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European Journal of Endocrinology Feb 2022This European expert consensus statement provides recommendations for the diagnosis and management of primary hyperparathyroidism (PHPT), chronic hypoparathyroidism in...
European Expert Consensus on Practical Management of Specific Aspects of Parathyroid Disorders in Adults and in Pregnancy: Recommendations of the ESE Educational Program of Parathyroid Disorders.
This European expert consensus statement provides recommendations for the diagnosis and management of primary hyperparathyroidism (PHPT), chronic hypoparathyroidism in adults (HypoPT), and parathyroid disorders in relation to pregnancy and lactation. Specified areas of interest and unmet needs identified by experts at the second ESE Educational Program of Parathyroid Disorders (PARAT) in 2019, were discussed during two virtual workshops in 2021, and subsequently developed by working groups with interest in the specified areas. PHPT is a common endocrine disease. However, its differential diagnosing to familial hypocalciuric hypercalcemia (FHH), the definition and clinical course of normocalcemic PHPT, and the optimal management of its recurrence after surgery represent areas of uncertainty requiring clarifications. HypoPT is an orphan disease characterized by low calcium concentrations due to insufficient PTH secretion, most often secondary to neck surgery. Prevention and prediction of surgical injury to the parathyroid glands are essential to limit the disease-related burden. Long-term treatment modalities including the place for PTH replacement therapy and the optimal biochemical monitoring and imaging surveillance for complications to treatment in chronic HypoPT, need to be refined. The physiological changes in calcium metabolism occurring during pregnancy and lactation modify the clinical presentation and management of parathyroid disorders in these periods of life. Modern interdisciplinary approaches to PHPT and HypoPT in pregnant and lactating women and their newborns children are proposed. The recommendations on clinical management presented here will serve as background for further educational material aimed for a broader clinical audience, and were developed with focus on endocrinologists in training.
Topics: Adult; Calcium; Female; Humans; Hypercalcemia; Hyperparathyroidism, Primary; Hypoparathyroidism; Infant, Newborn; Lactation; Parathyroid Diseases; Parathyroid Hormone; Pregnancy
PubMed: 34863037
DOI: 10.1530/EJE-21-1044 -
Gland Surgery Feb 2020Although untreated primary hyperparathyroidism is associated with increased cardiovascular mortality, controversy exists regarding the therapeutic effects of... (Review)
Review
Although untreated primary hyperparathyroidism is associated with increased cardiovascular mortality, controversy exists regarding the therapeutic effects of parathyroidectomy on cardiovascular health. This review will examine the evidence linking primary hyperparathyroidism (PHPT) and cardiovascular disease, specifically hypertension, and evaluate the available literature regarding the natural history of hypertension after successful parathyroidectomy.
PubMed: 32206606
DOI: 10.21037/gs.2019.10.21 -
Archives of Endocrinology and Metabolism Nov 2022Primary hyperparathyroidism (PHPT) is a hypercalcemic disorder that occurs when one or more parathyroid glands produces excessive parathyroid hormone (PTH). PHPT is... (Review)
Review
Primary hyperparathyroidism (PHPT) is a hypercalcemic disorder that occurs when one or more parathyroid glands produces excessive parathyroid hormone (PTH). PHPT is typically treated with surgery, and it remains the only definitive therapy, whose techniques have evolved over previous decades. Advances in preoperative localization exams and the intraoperative PTH monitoring have become the cornerstones of recent parathyroidectomy techniques, as minimally invasive techniques are appropriate for most patients. Nevertheless, these techniques, are not suitable for PHPT patients who are at risk for multiglandular disease, especially in those who present with familial forms of PHPT that require bilateral neck exploration. This manuscript also explores other conditions that warrant special consideration during surgical treatment for PHPT: normocalcemic primary hyperparathyroidism, pregnancy, reoperation for persistent or recurrent PHPT, parathyroid carcinoma, and familial and genetic forms of hyperparathyroidism.
