eMedicine Specialties > Endocrinology > Parathyroid Gland

Hypoparathyroidism

J Michael Gonzalez-Campoy, MD, PhD, FACE, Medical Director and CEO, MN Center for Obesity, Metabolism, and Endocrinology

Updated: Jul 17, 2009

Introduction

Background

Hypoparathyroidism is a condition of parathyroid hormone (PTH) deficiency.

Primary hypoparathyroidism is a state of inadequate PTH activity. In the absence of adequate PTH activity, the ionized calcium concentration in the extracellular fluid falls below the reference range. Primary hypoparathyroidism, the subject of this article, is a syndrome resulting from iatrogenic causes or one of many rare diseases.

Secondary hypoparathyroidism is a physiologic state in which PTH levels are low in response to a primary process that causes hypercalcemia. The primary processes that lead to hypercalcemia are discussed in other articles (see Hypercalcemia).

Pathophysiology

The ionized calcium concentration in the extracellular fluid (ECF) remains nearly constant, at a level of approximately 1 mM. Ionized calcium in the ECF is in equilibrium with ionized calcium in storage pools such as bone, proteins in the circulation, and within the intracellular fluid. The intracellular fluid concentration of calcium is more than 10,000-fold lower than in the ECF. The maintenance of ionized calcium concentrations in the intracellular and extracellular fluids is highly regulated and modulates the functions of bone, renal tubular cells, clotting factors, adhesion molecules, excitable tissues, and a myriad of intracellular processes.

An extracellular calcium-sensing receptor has been isolated from parathyroid, kidney, and brain cells. The extracellular calcium-sensing receptor is G protein coupled. Mutations in the extracellular calcium-sensing receptor have been demonstrated to result in hypercalcemic or hypocalcemic states. Normally, the extracellular calcium-sensing receptor is extremely sensitive and responds to changes in the ECF calcium ion concentration as small as 2%.

In parathyroid cells, the extracellular calcium-sensing receptor regulates the secretion of PTH. Inactivating mutations of the extracellular calcium-sensing receptor lead to hypercalcemia, as observed in familial hypocalciuric hypercalcemia (heterozygous mutation) and neonatal severe hyperparathyroidism (homozygous mutation). Conversely, activating mutations of the extracellular calcium-sensing receptor lead to hypocalcemia, as observed in some families with autosomal-dominant hypocalcemia.

The intracellular mechanism(s) whereby activation of the extracellular calcium-sensing receptor leads to inhibition of PTH exocytosis is unknown. Because pertussis toxin blocks the inhibition of cyclic adenosine monophosphate (cAMP), but not PTH, in response to a high ECF ionized calcium concentration, cAMP is probably not an important second messenger for the extracellular calcium-sensing receptor. Candidate second messengers include protein kinase C, phospholipase A2, and intracellular calcium.

Conversely, a fall in ECF ionized calcium concentration leads to exocytosis of PTH. PTH has the overall effect of returning the ECF ionized calcium concentration to the reference range by its effects on the kidneys and the skeleton.

PTH activates osteoclasts. Osteoclast activation results in bone resorption and a release of ionized calcium into the ECF. Evidence suggests that small pulse doses of PTH activate osteoblasts, with ensuing bone deposition. The effect of PTH on osteoclasts seems more important than the effect on osteoblasts.

PTH inhibits the proximal tubular transport of phosphate from the lumen to the interstitium. In conditions of primary PTH excess, hypophosphatemia tends to occur. Conversely, in hypoparathyroidism, the phosphate concentration in the plasma is within the reference range or slightly elevated.

PTH has a calcium-retaining effect on the distal tubule. The PTH-mediated calcium reabsorption is independent of any effects on sodium or water reabsorption. This effect of PTH is important in hypoparathyroidism because, in the absence of this distal tubular calcium reabsorption, the kidneys waste calcium. This depletes the ECF ionized calcium and increases the urinary calcium concentration.

PTH stimulates renal 1-alpha-hydroxylase, the enzyme that synthesizes formation of 1,25-dihydroxy vitamin D; 1,25-dihydroxy vitamin D allows for better dietary calcium absorption. Thus, 1,25-dihydroxy vitamin D has a synergistic effect with PTH; both contribute to a rise in the ECF ionized calcium concentration.

