eMedicine Specialties > Emergency Medicine > Endocrine & Metabolic

Hypoparathyroidism

Author: David J Wallace, MD, MPH, Critical Care Medicine Fellow, University of Pittsburgh Medical Center
Coauthor(s): Agnieszka Gliwa, MD, Assistant Professor of Medicine, State University of New York Downstate Medical Center College of Medicine, Brooklyn; Attending Physician and Endocrinologist, Staten Island University Hospital
Contributor Information and Disclosures

Updated: Apr 15, 2009

Introduction

Background

Hypoparathyroidism describes a condition in which there are low circulating levels of parathyroid hormone (PTH) or insensitivity to its action.1  The causes of hypoparathyroidism vary; however, they all share a common feature of hypocalcemia. The presentation of hypoparathyroidism also varies depending on the chronicity of the resultant hypocalcemia. Muscle spasms/tetany, paresthesias, and seizures may occur in an acute onset, whereas chronic hypoparathyroidism may only be evidenced by visual impairment due to cataract formation.

See Hypocalcemia for more information.

Pathophysiology

Many underlying pathologic etiologies of hypoparathyroidism exist.

  • The most common causes are neck surgery and autoimmune processes. Hypoparathyroidism resulting from thyroid or parathyroid surgery can become clinically apparent 1-2 days after the procedure or follow the operation by many years. The incidence of permanent hypoparathyroidism varies with the extent of the procedure, the surgeon’s experience, and the underlying disease process being treated. Rarely, hypoparathyroidism can be a complication of radioactive iodine treatment of external localized radiotherapy.2
  • Autoimmune insult to the parathyroid gland can be isolated or associated with a variety of polyglandular syndromes. Antibodies to the parathyroids have been detected in up to 30% of patients with isolated hypoparathyroidism and 40% of patients with polyglandular disease.3 The calcium sensor-receptor is another target of autoantibodies in hypoparathyroidism. In patients with polyglandular autoimmune syndrome type 1, more than 50% will have this antibody. See Polyglandular Autoimmune Syndrome, Type I.
  • Maternal hyperparathyroidism can result in transient neonatal hypoparathyroidism.4,5,6 Maternal PTH suppresses neonatal parathyroid activity; however, this resolves rapidly after birth and removal from excessive maternal PTH.
  • Both hypermagnesemia and hypomagnesemia can result in decreased PTH secretion. In the case of hypermagnesemia, elevated magnesium levels result in stimulation of a calcium-sensing receptor on the pituitary. This, in turn, attenuates PTH secretion. In the case of chronic alcoholics with hypomagnesemia, there is diminution of PTH secretion levels and a resistance to hormone activity.7 See Hypermagnesemia and Hypomagnesemia.
  • This condition is characterized by thymus and parathyroid dysgenesis, cardiac malformation, and facial dysmorphogenesis.8 Other complex syndromes associated with hypoparathyroidism have been described and include Sanjat-Sakati syndrome, HDR syndrome, Kenny-Caffey syndrome, Kearns-Sayre syndrome, and Pearson marrow-pancreas syndrome.9 See DiGeorge Syndrome and Kearns-Sayre Syndrome.
  • Infiltration of the parathyroid gland can lead to clinically significant hypoparathyroidism. Causes include metastatic carcinoma, hemochromatosis, transfusion-related iron overload, Wilson disease10 , and sarcoidosis11 . See Hemochromatosis, Wilson Disease, and Sarcoidosis.

PTH functions to maintain plasma calcium levels by withdrawing calcium from bone tissue, glomerular filtrate reabsorption, and indirectly through increased intestinal absorption of calcium by activation of vitamin D-1,25. Insufficient production of PTH is known as true hypoparathyroidism, while decreased action on target tissues iscalled pseudohypoparathyroidism.3 See Pseudohypoparathyroidism.

Frequency

United States

Primary hypoparathyroidism is rare. Familial cases occur with autosomal dominant, autosomal recessive, and X-linked transmission.

Mortality/Morbidity

Acute hypocalcemia can be treated with good outcome. The mortality rate of hypoparathyroidism depends on the underlying cause.

Sex

With the exception of X-linked transmitted syndromes, no sex predilection exists.

Age

  • Maternal hyperparathyroidism resulting in newborn hypoparathyroidism usually manifests by the third week of life;4,5 however, cases have been reported as late as 2 months of age.6
  • Patients with DiGeorge syndrome present for clinical evaluation between birth and 3 months of age with a variety of symptoms.
  • Patients with polyglandular autoimmune syndrome type I present early in life. These patients typically have candidiasis by age 5 years and hypoparathyroidism by age 10 years.
  • For other forms of hypoparathyroidism, no age predilection is noted.

Clinical

History

  • A full surgical and family history is essential in cases of suspected hypoparathyroidism.
  • Neuromuscular irritability, arising from hypocalcemia, is the hallmark of the condition. These features can range from mild-to-moderate paresthesias of the extremities or lips to painful muscle cramps. In severe cases, tetany can result in carpopedal spasm, laryngospasm,12 or generalized seizures. Recurrent laryngospasm should prompt an investigation of underlying hypoparathyroidism.13
  • Additionally, severe hypocalcemia can result in neuropsychiatric and cardiovascular abnormalities. Neuropsychiatric manifestations include irritability, anxiety, psychosis, dementia, hallucinations, depression, and confusion. The cardiovascular effects of hypocalcemia are usually bradydysrhythmias or prolongation of the QT interval. Severe hypocalcemia can rarely mimic myocardial infarction.14
  • Gastrointestinal complaints may result from hypocalcemia as well. Smooth muscle spasms can result in intestinal and biliary cramping. Several cases of dysphagia have been described in the setting of hypocalcemia.15
  • Symptoms are rare unless the ionized calcium level drops below 2.8 mg/dL.3

Physical

The clinical manifestation of hypoparathyroidism is due to hypocalcemia.

