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F1000Research 2020Hypoparathyroidism is a rare endocrine disorder which leads to hypocalcemia, hypercalciuria, and hyperphosphatemia. Complications include nephrocalcinosis with renal... (Review)
Review
Hypoparathyroidism is a rare endocrine disorder which leads to hypocalcemia, hypercalciuria, and hyperphosphatemia. Complications include nephrocalcinosis with renal dysfunction, reduced quality of life, and abnormal skeletal properties. Conventional therapy with calcium and vitamin D analogs addresses hypocalcemia but has important limitations. Parathyroid hormone (PTH) therapy is a fundamental advance, although the effects of PTH on long-term complications require additional testing. Continuous PTH therapy is likely to be particularly advantageous for addressing renal, quality of life, and skeletal complications. Overall, much progress has been made, yet more information is needed to improve our understanding and management of hypoparathyroidism.
Topics: Calcium; Humans; Hypocalcemia; Hypoparathyroidism; Parathyroid Hormone; Quality of Life
PubMed: 32765831
DOI: 10.12688/f1000research.22717.1 -
Hormones (Athens, Greece) Mar 2024Primary hyperparathyroidism (PHPT), a relatively common disorder characterized by hypercalcemia with raised or inappropriately normal serum parathyroid hormone (PTH)... (Review)
Review
Primary hyperparathyroidism (PHPT), a relatively common disorder characterized by hypercalcemia with raised or inappropriately normal serum parathyroid hormone (PTH) concentrations, may occur as part of a hereditary syndromic disorder or as a non-syndromic disease. The associated syndromic disorders include multiple endocrine neoplasia types 1-5 (MEN1-5) and hyperparathyroidism with jaw tumor (HPT-JT) syndromes, and the non-syndromic forms include familial hypocalciuric hypercalcemia types 1-3 (FHH1-3), familial isolated hyperparathyroidism (FIHP), and neonatal severe hyperparathyroidism (NS-HPT). Such hereditary forms may occur in > 10% of patients with PHPT, and their recognition is important for implementation of gene-specific screening protocols and investigations for other associated tumors. Syndromic PHPT tends to be multifocal and multiglandular with most patients requiring parathyroidectomy with the aim of limiting end-organ damage associated with hypercalcemia, particularly osteoporosis, nephrolithiasis, and renal failure. Some patients with non-syndromic PHPT may have mutations of the MEN1 gene or the calcium-sensing receptor (CASR), whose loss of function mutations usually cause FHH1, a disorder associated with mild hypercalcemia and may follow a benign clinical course. Measurement of the urinary calcium-to-creatinine ratio clearance (UCCR) may help to distinguish patients with FHH from those with PHPT, as the majority of FHH patients have low urinary calcium excretion (UCCR < 0.01). Once genetic testing confirms a hereditary cause of PHPT, further genetic testing can be offered to the patients' relatives and subsequent screening can be carried out in these affected family members, which prevents inappropriate testing in normal individuals.
Topics: Infant, Newborn; Humans; Hyperparathyroidism, Primary; Calcium; Hypercalcemia; Adenoma; Fibroma; Hyperparathyroidism; Jaw Neoplasms
PubMed: 38038882
DOI: 10.1007/s42000-023-00508-9 -
Seminars in Ultrasound, CT, and MR Dec 1996Primary hyperparathyroidism resulting in hypercalcemia is the most common presentation of parathyroid pathology and usually is related to a parathyroid adenoma and, less... (Review)
Review
Primary hyperparathyroidism resulting in hypercalcemia is the most common presentation of parathyroid pathology and usually is related to a parathyroid adenoma and, less commonly, to hyperplasia. Imaging of the parathyroid glands focuses on the detection of adenomas in patients with primary hyperparathyroidism. The role of imaging (as well as the modality used) for preoperative localization of the parathyroid glands continues to be controversial. The embryology, anatomy, and physiology of the parathyroid glands are reviewed. The diagnostic utility of radiological imaging-including ultrasonography, CT, MR imaging, and nuclear scintigraphy-are discussed, particularly as it pertains to the evaluation of primary hyperparathyroidism.