In the absence of PTH, bone resorption, phosphaturic effect, renal distal tubular calcium reabsorption, and 1,25-dihydroxy vitamin D–mediated dietary calcium absorption cannot occur. Therefore, the consequence of PTH deficiency is hypocalcemia.

Clinical

History

Hypoparathyroidism results in hypocalcemia, which may be variably symptomatic. The history should focus on eliciting signs and symptoms of neuromuscular irritability, including the following:

Paresthesias (involving fingertips, toes, perioral area)
Hyperirritability
Fatigue
Anxiety
Mood swings and/or personality disturbances
Seizures (especially in patients with epilepsy)
Hoarseness (due to laryngospasm)
Wheezing and dyspnea (due to bronchospasm)
Muscle cramps, diaphoresis, and biliary colic
Hypomagnesemia, hypokalemia, and alkalosis (eg, hyperventilation), which worsen signs and symptoms of hypocalcemia

Physical

Muscle cramps involving the lower back, legs, and feet are common in patients with hypoparathyroidism and hypocalcemia. Tetany develops if hypocalcemia is severe. In some patients, laryngospasm and bronchospasm may be life threatening.
Increased neuromuscular irritability from hypoparathyroidism-induced hypocalcemia may be demonstrated at the bedside by eliciting the following signs:
- Chvostek sign: Facial twitching, especially around the mouth, is induced by gently tapping the ipsilateral facial nerve as it courses just anterior to the ear.
- Trousseau sign: Carpal spasm is induced by inflating a blood pressure cuff around the arm to a pressure 20 mm Hg above obliteration of the radial pulse for 3-5 minutes.
Hypocalcemia of primary hypoparathyroidism may cause extrapyramidal choreoathetoid syndromes in patients with basal ganglia calcifications.
Parkinsonism, dystonia, hemiballismus, and oculogyric crises may occur in approximately 5% of patients with idiopathic hypoparathyroidism.¹
Spastic paraplegia, ataxia, dysphagia, and dysarthria have been documented in association with hypoparathyroidism-induced hypocalcemia. Severe hypocalcemia causes papilledema, which improves with treatment of the calcium derangement.
Emotional instability, anxiety, depression, confusion, hallucinations, and psychosis have been described in patients with hypoparathyroidism when the calcium level is low. Normocalcemia corrects these conditions.
Chronic hypocalcemia, as observed in primary hypoparathyroidism, is also associated with ocular cataracts; abnormal dentition; and dry, puffy, coarse skin. In severe hypocalcemia, a prolongation of the QT interval is observed on ECG, and congestive heart failure may develop. Correction of hypocalcemia reverses the cardiac effects of hypoparathyroidism.
In patients with autoimmune polyglandular syndrome, idiopathic hypoparathyroidism is associated with adrenal insufficiency and moniliasis. Moniliasis may affect the skin, nails, oral cavity, and vaginal cavity. It is frequently intractable. The underlying etiology is likely a defect in cellular immunity. Some authors advocate the term HAM syndrome, ie, hypoparathyroidism, Addison disease, and moniliasis (HAM), to denote these cases.
In a study of 33 patients with hypoparathyroidism, Rubin et al concluded that the disease causes bone to assume unusual structural and dynamic properties.² Examining biopsies of the iliac crest, the investigators found that, in comparison with biopsies from 33 patients with no known metabolic diseases, the individuals with hypoparathyroidism had greater cancellous bone volume, trabecular width, and cortical width. Moreover, the patients with hypoparathyroidism demonstrated profound suppression of dynamic skeletal indices, including mineralizing surface and bone formation rate.

Causes

Most people have 4 parathyroid glands; consequently, primary hypoparathyroidism is uncommon. Hypocalcemia from hypoparathyroidism requires all 4 parathyroid glands to be affected. Primary hypoparathyroidism may be permanent or reversible. Permanent primary hypoparathyroidism may be congenital or acquired.

