be identified in up to 1/3 of young patients with manifestation of the disease at <40 years of age. Parathyroid carcinomas or atypical parathyroid adenomas in young patients are frequently associated with CDC73 mutations.
| S-EPMC5633061 | biostudies-literature
Project description:ImportanceHyperparathyroidism is associated with cardiovascular disease. However, evidence for a beneficial consequence of parathyroidectomy on hypertension is limited.ObjectiveTo investigate if parathyroidectomy improves hypertension in patients with primary hyperparathyroidism (PHPT).Design, setting, and participantsIn this cohort study and retrospective database review, patients with PHPT and hypertension between January 1, 2008, and December 31, 2016, were identified. The mean arterial pressure (MAP) and number of antihypertensive medications were compared between those who did and did not undergo parathyroidectomy. The setting was a large health care system. Primary hyperparathyroidism was defined using biochemical data, and hypertension was identified by International Classification of Diseases, Ninth Revision codes.ExposureParathyroidectomy was identified in the database by Current Procedural Terminology codes.Main outcomes and measuresThe MAP and use of antihypertensive medications were compared for patients who underwent parathyroidectomy and those who did not at 6 months, 1 year, and 2 years. Multivariable logistic regression was used to assess the adjusted odds ratios for both increased and decreased use of antihypertensive medications.ResultsIn this cohort study of 2380 participants (79.0% female), patients undergoing parathyroidectomy (n = 501) were younger (mean [SD] age, 65.3 [9.7] vs 71.9 [10.4] years; P < .001) and took fewer antihypertensive medications at baseline (mean [SD] number of medications, 1.2 [1.1] vs 1.5 [1.3], P < .001) than nonsurgical patients (n = 1879). Patients with parathyroidectomy showed greater improvement in their MAP at all follow-up time points (the median [SD] MAP change from baseline to 1 year was 0.1 [8.7] mm Hg without parathyroidectomy vs -1.2 [7.7] mm Hg after parathyroidectomy, P = .002). Nonsurgical patients were more likely vs those with parathyroidectomy to require more antihypertensive medications at 6 months (15.9% [n = 298] vs 9.8% [n = 49], P = .001), 1 year (18.1% [n = 340] vs 10.8% [n = 54], P < .001), and 2 years (17.6% [n = 330] vs 12.2% [n = 61], P = .004). By multivariable analysis, parathyroidectomy was independently associated with freedom from an increased number of antihypertensive medications at all periods (eg, adjusted odds ratio, 0.49; 95% CI, 0.34-0.70; P < .001 at 1 year). Among patients who were initially not taking antihypertensive medications, patients with parathyroidectomy were less likely vs no surgery to start antihypertensive medication treatment at all periods (eg, 10.2% [13 of 127] vs 30.4% [136 of 447], P < .001 at 1 year).Conclusions and relevanceThis study's findings suggest that, among hypertensive patients with PHPT, parathyroidectomy may be associated not only with greater decreases in their MAP but also with reduced requirements for antihypertensive medications. Parathyroidectomy decreased the number of patients who began taking antihypertensive medications. Additional study will be required to find whether there are downstream cardiovascular benefits of parathyroidectomy. Preexisting hypertension, particularly in those not already taking antihypertensive medications, should be considered when weighing surgical treatment.
| S-EPMC6802266 | biostudies-literature
Project description:Patients with persistent primary hyperparathyroidism (PHPT) after parathyroidectomy or with contraindications to parathyroidectomy often require chronic treatment for hypercalcemia.The objective of the study was to assess the ability of the calcimimetic, cinacalcet, to reduce serum calcium in patients with intractable PHPT.This was an open-label, single-arm study comprising a titration phase of variable duration (2-16 wk) and a maintenance phase of up to 136 wk.The study was conducted at 23 centers in Europe, the United States, and Canada.The study included 17 patients with intractable PHPT and serum calcium greater than 12.5 mg/dl (3.1 mmol/liter).During the titration phase, cinacalcet dosages were titrated every 2 wk (30 mg twice daily to 90 mg four times daily) for 16 wk until serum calcium was 10 mg/dl or less (2.5 mmol/liter). If serum calcium increased during the maintenance phase, additional increases in the cinacalcet dose were permitted.The primary end point was the proportion of patients experiencing a reduction in serum calcium of 1 mg/dl or greater (0.25 mmol/liter) at the end of the titration phase.Mean +/- sd baseline serum calcium was 12.7 +/- 0.8 mg/dl (3.2 +/- 0.2 mmol/liter). At the end of titration, a 1 mg/dl or greater reduction in serum calcium was achieved in 15 patients (88%). Fifteen patients (88%) experienced treatment-related adverse events, none of which were serious. The most common adverse events were nausea, vomiting, and paresthesias.In patients with intractable PHPT, cinacalcet reduces serum calcium, is generally well tolerated, and has the potential to fulfill an unmet medical need.
