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Primary hyperparathyroidism (PHPT) is one of the most frequent endocrine disease in developed countries. It mainly occurs as sporadic cases (about 90-95% of cases), while only the remaining 5-10% is represented by familial inherited parathyroid disorders due to causative mutations in specific target genes. Clinical variability among the different familial parathyroid syndromes is generally linked to the specific mutated gene and it can predispose subjects to different manifestations of parathyroid pathology, various degrees of PHPT severity, persistence and/or after-surgery recurrences. Genetic tests is helpful in differential diagnosis favouring the recognition of the specific familial PHPT syndrome and, subsequently, in planning the most suitable surgical procedures and/or pharmacological interventions. Moreover, genetic test is important to recognise mutation carriers, within PHPT familial forms, even before the appearance of biochemical and/or clinical symptoms. This review resumes general concepts about genetic diagnosis of PHPT in familial hereditary syndromes, specifically describing why, when, and which genetic screenings should be performed in every specific PHPT-associated parathyroid disease. more...

The parathyroid glands are critical to maintaining calcium homeostasis through actions of parathyroid hormone (PTH). Recent clinical and molecular research has shown that direct and indirect actions of PTH also affect the heart and vasculature through downstream actions of G protein-coupled receptors in the myocardium and endothelial cells. Patients with disorders of the parathyroid gland have higher incidences of hypertension, arrhythmias, left ventricular hypertrophy, heart failure, and calcific disease which translate into increased cardiac morbidity and mortality. Importantly, clinical research also suggests that early treatment of parathyroid disorders through medical or surgical management may reverse cardiovascular remodeling and mitigate cardiac risk factors. more...

Ultrasound in the head and neck has utility beyond evaluation of thyroid and parathyroid disorders. It offers a low-cost method of evaluating congenital lesions like branchial cleft anomalies, dysontogenetic cysts, and thyroglossal duct cysts. Given that these lesions are often found in younger patients, ultrasound has the advantage of not exposing the patient to ionizing radiation and does not require sedation as might sometimes be required with MRI. Ultrasound can also be a useful modality in evaluating benign neoplastic lesions of the head and neck such as lipomas, schwannomas, benign salivary gland tumors, and vascular anomalies. Though it is often not the most common modality used, malignant non-thyroid lesions, including squamous cell carcinoma—the most common metastatic neck mass—lymphoma, and salivary gland malignancies, are also easily evaluated with ultrasound. Inflammatory conditions such as sarcoidosis and sialadenitis and acquired conditions like laryngoceles can also be adequately evaluated with ultrasound. more...

I have been invited to reflect on in this chapter what stimulated me to develop an interest in office-based ultrasound in lieu of continuing with the standard imaging by radiology. I learned the methodology from a skilled, highly published radiologist who was also department chair of diagnostic imaging and intervention. He concentrated his imaging interests to the head and neck to include MRI, PET scan, CT scan, and all aspects of ultrasound. His belief was that no one specialty owns ultrasound and in fact the technology belongs best in the hands of the clinician. For 12 years prior to my retirement from clinical practice in 2009, head and neck ultrasound had been an integral part of the assessment of every head and neck patient. With a practice heavily skewed toward thyroid and parathyroid disorders, ultrasound is integral to diagnosis, biopsy, and follow-up. Less common conditions such as lymphoma, inflammatory nodal enlargement, metastatic lymphadenopathy, cysts, congenital masses, hematoma, and abscess formation similarly are amenable to first-visit ultrasound. There are many reasons to develop this tool and it is instructive to examine the pros and cons along with considerations for the future. more...

The parathyroid glands regulate calcium metabolism through release of parathyroid hormone (PTH). Because most parathyroid disorders present with abnormalities of serum calcium, they commonly appear in differential diagnoses [1]. Based on that, every patient with a calcium abnormality should be investigated for parathyroid disorders. Moreover, the prevalence of parathyroid diseases is still unclear in some environments, and understanding the diagnosis and treatment of parathyroid disorders is very important for the head and neck surgeon. more...

Germline genetic tests can be extremely helpful for clinical diagnosis of patients and their families when a known deleterious change is found, but there are many reasons why gene testing and interpretation is not straightforward. This chapter pertains to monogenic conditions in which the genetic contribution to a parathyroid disorder is well-established. These disorders include: familial hypocalciuric hypercalcemia, neonatal severe hyperparathyroidism, hyperparathyroidism-jaw tumor syndrome and parathyroid carcinoma and multiple endocrine neoplasia types 1, 2, and 4. more...

Intraoperative PTH monitoring is now a commonly utilized tool in the surgical management of patients with sporadic primary hyperparathyroidism. Its use during parathyroidectomy guides the surgeon in operative decision making and is an often critical aspect of the surgery itself. The use of intraoperative PTH monitoring has several benefits over traditional bilateral neck exploration, including shorter operative times and hospital stays, and the reduced potential for complications. Most surgeons recommend using a greater than 50 % PTH drop criterion from the pre-incision PTH value to a 10 min post-excision PTH value for measuring operative success. Additionally, many surgeons recommend a post-excision PTH value that returns to the normal reference range. This criterion has improved accuracy in identifying parathyroid adenomas with reduction of unnecessary bilateral neck exploration. Further investigation is required into the utility and efficacy of intraoperative PTH monitoring for parathyroid disorders other than sporadic primary hyperparathyroidism. more...