Topics: Diagnostic Imaging; Humans; Parathyroid Diseases; Parathyroid Glands
PubMed: 9023868
DOI: 10.1016/s0887-2171(96)90004-9 -
The Journal of International Medical... Jan 2020In this review, parathyroid mass and its nonspecific symptoms are discussed. In daily clinical practice, patients present with varying symptoms, including depression,... (Review)
Review
In this review, parathyroid mass and its nonspecific symptoms are discussed. In daily clinical practice, patients present with varying symptoms, including depression, chest pain, pancreatitis, or nonspecific fatigue. If the patient is not tested for a parathyroid mass along with performing routine electrolyte tests, diagnosing such a patient with a parathyroid mass may take several years. This issue and situation are discussed in this review.
Topics: Cysts; Humans; Hyperparathyroidism; Parathyroid Glands; Parathyroid Neoplasms
PubMed: 30791798
DOI: 10.1177/0300060519827169 -
World Journal of Gastroenterology Sep 2011The parathyroid glands are the main regulator of plasma calcium and have a direct influence on the digestive tract. Parathyroid disturbances often result in unknown... (Review)
Review
The parathyroid glands are the main regulator of plasma calcium and have a direct influence on the digestive tract. Parathyroid disturbances often result in unknown long-standing symptoms. The main manifestation of hypoparathyroidism is steatorrhea due to a deficit in exocrine pancreas secretion. The association with celiac sprue may contribute to malabsorption. Hyperparathyroidism causes smooth-muscle atony, with upper and lower gastrointestinal symptoms such as nausea, heartburn and constipation. Hyperparathyroidism and peptic ulcer were strongly linked before the advent of proton pump inhibitors. Nowadays, this association remains likely only in the particular context of multiple endocrine neoplasia type 1/Zollinger-Ellison syndrome. In contrast to chronic pancreatitis, acute pancreatitis due to primary hyperparathyroidism is one of the most studied topics. The causative effect of high calcium level is confirmed and the distinction from secondary hyperparathyroidism is mandatory. The digestive manifestations of parathyroid malfunction are often overlooked and serum calcium level must be included in the routine workup for abdominal symptoms.
Topics: Celiac Disease; Gastrointestinal Diseases; Humans; Pancreas; Pancreatitis; Parathyroid Diseases; Steatorrhea; Zollinger-Ellison Syndrome
PubMed: 22039319
DOI: 10.3748/wjg.v17.i36.4063 -
Medicine Apr 2023Chronic kidney disease-mineral and bone disorder has complex and diverse clinical manifestations, including the simplest abnormalities of calcium, phosphorus and... (Review)
Review
Chronic kidney disease-mineral and bone disorder has complex and diverse clinical manifestations, including the simplest abnormalities of calcium, phosphorus and parathyroid hormone detected in blood, abnormalities of bone transformation and mineralization in bone, and calcification of blood vessels or other soft tissues detected on imaging. Patients with CKD-MBD combined low bone mineral density and fragility fractures are referred to as CKD-MBD with low bone mineral density. Vascular calcification refers to ectopic deposition of calcium phosphate in the blood vessel walls and heart valves. The degree of vascular calcification was inversely proportional to bone mineral density. The more severe the degree of vascular calcification, the lower the bone mineral density, and the higher the risk of death, indicating that the bone-vascular axis exists. Activation and alteration of the Wnt signaling pathway are central to the treatment of vascular diseases in uremia. Vitamin D supplementation can prevent secondary hyperparathyroidism, activate osteoblasts, relieve muscle weakness and myalgia, and reduce vascular calcification. Nutritional vitamin D may improve vascular calcification in uremia patients by regulating Wnt signaling pathway.
