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| '''For patient information click [[{{PAGENAME}} (patient information)|here]]'''
| | #Redirect [[Hyperparathyroidism]] |
| {{Infobox_Disease |
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| Name = Primary hyperparathyroidism |
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| Image = Illu thyroid parathyroid.jpg |
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| Caption = Thyroid and parathyroid. |
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| DiseasesDB = 6283 |
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| ICD10 = {{ICD10|E|21|0|e|20}} |
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| ICD9 = {{ICD9|252.01}} |
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| ICDO = |
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| OMIM = |
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| MedlinePlus = |
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| eMedicineSubj = |
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| eMedicineTopic = |
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| MeshID = D049950 |
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| }}
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| {{SI}}
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| {{GS}}
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| {{Editor Join}}
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| ==Overview==
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| '''Primary hyperparathyroidism''' causes [[hypercalcemia]] (elevated blood calcium levels) through the excessive secretion of [[parathyroid hormone]] (PTH), usually by an [[adenoma]] (benign tumors) of the [[parathyroid gland]]s. Its incidence is approximately 42 per 100,000 people. It is almost exactly three times as common in women as men.
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| ==Signs and Symptoms==
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| The signs and symptoms of primary hyperparathyroidism are those of hypercalcemia. They are classically summarized by the mnemonic "stones, bones, abdominal groans and psychic moans".
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| * "Stones" refers to [[kidney stones]], [[nephrocalcinosis]], and [[diabetes insipidus]] (polyuria and polydipsia). These can ultimately lead to [[renal failure]].
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| * "Bones" refers to bone-related complications. The classic bone disease in hyperparathyroidism is [[osteitis fibrosa cystica]], which results in pain and sometimes pathological fractures. Other bone diseases associated with hyperparathyroidism are [[osteoporosis]], [[osteomalacia]], and [[arthritis]].
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| * "Abdominal groans" refers to gastrointestinal symptoms of [[constipation]], [[indigestion]], [[nausea]] and [[vomiting]]. Hypercalcemia can lead to [[peptic ulcers]] and [[acute pancreatitis]].
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| * "Psychic moans" refers to effects on the [[central nervous system]]. Symptoms include lethargy, fatigue, depression, memory loss, psychosis, ataxia, delirium, and coma.
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| * [[Left ventricular hypertrophy]].<ref>{{cite journal |author=Stefenelli T, Abela C, Frank H, ''et al'' |title=Cardiac abnormalities in patients with primary hyperparathyroidism: implications for follow-up |journal=J. Clin. Endocrinol. Metab. |volume=82 |issue=1 |pages=106-12 |year=1997 |pmid=8989242 |doi= |url=http://jcem.endojournals.org/cgi/content/full/82/1/106}}</ref>
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| Other signs include proximal muscle weakness, itching, and band [[keratopathy]] of the eyes.
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| ==Diagnosis==
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| The diagnosis of primary hyperparathyroidism is made by blood tests. Serum calcium levels are elevated.
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| The serum chloride phosphate ratio is 33 or more in most patients with primary hyperparathyroidism. <ref name="pmid1155729">{{cite journal |author=Reeves CD, Palmer F, Bacchus H, Longerbeam JK |title=Differential diagnosis of hypercalcemia by the chloride/phosphate ratio |journal=Am. J. Surg. |volume=130 |issue=2 |pages=166-71 |year=1975 |pmid=1155729 |doi=}}<br><blockquote>This study found a ratio above 33 to have a [[sensitivity (tests)|sensitivity]] of 94% and a [[specificity (tests)|specificity]] of 96%.</blockquote></ref><ref name="pmid4405880">{{cite journal |author=Palmer FJ, Nelson JC, Bacchus H |title=The chloride-phosphate ratio in hypercalcemia |journal=Ann. Intern. Med. |volume=80 |issue=2 |pages=200-4 |year=1974 |pmid=4405880 |doi=}}</ref><ref name="pmid521012">{{cite journal |author=Broulík PD, Pacovský V |title=The chloride phosphate ratio as the screening test for primary hyperparathyroidism |journal=Horm. Metab. Res. |volume=11 |issue=10 |pages=577-9 |year=1979 |pmid=521012 |doi=}}<br><blockquote>This study found a ratio above 33 to have a [[sensitivity (tests)|sensitivity]] of 95% and a [[specificity (tests)|specificity]] of 100%.</blockquote></ref> However, [[thiazide]] medications have been reported to causes ratios above 33.<ref name="pmid6848626">{{cite journal |author=Lawler FH, Janssen HP |title=Chloride:phosphate ratio with hypercalcemia secondary to thiazide administration |journal=The Journal of family practice |volume=16 |issue=1 |pages=153-4 |year=1983 |pmid=6848626 |doi=}}</ref>
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| Urinary [[cAMP]] is occasionally measured; this is generally elevated.
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| ===Parathyroid hormone activity===
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| Intact PTH levels are also elevated.