Objectives: To determine the prevalence and risks factors of vitamin D deficiency, as well as its relationship with morbidity and mortality in a PICU. Materials and methods: An observational prospective study in a tertiary children's University Hospital PICU conducted in two phases: I: Cohorts study, and II: prevalence study. The study included 340 critically ill children with ages comprising 6 months to 16 years old. Exclusion criteria: Chronic kidney disease, known parathyroid disorders, and vitamin D supplementation. Total 25-hydroxyvitamin D [25(OH)D] was measured in the first 48 h of admission to a PICU. Parathormone, calcium, phosphate, blood gases, blood count, C-reactive protein, and procalcitonin were also analysed. A record was also made of demographic features, characteristics of the episode, and complications during the PICU stay. Results: The overall prevalence rate of vitamin D deficiency was 43.8%, with a mean of 22.28 (95% CI 21.15–23.41) ng/mL. Patients with vitamin D deficiency were older (61 vs 47 months, P = 0.039), had parents with a higher level of academic studies (36.5% vs 20%, P = 0.016), were admitted more often in winter and spring, had a higher PRISM-III (6.8 vs 5.1, P = 0.037), a longer PICU stay (3 vs 2 days, P = 0.001), and higher morbidity (61.1% vs 30.4%, P 6 meses, excluyendo a aquellos con enfermedad renal crónica, trastornos paratiroideos y suplementación con vitamina D. Se realizó medición de 25-hidroxivitamina D total (25[OH]D) en las primeras 48 h del ingreso, parathormona (PTH), calcio, fósforo, gasometría venosa, hemograma, proteína C reactiva y procalcitonina. Se registraron datos sociodemográficos, características del episodio y complicaciones. Resultados: La prevalencia de VDD ( more...

Introduction Renal dysfunction associated with primary hyperparathyroidism was studied mostly in patients with mildly asymptomatic or asymptomatic parathyroid disorders. Clinical and biochemical profile of asymptomatic and symptomatic disease vary grossly. The present study analyzed renal function in patients with primary hyperparathyroidism and the relation with clinical features, biochemical features and sonologic changes of kidneys in a cohort of primary symptomatic hyperparathyroidism. Study design Cross-sectional study was done in a cohort of surgically proven patients of primary symptomatic hyperparathyroidism. Urinary tract was evaluated with high definition Ultrasonography and renal function was estimated using Modification of Diet in Renal disease (MDRD) Study equation before and after parathyroidectomy. Result Case records 145 patients were studied and there were 44 patients (30.4%) with estimated Glomerular Filtration Rate (eGFR) less than 60 mL/min/1.73 m 2 . Associated renal complications, coincident hypertension and high serum level of calcium were strongly associated with renal dysfunction. Renal dysfunction marginally improved after 1 year following surgical cure of hyperparathyroidism in 25% of patients with renal dysfunction. There was no detectable change in renal function during the follow up period. Conclusion There was high prevalence of renal dysfunction among symptomatic hyperparathyroidism. Patients with primary hyperparathyroidism should undergo complete sonological assessment of kidneys which may include presence of nephrocalcinosis and cortical echogenicity. Successful parathyroidectomy prevented deterioration of renal function in most of patients and a significant subset with renal dysfunction showed improvement of functional status. more...

The pathogenesis of obstructive sleep apnoea/hypopnoea syndrome (OSAHS) is uncertain and likely to be a multifactorial process. The molecular mechanisms of the relationship between vitamin D status and inflammatory biomarkers are still not well understood. We aimed to investigate the relationship between Interleukin 17 (IL-17) and vitamin D among severe OSAHS patients. It was a coss-sectional survey carried between September 2014 and September 2015 in RABTA Pulmonary Department, Tunis. Patients with a severe OSAHS were randomly enrolled. Included patients had to show none of the following criteria: clinical signs of heart failure, autoimmune or acute inflammatory disease, cancer, parathyroid disorders, chronic liver disease, chronic renal disease, sarcoidosis, or osteoporosis. Anthropometric parameters and medical history were collected. The serum levels of IL-17 and 25-hydroxyvitamin D (25(OH) D) were determined by ELISA. Ninety two patients were enrolled with a mean age of 52.3 years (52% male, 48% female). All patients had a vitamin D deficiency (mean level: 7.9ng/ml). IL-17 levels were elevated (mean level: 20.3pg/ml). A negative association was observed between plasma IL-7 levels and serum 25(OH)D (r=-0,64540, p -4 ). The level of 25(OH)D was positively correlated with hypertension (p=0,026), diabetes (p=0.006), nycturia (p=0,026), the mean O2 saturation (p=0,034) and lowest O2 saturation (p=0,015). IL-17 levels were positively correlated only with nycturia (p=0,024). Both 25(OH)D and IL-17 may be involved in the pathogenesis of OSAHS and may represent a target for therapeutic intervention. Furthers studies are needed to clarify this association. more...

Fahr’s disease is a rare degenerative neurological disorder characterized by the presence of abnormal calcium deposition and associated cell loss in the areas of the brain that control movement, including the basal ganglia and cerebral cortex. Clinical findings associated with Fahr's disease include parkinsonism, dystonia, chorea, ataxia and psychiatric symptoms. Fahr's disease may result from metabolic disorders, especially parathyroid disorders. We report our experience with 4 patients admitted to our emergency department with complaints such as convulsions, hand spasms, loss of consciousness, and weakness. Computed tomography of all patients showed calcification in the bilateral basal ganglia. The purpose of this paper is to draw attention to a rare disorder involved in the etiology of elderly patients admitted to the emergency department for seizure and/or unconsciousness. more...

