Primary hyperparathyroidism: review and recommendations on evaluation, diagnosis, and management. A Canadian and international consensus

Khan, Aliya; Hanley, David A.; Rizzoli, René; Bollerslev, Jens; Young, Jack; Rejnmark, Lars; Thakker, Rajesh; D'Amour, P; Paul, Terri; Uum, Stan Van; Shrayyef, M Zakaria; Goltzman, David; Kaiser, Stéphanie; Cusano, Natalie E.; Bouillon, Roger; Mosekilde, Leif; Kung, A W; Rao, Sudhaker D; Bhadada, Sanjay Kumar; Clarke, B.L.; Liu, J; Duh, Quan‐Yang; Lewiecki, E. Michael; Bandeira, Francisco; Eastell, Richard; Marcocci, Claudio; Silverberg, Shonni J.; Udelsman, Robert; Davison, K. Shawn; Potts, John T.; Brandi, Maria Luisa; Bilezikian, John P.

doi:10.1007/s00198-016-3716-2

Cited by 378 publications

(339 citation statements)

References 130 publications

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“…This drug neither impacts BMD value nor lowers biochemical markers of bone turnover. There are no documented effects on hypercalcemic symptoms, renal stones or QoL (Khan et al 2017). A study conducted on the patients with endstage renal disease (ESRD) and related PHPT showed that parathyroidectomy improved QoL, whereas cinacalcet did not (van der Plas et al 2017).…”

Section: Parathyroid Adenomasmentioning

confidence: 99%

Management impact: effects on quality of life and prognosis in MEN1

Marini¹,

Giusti²,

Tonelli³

et al. 2017

Endocrine-Related Cancer

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Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant endocrine tumor syndrome, caused by inactivating mutations of the MEN1 tumor suppressor gene at 11q13 locus, which predisposes to develop tumors in target neuroendocrine tissues. As the positional cloning and identification of the causative gene in 1997, genetic diagnosis, by the sequencing-based research of gene mutations, has become an important tool in the early and differential diagnosis of the disease. Application of the genetic test, in MEN1 index cases and in first-degree relatives of mutated patients, has been constantly increasing during the last two decades, also thanks to the establishment of multidisciplinary referral centers and specific genetic counseling, and thanks to the wide availability of high throughput instruments for gene sequencing and gene mutation identification. The MEN1 genetic test helps the specific diagnosis of probands, and allows the early identification of asymptomatic carriers, strongly contributing, together with progressions in tumor diagnostic techniques and in pharmacological and surgical therapeutic approaches, to the reduction of morbidity and mortality associated with the syndrome. International clinical guidelines for MEN1 have been drafted by panels of specialists in the field, with the main goal to improve the management of the disease and grant patients a better quality of life. Here, we review main recommendations and suggestions derived by the last published general guidelines in 2012, and by most recent published studies about MEN1 syndrome diagnosis, clinical management, therapeutic approaches and patients' quality of life.

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Section: Parathyroid Adenomasmentioning

confidence: 99%

Management impact: effects on quality of life and prognosis in MEN1

Marini¹,

Giusti²,

Tonelli³

et al. 2017

Endocrine-Related Cancer

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show abstract

“…In 15% of individuals, it can be associated with hyperplasia, and, fortunately, parathyroid carcinoma is rare. 1 PHPT is diagnosed in the presence of hypercalcemia and an elevated or inappropriately normal parathyroid hormone (PTH) levels, following exclusion of other conditions that can mimic PHPT. 1,2 The use of hydrochlorothiazide and lithium can result in elevations in serum calcium and PTH levels.…”

Section: Diagnosis Primary Hyperparathyroidism (Phpt)mentioning

confidence: 99%

“…1 Cinacalcet is an allosteric modulator of the calcium-sensing receptor and increases its sensitivity to extracellular calcium.…”

