[The role of calcium sensitive and vitamin D receptors in the pathogenesis of sporadic multiple parathyroid gland disease].

BACKGROUND: Sporadic multiple parathyroid gland disease is » cases of primary hyperparathyroidism (PHPT). However, a single tactic for diagnosing and operating volume in patients with this variant of PHPT has not yet been developed. One of the possible directions in the search for pathogenetically substantiated methods of diagnosis and treatment is the study of the molecular genetic features of the disease and associated clinical and laboratory factors. AIM: To study the features of the expression of calcium sensitive (CaSR) and vitamin D (VDR) receptors on the surface of parathyroid cells in primary hyperparathyroidism with solitary and multiple lesions of the parathyroid glands, as well as its changes under the influence of a decrease in the filtration function of the kidneys. MATERIALS AND METHODS: In a single center observational prospective study with retrospective data collection, there were patients who during 2019-2021. operated on for PHPT, secondary hyperparathyroidism (SHPT) and all cases of tertiary hyperparathyroidism (THPT) operated during 2014-2021. The expression of CaSR, VDR and its relationship with the main laboratory parameters, the clinical variant of hyperparathyroidism, and the morphological substrate were studied. RESULTS: The study included 69 patients: 19 with multiple and 25 with solitary PTG near PHPT, 15 with SHPT, 10 with THPT. A statistically significant decrease in the frequency of detection of normal expression of CaSR and VDR receptors occurs in any morphological variant of hyperparathyroidism and is observed in 93-60% of drugs. A decrease in the normal expression of CaSR in hyperplasia is detected statistically significantly less frequently than in adenoma (p≤0.01). The median expression intensity in adenoma was 2.5 (2:3), in hyperplasia 3.5 (3-4) (p≤0.01). The difference in the molecular mechanisms of the development of hyperparathyroidism with a predominance of a morphological substrate in the form of adenoma (PHPT with solitary adenoma) or hyperplasia (SHPT and PHPT with multiple PTG lesions) is realized in the frequency of maintaining normal CaSR expression in the PTG tissue. These mechanisms are implemented at the local level, their variability does not change under the influence of RRT. A common molecular genetic mechanism for the development of hyperparathyroidism with a predominance of a morphological substrate in the form of adenoma or hyperplasia has been found to reduce the frequency of maintaining normal VDR expression in PTG (up to 7-13%), p<0.01. This mechanism is implemented at the local level, its variability changes under the influence of RRT, reaching statistically significant differences in patients with THPT. CONCLUSION: The study demonstrates the features of changes in the expression of CaSR and VDR in PHPT with multiple lesions of the parathyroid glands. The relationship between the expression of these receptors and the clinical variant of hyperparathyroidism, the morphological substrate, the main laboratory parameters, and renal function was shown.

Parathyroid Disorders.

Parathyroid disorders are most often identified incidentally by abnormalities in serum calcium levels when screening for renal or bone disease or other conditions. Parathyroid hormone, which is released by the parathyroid glands primarily in response to low calcium levels, stimulates osteoclastic bone resorption and serum calcium elevation, reduces renal calcium clearance, and stimulates intestinal calcium absorption through synthesis of 1,25-dihydroxyvitamin D. Primary hyperparathyroidism, in which calcium levels are elevated without appropriate suppression of parathyroid hormone levels, is the most common cause of hypercalcemia and is often managed surgically. Indications for parathyroidectomy in primary hyperparathyroidism include presence of symptoms, age 50 years or younger, serum calcium level more than 1 mg per dL above the upper limit of normal, osteoporosis, creatinine clearance less than 60 mL per minute per 1.73 m2, nephrolithiasis, nephrocalcinosis, and hypercalciuria. Secondary hyperparathyroidism is caused by alterations in calcium, phosphate, and vitamin D regulation that result in elevated parathyroid hormone levels. It most commonly occurs with chronic kidney disease and vitamin D deficiency, and less commonly with gastrointestinal conditions that impair calcium absorption. Secondary hyperparathyroidism can be managed with calcium and vitamin D replacement and reduction of high phosphate levels. There is limited evidence for the use of calcimimetics and vitamin D analogues for persistently elevated parathyroid hormone levels. Hypoparathyroidism, which is most commonly caused by iatrogenic surgical destruction of the parathyroid glands, is less common and results in hypocalcemia. Multiple endocrine neoplasia types 1 and 2A are rare familial syndromes that can result in primary hyperparathyroidism and warrant genetic testing of family members, whereas parathyroid cancer is a rare finding in patients with hyperparathyroidism.

