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Hyperparathyroidism

Hyperparathyroidism is an increase in parathyroid hormone (PTH) levels in the blood. This occurs from a disorder either within the parathyroid glands or as response to external stimuli. Symptoms of hyperparathyroidism are caused by inappropriately elevated blood calcium excreted from the bones into the blood stream in response to increased production of parathyroid hormone. In healthy people, when blood calcium levels are high, parathyroid hormone levels should be low. With long-standing hyperparathyroidism, the most common symptom is kidney stones. Other symptoms may include bone pain, weakness, depression, confusion, and increased urination. Both primary and secondary may result in osteoporosis.

Signs and symptoms
In primary hyperparathyroidism, about 75% of people are "asymptomatic". Common manifestations of hypercalcemia include constipation, vomiting, weakness, lethargy, fatigue, depression, bone pain, muscle soreness (myalgias), joint pain, decreased appetite, feelings of nausea, abdominal pain, pancreatitis, polyuria, polydipsia, cognitive impairment, kidney stones (), vertigo and osteopenia or osteoporosis. A history of acquired racquet nails (brachyonychia) may be indicative of bone resorption. Radiographically, hyperparathyroidism has a pathognomic finding of rugger jersey spine. Parathyroid adenomas are very rarely detectable on clinical examination. Surgical removal of a parathyroid tumor eliminates the symptoms in most patients. In secondary hyperparathyroidism due to lack of vitamin D absorption, the parathyroid gland is behaving normally; clinical problems are due to bone resorption and manifest as bone syndromes such as rickets, osteomalacia, and renal osteodystrophy. ==Causes==
Causes
Causes of primary hyperparathyroidism include parathyroid adenoma (80% of patients), multiglandular disease usually seen as hyperplasia of the 4 parathyroid glands (15–20% of patients), parathyroid carcinoma (less than 1% of patients). Primary hyperparathyroidism occurs sporadically and most patients do not have a family history. and thiazide diuretics exposure. A number of genetic conditions including multiple endocrine neoplasia syndromes, hyperparathyroidism-jaw tumor syndrome, familial hypocalciuric hypercalcemia, neonatal severe hyperparathyroidism also increase the risk. The most common causes for secondary hyperparathyroidism include vitamin D deficiency, chronic kidney disease, inadequate calcium intake, malabsorption. Tertiary hyperparathyroidism most commonly occurs from prolonged secondary hyperparathyroidism. == Development ==
Development
The parathyroid is composed of 4 glands with 2 located superiorly and 2 located inferiorly. The parathyroid glands are located on the posterior thyroid and are derived from the endoderm of the 3rd and 4th pharyngeal pouches. The ultimopharyngeal body is derived from the 4th pharyngeal pouch ventral wing and the parafollicular cells ( C-cells) are derived when the ultimopharyngeal bodies fuse with the posterolateral thyroid. The parathyroid glands separates from the pharyngeal wall and attaches to the posterior thyroid during the 7th week of human embryonic development. ==Mechanism==
Mechanism
Normal parathyroid glands measure the ionized calcium (Ca2+) concentration in the blood and secrete parathyroid hormone accordingly; if the ionized calcium rises above normal, the secretion of PTH is decreased, whereas when the Ca2+ level falls, parathyroid hormone secretion is increased. Alternatively, prolonged changes in serum calcium influences mRNA-binding proteins altering the encoding of PTH mRNA. There are also calcium independent mechanisms which include repression of PTH transcription through 1α,25-dihydroxyvitamin D binding with the vitamin D receptor. 2) PTH inhibits secretion of osteoprotegerina to allow for osteoclast differentiation. PTH acts on the distal convoluted tubule and collecting duct to increase calcium reabsorption in the nephron. The resulting hypovitaminosis D is usually due to a partial combination of both factors. Vitamin D3 (or cholecalciferol) is converted to 25-hydroxyvitamin D (or calcidiol) by the liver, from where it is transported via the circulation to the kidneys, and it is converted into the active hormone, 1,25 dihydroxyvitamin D3. Thus, a third cause of secondary hyperparathyroidism is chronic kidney disease. Here the ability to manufacture 1,25 dihydroxyvitamin D3 is compromised, resulting in hypocalcemia. ==Diagnosis==
Diagnosis
The gold standard of diagnosis is the PTH immunoassay. Once an elevated PTH has been confirmed, the goal of diagnosis is to determine the type of hyperparathyroidism (primary, secondary, or tertiary hyperparathyroidism) by obtaining a serum calcium, phosphate, and PTH levels. Primary hyperparathyroidism has high calcium, vitamin D, and PTH levels and a low phosphate level. Additionally a CT scan without contrast or renal ultrasound can be done to assess for nephrolithiasis and/or nephrocalcinosis if there is concern for it. Additionally, familial benign hypocalciuric hypercalcamia can present with similar lab changes. Calcium levels In cases of primary hyperparathyroidism or tertiary hyperparathyroidism, heightened PTH leads to increased serum calcium (hypercalcemia) due to: • increased bone resorption, allowing the flow of calcium from bone to blood • reduced kidney clearance of calcium • increased intestinal calcium absorption Serum phosphate In primary hyperparathyroidism, serum phosphate levels are abnormally low as a result of decreased reabsorption of phosphate in the kidney tubules. However, this is only present in about 50% of cases. This contrasts with secondary hyperparathyroidism and tertiary hyperparathyroidism, in which serum phosphate levels are generally elevated because of kidney disease. Alkaline phosphatase Alkaline