Iatrogenic causes
- The most common cause of primary hypoparathyroidism is excision of all parathyroid glands via surgery in the treatment of thyroid, laryngeal, or other neck malignancy. Patients with parathyroid hyperplasia, as observed in the multiple endocrine neoplasia (MEN) syndromes, are treated by surgical removal of the parathyroid glands. Attempts at restoring normal PTH levels and normocalcemia by autotransplantation³ of a fraction of one of the parathyroid glands sometimes are effective, but many patients become hypoparathyroid. Repeated neck explorations for primary hyperparathyroidism caused by parathyroid adenoma may also cause hypoparathyroidism.
- Extensive irradiation to the face, neck, or mediastinum may cause destruction of all 4 parathyroid glands, with ensuing primary hypoparathyroidism and hypocalcemia.
- The "hungry bone syndrome" develops after a parathyroidectomy for hyperparathyroidism. The body has been accustomed to high levels of PTH, causing hypercalcemia. Much of this hypercalcemic effect is because of resorption of bone. When the parathyroid gland or glands responsible for the hypersecretion of PTH are removed, the PTH level in the blood drops suddenly, and the patient experiences transient hypoparathyroidism. The bone, which has been starved of calcium, avidly retains it under the influence of osteoblasts. Without PTH and with bone now using calcium to remineralize, the ECF ionized calcium level falls. Postoperatively, patients require aggressive treatment with calcium for several hours to several days. Eventually, the hypoparathyroid state resolves, and calcium homeostasis is re-achieved.
Autoimmune causes⁴
- Type 1 autoimmune polyglandular syndrome (also referred to as HAM syndrome) includes primary hypoparathyroidism that is due to destruction of the parathyroid glands. On average, these patients develop primary hypoparathyroidism by age 10 years.
- Autoimmune hypoparathyroidism may exist alone or in sporadic or familial forms. For patients with autoimmune primary hypoparathyroidism, the average age for development of hypocalcemia is 7 years, with a range of 6 months to 20 years.
Congenital causes
- Numerous conditions are described in the literature that result in congenital agenesis or hypoplasia and, therefore, can produce primary hypoparathyroidism with symptomatic hypocalcemia at birth or in the newborn period. These conditions, which are summarized from Goltzman and Cole (1996), are as follows:⁵
  - Isolated primary hypoparathyroidism
  - X-linked primary hypoparathyroidism (band Xq26-Xq27)
  - X autosomal-recessive primary hypoparathyroidism
  - Branchial dysgenesis (DiGeorge syndrome)
  - Chromosomal defects dup(1q),del(5p),dup(8q),del(10q),del(22q)
  - Monogenic hypoparathyroidism
  - Isolated autosomal-dominant conditions
  - Isolated autosomal-recessive conditions
  - Velocardiofacial (Shprintzen) syndrome (CATCH 22 [for cardiac, abnormal facies, thymic aplasia, cleft palate, and hypocalcemia with 22q deletion] is a mnemonic for the features of this syndrome.)
  - Zellweger syndrome
  - Teratogenic effects
  - Diabetic embryopathy
  - Fetal alcohol syndrome
  - Retinoid embryopathy
  - Associational arhinencephalia and/or DiGeorge syndrome and the coloboma, heart disease, choanal atresia, retarded growth and development, genital anomalies, ear anomalies (CHARGE) syndrome and/or DiGeorge syndrome
  - Cardiofacial–DiGeorge–Kenny-Caffey syndrome (ie, absent parathyroid tissue, growth retardation, medullary stenosis of tubular bones)
  - Kearns-Sayre syndrome (ie, mitochondrial myopathy, ophthalmoplegia, retinal degeneration, cardiac conduction defects, primary hypoparathyroidism)
  - Barakat syndrome (ie, primary hypoparathyroidism, nerve deafness, steroid-resistant nephrosis)
  - Hypoparathyroidism with short stature, mental retardation, and seizures
- In addition to the above list, several other genetic defects cause primary hypoparathyroidism. As opposed to the conditions listed above, no agenesis or hypoplasia of the parathyroid glands occurs in these other genetic defects. These mutations are functional, not anatomic, and are listed as follows:
  - Mutation of chromosome arm 3q has been demonstrated to cause primary hypoparathyroidism in several kindreds because of activation of the parathyroid extracellular calcium-sensing receptor. These patients have mild-to-moderate hypocalcemia, urinary calcium excretion that is high relative to serum calcium (presumably the extracellular calcium-sensing receptor in the kidney contributes to this), and serum PTH concentration that is within the reference range (but is inappropriately low).
  - Familial isolated hypoparathyroidism is a heterogenous mix of disorders as follows: autosomal dominant abnormal prepro-PTH allele (C-to-T substitution in codon 18 of the prepeptide encoding region does not allow for cleavage to pro-PTH) and autosomal recessive abnormal prepro-PTH allele (C-to-G substitution in the first nucleotide position of prepro-PTH intron 2).
Causes related to metal overload (ion deficiency)
- Hemochromatosis and thalassemia, both of which are associated with iron overload, may result in primary hypoparathyroidism.
- Wilson disease, with copper overload, may also cause primary hypoparathyroidism.
- Hypermagnesemia has been demonstrated to decrease PTH release. Correction of hypermagnesemia leads to correction of the primary hypoparathyroidism.
- Aluminum deposition within the parathyroid glands may cause primary hypoparathyroidism in patients with end-stage renal disease who are on hemodialysis.
- Hypomagnesemia causes reversible functional primary hypoparathyroidism.
Causes related to infiltration of the parathyroid glands
- In addition to hemochromatosis and Wilson disease, parathyroid gland destruction has been reported as a result of metastatic disease, granulomatous disease, amyloidosis, syphilis, and progressive systemic sclerosis.
- Of note, clinically significant hypocalcemia is not always apparent in these patients.
Neonatal causes
- The unborn baby of a mother with hypercalcemia has chronic suppression of parathyroid gland function. In the worst circumstances, the parathyroid glands may become atrophic.
- At birth, the maternal calcium excess is eliminated, and newborns are at risk of hypocalcemia caused by primary hypoparathyroidism.
- Clinically significant hypocalcemia may develop within the first 3 weeks of life but may occur as late as 1 year after birth. The primary hypoparathyroidism in these patients is self-limited.