Parathyroid disorder associated with hypercalcemia during pregnancy reduces the normal foetal growth, skeletal mineralization and serum calcium concentration. Hypercalcemia increases the risk of birth loss and foetal postpartum complications which includes intrauterine growth retardation, low birth weight, preterm delivery, neonatal hypocalcaemia, tetany and still-birth. Human and animal study exhibit benefits from local hyperthermia induced by ultrasound exposure. Ten-month-old primiparous New Zealand White (NZW) does were assigned into three different groups; control (C), healthy NZW does and free from ultrasound insonation; hyperparathyroidism (HPT), a positive control group having hypercalcemia conditions established through administering phosphate intravenously for 5 months and free from parathyroid ultrasound insonation; treatment (T), an experimental group experiencing hypercalcemia condition and receiving parathyroid ultrasound insonation during pregnancy as intervention to hyperparathyroidism. In treatment group rabbit were exposed for 30, 60 and 90 minutes of parathyroid ultrasound exposure on 1st, 2nd and 3rd gestational stage accordingly. Following birth, foetal serum calcium (SCa), body weight (BW), crown-to-rump length (CRL), total body length (TBL), bi-parietal diameter (BPD) and femur length (FL) of the foetal were measured. Maternal hypercalcemia during pregnancy produced small to gestational age (SGA) progeny with significant reduction of BW, CRL, TBL, BPD, FL and SCa compared to normal. While the outcome of ultrasound exposure given during middle 2nd gestational stage resulted in significantly increase in progeny mean average BW, CRL, TBL, BPD FL and SCa compared to HPT group. Ultrasound induced local heating demonstrated an improved in the physiological development of progeny in the cases of maternal hypercalcemia condition. more...

Introduction: Patients with thyroid tumor especially those having thyroid cancer with lymph nodes metastasis may benefit thyroidectomy with lymph nodes dissection. The surgery requires exa...

Introduction: Preoperative and intraoperative localization strategies have allowed the development of minimally invasive surgical techniques for the treatment of primary hyperparathyroidis...

BackgroundNormocalcemic primary hyperparathyroidism (PHPT-N) is a condition that may have similar long-term implications to primary hyperparathyroidism (PHPT); however, differential diagnosis and treatment for parathyroid disorders are not clearly defined. We investigated the effect of an oral peptone and an oral calcium load on calcium-regulating hormones in PHPT-N compared with PHPT and healthy controls to provide a new potential diagnostic tool.DesignCase–control study.MethodsWe evaluated serum gastrin, PTH, ionized calcium, and phosphate responses to oral calcium (1 g) and peptone (10 g) load in 22 PHPT and 20 PHPT-N patients matched for PTH serum values. Moreover, 30 healthy subjects were enrolled as controls. In 12 patients for each group, we also performed the oral peptone test adding aluminum hydroxide (AH) to suppress phosphate absorption.ResultsIn PHPT patients, PTH increased significantly 30 min after the oral peptone load, while no significant increase was found in PHPT-N and controls. After oral calcium load, PTH remained stable in PHPT while it decreased dramatically in PHPT-N patients, and ionized calcium increased significantly in each of the three groups. Peptones plus AH induced a blunted PTH increase in the three groups.ConclusionsConsidering the marked difference in PTH response elicited by peptones in PHPT compared with PHPT-N, we suggest that the oral peptone test could be added to the diagnostic evaluation of PHPT patients. In case of absent response to peptones, patients should have their serum calcium levels assessed twice a year in accordance with recent guidelines. more...

The parathyroid disorder is an important problem in clinical endocrinology. There are several possible causes of parathyroid disorder. Infection is an uncommon cause of parathyroid disorder. The parasitic infection induced parathyroid disorder is an important problem in tropical medicine. In this paper, the authors briefly summarize and discuss on the important abnormalities of parathyroid due to important tropical parasitic infections. more...

‘inflammation of parathyroid gland’, ‘lymphocytic infiltrate’, ‘tuberculosis of the parathyroid’, ‘sarcoidosis’, and ‘graulomatous inflammation’. Three autopsy series, 27 articles and 96 case reports with inflammatory parathyroid disorders were identified. Autopsy series showed lymphocytic infiltrates in up to 16% of all cases. The entire material reported lymphocytic infiltrates (n = 69), parathyroiditis with germinal centres (n = 15), sarcoidosis (n = 6), tuberculosis (n = 4), and other granulomatous diseases (n = 2). Conclusions: Distinct inflammatory and granulomatous processes in the parathyroid gland are rare. Scanty lymphocytic infiltrates are common, and occur in generalized inflammatory conditions or venous congestion. We note the surprising absence of an association between histological proof of parathyroiditis and hypoparathyroidism. more...

Pregnancy and lactation are associated with remarkable changes in calcium homeostasis, resulting from pregnancy-induced alterations in the production, metabolism and excretion of calcium and calcitropic hormones. Therefore, the clinical course of disorders affecting the parathyroid glands during pregnancy and lactation is often atypical. As the symptoms experienced by patients with parathyroid disorders are not specific, their diagnosis during gestation and breastfeeding may be sometimes very difficult. If left untreated, parathyroid disorders may pose a significant risk to the mother and fetus associated with increased perinatal and maternal morbidity and mortality. Hypoparathyroidism is a disorder rarely observed during pregnancy, resulting in most cases from surgical thyroidectomy. In our article, we present the case of a 32-year-old female with primary hypoparathyroidism diagnosed for the first time during pregnancy. We describe in details the treatment strategies undertaken in this patient and their impact on the outcome of pregnancy and lactation. more...