phosphatase levels are usually high in hyperparathyroidism due to high bone turn over. In primary hyperparathyroidism, levels may remain within the normal range, but this is inappropriately normal given the increased levels of plasma calcium. Nuclear medicine Nuclear medicine imaging methods are used by surgeons to locate which parathyroid gland is responsible for hyperparathyroidism or to find ectopic parathyroid adenomas, most commonly found in the anterior mediastinum. Historically, technetium sestamibi scintigraphy was the main method used or this indication. Recently 18F-fluorocholine PET/CT tend to be more and more performed due to excellent diagnostic performance. Classification Primary . Primary hyperparathyroidism results from a hyperfunction of the parathyroid glands themselves. The oversecretion of PTH is due to a parathyroid adenoma, parathyroid hyperplasia, or rarely, a parathyroid carcinoma. This disease is often characterized by the quartet stones, bones, groans, and psychiatric overtones referring to the presence of kidney stones, hypercalcemia, constipation, and peptic ulcers, as well as depression, respectively. In a minority of cases, this occurs as part of a multiple endocrine neoplasia (MEN) syndrome, either type 1 (caused by a mutation in the gene MEN1) or type 2a (caused by a mutation in the gene RET), which is also associated with the adrenal tumor pheochromocytoma. Other mutations that have been linked to parathyroid neoplasia include mutations in the genes HRPT2 and CASR. Patients with bipolar disorder who are receiving long-term lithium treatment are at increased risk for hyperparathyroidism. Elevated calcium levels are found in 15% to 20% of patients who have been taking lithium long-term. However, only a few of these patients have significantly elevated levels of parathyroid hormone and clinical symptoms of hyperparathyroidism. Lithium-associated hyperparathyroidism leads to hypercalcemia in about 4% of lithium-treated patients. Calcium levels should be checked for patients undergoing long-term lithium treatment. (caused by lack of sunlight, diet or malabsorption) and chronic kidney failure. Vitamin D deficiency can result from malabsorption or decreased vitamin D intake such as with gastric bypass, small bowel disease, pancreatic disease, and dietary causes. Other causes include decreased skin synthesis of vitamin D such as decreased exposure to sunlight and skin disorders. Insufficient vitamin D synthesis such as defective 25-hydroxylation, 1-alpha hydroxylase, and 1-alpha 25-hydroxylation can also contribute to vitamin D deficiency. Lack of vitamin D leads to reduced calcium absorption by the intestine leading to hypocalcemia and increased parathyroid hormone secretion. This increases bone resorption. In chronic kidney failure the problem is more specifically failure to convert vitamin D to its active form in the kidney. The bone disease in secondary hyperparathyroidism caused by kidney failure is termed renal osteodystrophy. Tertiary Tertiary hyperparathyroidism is seen in those with long-term secondary hyperparathyroidism, which eventually leads to hyperplasia of the parathyroid glands and a loss of response to serum calcium levels. This disorder is most often seen in patients with end-stage kidney disease and is an autonomous activity. Patients with late-stage kidney disease have an increased likelihood of developing tertiary hyperparathyroidism if not promptly corrected. ==Treatment==
Treatment
Treatment depends on the type of hyperparathyroidism encountered. Primary Parathyroidectomy is a curative therapy for symptomatic hyperparathyroidism. Additionally, it decreases the risk of nephrolithiasis, osteoporosis, fragility fractures, and improves bone mineral density. Studies have also found that parathyroidectomy for hyperparathyroidism improves fatigue, weakness, depression, and memory. While parathyroidectomy is recommended for all patients with hyperparathyroidism who are symptomatic, indications of surgery for those who are asymptomatic include the following: • Asymptomatic hyperparathyroidism with any of the following: • 24-hour urinary calcium >250 mg/day in women and >300 mg/day in men (see footnote, below) • serum calcium > 1 mg/dl above upper limit of normal • Creatinine clearance > 30% below normal for patient's age • Estimated glomerular filtration rate 2.5 standard deviations below peak (i.e., T-score of −2.5) • People age 11.0 mg/ dL), calciphylaxis, bone pain, or pathological fracture. A systematic review found surgical treatment to be superior regarding cure rates than medical therapy with cinacalcet with lower risk of complications. Calcimimetics A calcimimetic (such as cinacalcet) is a potential therapy for some people with severe hypercalcemia and primary hyperparathyroidism who are unable to undergo parathyroidectomy, and for secondary hyperparathyroidism on dialysis. Treatment of secondary hyperparathyroidism with a calcimimetic in those on dialysis for CKD does not alter the risk of early death; however, it does decrease the likelihood of needing a parathyroidectomy. == Epidemiology ==
Epidemiology
In the developed world, between one and four per thousand people are affected. Primary hyperparathyroidism is the most common type. The United States prevalence of primary hyperparathyroidism from 2010 was 233 per 100,000 women and 85 per 100,000 men. Black and white women aged 70–79 have the highest overall prevalence. Secondary hyperparathyroidism is most commonly caused by chronic kidney disease and vitamin D deficiency. The prevalence of vitamin D deficiency is about 50% of the world population and chronic kidney disease prevalence is 15% of the United States population. ==History==
History
The oldest known case was found in a cadaver from an Early Neolithic cemetery in southwest Germany. ==Notes==
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