Secondary hypoparathyroidism due to impaired parathormon secretion

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ORPHA:140286E20.8
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Overview

Secondary hypoparathyroidism due to impaired parathormone (PTH) secretion (Orphanet code 140286) is a rare endocrine disorder characterized by insufficient production or release of parathyroid hormone from the parathyroid glands, occurring as a secondary consequence of an underlying condition rather than a primary defect of the glands themselves. Common causes include hypomagnesemia (severe magnesium deficiency), which impairs the ability of the parathyroid glands to secrete PTH adequately, as well as certain infiltrative diseases or other metabolic disturbances that affect parathyroid gland function. The condition primarily affects calcium and phosphorus homeostasis, leading to hypocalcemia (low blood calcium levels) and hyperphosphatemia (elevated blood phosphorus levels). Key symptoms result from low calcium levels and may include muscle cramps, tingling or numbness in the fingers, toes, and around the mouth (perioral paresthesias), muscle spasms (tetany), fatigue, and in severe cases, seizures. Cardiac complications such as prolonged QT interval on electrocardiogram may also occur. Chronic hypocalcemia can lead to dental abnormalities, cataracts, and neuropsychiatric symptoms including anxiety and depression. The diagnosis is established through blood tests showing low calcium, elevated phosphorus, and inappropriately low or undetectable PTH levels, along with identification of the underlying cause. Treatment focuses on correcting the underlying cause of impaired PTH secretion. For example, if hypomagnesemia is the trigger, magnesium replacement therapy often restores normal parathyroid function. Acute symptomatic hypocalcemia is managed with intravenous calcium supplementation, while chronic management typically involves oral calcium and active vitamin D analogs (such as calcitriol) to maintain adequate calcium levels. In cases where the underlying cause can be fully corrected, the hypoparathyroidism may be reversible. Regular monitoring of serum calcium, phosphorus, magnesium, and renal function is essential for long-term management.

Clinical phenotype terms— hover any for plain English:

Abnormality of the parathyroid glandHP:0000828HypoparathyroidismHP:0000829Basal ganglia calcificationHP:0002135HyperphosphatemiaHP:0002905Decreased circulating parathyroid hormone levelHP:0031817RicketsHP:0002748OsteomalaciaHP:0002749Bipolar affective disorderHP:0007302Posterior subcapsular cataractHP:0007787LaryngospasmHP:0025425
Age of Onset

Variable

Can begin at different ages, from infancy through adulthood

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Secondary hypoparathyroidism due to impaired parathormon secretion.

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No specialists are currently listed for Secondary hypoparathyroidism due to impaired parathormon secretion.

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Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Travel Grants

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Common questions about Secondary hypoparathyroidism due to impaired parathormon secretion

What is Secondary hypoparathyroidism due to impaired parathormon secretion?

Secondary hypoparathyroidism due to impaired parathormone (PTH) secretion (Orphanet code 140286) is a rare endocrine disorder characterized by insufficient production or release of parathyroid hormone from the parathyroid glands, occurring as a secondary consequence of an underlying condition rather than a primary defect of the glands themselves. Common causes include hypomagnesemia (severe magnesium deficiency), which impairs the ability of the parathyroid glands to secrete PTH adequately, as well as certain infiltrative diseases or other metabolic disturbances that affect parathyroid gland f