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Severe Combined Immunodeficiency, Pediatric

Reviewed 10/2018
 


BASICS

DESCRIPTION

Severe combined immunodeficiency (SCID) is a primary immunodeficiency disease characterized by functional defects in both the humoral and cellular immune systems. Most babies present...

DIAGNOSIS

HISTORY

  • Infants who have not been identified with newborn screening tests, usually present in the 1st year of life as their immunity from maternal antibody wanes.

  • Failure to thrive

  • Chronic diar...

TREATMENT

  • Hematopoietic (bone marrow) stem cell transplantation is curative for all types of SCID.

    • Success rates >70% have been reported and increase to 96% when the transplant is done before the ag...

ONGOING CARE

  • Pretransplant supportive care

    • Pneumocystis prophylaxis with trimethoprim/sulfamethoxazole

    • Immunoglobulin replacement therapy (IV every 3 to 4 weeks, subcutaneously every 1 to 2 weeks)

    • Palivi...

ADDITIONAL READING

  • Buckley RH. Transplantation of hematopoietic stem cells in human severe combined immunodeficiency: longterm outcomes. Immunol Res.  2011;49(1–3):25–43. [View Abstract on OvidIn...

CODES

ICD9

279.2 Combined immunity deficiency 

ICD10

  • D81.9 Combined immunodeficiency, unspecified

  • D81.1 Severe combined immunodeficiency w low T- and B-cell numbers

  • D81.2 Severe combined immunodef w low or...

FAQ

  • Q: What should I do if I am notified that a newborn screen has identified a low number of TRECs?

  • A: As a precaution while the child is being evaluated, prevent the infant’s exposure to sick contact...

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