Current multimodal imaging techniques offer practicing providers the adequate framework to plan and accomplish care for patients with thyroid and parathyroid disorders. Available imaging modalities include ultrasonography (US), nuclear scintigraphy, computed tomography (CT), and magnetic resonance imaging (MRI). US remains the most cost-effective and the safest approach for the initial evaluation of the thyroid gland. Parathyroid subtraction scintigraphy provides localization of pathologic parathyroid glands with the greatest sensitivity. Localizing imaging studies allow for surgical planning with minimal risk and morbidity to the patient. CT scan and MRI play an adjunctive role in the further characterization of neck pathology. more...

The G-protein-coupled calcium-sensing receptor (CaSR), upon activation by Ca(2+) or other physiologically relevant polycationic molecules, performs diverse functions in the brain. The CaSR is widely expressed in the central nervous system (CNS) and is characterized by a robust increase in its expression during postnatal brain development over adult levels throughout the CNS. Developmental increases in CaSR levels in brain correlate with myelinogenesis. Indeed, neural stem cells differentiating to the oligodendrocyte lineage exhibit the highest CaSR expression compared with those differentiating to astrocytic or neuronal lineages. In adult CNS, CaSR has broad relevance in maintaining local ionic homeostasis. CaSR shares an evolutionary relationship with the metabotropic glutamate receptor and forms heteromeric complexes with the type B-aminobutyric acid receptor subunits that affects its cell surface expression, activation, signaling, and functions. In normal physiology as well as in pathologic conditions, CaSR is activated by signals arising from mineral ions, amino acids, polyamines, glutathione, and amyloid-beta in conjunction with Ca(2+) and other divalent cationic ligands. CaSR activation regulates membrane excitability of neurons and glia and affects myelination, olfactory and gustatory signal integration, axonal and dendritic growth, and gonadotrophin-releasing hormonal-neuronal migration. Insofar as the CaSR is a clinically important therapeutic target for parathyroid disorders, development of its agonists or antagonists as therapeutics for CNS disorder could be a major breakthrough. more...

Technological developments have significantly contributed to the rapid evolution of the surgical management of parathyroid disorders. The ability to physiologically determine the intraoperative status of the patient is now possible through the assessment of changing levels of intact parathyroid hormone (PTH) during surgery. In most patients with primary hyperparathyroidism, this method provides biochemical confirmation of hyperfunctional gland removal, and is predictive of a eucalcemic state and surgical cure. Patients with renal-induced disease (excluding tertiary hyperparathyroidism) do not follow the same kinetic decline in PTH and are therefore less likely to benefit from this modality. An emerging population of patients with primary disease and lower baseline levels of intact PTH appear to demonstrate a greater likelihood of manifesting multiple gland disease and do not follow a consistently predictable kinetic degradation profile. Although this category of patients will require further investigation, potentially resulting in modification of the utility of intraoperative determination of PTH levels, it is clear that this modality represents a very powerful technique which has significantly enhanced the surgical treatment of parathyroid disease. more...

Hyperparathyroidism (HPT) is a common complication in haemodialysis patients, associated with morbidity and sometimes mortality [1]. In the majority of patients with secondary hyperparathyroidism (2HPT), this can be managed by medical treatment but this does not always give adequate control of the parathyroid disorder. Some patients require intervention treatment of the parathyroid glands including parathyroidectomy (PTx) and percutaneous ethanol injection therapy (PEIT). Successful surgical treatment achieves a dramatic drop of the parathyroid hormone (PTH) level, relieves the patient from clinical symptoms and reduces mortality [2]. Surgical indications in 2HPT and the frequency of PTx might be influenced by medical therapy, i.e. vitamin D and vitamin D analogues injection therapy, sevelamer hydrochloride, calcimimetics, etc., and the composition of the haemodialysis patients, i.e. race, gender, age and duration of haemodialysis. In this review the current status of PTx for 2HPT in our country is presented including the experiences from our department. more...

Successful parathyroidectomy requires advanced surgeon experience. We aim to examine population characteristics at risk of being managed by low-volume surgeons.A cross-sectional study was performed utilizing the Nationwide Inpatient Sample database, 2004 to 2009. The study population included adult inpatients who underwent parathyroidectomy for primary hyperparathyroidism.A total of 3,503 discharge records were included. Men, Hispanics, and those with Medicaid/Medicare health coverage were more likely to be managed by low-volume surgeons (P.05 each). Low-volume surgeons were more likely to operate in rural (odds ratio [OR], 3.99; 95% confidence interval [CI], 1.95 to 8.16; P.001) or nonteaching hospitals (OR, 2.15; 95% CI, 1.42 to 3.27; P.001). Southern region of the United States had a high prevalence of low-volume surgeons compared with other regions (Southern: 51.3%, Northeast: 24.3%, Midwest: 25.6%, and West: 27.6%, P.001). Operations by the low-volume surgeons associated with a higher risk of postoperative complications (OR, 1.81; 95% CI, 1.11 to 2.97) and a hospital stay more than 2 days (OR, 7.12; 95% CI, 3.75 to 13.45; P.001).Certain populations are at risk of management by low-volume surgeons based on their demographic and economic characteristics. more...