Topics: Humans; Chronic Kidney Disease-Mineral and Bone Disorder; Vitamin D; Calcium; Parathyroid Hormone; Vascular Calcification; Bone Diseases, Metabolic; Vitamins; Uremia; Renal Insufficiency, Chronic
PubMed: 37026958
DOI: 10.1097/MD.0000000000033477 -
Drugs Apr 2019Significant advances in immunosuppressive therapies have been made in renal transplantation, leading to increased allograft and patient survival. Despite improvement in... (Review)
Review
Significant advances in immunosuppressive therapies have been made in renal transplantation, leading to increased allograft and patient survival. Despite improvement in overall patient survival, patients continue to require management of persistent post-transplant hyperparathyroidism. Medications that treat persistent hyperparathyroidism include vitamin D, vitamin D analogues, and calcimimetics. Medication side effects such as hypocalcemia or hypercalcemia, and adynamic bone disease, may lead to a decrease in the drugs. When medical management fails to control persistent post-transplant hyperparathyroidism, treatment is a parathyroidectomy. Surgical techniques are not uniform between centers and surgeons. Undergoing the surgery may include a subtotal technique or a technique including total parathyroid gland resection with partial heterotopic gland reimplantation. In addition, there are possible post-surgical complications. The ideal treatment for persistent post-transplant hyperparathyroidism is the treatment and prevention of the condition while patients are being managed for their late-stage chronic kidney disease and end-stage renal disease.
Topics: Bone Diseases; Calcimimetic Agents; Humans; Hyperparathyroidism; Hyperparathyroidism, Secondary; Kidney Transplantation; Parathyroidectomy; Transplantation, Homologous; Treatment Outcome; Vitamin D
PubMed: 30811012
DOI: 10.1007/s40265-019-01074-4 -
Seminars in Oncology Dec 2010Parathyroid cancer is an uncommon malignancy and rare cause of primary hyperparathyroidism (HPT) with a high morbidity and patient death in advanced cases usually... (Review)
Review
Parathyroid cancer is an uncommon malignancy and rare cause of primary hyperparathyroidism (HPT) with a high morbidity and patient death in advanced cases usually resulting from intractable hypercalcemia. Inactivation of the HRPT2/CDC73 gene, encoding the putative tumor-suppressor protein parafibromin and discovered in the context of the hyperparathyroidism-jaw tumor (HPT-JT) syndrome, is a common, somatic event in most parathyroid cancers. Approximately 25% of patients with apparently sporadic parathyroid cancer carry germline HRPT2/CDC73 mutation. Germline DNA analysis for HRPT2/CDC73 mutation is recommended in all patients with parathyroid cancer because of the potential benefit for first-degree relatives, who should nevertheless undergo serum calcium screening. The histopathologic diagnosis of parathyroid cancer is nonspecific unless vascular, lymphatic, capsular, or soft tissue invasion is seen, or metastases are clinically evident. Immunohistochemical analysis of parathyroid tumors for loss of parafibromin expression offers promise as a diagnostic tool. En bloc tumor resection offers the highest chance of cure in patients with suspected parathyroid carcinoma. No adjuvant chemotherapy regimen has yet proven effective, and the role of local adjuvant radiotherapy is being evaluated. Metastatic disease can be palliated with surgical debulking. Medical therapy with the calcimimetic cinacalcet and bisphosphonates can ameliorate hypercalcemia in patients with inoperable disease.
Topics: Biomarkers, Tumor; Disease Progression; Humans; Hypercalcemia; Mutation; Parathyroid Neoplasms; Parathyroidectomy; Prognosis; Tumor Suppressor Proteins
PubMed: 21167377
DOI: 10.1053/j.seminoncol.2010.10.013 -
Endokrynologia Polska 2022Parathyroid carcinoma is a rare condition and accounts for < 1% of cases of sporadic primary hyperparathyroidism. It accounts for 0.005% of all cancers. Often the...