Differential Diagnoses

Hypocalcemia
Pseudohypoparathyroidism

Workup

Laboratory Studies

Parathyroid hormone
- Primary hypoparathyroidism is defined by a low concentration of PTH with a concomitant low calcium level.
- In pseudohypoparathyroidism, the serum PTH concentration is elevated as a result of resistance to PTH caused by mutations in the PTH receptor system.
- In secondary hypoparathyroidism, the serum PTH concentration is low and the serum calcium concentration is elevated.
Calcium
- The calcium ion is highly bound to protein. A total calcium level cannot be interpreted without a total protein or albumin level.
- Hypoalbuminemia causes a drop in total calcium concentration, but the ionized fraction may be within the reference range. Elevated protein states, such as multiple myeloma and paraproteinemias, may cause an elevation of the total calcium concentration, but the ionized fraction may be within the reference range.
- Conversely, in the presence of albumin or protein excess, low ionized calcium levels with reference range levels of total calcium are possible. Likewise, if the patient is hypoalbuminemic, high ionized calcium levels with a reference range level of total calcium are possible.
- Measurement of ionized calcium concentration in the plasma is ideal; however, it is not readily available in many places.
- The relationship between total serum calcium and albumin is defined by the following simple rule: the serum total calcium concentration falls by 0.8 mg/dL for every 1-g/dL fall in serum albumin concentration. This rule assumes that normal albumin equals 4.0 g/dL and normal calcium is 10.0 mg/dL.
- Alkalosis causes ionized calcium to bind to albumin more strongly. This causes a decrease in the ionized calcium and may trigger symptoms of hypocalcemia.
Measurement of 25-hydroxy vitamin D: This measurement is important to exclude vitamin D deficiency as a cause of hypocalcemia.
Serum magnesium: Hypomagnesemia may cause PTH deficiency and subsequent hypocalcemia. Exclude it in any patient with primary hypoparathyroidism.
Serum phosphorus: PTH is a phosphaturic hormone. In its absence, phosphorus levels in the blood rise.

Treatment

Medical Care

PTH is commercially available for use in the treatment of osteoporosis. Its use for patients with hypoparathyroidism is not approved by the Food and Drug Administration.
Currently, treatment of patients with hypoparathyroidism involves correcting the hypocalcemia by administering calcium and vitamin D.⁶

Surgical Care

Patients undergoing parathyroidectomy for parathyroid hyperplasia are at high risk of developing permanent primary hypoparathyroidism.
Patients may be treated with an autotransplant of a segment of parathyroid gland to prevent hypoparathyroidism.³ This autotransplant is usually placed subcutaneously in the forearm or in the neck.
If the autotransplantation fails, patients receive the same treatment that is administered to other patients with hypoparathyroidism.

Consultations

An endocrinologist should be involved in the care of all patients who have primary hypoparathyroidism or who are at risk of developing it.

