Impaired geometry, volumetric density, and microstructure of cortical and trabecular bone assessed by HR-pQCT in both sporadic and MEN1-related primary hyperparathyroidism

Wang, W.; Nie, Min; Jiang, Yan; Li, M.; Meng, Xunwu; Xing, Xiaoping; Wang, O.; Xia, Weibo

doi:10.1007/s00198-019-05186-1

Cited by 20 publications

(16 citation statements)

References 24 publications

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“…As opposed to other studies [3,9], no significant differences in BMD values and T-score were reported between our MEN1 PHPT and sPHPT patients at all the analyzed bone sites. However, according to results from Wang et al [12], our MEN1 PHPT patients had a significantly lower Z-score at lumbar spine and femoral neck than sPHPT (data not shown). Since Z-score represents the standard deviation of the attenuation of X-ray passage through bone tissue compared to that of the healthy population of the same age, our results confirmed that MEN1 PHPT is responsible for reduction of bone mass, both at cortical and trabecular compartments, at any age, leading to a demineralized bone compared with the average bone density of healthy people of the same age and gender, and increasing the risk for early fragility fractures, significantly more than sPHPT.…”

Section: Discussionsupporting

confidence: 50%

“…By the age of 20-35 years, MEN1 women with PHPT showed a higher incidence of osteopenia and osteoporosis compared to the general population of the same age, with a consequent increased risk of fragility fracture [3,7,11]. A more severe degree of trabecular bone demineralization and lower values of both spine and femur bone mineral density (BMD) were reported in MEN1 PHPT compared to sPHPT patients, despite a significantly younger age, lower serum levels of PTH, and usually a milder hypercalcemia [3,9,12]. The earlier occurrence of MEN1 PHPT, about three decades before sPHPT, leading to persistent and prolonged increased levels of PTH, starting during late childhood, adolescence or early adulthood, may negatively affect the normal acquisition of bone mass peak, while in adults the increased bone resorption will be responsible for bone mass loss and increased bone fragility.…”

Section: Introductionmentioning

confidence: 99%

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Bone and Mineral Metabolism Phenotypes in MEN1-Related and Sporadic Primary Hyperparathyroidism, before and after Parathyroidectomy

Marini

Giusti

Cioppi

et al. 2021

Cells

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Primary hyperparathyroidism (PHPT) is the most common endocrinopathy in multiple endocrine neoplasia type 1 (MEN1). Persistent levels of increased parathyroid hormone (PTH) result in a higher incidence of osteopenia and osteoporosis compared to the general population. Surgical removal of hyper-functioning parathyroid tissue is the therapy of choice. This retrospective study evaluated the effect of parathyroidectomy (PTX) on bone metabolism and bone mass in two series of patients with MEN1 PHPT and sporadic PHPT (sPHPT) by comparing bone metabolism-related biochemical markers and bone mineral density (BMD) before and after surgery. Our data confirmed, in a higher number of cases than in previously published studies, the efficacy of PTX, not only to rapidly restore normal levels of PTH and calcium, but also to normalize biochemical parameters of bone resorption and bone formation, and to improve spine and femur bone mass, in both MEN1 PHPT and sPHPT. Evaluation of single-patient BMD changes after surgery indicates an individual variable bone mass improvement in a great majority of MEN1 PHPT patients. In MEN1 patients, PTX is strongly suggested in the presence of increased PTH and hypercalcemia to prevent/reduce the early-onset bone mass loss and grant, in young patients, the achievement of the bone mass peak; routine monitoring of bone metabolism and bone mass should start from adolescence. Therapy with anti-fracture drugs is indicated in MEN1 patients with BMD lower than the age-matched normal values.

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Section: Discussionsupporting

confidence: 50%

Section: Introductionmentioning

confidence: 99%

Bone and Mineral Metabolism Phenotypes in MEN1-Related and Sporadic Primary Hyperparathyroidism, before and after Parathyroidectomy

Marini

Giusti

Cioppi

et al. 2021

Cells

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“…Therefore, increased severity of bone loss in MHPT patients could be partially attributed to the combination of other endocrine dysfunction components, including hypercortisolism, hyperprolactinemia, hypogonadism, and GH deficiency, which also exert negative effects on bone mineralization (29). However, in the previous study of our center, there were no differences between MHPT and SHPT were observed in bone indices as measured using both DXA and high-resolution peripheral computed tomography (HR-pQCT) (4,30). Therefore, more research is needed in the future to clarify the bone changes in MHPT patients, especially MEN1-PC/APN patients.…”

