Publications by authors named "Mary Dell Railey"

Background: Severe combined immunodeficiency (SCID) is a syndrome uniformly fatal during infancy unless recognized and treated successfully by bone marrow transplantation or gene therapy. Because infants with SCID have no abnormal physical appearance, diagnosis is usually delayed unless newborn screening is performed.

Objective: In this study, we sought to evaluate the presenting features of all 172 patients with SCID transplanted at this institution over the past 31 years.

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Food allergy is a life-changing and potentially life-threatening diagnosis, affecting approximately 6% of children and 4% of adults in the United States. A small number of foods account for the vast majority of food allergies, and the reactions after ingestion of a food to which a person is allergic are varied. At present, the standard of care for food-allergic patients is strict avoidance along with immediate access to self-injectable epinephrine and antihistamines.

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Objective: To determine long-term health benefits of nonablative bone marrow transplantation for severe combined immunodeficiency (SCID), we investigated our cohort of 161 related donor bone marrow-transplanted patients with SCID. Only 16 (10%) had HLA-identical donors.

Study Design: All 124 survivors were sent questionnaires about their current clinical statuses.

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