DiGeorge Syndrome Medication

Updated: Oct 14, 2021
  • Author: Erawati V Bawle, MD, FAAP, FACMG; Chief Editor: Harumi Jyonouchi, MD  more...
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Medication Summary

Medications are necessary when hypocalcemia or immunodeficiency is present. Treat patients with severely impaired T-cell function or profound lymphopenia prophylactically with trimethoprim/sulfamethoxazole, as directed by the immunologist. In patients with primary immune deficiencies, an immunologist should decide whether to initiate replacement therapy with intravenous (IV) immunoglobulin.

Calcium supplementation is necessary in patients with hypocalcemia. In rare cases in which calcium supplementation may not suffice, vitamin D may also be administered.


Regenerative Therapy

Class Summary

Surgically implanted allogeneic processed thymic tissue is intended to reconstitute immunity in children who are athymic. 

Allogeneic processed thymus tissue (Rethymic)

Indicated for immune reconstitution in pediatric patients with congenital athymia. 



Class Summary

These agents are used prophylactically in patients with immunodeficiency.

Sulfamethoxazole and trimethoprim (Bactrim, Septra)

This combination is used for prophylaxis. It inhibits bacterial growth by inhibiting the synthesis of dihydrofolic acid. Sulfamethoxazole/trimethoprim should be prescribed based on recommendations from an immunologist.


Vitamin and Mineral Supplements

Class Summary

Hypocalcemia may occur, requiring supplementation with calcium. In patients with symptoms refractory to calcium, supplementation with a vitamin D analog may also be necessary.

Calcium carbonate (Oystercal, Caltrate)

Calcium carbonate is used for the treatment and prevention of calcium depletion. Calcium moderates nerve and muscle performance by regulating the action potential excitation threshold. One gram of calcium carbonate equals 400 mg of elemental calcium. Calcium carbonate has higher oral bioavailability than other orally administered calcium salt products.

Calcitriol (Rocaltrol)

This agent is a vitamin D analogue and the primary active metabolite of vitamin D-3. Calcitriol increases calcium levels by promoting the absorption of calcium in the intestines and the retention of calcium in the kidneys. Its use should be initiated only upon an endocrinologist's recommendation.

Calcium gluconate (Kalcinate)

Calcium gluconate moderates nerve and muscle performance and facilitates normal cardiac function. It can initially be administered intravenously; a high-calcium diet can be used to maintain calcium levels. Some patients require oral calcium supplementation. The 10% IV solution provides 100 mg/mL of calcium gluconate, which equals 9 mg/mL (0.46 mEq/mL) of elemental calcium.

Ergocalciferol, vitamin D-2 (Drisdol)

This vitamin D-2 analogue is converted in the liver to an active intermediate and then further converted to its most active form in kidneys. Ergocalciferol effectively increases renal reabsorption of calcium, intestinal absorption of calcium, and calcium mobilization from bone to plasma.