Section: Discussionmentioning

confidence: 78%

Prevalence of Parathyroid Carcinoma and Atypical Parathyroid Neoplasms in 153 Patients With Multiple Endocrine Neoplasia Type 1: Case Series and Literature Review

et al. 2020

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The occurrence of parathyroid carcinoma (PC) and atypical parathyroid neoplasm (APN) in multiple endocrine neoplasia type 1 (MEN1) is rare. The present paper reports the cases of 3 MEN1-PC/APN patients at our center and discusses the prevalence in a Chinese MEN1 cohort. Methods: This report is a retrospective analysis of 153 MEN1-associated primary hyperparathyroidism (MEN1-HPT) patients at our center, which included 3 MEN1-associated PC/APN (MEN1-PC/APN) patients. The clinical manifestations, biochemical indices, pathological findings, and therapy have been summarized along with the report of the genetic testing of the 3 patients. Results: Of the 153 MEN1-HPT patients, 1 (0.7%) was histopathologically diagnosed with PC and 2 (1.3%) with APN. Three heterozygous mutations were identified in the 3 MEN1-PC/APN patients (c.917 T > G, c.431T > C, and c.549 G > C). The cumulative findings of 3 cases with 18 previously reported MEN1-PC/APN cases revealed that the mean serum calcium (Ca) level was 3.15 ± 0.44 mmol/L and the median parathyroid hormone (PTH) level was 327 pg/mL (214.1, 673.1), both of which were significantly higher as compared to the respective levels in non-PC/APN MEN1 patients at our center [Ca: 2.78 mmol/L [2.61, 2.88], PTH: 185.5 pg/mL [108.3, 297.0]; P = 0.0003, 0.0034, respectively]. Conclusion: MEN 1-PC/APN is a rare disease, with a prevalence of only 2.0% among the MEN1-HPT cohort at our center. The affected patients recorded higher serum Ca level and PTH levels than those with MEN1-associated benign tumors. However, the diagnosis of MEN1-PC/APN is based upon pathology most of the times.

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“…С одной стороны, непосредственно молодой возраст пациентов с МЭН1 может объяснить высокие уровни BALP, однако авторы не исключают прямую роль дефицита менина. При инструментальной оценке, сопоставимо с результатами исследований Wang et al [31], пациенты с ПГПТ при МЭН1 в сравнении со спорадической формой имели более низкие показатели МПК по Z-критерию в поясничном отделе позвоночника и шейке бедренной кости, при этом обе группы не различались по средним показателям МПК по Т-критерию. Сравнение совокупных данных денситометрии при МЭН1 до и после операции показало, что ПТЭ улучшала прирост костной массы во всех оцениваемых участках.…”

Section: Discussionunclassified

Severe bone complications of primary hyperparathyroidism in a young patient with the rare verified mutation of <i>MEN1</i>

Eremkina

Sazonova

Bibik

et al. 2022

Probl Endokrinol (Mosk)

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Multiple endocrine neoplasia type 1 syndrome (MEN1) is a rare inherited disorder that can include combinations of more than 20 endocrine and non-endocrine tumors. Unfortunately, none of the described MEN1 mutations has been associated with a peculiar clinical phenotype, even within members of the same family, thus a genotype-to-phenotype correlation does not exist. MEN1 syndrome is the most common cause of hereditary primary hyperparathyroidism (PHPT), the disease penetrance of which exceeds 50% by the age of 20 and reaches 95% by the age of 40. At the same time, PHPT with hyperplasia or adenomas of the parathyroid glands (PTG) is the most distinctive manifestation of the MEN1 syndrome. One of the main symptoms of PHPT, both in sporadic and hereditary forms of the disease, is bone damage. At the time of diagnosis in PHPT/MEN1, the bone mineral density is generally lower in comparison with the sporadic form of PHPT. This may be due to excessive secretion of parathyroid hormone during the period of peak bone mass, concomitant components of the syndrome, extended surgical treatment, and the direct effect of a mutation in the menin gene on bone remodeling. This clinical case describes a young patient with severe bone complications of PHPT and uncertain rare MEN1 mutation. PHPT was diagnosed five years later from the first onset of bone complications and repeated orthopedic operations. There was the «hungry bones» syndrome after successful surgery of PHPT, which was managed with vitamin D and calcium carbonate supplementation and there is a positive dynamic in increased bone mineral density in the main skeleton after 6 months.

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Impaired geometry, volumetric density, and microstructure of cortical and trabecular bone assessed by HR-pQCT in both sporadic and MEN1-related primary hyperparathyroidism

Cited by 20 publications

References 24 publications

Bone and Mineral Metabolism Phenotypes in MEN1-Related and Sporadic Primary Hyperparathyroidism, before and after Parathyroidectomy

Bone and Mineral Metabolism Phenotypes in MEN1-Related and Sporadic Primary Hyperparathyroidism, before and after Parathyroidectomy

Prevalence of Parathyroid Carcinoma and Atypical Parathyroid Neoplasms in 153 Patients With Multiple Endocrine Neoplasia Type 1: Case Series and Literature Review

Severe bone complications of primary hyperparathyroidism in a young patient with the rare verified mutation of <i>MEN1</i>

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