Topics: Humans; Hyperparathyroidism, Primary; Parathyroidectomy; Parathyroid Neoplasms; Parathyroid Hormone; Parathyroid Glands
PubMed: 36382757
DOI: 10.20945/2359-3997000000557 -
Indian Journal of Surgical Oncology Mar 2022Primary hyperparathyroidism (PHPT) is the unregulated overproduction of parathyroid hormone (PTH) resulting in abnormal calcium homeostasis. The disease profile has... (Review)
Review
Primary hyperparathyroidism (PHPT) is the unregulated overproduction of parathyroid hormone (PTH) resulting in abnormal calcium homeostasis. The disease profile has evolved over the last century from symptomatic hyperparathyroidism to asymptomatic hyperparathyroidism. Primary hyperparathyroidism is a biochemical diagnosis. Parathyroidectomy is the only established cure for this disease. Every effort is made to localize the offending gland preoperaively with various imaging modalities. These localization studies along with intraoperative parathormone monitoring will direct the surgeon to either a focused parathyroidectomy or a four gland exploration. All symptomatic patients and asymptomatic patients who meet the criteria for surgery should undergo parathyroidectomy if no medical contraindications exist. Pharmacologic approaches are reserved for those patients unfit or unwilling to undergo surgery.
PubMed: 35462664
DOI: 10.1007/s13193-021-01319-3 -
Journal of Bone and Mineral Research :... Nov 2022This narrative review summarizes data on classical and nonclassical manifestations of primary hyperparathyroidism (PHPT). It is based on a rigorous literature search,... (Review)
Review
This narrative review summarizes data on classical and nonclassical manifestations of primary hyperparathyroidism (PHPT). It is based on a rigorous literature search, inclusive of a Medline search for systematic reviews from 1940 to December 2020, coupled with a targeted search for original publications, covering four databases, from January 2013-December 2020, and relevant articles from authors' libraries. We present the most recent information, identify knowledge gaps, and suggest a research agenda. The shift in the presentation of PHPT from a predominantly symptomatic to an asymptomatic disease, with its varied manifestations, has presented several challenges. Subclinical nephrolithiasis and vertebral fractures are common in patients with asymptomatic disease. The natural history of asymptomatic PHPT with no end organ damage at diagnosis is unclear. Some observational and cross-sectional studies continue to show associations between PHPT and cardiovascular and neuropsychological abnormalities, among the different disease phenotypes. Their causal relationship is uncertain. Limited new data are available on the natural history of skeletal, renal, cardiovascular, neuropsychological, and neuromuscular manifestations and quality of life. Normocalcemic PHPT (NPHPT) is often diagnosed without the fulfillment of rigorous criteria. Randomized clinical trials have not demonstrated a consistent long-term benefit of parathyroidectomy (PTX) versus observation on nonclassical manifestations. We propose further refining the definition of asymptomatic disease, into two phenotypes: one without and one with evidence of target organ involvement, upon the standard evaluation detailed in our recommendations. Each of these phenotypes can present with or without non-classical manifestations. We propose multiple albumin-adjusted serum calcium determinations (albumin-adjusted and ionized) and exclusion of all secondary causes of high parathyroid hormone (PTH) when establishing the diagnosis of NPHPT. Refining the definition of asymptomatic disease into the phenotypes proposed will afford insights into their natural history and response to interventions. This would also pave the way for the development of evidence-based guidance and recommendations. © 2022 The Authors. Journal of Bone and Mineral Research published by Wiley Periodicals LLC on behalf of American Society for Bone and Mineral Research (ASBMR).
Topics: Humans; Hyperparathyroidism, Primary; Cross-Sectional Studies; Quality of Life; Systematic Reviews as Topic; Parathyroidectomy; Parathyroid Hormone; Calcium; Asymptomatic Diseases; Albumins
PubMed: 36245249
DOI: 10.1002/jbmr.4679 -
British Journal of Hospital Medicine... Dec 2023Thiazide diuretics exert a natriuretic and diuretic effect by inhibiting sodium reabsorption in the distal convoluted tubule. Furthermore, thiazide diuretics affect...