Diet

A diet rich in calcium content (ie, emphasizing dairy products) is recommended for patients with primary hypoparathyroidism.

Activity

Patients with symptomatic hypocalcemia develop tetany. Otherwise, no restriction in activity for these patients is necessary.

Medication

Calcium and vitamin D are the mainstays of treatment.

Calcium salts

Without PTH, the ionized calcium levels in the plasma drop. Bone becomes an inefficient source of calcium for plasma, and kidneys waste calcium. Calcium helps maintain the ionized calcium level close to the reference range.

Calcium carbonate (Tums Extra Strength, Cal-Plus, Caltrate, Os-Cal 500)

Moderates nerve and muscle performance and facilitates normal cardiac function. Many commercially available preparations exist. Titrate total daily dose of elemental calcium to minimize the daily dose of vitamin D and to keep patients asymptomatic. Ionized calcium is absorbed best in an acidic environment; 400 mg elemental calcium equals 1 g calcium carbonate.

Dosing

Adult

1-2 g/d elemental calcium PO
2.5-5 g/d calcium carbonate PO

Pediatric

Administer as in adults

Interactions

May decrease effects of tetracyclines, atenolol, salicylates, iron salts, and fluoroquinolones; IV administration antagonizes effects of verapamil; large intakes of dietary fiber may decrease calcium absorption and levels

Contraindications

Documented hypersensitivity; renal calculi; hypercalcemia; hypophosphatemia; renal or cardiac disease; patients with digitalis toxicity

Precautions

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Nephrocalcinosis and nephrolithiasis are potential complications of therapy; caution in patients who are digitalized and patients with respiratory failure or acidosis; in absence of PTH, may precipitate in urinary tract

Calcium citrate (Citracal, Cal-Citrate 250)

Moderates nerve and muscle performance and facilitates normal cardiac function; 210 mg of elemental calcium equals 1 g calcium citrate.

Dosing

Adult

1-2 g/d elemental calcium PO
4.5-9 g/d calcium citrate PO

Pediatric

Administer as in adults

Interactions

Contraindications

Documented hypersensitivity; renal calculi; hypophosphatemia; hypercalcemia

Precautions

Pregnancy

A - Fetal risk not revealed in controlled studies in humans

D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus

Precautions

Pregnancy category D if dosage exceeds RDA; nephrocalcinosis and nephrolithiasis are potential complications of therapy; caution in patients who are digitalized and patients with respiratory failure or acidosis; may precipitate in urinary tract in absence of PTH; adequate dietary calcium is needed for clinical response; maintain adequate fluid intake; calcium-phosphate product (serum calcium times phosphorus) not to exceed 70; avoid use with renal function impairment and secondary hyperparathyroidism; avoid hypercalcemia

Calcium gluconate (Kalcinate)

Moderates nerve and muscle performance and facilitates normal cardiac function. Available for IV use. Infuse slowly over 5-10 min; 10 mL calcium gluconate contains approximately 90 mg elemental calcium; 1000 mg of calcium gluconate equals 90 mg elemental calcium.

Dosing

Adult

90 mg elemental calcium (1 g calcium gluconate) IV over 5-10 min

Pediatric

Administer as in adults

Interactions

May decrease bioavailability of tetracyclines, fluoroquinolones, iron salts, salicylates, atenolol, and sodium polystyrene sulfonate; IV calcium may antagonize verapamil effects; large intake of dietary fiber may decrease calcium absorption; IV calcium may increase quinidine and digitalis effects

Contraindications

Documented hypersensitivity; ventricular fibrillation during cardiac resuscitation; digitalis toxicity; renal or cardiac disease; hypercalcemia; renal calculi; hypophosphatemia

Precautions

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Avoid rapid IV administration; caution in patients who are digitalized and patients with severe hyperphosphatemia; patients with respiratory failure or acidosis; avoid extravasation; may produce cardiac arrest; hypercalcemia may occur in renal failure; monitor serum calcium during early dosing period; nephrocalcinosis and renal lithiasis are potential adverse effects of chronic renal calcium loss

Vitamin D preparations

Vitamin D is synthesized by the kidneys, and the synthesis of 1,25-dihydroxy vitamin D is PTH dependent. In most patients with chronic hypoparathyroidism, treatment with the active vitamin D form is necessary.⁶

Ergocalciferol (Calciferol, Drisdol)

Stimulates absorption of calcium and phosphate from small intestine and promotes release of calcium from bone into blood.

