Severe combined immunodeficiency

Summary of Severe combined immunodeficiency
Severe combined immunodeficiency is the most severe form of primary immunodeficiencies, and may results from an X-linked recessive defect in IL-2R gamma chain or an autosomal recessive defect in adenosine deaminase deficiency. This disorder results in B-cell and T-cell disorders, resulting in the failure to thrive, chronic diarrhea, thrush, and recurrent infections.

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Severe combined immunodeficiency

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Severe combined immunodeficiency

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Severe combined immunodeficiency is a primary immunodeficiency that can develop from major histocompatibility complex (I/II) deficiency as it is required for CD4+ helper T cell activation and cytokine production.

Questions

USMLE® Step 1 style questions USMLE

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A 12-month-old boy is brought to the emergency room by his mother because of recurrent episodes of fever, chills, diarrhea, and failure to grow. He was born term and appeared normal at infancy; however, the mother explains she had not received prenatal care and had delivered her child at home. His temperature is 39.4°C (104.0°F), pulse is 140/min, respirations are 31/min, and blood pressure is 120/80 mm Hg. Chest X-ray shows an absent thymic shadow. The absolute lymphocyte count is well below the lower limit of normal. Medical records indicate he was diagnosed with severe combined immunodeficiency (SCID) when he was 5-months-old, but the patient was lost to follow-up. Hematopoietic cell transplantation is considered as the next step in management. Which of the following choices is true regarding bone marrow transplant in this patient?

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