Thiazide diuretics exert a natriuretic and diuretic effect by inhibiting sodium reabsorption in the distal convoluted tubule. Furthermore, thiazide diuretics affect renal calcium handling by increasing calcium reabsorption, leading to hypocalciuria. The effect that thiazide diuretics exert on parathyroid hormone secretion is controversial. Some studies found parathyroid hormone levels were suppressed with the use of thiazide diuretics, while others found that thiazides were associated with initial parathyroid hormone suppression followed by raised parathyroid hormone levels. This makes the relationship between thiazide diuretics and primary hyperparathyroidism interesting. If a patient is taking thiazide diuretics, this may make it harder to establish the aetiology of hypercalcaemia and may unmask normocalcaemic or mild primary hyperparathyroidism. Thiazide diuretics may have a beneficial role in the diagnosis of patients with concomitant hyperparathyroidism and hypercalciuria by distinguishing secondary hyperparathyroidism caused by hypercalciuria from normocalcaemic primary hyperparathyroidism. In addition, thiazide diuretics may have a role in managing patients with primary hyperparathyroidism who have an indication for parathyroidectomy in view of significant hypercalciuria, but are unfit for surgery.
Topics: Humans; Sodium Chloride Symporter Inhibitors; Calcium; Hyperparathyroidism, Primary; Hypercalciuria; Diuretics; Parathyroid Hormone
PubMed: 38153014
DOI: 10.12968/hmed.2023.0228 -
Reumatologia 2023Primary hyperparathyroidism (PHPT) is a frequent endocrine disease which mainly affects the skeletal system and kidney. Some of its signs and symptoms are similar to... (Review)
Review
Primary hyperparathyroidism (PHPT) is a frequent endocrine disease which mainly affects the skeletal system and kidney. Some of its signs and symptoms are similar to those seen in rheumatic diseases such as rheumatoid arthritis, Sjögren's disease, fibromyalgia, polymyalgia rheumatica, gout or systemic lupus erythematosus. Coexistence of primary hyperparathyroidism with those pathologies potentiate their effects on muscles, bones and joints, increasing the risk of complications such as osteoporosis and fractures. Therefore, rheumatologists should be familiar with symptoms and diagnostic criteria of PHPT and consider it in the differential diagnosis of rheumatic diseases. In 2022 the Fifth International Workshop guidelines on the PHPT evaluation and management were published. They are based on a profound analysis of advances in research concerning multiple fields, that include genetics, outcomes and new imaging modalities of PHPT. They have led to revision of previous renal indications for parathyroidectomy in PHPT. There is also more evidence for the other recommendations regarding evaluation of the disease. This article summarizes the most relevant elements of these recommendations and refers them to Polish realities. I focus on the symptoms of primary hyperparathyroidism and its diagnosis as I consider these areas to be the most important for non-endocrinologists.
PubMed: 37745146
DOI: 10.5114/reum/170705 -
Journal of Bone and Mineral Research :... Nov 2022Parathyroidectomy (PTX) is the treatment of choice for symptomatic primary hyperparathyroidism (PHPT). It is also the treatment of choice in asymptomatic PHPT with... (Review)
Review
Parathyroidectomy (PTX) is the treatment of choice for symptomatic primary hyperparathyroidism (PHPT). It is also the treatment of choice in asymptomatic PHPT with evidence for target organ involvement. This review updates surgical aspects of PHPT and proposes the following definitions based on international expert consensus: selective PTX (and reasons for conversion to an extended procedure), bilateral neck exploration for non-localized or multigland disease, subtotal PTX, total PTX with immediate or delayed autotransplantation, and transcervical thymectomy and extended en bloc PTX for parathyroid carcinoma. The systematic literature reviews discussed covered (i) the use of intraoperative PTH (ioPTH) for localized single-gland disease and (ii) the management of low BMD after PTX. Updates based on prospective observational studies are presented concerning PTX for multigland disease and hereditary PHPT syndromes, histopathology, intraoperative adjuncts, localization techniques, perioperative management, "reoperative" surgery and volume/outcome data. Postoperative complications are few and uncommon (<3%) in centers performing over 40 PTXs per year. This review is the first global consensus about surgery in PHPT and reflects the current practice in leading endocrine surgery units worldwide. © 2022 The Authors. Journal of Bone and Mineral Research published by Wiley Periodicals LLC on behalf of American Society for Bone and Mineral Research (ASBMR).