Is extensive surgery really necessary in patients with parathyroid carcinoma? Single-centre experience and a brief review of the literature.

AIM: Parathyroid carcinoma (PC) represents a rare cause of primary hyperparathyroidism (PHPT). In this paper, among patients who underwent surgery for PHPT, we compared those with benign parathyroid disease with those affected by PC in terms of demographic and preoperative biochemical features. Moreover, we singularly described all 10 cases of PC treated at our Institution (including a case that occurred in a patient with tertiary hyperparathyroidism) and a brief review of the literature. MATERIAL AND METHODS: Patients undergoing surgery for PHPT in our Unit between 2003 and 2018 were retrospectively analysed. They were divided into two groups: Group A (benign parathyroid disease), Group B (PC). The case of PC that occurred in the patient with tertiary hyperparathyroidism was not included into the two groups. RESULTS: Three hundred and eight patients were included: 299 in Group A and 9 in Group B. The mean preoperative serum PTH value and mean preoperative serum calcium level were significantly higher in Group B than in Group A (P = 0.018, P = 0.027; respectively). Including the case of PC that occurred in the patient with tertiary hyperparathyroidism, 10 patients with PC were treated at our Institution. Among these, 3 underwent a re-exploration. Disease recurrence occurred in 1 (10%) patient, who developed a local recurrence and distant metastases. CONCLUSIONS: In the presence of PHPT characterized by particularly high preoperative levels of serum PTH and calcium this malignancy should be suspected. On the basis of our experience, we believe that extensive surgery is not always necessary. KEY WORDS: Hyperparathyroidism, Parathyroid carcinoma, Parathyroid surgery.

Cardiovascular consequences of parathyroid disorders in adults.

PTH is a metabolic active hormone primarily regulating calcium and phosphate homeostasis in a very tight and short term-manner. Parathyroid disorders in adult patients reflect a variety of different conditions related either to the parathyroid glands itself or to the effects of the secreted hormone. The clinical spectrum varies from the common disease primary hyperparathyroidism (PHPT) to the orphan conditions pseudohypoparathyroidism (Ps-HypoPT) and chronic hypoparathyroidism (HypoPT). The purpose of this review is to describe the consequences of disturbances in levels or action of PTH for cardiac function and cardiovascular risk in adult patients with these disorders. Most patients with PHPT achieve the diagnose by chance and have minor or no specific symptoms. Still, these patients with mild PHPT do possess cardiovascular (CV) morbidity, however so far not proven ameliorated by surgery in controlled trials. In severe cases, the CV risk is increased and with a potential reversibility by treatment. Patients with Ps-HypoPT have resistance to PTH action, but not necessarily total resistance in all tissues. So far, no clear CV morbidity or risk has been demonstrated, but there are several aspects of interest for further studies. Most patients with HypoPT do get their hormonal deficiency syndrome following neck surgery. These patients do experience multiple symptoms and do have an increased CV-risk before the primary surgery. Based on existing data, their CV mortality do not deviate from the expected when adjusting for the preexisting increased risk. Patients with nonsurgical (NS-) HypoPT do demonstrate increased CV-risk also associated with exposure time. Endocrine disorders with alterations in PTH function have major impact on the cardiovascular system of importance for morbidity and mortality, wherefore management of these specific diseases should be optimized currently, as new data become available, however also avoiding over-treating asymptomatic patients.

Parathyroid hemangioma

Background Hemangiomas are benign neoplasms of capillary proliferation that arise from a developmental anomaly where angioblastic mesenchyme fails to form canals. Most hemangiomas arise in the head and neck region, either superficially in the skin or deeper within endocrine organs such as the parotid gland. Parathyroid hemangiomas, however, are extremely rare, with only five cases previously reported in the literature. Case presentation Herein, we present a case of a 68-year-old man with a hemangioma almost completely replacing the right upper parathyroid gland, grossly measuring 1.3 × 1.3 × 1.2 cm and weighing 700 mg, associated with primary hyperparathyroidism. Conclusions Parathyroid gland enlargement due to vascular neoplasms such as hemangiomas can mimic, both clinically and radiographically, hyperplasias and/or adenomas. Surgeons need to be aware of the presence of this entity and should consider it in the differential diagnosis of hyperparathyroidism or parathyroid gland enlargement.

Robotic resection of a mediastinal parathyroid cyst.