| S-EPMC3214593 | biostudies-literature
Project description:Context:Germline gain-of-function variants in the transcription factor GCM2 were found in 18% of kindreds with familial isolated hyperparathyroidism (FIHP). These variants [c.1136T>A (p.Leu379Gln) and c.1181A>C (p.Tyr394Ser)] were located in a 17-amino acid transcriptional inhibitory domain named C-terminal conserved inhibitory domain (CCID). Objective:We investigated the ethnicity of individuals with germline variants in the GCM2 CCID in our primary hyperparathyroidism (PHPT) patient samples and in the Genome Aggregation Database. Design:Ethnicity information was obtained from an in-house clinical database and genetic counseling. Sanger sequencing of blood DNA was used to determine the genotype of the GCM2 CCID region. Luciferase reporter assays were performed to determine the functional impact of GCM2 variants. Setting and Patients:National Institute of Diabetes and Digestive and Kidney Diseases endocrine clinic is a service that accepts PHPT referral patients. Results:The GCM2 p.Tyr394Ser variant was found in 41% [95% confidence interval (CI), 22% to 64%] of Ashkenazi Jewish (AJ) kindreds with FIHP and in 27% (95% CI, 17% to 40%) of AJ patients with sporadic PHPT. The p.Tyr394Ser variant was also found in sporadic PHPT patients of European ancestry, but at a lower prevalence. The p.Leu379Gln variant was found in 8% (95% CI, 1% to 26%) of European kindreds with FIHP and 0.5% (95% CI, 0% to 3.0%) of sporadic PHPT cases of European ancestry. The sporadic PHPT patients with GCM2-activating variants often had multigland involvement or postoperative recurrent or persistent disease. Conclusions:Specific GCM2-activating variants enriched among various ethnic backgrounds could contribute to a large number of cases with FIHP or sporadic PHPT.
| S-EPMC5686704 | biostudies-literature
Project description:BackgroundPrimary hyperparathyroidism is a prevalent endocrinopathy for which surgery is the only curative option. Parathyroidectomy is primarily recommended in younger and symptomatic patients, while there are still concerns regarding surgical complications in older patients. We therefore assessed the association of age with surgical outcomes in patients undergoing parathyroidectomy in a large population in Switzerland.MethodsPopulation-based cohort study of adult patients with primary hyperparathyroidism undergoing parathyroidectomy in Switzerland between 2012 and 2018. The cohort was divided into four age groups (<50 years, 50-64 years, 65-74 years, ≥75 years). The primary outcome was a composite of in-hospital postoperative complications. Secondary outcomes were intensive care unit (ICU) admission, unplanned 30-day-readmission, and prolonged length of hospital stay.ResultsWe studied 2642 patients with a median (IQR) age of 62 (53-71) years. Overall, 111 patients had complications including surgical re-intervention, hypocalcemia, and vocal cord paresis. As compared to <50 year-old patients, older patients had no increased risk for in-hospital complications after surgery (50-64 years: odds ratio (OR): 0.51 (95% CI, 0.28 to 0.92); 65-74 years: OR: 0.72 (95% CI, 0.39 to 1.33); ≥75 years: OR: 1.03 (95% CI, 0.54 to 1.95), respectively. There was also no association of age and rates of ICU-admission and unplanned 30-day-readmission, but oldest patients had longer hospital stays (OR: 2.38 (95% CI, 1.57 to 3.60)).Conclusion≥50 year-old patients undergoing parathyroidectomy had comparable risk of in-hospital complications as compared with younger ones. These data support parathyroidectomy in even older patients with primary hyperparathyroidism as performed in clinical routine.
| S-EPMC8558907 | biostudies-literature
Project description:Primary hyperparathyroidism is a common condition that affects 0.3% of the general population. Primary and tertiary care specialists can encounter patients with primary hyperparathyroidism, and prompt recognition and treatment can greatly reduce morbidity and mortality from this disease. In this paper we will review the basic physiology of calcium homeostasis and then consider genetic associations as well as common etiologies and presentations of primary hyperparathyroidism. We will consider emerging trends in detection and measurement of parathyroid hormone as well as available imaging modalities for the parathyroid glands. Surgical indications and approach will be reviewed as well as medical management of primary hyperparathyroidism with bisphosphonates and calcimimetics.
| S-EPMC3124277 | biostudies-other