mentioning

confidence: 99%

Primary hyperparathyroidism: diagnosis and management in 2017

Syed¹,

Khan²

2017

Polish Archives of Internal Medicine

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438creatinine × serum calcium). The ratio is lower than 0.01 in 80% of individuals with FHH. However, approximately 20% of individuals have the ratio between 0.01 and 0.02, and in these individuals, other factors that can lower the ratio need to be considered. Possible conditions include renal insufficiency or severe calcium or vitamin D deficiency. The calcium-to-creatinine clearance ratio can also be low in individuals of African descent. 5 On the other hand, 80% of individuals with PHPT have a calcium-to-creatinine clearance ratio exceeding 0.02; an overlap of PHPT and FHH can occur in 20% of individuals and the ratio can be between 0.01 and 0.02.5 FHH should be excluded prior to confirming the diagnosis of PHPT, because hypercalcemia will persist even after parathyroid surgery, and it is important not to refer individuals with FHH for surgery because it is unnecessary and of no benefit.As the focus of the article is on diagnosis and management of PHPT, calcium homeostasis is described only briefly. The main regulator of serum calcium is PTH. The synthesis and secretion of PTH is stimulated by a decrease in extracellular calcium levels. PTH enhances renal calcium reabsorption, bone resorption, and the hydroxylation of 25-hydroxyvitamin D in the kidney, resulting in elevations in serum calcium levels into the normal reference range (FIGURES 1 and 2). Extracellular calcium binds to the calcium-sensing receptor on the chief cells in the parathyroid glands and inhibits the synthesis and secretion of PTH (FIGURE 3). Diagnosis Primary hyperparathyroidism (PHPT)is caused by a single parathyroid adenoma in approximately 85% of patients. In 15% of individuals, it can be associated with hyperplasia, and, fortunately, parathyroid carcinoma is rare. 1 PHPT is diagnosed in the presence of hypercalcemia and an elevated or inappropriately normal parathyroid hormone (PTH) levels, following exclusion of other conditions that can mimic PHPT. 1,2 The use of hydrochlorothiazide and lithium can result in elevations in serum calcium and PTH levels. Hydrochlorothiazide should be discontinued and the laboratory profile should be repeated 3 months later to confirm the diagnosis of PHPT. Hydrochlorothiazide enhances renal calcium reabsorption; it can result in elevations in serum calcium levels and must be discontinued prior to confirming the diagnosis. Lithium elevates the set point for serum calcium levels. It may be difficult to discontinue lithium and the patient may require management decisions to be made during ongoing lithium use.Familial hypocalciuric hypercalcemia (FHH) is an autosomal dominant condition that can mimic PHPT. It is caused by an inactivating mutation of the calcium-sensing receptor gene. The less common variant is FHH2, which is caused by an inactivating mutation of the G protein alpha 11 subunit.3 The FHH3 variant is caused by inactivating mutations of the adaptor protein 2 sigma subunit.4 FHH is excluded by evaluating the calcium-to-creatinine clearance ratio, which is calculated using the following formula: ...

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“…PHPT is the most common cause of hypercalcemia and affects at least 1 in 1000 persons [1]. Although hypercalcemia may not always be present, PHPT is typically detected by hypercalcemia with increased, or inappropriately normal, plasma PTH [22]. The incidence of PHPT is low in younger populations and similar in men and women prior to 45 years of age.…”

Section: Primary Hyperparathyroidismmentioning

confidence: 99%

“…More rarely (in 10 to 15% of cases) a four-gland hyperplasia underlies the disease [1]. Current guidelines for PHPT treatment recommend parathyroidectomy as the only curative approach to this disease [22]. Because most patients with primary hyperparathyroidism are asymptomatic at the time of diagnosis [1] there is a potential for significant skeletal damage to accrue if PHPT ensues for long periods of time prior to diagnosis and therapeutic resolution.…”

Section: Primary Hyperparathyroidismmentioning

confidence: 99%

Parathyroid Diseases and T Cells

Weitzmann

Pacifici

2017

Curr Osteoporos Rep

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Purpose of review This review summarizes studies into the permissive role of T cells in the bone catabolic effects of hyperparathyroidism and parathyroid hormone (PTH). Recent findings Work in animals combined with recent translation studies in humans now highlight the potent amplificatory action of T cells on PTH-induced bone resorption. Mechanistic animal studies reveal a complex pathway by which PTH exploits natural self-renewal functions of CD4+ T cells, to drive TNFα production that promotes formation of IL-17A secreting Th17 T cells. TNFα and IL-17 further amplify osteoblastic RANKL production and down-modulate OPG, establishing conditions propitious for osteoclastic bone resorption. Summary These findings are consistent with, and add to, the traditional view of PTH-induced bone loss involving only osteoblast-lineage cells. T cells potently amplify traditional pathways and provide permissive costimulatory signals to bone marrow stromal cells, facilitating the development of an increased RANKL/OPG ratio favourable to bone resorption and bone loss.

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Primary hyperparathyroidism: review and recommendations on evaluation, diagnosis, and management. A Canadian and international consensus

Cited by 378 publications

References 130 publications

Management impact: effects on quality of life and prognosis in MEN1

Management impact: effects on quality of life and prognosis in MEN1

Primary hyperparathyroidism: diagnosis and management in 2017

Parathyroid Diseases and T Cells

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