The thyroid gland disease incidence in hyperparathyroidism (HPT) is higher than the incidence of thyroid disease in general population. Likewise, HPT is more frequent in patients primary admitted due to thyroid disease, than in general population. The aim of this study was to determine the incidence and clinical characteristics of concomitant HPT and thyroid disease, based on a single center experience. From 2009 to 2014, a total of 4882 patients underwent thyroidectomy and/or parathyroidectomy at the Center for Endocrine Surgery, Belgrade. We reviewed the database to find out indications for surgery, clinical characteristics, operative and histopathological findings. Out of 4033 patients, who underwent thyroidectomy, in 114 cases (2.8 %) parathyroidectomy was simultaneously performed. Out of these 114 patients, 42 patients (37 %) had normocalcemic HPT. Among 849 patients primary operated due to HPT, thyroid gland disease that required surgery was found in 224 (26.4 %). In patients primary seen for HPT, thyroid cancer was found in 22 (9.8 %), Hashimoto’s thyroiditis in 41 (18.3 %) and micropapillary carcinoma in 36 cases (16.1 %). Due to residual or recidivant HPT, 16 patients (15 who primary underwent parathyroidectomy and 1 primary seen for thyroid disease) needed a reoperation. There are a considerable number of patients with concomitant thyroid and parathyroid disease; this justifies the routine analyses of calcemia and PTH level in patients preparing for thyroidectomy, and sets up the ground for the thyroid investigations in HPT. more...

Introduction: Fine-needle aspiration with parathyroid aspiration has been described as a preoperative adjunct to confirm parathyroid tissue. However, intraoperative parathyroid hormone (IO...

Complications related to parathyroid surgery are rare but most commonly include recurrent laryngeal nerve injury, hematoma formation, and hypocalcemia. In highly experience centers, >95 % of patients are normocalcemic postoperatively. Parathyroid hormone (PTH) on the other hand remains elevated in a significant number of patients with normocalcemia after parathyroidectomy. Primary hyperparathyroidism (PHPT) is due to a single parathyroid adenoma in most patients, with a chief cell adenoma being the most common pathologic variant. However, oxyphil adenomas, lipoadenomas, clear cell adenomas, and atypical parathyroid adenomas can also be seen. Parathyroid carcinoma and parathyromatosis are other pathologic entities that should be considered in persistent and recurrent PHPT. Parathyroid hyperplasia is the cause of PHPT in approximately 15 % of patients and is seen in familial parathyroid disorders such as MEN1, MEN2A, MEN4, hyperparathyroidism-jaw tumor syndrome, and familial isolated PHPT. more...

Pituitary failure occurs due to multiple reasons including genetic abnormalities, trauma, infections, empty sella syndrome, Sheehan’s syndrome, etc.

Introduction: Four-dimensional CT (4D CT) can be used in the setting of hyperparathyroidism to localize parathyroid adenomas. In particular, 4D CT can help differentiate an adenoma from co...

OBJECTIVES The aim of our study was to evaluate the shear wave velocity (SWV) of parathyroid lesions by point shear wave elastography (SWE) and to compare their stiffness with that of thyroid nodules and normal thyroid parenchyma quantitatively. METHODS Thirty-six patients considered to have parathyroid disorders by clinical and laboratory tests and scintigraphy were enrolled in the study between January 2012 and February 2015. Conventional sonography, Doppler sonography, and the Virtual Touch tissue quantification (VTQ) method of point SWE (Siemens Medical Solutions, Mountain View, CA) were conducted with a linear transducer (4-9 MHz) while the patients were in the supine position. Then we compared our VTQ measurements with pathologic results. RESULTS The 36 patients included 31 female and 5 male patients with a mean age ± SD of 49 ± 15.7 years (range, 15-79 years). The mean SWV of parathyroid hyperplasia lesions (n = 4) was 1.46 ± 0.23 m/s, whereas the mean SWV of parathyroid adenomas (n = 32) was 2.28 ± 0.50 m/s. The mean SWV of normal thyroid parenchyma (n = 36) was 1.62 ± 0.20 m/s, and the mean SWV of benign thyroid nodules (n = 21) was 2.25 ± 0.51 m/s. A significant difference was found between SWV values of normal thyroid parenchyma and parathyroid adenoma (P < .001). A cutoff value of 1.73 m/s for adenomas led to 90.0% sensitivity and 80.6% specificity. CONCLUSIONS The VTQ method of point SWE may contribute to the discrimination of parathyroid adenomas from the thyroid gland. However, more comprehensive studies are needed. more...

Introduction Reoperative parathyroidectomy is required when there is persistent or recurrent hyperparathyroidism following the initial surgery (at least 5% of parathyroidectomies nationally). By convention, ‘persistent disease’ is defined as the situation where the patient has not been cured by the first operation. The term ‘recurrent hyperparathyroidism’ is used when the patient was confirmed to be biochemically cured for six months from the first operation but has hyperparathyroidism after this date. Reoperative surgery is associated with higher rates of postoperative complications as well as a greater rate of failure to cure. The aim of our study was to review our departmental experience of reoperative parathyroidectomy, with a view to identify patterns of disease persistence and recurrence. Methods Using a departmental database, patients were identified who had undergone reoperative parathyroidectomy between 2006 and 2014. All the pre, intra and postoperative information was documented including the operative note so as to record the location of the abnormal parathyroid gland found at reoperation. Results Almost two-thirds (63%) of patients had negative, equivocal or discordant conventional imaging so secondary investigative tools were required frequently. The majority of abnormal glands were found in eutopic locations. The most common locations for ectopic glands were intrathyroidal, mediastinal and intrathymic. A third (33%) of the patients had multigland disease and over a quarter (28%) had coexisting thyroid disease. Conclusions Persistent hyperparathyroidism represents a challenging patient subgroup for which access to all radiological modalities and intraoperative parathyroid hormone monitoring are required. Patient selection for reintervention is a key determinant in the reoperation cure rate. more...