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| ==Causes==
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| The most common cause of primary hyperparathyroidism is a sporadic, single parathyroid adenoma resulting from a clonal mutation (~97%). Less common are hyperplasia of all parathyroid glands (~2.5%), parathyroid [[carcinoma]] (malignant tumor), and adenomas in more than one gland (together ~0.5%). Primary hyperparathyroidism is also a feature of several familial endocrine disorders: [[Multiple endocrine neoplasia]] type 1 and type 2A ([[Multiple endocrine neoplasia type 1|MEN type 1]] and [[Multiple endocrine neoplasia type 2|MEN type 2A]]), and familial hyperparathyroidism.
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| In all cases, the disease is [[idiopathic]], but is thought to involve inactivation of tumor suppression genes.
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| ==Complications==
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| The classic bone disease in hyperparathyroidism is [[osteitis fibrosa cystica]], which results in pain and sometimes pathological fractures. Other bone diseases associated with hyperparathyroidism are [[osteoporosis]], [[osteomalacia]], and [[arthritis]].
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| ==Treatment==
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| Treatment is usually surgical removal of the gland(s) containing adenomas.
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| ===Medications===
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| Medications include estrogen replacement therapy in postmenopausal women and [[bisphosphonate]]s. [[Bisphosphonate]]s may improve bone turnover.<ref name="pmid15240609">{{cite journal |author=Khan AA, Bilezikian JP, Kung AW, ''et al'' |title=Alendronate in primary hyperparathyroidism: a double-blind, randomized, placebo-controlled trial |journal=J. Clin. Endocrinol. Metab. |volume=89 |issue=7 |pages=3319-25 |year=2004 |pmid=15240609 |doi=10.1210/jc.2003-030908}}</ref>
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| ===Surgery===
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| A consensus statement in 2002 recommended the following indications for surgery<ref name="pmid12466320">{{cite journal |author=Bilezikian JP, Potts JT, Fuleihan Gel-H, ''et al'' |title=Summary statement from a workshop on asymptomatic primary hyperparathyroidism: a perspective for the 21st century |journal=J. Clin. Endocrinol. Metab. |volume=87 |issue=12 |pages=5353-61 |year=2002 |pmid=12466320 |doi=}}</ref>:
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| * Serum calcium (above upper limit of normal): 1.0 mg/dl
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| * 24-h urinary calcium >400 mg
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| * Creatinine clearance reduced by 30% compared with age-matched subjects.
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| * Bone mineral density t-score <-2.5 at any site
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| * Age <50
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| More recently, three [[randomized controlled trial]]s have studied the role of surgery in patients with asymptomatic hyperparathyroidism. The largest study reported that surgery showed increase in bone mass, but no improvement in quality of life after one to two years among patients with<ref name="pmid17284629">{{cite journal |author=Bollerslev J, Jansson S, Mollerup CL, ''et al'' |title=Medical observation, compared with parathyroidectomy, for asymptomatic primary hyperparathyroidism: a prospective, randomized trial |journal=J. Clin. Endocrinol. Metab. |volume=92 |issue=5 |pages=1687-92 |year=2007 |pmid=17284629 |doi=10.1210/jc.2006-1836}}</ref>:
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| * Untreated, asymptomatic primary hyperparathyroidism
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| * Serum calcium between 2.60 - 2.85 mmol/liter (10.4 - 11.4 mg/dl)
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| * Age between 50 and 80 yr
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| * No medications interfering with Ca metabolism
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| * No hyperparathyroid bone disease
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| * No previous operation in the neck
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| * Creatinine level < 130 µmol/liter (<1.47 mg/dl)
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| Two other [[randomized controlled trial | trials]] reported improvements in bone density and some improvement in quality of life with surgery.<ref name="pmid17535997">{{cite journal |author=Ambrogini E, Cetani F, Cianferotti L, ''et al'' |title=Surgery or surveillance for mild asymptomatic primary hyperparathyroidism: a prospective, randomized clinical trial |journal=J. Clin. Endocrinol. Metab. |volume=92 |issue=8 |pages=3114-21 |year=2007 |pmid=17535997 |doi=10.1210/jc.2007-0219}}</ref><ref name="pmid15531491">{{cite journal |author=Rao DS, Phillips ER, Divine GW, Talpos GB |title=Randomized controlled clinical trial of surgery versus no surgery in patients with mild asymptomatic primary hyperparathyroidism |journal=J. Clin. Endocrinol. Metab. |volume=89 |issue=11 |pages=5415-22 |year=2004 |pmid=15531491 |doi=10.1210/jc.2004-0028}}</ref>
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| ===Future therapies===
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| Future developments such as calcimemetic agents (e.g. [[cinacalcet]]) which activate the parathyroid [[calcium-sensing receptor]] may offer a good alternative to surgery.
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| ==See also==
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| *[[Secondary hyperparathyroidism]]
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| *[[Tertiary hyperparathyroidism]]
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| ==References==
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| {{Reflist|2}}
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| {{Endocrine pathology}}
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| {{SIB}}
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| [[sv:Primär hyperparatyreos]]
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| [[Category:Endocrinology]]
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