INTRODUCTION
Parathyroid carcinoma is a rare condition and accounts for < 1% of cases of sporadic primary hyperparathyroidism. It accounts for 0.005% of all cancers. Often the differentiation between adenoma and carcinoma is challenging and requires multidisciplinary cooperation. Complete surgical resection is the treatment of choice. We present a retrospective analysis of 29 patients who were surgically treated for parathyroid cancer.
MATERIAL AND METHODS
Between the years 1983 and 2018, 71 (7.0%) patients were treated for suspicion of parathyroid cancer among a group of 1019 operated for primary hyperparathyroidism.
RESULTS
We confirmed the diagnosis of parathyroid cancer in 29 (2.8%) patients, 12 men and 17 women, aged 27 to 77 years, mean 55.1 years. That constituted 43.9% of the 71 patients with initial suspicion of cancer diagnosis. All operated patients were under long-term observation.
CONCLUSIONS
A diagnosis of parathyroid carcinoma should always be considered during surgery in patients diagnosed with primary hyperparathyroidism, especially in patients with severe hypercalcaemia, significantly enlarged neck circumference, and concomitant diseases of the renal and skeletal system. Parathyroid carcinoma is rarely definitively diagnosed preoperatively or even intraoperatively, and the final diagnosis can be made exclusively after operation. The optimal treatment is a complete surgical resection at a reference centre - specialized in parathyroid surgery - to improve outcomes and provide the best chance of recovery.
Topics: Adenoma; Adult; Aged; Female; Humans; Hyperparathyroidism; Male; Middle Aged; Parathyroid Neoplasms; Parathyroidectomy; Retrospective Studies
PubMed: 35156702
DOI: 10.5603/EP.a2022.0003 -
The Indian Journal of Medical Research Jan 2022Primary hyperparathyroidism (PHPT) is a common endocrine disorder caused by the elevated secretion of the parathormone (PTH). The aim of this study was to evaluate the...
BACKGROUND & OBJECTIVES
Primary hyperparathyroidism (PHPT) is a common endocrine disorder caused by the elevated secretion of the parathormone (PTH). The aim of this study was to evaluate the haematological manifestations of PHPT in patients with normal renal functions who were treated surgically for parathyroid adenomas.
METHODS
In this retrospective cross-sectional study, 134 patients with normal renal functions who underwent parathyroidectomies for PHPT were included. The haematological manifestations were evaluated in the total study cohort and in the two groups of different calcium (Ca) levels (Group 1 ≤11.2 mg/dl and Group 2 >11.2 mg/dl).
RESULTS
The overall prevalence of anaemia, leucopenia and thrombocytopenia was 20.1, 6.7 and 6.0 per cent, respectively. Normocytic anaemia was present in 19 (14.2%) patients. There were no significant differences in the prevalence of anaemia, leucopenia and thrombocytopenia between the two groups. There were no correlations between the PTH levels and the leukocyte, haemoglobin or platelet values. Six to 12 months after the parathyroidectomy (PTX), 35.7 per cent of the patients with anaemia, 85.7 per cent of the patients with leucopenia and 100 per cent of the patients with thrombocytopenia had recovered.
INTERPRETATION & CONCLUSIONS
In the present study, anaemia was seen with a variable frequency in PHPT, but there was no relationship between anaemia and high PTH or Ca levels. The development of anaemia can be seen regardless of the PTH levels in PHPT patients with normal renal functions. High-resolution rates after PTX indicate a possible association between PHPT and thrombocytopenia or leucopenia, although their prevalence is low in PHPT.
Topics: Anemia; Calcium; Cross-Sectional Studies; Humans; Hyperparathyroidism, Primary; Parathyroid Hormone; Parathyroidectomy; Retrospective Studies; Thrombocytopenia
PubMed: 35859427
DOI: 10.4103/ijmr.IJMR_1200_19