Dosing

Adult

50,000-100,000 U/d PO/IM

Pediatric

Administer as in adults

Interactions

Colestipol, mineral oil, and cholestyramine may decrease absorption from small intestine; thiazide diuretics may increase effects

Contraindications

Documented hypersensitivity; hypercalcemia; malabsorption syndrome

Precautions

Pregnancy

A - Fetal risk not revealed in controlled studies in humans

D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus

Precautions

Pregnancy category D if dosage exceeds RDA; caution in patients with impaired renal function, renal stones, heart disease, or arteriosclerosis

Dihydrotachysterol (DHT, Hytakerol)

Synthetic analog of vitamin D. Stimulates calcium and phosphate absorption from small intestine and promotes secretion of calcium from bone to blood. Promotes renal tubule resorption of phosphate.

Dosing

Adult

125-250 mcg/d PO

Pediatric

Administer as in adults

Interactions

Colestipol, mineral oil, and cholestyramine may decrease absorption from the small intestine; thiazide diuretics may increase effects of vitamin D

Contraindications

Documented hypersensitivity; hypercalcemia

Precautions

Pregnancy

A - Fetal risk not revealed in controlled studies in humans

D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus

Precautions

Pregnancy category D if dosage exceeds RDA; caution in impaired renal function, renal stones, heart disease, or arteriosclerosis

Calcifediol (Calderol)

Promotes absorption of calcium and phosphorus in the small intestine. Promotes renal tubule resorption of phosphate. Increases rate of accretion and resorption in bone minerals.

Dosing

Adult

50-220 mcg/d PO

Pediatric

Administer as in adults

Interactions

Cholestyramine and colestipol decrease effects; thiazide diuretics increase effect

Contraindications

Documented hypersensitivity; hypercalcemia

Precautions

Pregnancy

A - Fetal risk not revealed in controlled studies in humans

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus

Precautions

Pregnancy category A per expert analysis; pregnancy category C per manufacturer; pregnancy category D if dosage exceeds RDA; adequate dietary calcium needed for clinical response; maintain adequate fluid intake; calcium-phosphate product (serum calcium times phosphorus) not to exceed 70; avoid use with renal function impairment and secondary hyperparathyroidism; avoid hypercalcemia

Calcitriol (Rocaltrol, Calcijex)

Promotes absorption of calcium in intestines and retention at kidneys to increase calcium levels in serum. Decreases excessive serum phosphatase levels and parathyroid levels. Decreases bone resorption.

Dosing

Adult

0.5-1 mcg/d PO

Pediatric

Administer as in adults

Interactions

Cholestyramine and colestipol decrease effects; thiazide diuretics increase effects; magnesium-containing antacids have additive effects

Contraindications

Documented hypersensitivity; hypercalcemia; vitamin D toxicity; malabsorption syndrome

Precautions

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus

Precautions

Pregnancy category D if dosage exceeds RDA; adequate dietary calcium is needed for clinical response; maintain adequate fluid intake; calcium-phosphate product (serum calcium times phosphorus) not to exceed 70; avoid use with renal function impairment and secondary hyperparathyroidism; avoid hypercalcemia

Follow-up

Further Outpatient Care

Patients with primary hypoparathyroidism have a lifelong risk of symptomatic tetany.
Without access to calcium, a patient may die.
All patients should wear a chain or bracelet that identifies them as having primary hypoparathyroidism.

Complications

Nephrocalcinosis
Nephrolithiasis

Patient Education

Diuretic use: The use of any diuretic medication may alter calcium homeostasis. Patients must know this and should remind their practitioner whenever new medications are prescribed.
Pregnancy: Medications that alter the synthesis of proteins and albumin in the liver and/or hyperestrogenic states, such as pregnancy, may lead to alterations in calcium homeostasis. Instruct patients to consult their doctor prior to any changes in any medications.

Miscellaneous

Medicolegal Pitfalls

In discussing the risks of untreated primary hypoparathyroidism with patients, document the signs and symptoms of severe hypocalcemia.
Direct patients to the emergency department for treatment if mental status changes or respiratory distress develops.
Recommend that patients wear a chain or bracelet identifying them as having primary hypoparathyroidism; document this recommendation in the chart.