Introduction: Subtotal parathyroid resection is indicated when secondary or tertiary hyperparathyroidism (HPT) develops and may be indicated also in patients with primary HPT and multiglandular disease. Three different surgical procedures are used to treat diffuse parathyroid hyperplasia: total parathyroidectomy with or without autotransplantation, and subtotal parathyroidectomy. One of the main complications is transient or persistent hypoparathyroidism. In this video, we show our technique of subtotal parathyroidectomy using a fluorescent dye (indocyanine green [ICG]) to check for the vascularization of the parathyroid remnant, to avoid definitive postoperative hypoparathyroidism. Methods: We present a 64-year-old man with end-stage chronic kidney disease dialyzed since 2008. His parathyroid hormone (PTH) level was 106 pmol/L, corrected calcium level was 2.29 mmol/L and phosphate 1.63 mmol/L under maximal medical treatment, and he had significant bone disease. A subtotal parathyroidectomy was scheduled. After reclining pre-thyroid muscles, we medialized the right thyroid lobe to expose the right parathyroid glands. The superior one was a good candidate to be preserved partially because it looked hyperplastic, but without a macroscopic nodule and was the smallest of the four parathyroid glands. The inferior one was located deep in the mediastinum, in the thymus, and was therefore not suitable for subtotal resection. The procedure was the same for the left side. The inferior parathyroid gland harbored nodular hyperplasia and, therefore, was not very suitable for partial resection, but the superior one looked as a good candidate for subtotal resection too. We started reducing the volume of the parathyroid glands with clips, preserving carefully each parathyroid's vascular pedicle. Then, we intravenously injected 3.5 mL of indocyanine green solution to check the perfusion of the parathyroid remnant, using a fluorescent imaging camera (PINPOINT camera; Novadaq, Mississauga, ON, Canada). The perfusion can be seen as green or white, depending on the selected image mode. We finally chose the right superior parathyroid gland and resected the gland outside of the clips. The other glands have finally been entirely removed. Results: The postoperative course was uneventful except for hypocalcemia needing intravenous calcium for 48 hours. On the first postoperative day, corrected calcium level was 1.93 mmol/L and PTH level was 8 pmol/L. The two inferior parathyroid glands showed nodular hyperplasia at pathologic examination and the two superior glands showed diffuse hyperplasia without nodules. Conclusion: With this new procedure, subtotal parathyroidectomy under ICG angiography, we can check for the good vascularization of the parathyroid remnant before resecting the other parathyroid glands. Therefore, we can intraoperatively guarantee the absence of definitive hypoparathyroidism. This technique is safe, reproducible, and its easy use makes it the procedure of choice in these situations, when the device is available. No competing financial interests exist. Runtime of video: 6 mins 33 secs more...

Introduction: To minimize risk and improve outcomes after parathyroidectomy, a number of tools are used to distinguish the diseased parathyroid gland from its surrounding structures.1–3 Ex...

Introduction: Parathyroid surgery has evolved considerably over the past decade. It is now often performed as an image-directed, focused exploration and accomplished on an outpatient basis...

Introduction: Ectopic parathyroid adenomas can be located within the residual thymic tissue, in the aortopulmonary window (APW), in the posterior mediastinum, or within the parenchyma of t...

Purpose of review The importance of parathyroid hormone in maintaining bone health has long been appreciated. Recent advances in the understanding of the cellular and molecular actions of this hormone have enhanced our treatment of parathyroid disorders and osteoporosis. Recent findings Parathyroid hormone has been shown to act at the cellular level through the activation of the RANK ligand system, which is a new target for drug development for osteoporosis. Our understanding of the natural history of primary hyperparathyroidism and the potential for medical management has recently come under greater scrutiny. An increased recognition of vitamin D inadequacy is changing our understanding of secondary hyperparathyroidism. Newer calcimimetic drugs are enhancing our therapy of both primary and secondary hyperparathyroidism. Synthetic parathyroid hormone has been shown to be both safe and effective in the treatment of osteoporosis. Summary Synthetic parathyroid hormone, the first anabolic agent for osteoporosis, has changed the therapeutic approach in severe osteoporosis. Although newer drugs have been used for primary hyperparathyroidism, surgery remains the definitive therapy. Increased understanding of the molecular biology of parathyroid hormone has allowed the development of new classes of therapeutic agents for osteoporosis. more...

The aim of this study is to determine the incidence and to quantify the risk factors for developing long-term regional osteopenia/osteoporosis (RO) following tibial fractures.We studied 42 adult subjects (8 females and 34 males) who had sustained a tibial fracture 16 yr prior to the study. Mechanism and type of injury, method of treatment, length of immobilization, weight-bearing status, and healing time were determined from the patient records. A questionnaire covering known causes of generalized osteoporosis (GO), including history of smoking, alcohol consumption, medications, other fractures, thyroid/parathyroid disorders, epilepsy, and renal disorders, was answered by all the subjects. Bone mineral density (BMD) of lumbar vertebrae 1‐4 and both proximal femurs was assessed using dual-energy X-ray absorptiometry (DXA) scanning. T- and Z-scores were generated. Assessment of risk factors was done by calculating the odds ratio (OR) and 95% confidence interval (CI). The incidence of significant loss of BMD as defined by the World Health Organization (T-score 40), alcohol >20 units/wk (OR 11, 95% CI 2 = 2‐54), open fracture (OR 17, 95% CI = 2.9 ‐ >40), nonweight bearing >4 wk (OR 15, 95% CI 2.9‐ >40), and delayed union defined as healing time more than 6 mo (OR 15, 95% CI 1.54 ‐ >40). Permanent regional loss of BMD occurs in a significant proportion of tibial shaft fracture patients. Modern fracture management should include identifying “at-risk” patients and appropriate preventive measures to prevent fragility fractures. more...

