eMedicine Specialties > Endocrinology > Parathyroid Gland
Pseudohypoparathyroidism: Treatment & Medication
Updated: Aug 5, 2009
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
Treatment
Medical Care
All patients with severe symptomatic hypocalcemia should be initially treated with intravenous calcium. Administration of oral calcium and 1alpha-hydroxylated vitamin D metabolites, such as calcitriol, remains the mainstay of treatment and should be initiated in every patient with a diagnosis of PHP. The goals of therapy are to maintain serum total and ionized calcium levels within the reference range to avoid hypercalciuria and to suppress PTH levels to normal. This is important because elevated PTH levels in patients with PHP could cause increased bone remodeling and can lead to hyperparathyroid bone disease.
- In adults, infuse approximately 100 mg of elemental calcium (either calcium chloride or calcium gluconate) over 10-20 minutes. If this measure does not alleviate the clinical manifestation, 100 mg/h of elemental calcium can be infused (in adults), with close monitoring of calcium levels. Do not rapidly infuse calcium because of the possible adverse effects of cardiac conduction defects; cardiac monitoring may help guide therapy. The 2 most readily available formulations for parenteral use are calcium chloride and calcium gluconate; a 10-mL ampule of 10% calcium chloride contains 360 mg of elemental calcium, and a 10-mL ampule of 10% calcium gluconate contains 93 mg of elemental calcium.
- For neonates, infants, and children, the recommended initial dose is 0.5-1 mL/kg of 10% calcium gluconate administered over 5 minutes.
Surgical Care
Rarely, extraskeletal osteomas require surgical removal to relieve pressure symptoms.
Diet
No restrictions are necessary.
Activity
No restrictions are necessary.
Medication
The goals of pharmacotherapy are to correct calcium deficiency, to prevent complications, and to reduce morbidity.
Calcium salts
Used for calcium electrolyte supplementation.
Calcium chloride
Improves nerve and muscle performance by regulating action potential excitation threshold affected by calcium deficiency.
Adult
0.5-1 g (7-14 mEq) IV; repeat q1-3d prn
Pediatric
0.2 mL (20 mg of calcium chloride)/kg IV; not to exceed 1-10 mL/d (10% solution); repeat q1-3d prn
Coadministration with digoxin may cause arrhythmias; with thiazides, may induce hypercalcemia; may antagonize effects of calcium channel blockers, atenolol, and sodium polystyrene sulfonate
Documented hypersensitivity; ventricular fibrillation not associated with hyperkalemia; digitalis toxicity; hypercalcemia; renal insufficiency; cardiac disease
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
Administer slowly (not to exceed 0.5-1 mL/min) to avoid extravasation; hypercalcemia may occur in patients with renal failure
Calcium gluconate (Kalcinate)
Moderates nerve and muscle performance and facilitates normal cardiac function. Can be initially administered IV, and calcium levels can be maintained with high-calcium diet. Some patients require PO calcium supplementation.
Adult
100-300 mg elemental calcium IV diluted in 150 mL D5W over 10 min; initial rate of infusion should be 0.3-2 mg of elemental calcium/kg/h
Pediatric
2 mg/kg IV of elemental calcium (about 20 mg/kg of calcium gluconate 10%)
May decrease effects of tetracyclines, atenolol, salicylates, iron salts, and fluoroquinolones; antagonizes effects of verapamil; large intakes of dietary fiber may decrease calcium absorption and levels
Renal calculi; hypercalcemia; hypophosphatemia; renal or cardiac disease; digitalis toxicity
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Caution in digitalized patients and patients with respiratory failure, acidosis, or severe hyperphosphatemia
Calcium carbonate (Oystercal, Caltrate, Os-Cal, Tums)
For supplementation of IV therapy in hypocalcemia. Calcium moderates nerve and muscle performance by regulating action potential excitation threshold.
Adult
1-2 g/d PO divided bid/qid at meal times
Pediatric
45-65 mg/kg/d PO divided qid
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
Documented hypersensitivity; renal calculi; hypercalcemia; hypophosphatemia; renal or cardiac disease; digitalis toxicity
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
Caution in digitalized patients and in patients with respiratory failure or acidosis; hypercalcemia or hypercalciuria may occur when therapeutic amounts are administered
Vitamins
Supplementation increases calcium levels in the serum by improving calcium absorption and retention.
Calcitriol (Calcijex, Rocaltrol)
Increases calcium levels by promoting calcium absorption in intestines and retention in kidneys.
Adult
0.25 mcg PO qd; increase at 4- to 8-wk intervals by 0.25 mcg prn
Pediatric
Initial: 15 ng/kg/d PO
Maintenance: 5-40 ng/kg/d PO
Cholestyramine and colestipol decrease absorption; magnesium-containing antacids and thiazide diuretics can increase effects
Documented hypersensitivity; hypercalcemia; malabsorption syndrome
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
Adequate response depends on adequate dietary calcium intake; maintain adequate fluid intake
More on Pseudohypoparathyroidism |
| Overview: Pseudohypoparathyroidism |
| Differential Diagnoses & Workup: Pseudohypoparathyroidism |
 Treatment & Medication: Pseudohypoparathyroidism |
| Follow-up: Pseudohypoparathyroidism |
| Multimedia: Pseudohypoparathyroidism |
| References |
| Further Reading |
| « Previous Page | Next Page » |
References
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Further Reading
Related eMedicine topics:
Embryology of the Thyroid and Parathyroids
Hyperphosphatemia [Emergency Medicine]
Hyperphosphatemia [Nephrology]
Hypocalcemia [Emergency Medicine]
Hypocalcemia [Nephrology]
Hypocalcemia [Pediatrics: General Medicine]
Hypoparathyroidism [Emergency Medicine]
Hypoparathyroidism [Endocrinology]
Hypoparathyroidism [Pediatrics: General Medicine]
Parathyroid Physiology
Clinical trials:
Resistance to Vitamin D or Parathyroid Hormone
Study of Growth Hormone Use in Pseudohypoparathyroidism Type 1a and Pseudopseudohypoparathyroidism (Albright Hereditary Osteodystrophy)
Keywords
pseudohypoparathyroidism, parathyroid, parathyroid hormone, hypocalcemia, parathyroid gland, parathyroid glands, PTH, pseudopseudohypoparathyroidism, hyperphosphatemia, Albright hereditary osteodystrophy, Albright's hereditary osteodystrophy, pseudo-PHP, stimulatory G protein, Gsa, GNAS1, testotoxicosis, dental hypoplasia, brachymetacarpals, brachymetatarsals, brachydactyly