Topics: Humans; Hyperparathyroidism, Primary; Parathyroidectomy; Parathyroid Neoplasms; Postoperative Complications; Parathyroid Hormone; Observational Studies as Topic
PubMed: 36054175
DOI: 10.1002/jbmr.4689 -
Endocrine Reviews Sep 2023Recent data suggest an increase in the overall incidence of parathyroid disorders, with primary hyperparathyroidism (PHPT) being the most prevalent parathyroid disorder....
Recent data suggest an increase in the overall incidence of parathyroid disorders, with primary hyperparathyroidism (PHPT) being the most prevalent parathyroid disorder. PHPT is associated with morbidities (fractures, kidney stones, chronic kidney disease) and increased risk of death. The symptoms of PHPT can be nonspecific, potentially delaying the diagnosis. Approximately 15% of patients with PHPT have an underlying heritable form of PHPT that may be associated with extraparathyroidal manifestations, requiring active surveillance for these manifestations as seen in multiple endocrine neoplasia type 1 and 2A. Genetic testing for heritable forms should be offered to patients with multiglandular disease, recurrent PHPT, young onset PHPT (age ≤40 years), and those with a family history of parathyroid tumors. However, the underlying genetic cause for the majority of patients with heritable forms of PHPT remains unknown. Distinction between sporadic and heritable forms of PHPT is useful in surgical planning for parathyroidectomy and has implications for the family. The genes currently known to be associated with heritable forms of PHPT account for approximately half of sporadic parathyroid tumors. But the genetic cause in approximately half of the sporadic parathyroid tumors remains unknown. Furthermore, there is no systemic therapy for parathyroid carcinoma, a rare but potentially fatal cause of PHPT. Improved understanding of the molecular characteristics of parathyroid tumors will allow us to identify biomarkers for diagnosis and novel targets for therapy.
Topics: Humans; Adult; Hyperparathyroidism, Primary; Parathyroid Neoplasms; Parathyroidectomy; Genetic Testing; Renal Insufficiency, Chronic
PubMed: 36961765
DOI: 10.1210/endrev/bnad009 -
Hormone and Metabolic Research =... Aug 2020Calcium homeostasis is maintained by the actions of the parathyroid glands, which release parathyroid hormone into the systemic circulation as necessary to maintain the... (Review)
Review
Calcium homeostasis is maintained by the actions of the parathyroid glands, which release parathyroid hormone into the systemic circulation as necessary to maintain the serum calcium concentration within a tight physiologic range. Excessive secretion of parathyroid hormone from one or more neoplastic parathyroid glands, however, causes the metabolic disease primary hyperparathyroidism (HPT) typically associated with hypercalcemia. Although the majority of cases of HPT are sporadic, it can present in the context of a familial syndrome. Mutations in the tumor suppressor genes discovered by the study of such families are now recognized to be pathogenic for many sporadic parathyroid tumors. Inherited and somatic mutations of proto-oncogenes causing parathyroid neoplasia are also known. Future investigation of somatic changes in parathyroid tumor DNA and the study of kindreds with HPT yet lacking germline mutation in the set of genes known to predispose to HPT represent two avenues likely to unmask additional novel genes relevant to parathyroid neoplasia.
Topics: Genetic Predisposition to Disease; Humans; Hypercalcemia; Hyperparathyroidism, Primary; Mutation; Prognosis; Tumor Suppressor Proteins
PubMed: 32227318
DOI: 10.1055/a-1132-6223