UNLABELLED 99mTc-pertechnetate scintigraphy plays an essential role in the management of a variety of thyroid and parathyroid disorders. The authors report an unusual case of mediastinal tracer distribution of 99mTc-pertechnetate and 99mTc-MIBI in relation to an oesophageal conduit following oesophago-gastrectomy and reconstructive surgery on thyroid scintigraphy. This is a rare but important cause of diffuse mediastinal uptake on thyroid scintigraphy. An awareness of abnormal anatomy as well as altered physiological tracer uptake would help to avoid any diagnostic pitfall. CONFLICT OF INTEREST None declared. more...

The three calcitropic hormones, parathyroid hormone (PTH), 1,25-dihydroxycholecalciferol and calcitonin are together responsible for calcium homeostasis in the mammal. Feline PTH is an 84 amino acid, single chain polypeptide with a molecular weight of 9449, which is secreted by the parathyroid glands. The principle secretagogue for PTH is a low plasma ionised calcium concentration, although both 1,25-dihydroxycholecalciferol and phosphate have significant roles in regulating PTH secretion. The ability to accurately measure circulating PTH in the cat has simplified the evaluation of disorders of calcium metabolism in this species. In primary parathyroid disorders the lesion is located within the parathyroid gland, with parathyroid secretion being inappropriate to the prevailing mineral balance. By contrast, in secondary conditions a pathological state out with the parathyroid gland alters mineral homeostasis and the parathyroid gland responds in an appropriate manner. The measurement of circulating PTH may then be used to determine if PTH secretion is appropriate to the prevailing calcium concentrations to differentiate primary from secondary disorders. Although primary hyper and hypoparathyroidism are generally considered rare endocrine conditions of the cat, the ability to measure PTH has led to their increasing recognition. more...

Specific adaptations are invoked during pregnancy and lactation to enable women to supply calcium and other minerals needed to support rapid growth and mineralization of the fetal and neonatal skeletons. These adaptations alter the expected normal values for serum minerals and calciotropic hormones and can occasionally lead to maternal hypercalcemia or fractures. Moreover, the presentation, diagnosis, and management of hypoparathyroidism and pseudohypoparathyroidism can also be altered by these adaptations. Fetal mineral metabolism is characterized by low levels of parathyroid hormone and calcitriol as compared to adult values. The fetus can be adversely affected by maternal hypocalcemia or hypercalcemia and genetic parathyroid disorders. The blood calcium normally falls after birth, and this triggers an upregulation in parathyroid function and calcitriol synthesis. Neonatal mineral homeostasis can be impacted by genetic parathyroid disorders and by prior exposure in utero to abnormal mineral concentrations in the mother. more...

The past six decades have witnessed the initial clinical descriptions of neonatal severe hyperparathyroidism (NSHPT) and familial hypocalciuric hypercalcemia (FHH), biochemical and physiologic studies suggesting abnormal calcium sensing by parathyroid glands and kidneys, and finally the description of three distinct molecular causes of FHH. FHH1 results from heterozygous inactivating mutations in the extracellular calcium-sensing receptor (CaSR), usually manifesting as mild to moderate resistance of parathyroid and kidney to calcium, mild PTH-dependent hypercalcemia, and relative hypocalciuria. NSHPT is caused by more severe calcium resistance owing to heterozygous, compound heterozygous, or homozygous CaSR mutations. Recent studies have identified the molecular bases for two additional forms of FHH linked to chromosome 19, FHH2 and FHH3, caused by mutations in downstream proteins in the CaSR signaling pathway. Further studies will likely further expand the range of clinical presentations of FHH, enhance our diagnosis of these conditions, and improve therapies for treating NSHPT and the occasional FHH patients requiring medical or surgical therapy. more...

Endocrine surgeries present a unique challenge to the attending surgeon and anesthesiologist and parathyroid adenoma is no exception. Parathyroid disorders are rare in children. Most striking disturbance in this disorder is the imbalance of calcium levels in body and main emphasis of management revolves around the same in perioperative period. We are reporting the anesthesia management for parathyroidectomy with total intra-venous anesthesia (TIVA). more...