Mediastinal parathyroid cysts (PC) are rare, benign lesions, reported in fewer than 150 cases worldwide. Although most are asymptomatic and discovered incidentally on imaging, symptoms of dyspnea, dysphagia, hoarseness, palpitations, hypercalcemia, and innominate or jugular venous thrombosis have been reported. Sternotomy or thoracotomy has traditionally been the approach used to resect mediastinal PCs. We describe the first reported case of a robot-assisted resection of a mediastinal PC.

[Parathyroid disease: The full spectrum, from adenoma to carcinoma. Report of 3 cases]. / Enfermedad paratiroidea: el espectro completo, de adenoma a carcinoma. Reporte de 3 casos.

BACKGROUND: Primary hyperparathyroidism is a disease characterised by the autonomous production of parathyroid hormone. The most common cause is an adenoma, followed by hyperplasia, and rarely carcinoma. CLINICAL CASES: Three cases are presented. The first case is associated with a brown tumour that was diagnosed as hyperplasia after study and surgery. The second case was related to pathological fractures, and a lower right adenoma 236 times bigger than a normal parathyroid was excised. The last case presented with abdominal pain and heartburn. Histopathology reported a carcinoma, which was removed using surgery en bloc. All patients have improved. CONCLUSION: Hyperparathyroidism symptoms are very difficult to identify and diagnose, thus a detailed and broad approach is needed when hyperparathyroidism is suspected.

The eye and the skin in endocrine metabolic diseases.

The eye and skin may offer critical clues to the diagnosis of a varied spectrum of metabolic diseases from endocrine origin and their different stages of severity, such as diabetes mellitus and Graves disease. On the other hand, such entities may compromise the eye and visual function severely, and awareness of these possible associations is an important step in their diagnosis and management. A large number of less common endocrine diseases may also have significant ocular/visual or skin involvement. Often the etiologic relationship between the endocrine metabolic disease and the ocular compromise is unknown, but diverse pathogenetic mechanisms may act through a common pathologic pathway producing ocular damage, as occur in diabetic retinopathy. This review emphasizes the ocular and skin manifestations of different metabolic diseases of endocrine origin.

Electrocardiographic changes in the most frequent endocrine disorders associated with cardiovascular diseases. Review of the literature.

Car- diovascular abnormalities associated with endocrine diseases are often frequent and due to complex relationships between endocrine glands (with internal secretion) and cardiovascular system (heart and vessels). Certain hormones secreted by the endocrine glands (particularly the thyroid and pituitary gland) excesses or deficiencies, are involved in morphogenesis, growth processes and activity regulation of cardiovascular system, most often in connection with the autonomic nervous system. There are also a lot of electrocardiographic changes caused by hormonal disorders that requires differential diagnosis and represents the source of erroneous diagnosis. Endocrine pathology occurred later than a heart disease, may worse heart function. Ignoring the cardiovascular events that may occur in the evolution of endo- crine diseases, may induce increased mortality due to cardiovascular complications.

A rare case of a parathyroid adenoma inside a parathyroid cyst.

Parathyroid cysts (PCs) are rare lesions, located in the neck and anterior mediastinal region. The vast majority are non-functioning, presented as nodular cervical lesions. Large, non-functioning PCs can manifest with compressive symptoms of the surrounding tissues. Rarely, PCs produce excessive amounts of parathyroid hormone (PTH), resulting in primary hyperparathyroidism. We report a case of functional PC, describing its diagnostic and therapeutic approach.

A rare case of a parathyroid adenoma inside a parathyroid cyst / Um caso raro de adenoma de paratireoide em um cisto de paratireoide

Parathyroid cysts (PCs) are rare lesions, located in the neck and anterior mediastinal region. The vast majority are non-functioning, presented as nodular cervical lesions. Large, non-functioning PCs can manifest with compressive symptoms of the surrounding tissues. Rarely, PCs produce excessive amounts of parathyroid hormone (PTH), resulting in primary hyperparathyroidism. We report a case of functional PC, describing its diagnostic and therapeutic approach.

Os cistos de paratireoide (PCs) são lesões raras, localizadas no pescoço e na região do mediastino anterior. A grande maioria é não funcionante, apresentando-se como lesões cervicais nodulares. PCs não funcionantes de grandes dimensões podem se manifestar com sintomas compressivos. Raramente, PCs produzem quantidades excessivas de hormônio da paratireoide (PTH), o que resulta em hiperparatiroidismo primário. Relatamos um caso de PC funcional, descrevendo sua abordagem diagnóstica e terapêutica.

[Mediastinal parathyroid cyst - a case report]. / Kalkkirtlablaðra í midmæti - sjúkratilfelli.

