Treatment algorithm

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer

ACUTE

symptomatic hypocalcemia

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intravenous calcium gluconate or calcium chloride + ECG monitoring

Symptomatic hypocalcemia is an acute medical emergency, and intravenous calcium gluconate or calcium chloride should be started as soon as possible.

Calcium should be given in an infusion to reduce fluctuations in levels.

ECG monitoring is required during the administration of intravenous calcium to detect calcium-induced cardiac conduction defects. Rapid administration may cause bradycardia, hypotension, and vasodilation. Infiltration of intravenous calcium may cause severe tissue necrosis and sloughing.

Calcium levels should be monitored regularly.

The patient should be transitioned to oral calcium supplements as soon as the acute symptoms have resolved.

Consult local protocol for dosing and administration guidelines.

Primary options

calcium gluconate

OR

calcium chloride

ONGOING

asymptomatic hypocalcemia

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oral calcium supplements

Calcium carbonate and calcium acetate are preferred to dietary sources for maintaining a normal serum calcium.

Dietary sources of calcium also contain high levels of phosphate, which increases the risk of extraskeletal calcification.

Calcium carbonate and calcium acetate act as phosphate binders and reduce the absorption of phosphate from the gut. They can therefore lower serum phosphate levels and reduce the risk of extraskeletal calcification.

Primary options

calcium carbonate: children: 45-65 mg/kg/day orally given in 4 divided doses; adults: 1-2 g/day orally given in 3-4 divided doses

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OR

calcium acetate: adults: 1-2 g/day orally given in 3-4 divided doses

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calcitriol

Treatment recommended for SOME patients in selected patient group

If the response to calcium supplements is inadequate, calcitriol (a vitamin D metabolite) can be added to improve calcium absorption from the gut.

Patients with PHP do not have vitamin D deficiency, and care must be taken to avoid vitamin D overdosing.

Toxicity is easier to reverse with calcitriol because it has a short half-life and is not stored in adipose tissue. Calcitriol is therefore preferred to cholecalciferol or ergocalciferol.

Primary options

calcitriol: children <1 year of age: consult specialist for guidance on dose; children 1-5 years of age: 0.25 to 0.75 micrograms orally once daily; children >5 years of age and adults: 0.25 micrograms orally once daily

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thiazide diuretic

Treatment recommended for SOME patients in selected patient group

Thiazide diuretics reduce the urinary excretion of calcium and can be added to further increase calcium levels.

Primary options

hydrochlorothiazide: children: 1-2 mg/kg/day orally given in 2 divided doses; adults: 12.5 mg orally once daily

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Plus – 

levothyroxine

Treatment recommended for ALL patients in selected patient group

Hypothyroidism is the most common associated endocrinopathy and requires lifelong treatment with levothyroxine.

Primary options

levothyroxine: children: consult specialist for guidance on dose; adults: 1.7 micrograms/kg/day orally initially, increase by 12.5 to 25 micrograms/day increments every 2-4 weeks according to response

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Consider – 

growth hormone therapy

Treatment recommended for SOME patients in selected patient group

Growth hormone therapy may be beneficial in children with short stature due to growth hormone deficiency.

The onset of growth hormone deficiency in PHP type Ia is variable, making the time interval to treat short stature limited. Attempts to determine the etiology and best treatment regimen are ongoing. The effect of human growth hormone in this group has been studied and researchers demonstrated successful attainment of height velocity in prepubertal children.[49] In this small series only one child attained normal final height. The findings suggested the lack of sufficient growth hormone-releasing hormone effect as a major component of short stature. For more information on treatment of growth hormone deficiency, please see Growth hormone deficiency in children.

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Consider – 

testosterone or estrogen

Treatment recommended for SOME patients in selected patient group

Children with delayed maturation may require exogenous testosterone or estrogen. For more information on treatment of delayed puberty, please see Delayed puberty.

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Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer

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