References

Goswami R, Goel S, Tomar N, et al. Prevalence of clinical remission in patients with sporadic idiopathic hypoparathyroidism. Clin Endocrinol (Oxf). Jun 22 2009;[Medline].
Rubin MR, Dempster DW, Zhou H, et al. Dynamic and structural properties of the skeleton in hypoparathyroidism. J Bone Miner Res. Dec 2008;23(12):2018-24. [Medline].
Ebrahimi H, Edhouse P, Lundgren CI, et al. Does autoimmune thyroid disease affect parathyroid autotransplantation and survival?. ANZ J Surg. May 2009;79(5):383-5. [Medline].
Brown EM. Anti-parathyroid and anti-calcium sensing receptor antibodies in autoimmune hypoparathyroidism. Endocrinol Metab Clin North Am. Jun 2009;38(2):437-45, x. [Medline].
Goltzman D, Cole DEC. Hypoparathyroidism. In: Favus MJ, ed. Primer on the Metabolic Bone Diseases and Disorders of Mineral Metabolism. Philadelphia, Pa: Lippincott-Raven; 1996:220-3.
Cheung M. Drugs used in paediatric bone and calcium disorders. Endocr Dev. 2009;16:218-232. [Medline].
Brown EM, Harris HW, Vassilev PM. The biology of the extracellular Ca2+-sensing receptor. In: Bilezikian JP, ed. Principles of Bone Biology. San Diego, Calif: Academic Press; 1996:243-62.
Cole DEC, Hendy GN. Hypoparathyroidism and pseudohypoparathyroidism. Endotext.com. 2005, Available at. [Full Text].
Marx SJ. Hyperparathyroid and hypoparathyroid disorders. N Engl J Med. Dec 21 2000;343(25):1863-75. [Medline].
Thakker RV. Molecular basis of PTH underexpression. In: Bilezikian JP, et al, eds. Principles of Bone Biology. San Diego, Calif: Academic Press; 1996:837-51.

Keywords

hypoparathyroidism, parathyroid, PTH, hyperparathyroidism, hypocalcemia, parathyroid hormone, tetany, parathyroid glands, parathyroid gland, surgery parathyroid, parathyroid surgery, parathyroidectomy, hypoparathyroid, parathyroid hormone deficiency, PTH deficiency, primary hypoparathyroidism, inadequate PTH activity, secondary hypoparathyroidism, hypercalcemia

Contributor Information and Disclosures

Author

J Michael Gonzalez-Campoy, MD, PhD, FACE, Medical Director and CEO, MN Center for Obesity, Metabolism, and Endocrinology
J Michael Gonzalez-Campoy, MD, PhD, FACE is a member of the following medical societies: American Association of Clinical Endocrinologists, American Medical Association, and Minnesota Medical Association
Disclosure: Nothing to disclose.

Medical Editor

David S Schade, MD, Chief, Division of Endocrinology and Metabolism, Professor, Department of Internal Medicine, University of New Mexico School of Medicine and Health Sciences Center
David S Schade, MD is a member of the following medical societies: American College of Physicians, American Diabetes Association, American Federation for Medical Research, Endocrine Society, New Mexico Medical Society, New York Academy of Sciences, and Society for Experimental Biology and Medicine
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Yoram Shenker, MD, Chief of Endocrinology Section, Veterans Affairs Medical Center of Madison; Interim Chief, Associate Professor, Department of Internal Medicine, Section of Endocrinology, Diabetes and Metabolism, University of Wisconsin at Madison
Yoram Shenker, MD is a member of the following medical societies: American Heart Association, Central Society for Clinical Research, and Endocrine Society
Disclosure: Nothing to disclose.

CME Editor

Mark Cooper, MBBS, PhD, FRACP, Head, Diabetes & Metabolism Division, Baker Heart Research Institute, Professor of Medicine, Monash University
Disclosure: Nothing to disclose.

Chief Editor

George T Griffing, MD, Professor of Medicine, St Louis University School of Medicine
George T Griffing, MD is a member of the following medical societies: American Association for the Advancement of Science, American College of Medical Practice Executives, American College of Physician Executives, American College of Physicians, American Diabetes Association, American Federation for Medical Research, American Heart Association, Central Society for Clinical Research, Endocrine Society, International Society for Clinical Densitometry, and Southern Society for Clinical Investigation
Disclosure: Nothing to disclose.

Further Reading