  • Head, ears, eyes, nose, and throat signs
    • Surgical/traumatic scars
    • Mucocutaneous candidiasis (in the setting of polyglandular failure type 116 )
  • Neurologic signs
    • Hyperreflexia
    • Tetany
    • Chvostek sign - Chvostek sign has low sensitivity and specificity. Twenty-five percent of healthy persons will have a positive result; 29% of hypocalcemic patients will have a negative result.3
    • Trousseau sign (carpal spasm caused by occluding the brachial artery) - Trousseau sign is more reliable. Only 1-4% of healthy persons will have a positive sign; 94% of hypocalcemic persons will have a positive sign.3
    • Seizures
    • Altered mental status
  • Cardiovascular signs
    • Heart failure17,18
    • Bradycardia19
    • Hypotension not responsive to fluids or pressors20
  • Ophthalmologic signs - Cataracts21
  • Signs in infants
    • Vomiting
    • Abdominal distention
    • Apneic spells
    • Intermittent cyanosis
    • Twitching, tremors, and seizures

Causes

Hypoparathyroidism has multiple etiologies:

  • Postsurgical
  • Autoimmune
  • Sporadic22
  • Polyglandular syndromes
  • Activating antibodies to the calcium-sensing receptor23,24
  • Infiltration
  • Parathyroid destruction
  • Copper10
  • Malignancy
  • Granulomatous disease11
  • Mitochondrial neuropathies
  • Inactivating mutations of the PTH gene22
  • DiGeorge syndrome8
  • Impaired secretion and/or action of PTH
  • Hypomagnesemia25
  • Pseudohypoparathyroidism
  • Hemochromatosis26
  • Infarction27
  • Hypermagnesemia28
  • Medication induced (aluminum,29 doxorubicin,25  aminoglycoside,30 cimetidine,31 alendronate32 , omeprazole33 )

More on Hypoparathyroidism

Overview: Hypoparathyroidism
Differential Diagnoses & Workup: Hypoparathyroidism
Treatment & Medication: Hypoparathyroidism
Follow-up: Hypoparathyroidism
References
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References

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Further Reading

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Keywords

hypoparathyroidism, parathyroid hormone, PTH, hypocalcemia, hypomagnesemia, pseudohypoparathyroidism, parathyroid glands, parathyroid aplasia, DiGeorge syndrome, congenital hypoparathyroidism, parathyroid adenoma, sarcoidosis, Wilson disease, hemochromatosis, metastatic carcinoma, hypermagnesemia, autoimmune polyglandular syndrome type 1, Kenny syndrome, drug-induced hypoparathyroidism, suppression of parathyroid gland, Sanjat-Sakati syndrome, HDR syndrome, Kenny-Caffey syndrome, Pearson's marrow-pancreas syndrome, Pearson marrow-pancreas syndrome

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Contributor Information and Disclosures

Author

David J Wallace, MD, MPH, Critical Care Medicine Fellow, University of Pittsburgh Medical Center
David J Wallace, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American College of Emergency Physicians, American Medical Association, Emergency Medicine Residents Association, Society for Academic Emergency Medicine, and Society of Critical Care Medicine
Disclosure: Nothing to disclose.

Coauthor(s)

Agnieszka Gliwa, MD, Assistant Professor of Medicine, State University of New York Downstate Medical Center College of Medicine, Brooklyn; Attending Physician and Endocrinologist, Staten Island University Hospital
Agnieszka Gliwa, MD is a member of the following medical societies: American Association of Clinical Endocrinologists, American College of Physicians-American Society of Internal Medicine, American Medical Association, and Endocrine Society
Disclosure: Nothing to disclose.

Medical Editor

Erik D Schraga, MD, Consulting Staff, Department of Emergency Medicine, Mills-Peninsula Emergency Medical Associates; Consulting Staff, Permanente Medical Group, Kaiser Permanente, Santa Clara Medical Center
Disclosure: Nothing to disclose.

Pharmacy Editor

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

Managing Editor

Howard A Bessen, MD, Professor of Medicine, Department of Emergency Medicine, UCLA School of Medicine; Program Director, Harbor-UCLA Medical Center
Howard A Bessen, MD is a member of the following medical societies: American College of Emergency Physicians
Disclosure: Nothing to disclose.

CME Editor

John D Halamka, MD, MS, Associate Professor of Medicine, Harvard Medical School, Beth Israel Deaconess Medical Center; Chief Information Officer, CareGroup Healthcare System and Harvard Medical School; Attending Physician, Division of Emergency Medicine, Beth Israel Deaconess Medical Center
John D Halamka, MD, MS is a member of the following medical societies: American College of Emergency Physicians, American Medical Informatics Association, Phi Beta Kappa, and Society for Academic Emergency Medicine
Disclosure: Nothing to disclose.

Chief Editor

Erik D Schraga, MD, Consulting Staff, Department of Emergency Medicine, Mills-Peninsula Emergency Medical Associates; Consulting Staff, Permanente Medical Group, Kaiser Permanente, Santa Clara Medical Center
Disclosure: Nothing to disclose.

 
 
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