The calcium-sensing receptor (CaSR) is a G-protein-coupled receptor with a crucial role in calcium homeostasis. Mutations in the CaSR gene may lead to specific parathyroid disorders due to either gain-of-function (autosomal dominant hypercalciuric hypocalcemia; ADHH) or loss-of-function (familial hypocalciuric hypercalcemia; FHH). Our aim was to evaluate CaSR mutations as a cause of disease in selected patients. We identified and recruited patients with phenotypes suggestive of CaSR-related parathyroid disorders. DNA was extracted, and CaSR gene was sequenced. Live-ratiometric measurements of intracellular [Ca(2+)] and Western blot assays for evaluation of MAPK phosphorylation in response to changes in extracellular [Ca(2+)] were performed in transiently transfected HEK-293T cells to functionally characterize mutants. A total of 21 patients were evaluated, seven of them with idiopathic hypoparathyroidism (suspected ADHH) and 14 with hyperparathyroidism (suspected FHH). In the latter group two patients were found to harbor missense mutations: a novel heterozygous I32 V mutation in a female index case and a sporadic known R185Q mutation in a 1-year-old girl. In-vitro functional studies showed that I32 V is an inactivating mutation. In our study, most patients had normal CaSR sequencing. This suggests that phenotypic pitfalls may occur at time of patients' selection for CaSR sequencing. In one patient with strong positive pre-test probability based on both familial history and appropriate phenotype, a novel I32 V mutation leading to FHH was identified and characterized. In cases of familial parathyroid disorders, CaSR sequencing should be performed, but if negative, one should consider involvement of alternative genes or mechanisms. more...

Fahr’s disease (FD), also called idiopathic striopallidodentate calcinosis, nonarteriosclerotic cerebral calcification or idiopathic basal ganglia calcification1,2, is a rare clinical entity characterized by movement disorders, dementia and behavioral disorders related to symmetric and bilateral calcifications of the basal ganglia. Sporadic and family cases have been reported1-3. FD should be differentiated from Fahr’ syndrome. The latter is defined as symmetric and bilateral calcifications of the basal ganglia associated with neuropsychiatric manifestations that preferentially occur in patients with parathyroid disorders, especially hypoparathyroidism2 [...] more...

Hyperparathyroidism represents a wide spectrum of parathyroid disorders from hyperplasia to adenoma and carcinoma. These disorders can be present as primary or secondary, and as sporadic or hereditary forms. Although the incidence of hyperparathyroidism has increased dramatically, the interpretation of the parathyroid pathology is still of considerable controversy and confusion. Over the past few years, multiple genetic abnormalities have been characterized in sporadic parathyroid tumors. A majority of secondary parathyroid hyperplasia have been shown to be of monoclonal origins by clonal analysis. The ret oncogene responsible for the pathogenesis of multiple endocrine neoplasia type 2 and multiple endocrine neoplasia (MEN) 1 gene for type 1 have been cloned in succession. Mutation of the calcium-sensing receptor gene has been identified to be thegenetic trigger of neonatal severe hyperparathyroidism and familial hypocalciuric hypercalcemia. HRPT 2 gene designated for the syndrome of hereditary hyperparathyroidism and jaw tumors has recently been localized on the 1q21-23, and linkage analysis has established the presence of familial hyperparathyroidism as a distinct entity. These advances provide new insights for understanding the molecular pathology of various types of hyperparathyroidisms and allow us a precise differentiation among them. Based on the results of our study and those reported in literature, this article reviews the recent advances in the molecular basis of various hyperparathyroidisms. more...

マグネシウム(Mg)は細胞内に多く存在して生理的に重要な役割を果たしている.従って, Mgの欠乏状態や過剰状態では多彩な症状を呈するので, Mg代謝異常を正確に把握することは臨床上非常に重要である.しかし, Mg代謝異常は単独で存在する場合もあるが, Ca, K, Pなどその他の電解質異常と合併して認められる場合も多く,電解質異常を認める際には必ずMgも同時に測定することによって, Mg代謝異常があるか否かを確認する必要がある. more...

多発性内分泌腫瘍症(multiple endocrine neoplasia, MEN)のうち,副甲状腺腫瘍による原発性副甲状腺機能亢進症はMEN 1型およびMEN 2A型に認められる. MEN 1型においては,副甲状腺病変が最も出現頻度が高く,しかも若年で発症する. 1997年に単離されたMEN1遺伝子の胚細胞変異は約90%のMEN 1型症例に認められ,癌抑制遺伝子としての性質を有する,副甲状腺腫瘍を含めた一部の散発性腫瘍においても体細胞変異が認められ,腫瘍化に関与していること明らかにされている. MEN 2A型においては副甲状腺病変は約10%に認められるが,血清カルシウム値は正常のことが多い. RET遺伝子変異検出による保因者の遺伝子診断,それに基づく予防的甲状腺全摘術の有用性が確立されている. more...

腎不全患者では,低カルシウム血症が補正され,生理量の活性型ビタミンD製剤が投与されているにもかかわらず, PTH分泌が亢進し,副甲状腺過形成が生ずる.これは副甲状腺のビタミンDに対する抵抗性,カルシウムイオンに対する感受性の異常,リンの直接作用などによる考えられており,病態に基づいた治療法が開発されている.一方,腎不全では骨のPTHに対する抵抗性も存在するので, PTH分泌の抑制目標は正常人と異なる. more...

高Ca血症を来す病態は原発性副甲状腺機能亢進症(I°-HPT)と悪性腫瘍に伴う高Ca血症(MAHC)で全体の約80%を占め,両者の比率は同等である.次いでビタミンD製剤の過剰投与によるビタミンD中毒,症例数は少ないが家族性低Ca尿性高Ca血症(FHH)がある.多彩な臨床症状・病歴の注意深い観察は診断のきっかけとなる. MAHCでは症状が急速に進行し,高Ca血症が直接死因となり得る. I°-HPTではPTHの上昇, MAHCはHHMとLOHに大別され,前者でPTHrPの上昇が見られる.症状の少ないFHHはCca/Ccr値の著明低値で確診される. more...