The most common causes of mediastinal masses are thymomas, lymphomas and neuromas. Mediastinal cysts, such as bronchogenic cysts, which are usually benign, are less common. We report the case of a 59-year-old woman with a history of progressive dysphagia. A computed tomography scan revealed a cystic lesion in the anterior mediastinum. The cyst was surgically resected and turned out to be a benign parathyroid cyst. The patient's symptoms disappeared after surgical removal. Both the serum calcium and parathyroid hormone levels were normal before, and after surgery. Parathyroid cysts are rare lesions of the mediastinum and only around 100 cases have been reported in literature. Here we report the first case of a mediastinal parathyroid cyst in Iceland.

[Bilateral neck exploration with intraoperative iPTH assay in patients not eligible for minimally invasive parathyroidectomy]. / Obustronna eksploracja szyi ze sródoperacyjnym monitoringiem stezenia parathormonu u chorych niekwalifikujacych sie do maloinwazyjnej paratyreoidektomii.

BACKGROUND AND AIMS: Bilateral neck exploration (BNE) is the preferred surgical technique in patients with primary hyperparathyroidism (pHPT) not eligible for minimally invasive parathyroidectomy (MIP). The aim of this study was to assess indications for BNE in the era of MIP, including short-term outcomes of surgery with intraoperative intact parathyroid hormone (iPTH) monitoring added-value. METHODS: Data of 155 patients with pHPT qualified for BNE with intraoperative iPTH monitoring and treated in 2003-2012 were retrospectively analysed. All patients underwent biochemical and imaging testing in the preoperative work-up. The following endpoints were analysed in this study: indications for BNE, short-term outcomes of surgery, and intraoperative iPTH monitoring added-value. RESULTS: Indications for BNE were: negative preoperative imaging in 65 (41.9%) patients, concomitant goitre necessitating surgical removal in 51 (32.9%) patients, MEN 1 syndrome in 17 (11.0%) patients, lithium treatment in 12 (7.7%) patients, lacking consent for MIP in 5 (3.2%) patients, and MEN 2A syndrome in 5 (3.2%) patients. The extent of parathyroidectomy was a solitary parathyroid adenoma removal in 97 (62,6%) patients, subtotal parathyroidectomy in 41 (26.4%) patients, and double-parathyroid adenoma removal in 17 (11,0%) patients. Use of intraoperative iPTH monitoring influenced on the extent of parathyroid tissue resection in 16(10.3%) patients. Normalised total serum calcium values were observed in 154 (99.4%) patients during a 6-month follow-up. CONCLUSIONS: BNE in patients with pHPT is the preferred surgical technique in the following circumstances: a suspicion of multiglandular parathyroid disease (MEN 1 or 2A syndrome, familial hyperparathyroidism, lithium therapy), a negative preoperative imaging, in patients not consenting for MIP, and in cases with concomitant goitre necessitating surgical treatment. Use of intraoperative iPTH monitoring influences on the extent of parathyroid tissue resection in one often patients, hence assuring the highest quality of surgical treatment.

Coexisiting adenoma and granuloma involving the right inferior parathyroid gland with adjacent ectopic thymic tissue.

Inflammatory lesions, particularly granulomas, involving adenoma of the parathyroid gland are rare. Ectopic thymic tissue is commonly associated with the thyroid and/or parathyroid gland due to their close embryonic relationship. We report a rare case of coexisting adenoma and granuloma of the parathyroid gland with adjacent ectopic thymic tissue.

[A case of idiopathic neck bleeding from a parathyroid cyst].

Bleeding from parathyroid cysts is rare. The retropharyngeal space has a very soft structure and if bleeding spreads to this space, airway obstruction can easily occur. We report on a 50-year-old female case with idiopathic neck bleeding from a left parathyroid cyst without any episode of injury. The patient complained of neck swelling after exercise and went to a nearby hospital. At the hospital, the doctor thought this swelling was caused by retropharyngeal bleeding from a tumor behind the left thyroid gland. Embolization of the left thyroid artery was performed. However, the next day, airway obstruction.was occurred and she was brought to our hospital. An emergency operation was performed to open the left neck swelling region. The operation findings and pathological examination showed that the bleeding was caused by a parathyroid cyst and airway obstruction had occurred because of retropharyngeal edema. In this case, the bleeding may have been caused by torsion of the neck when the patient exercised. The retropharyngeal edema probably resulted from the delay of delivery of the lymphatic and venous return and the arterial embolization. 1 year after the operation, the patient is very well and there has been no recurrence.