Thursday, 28 March 2019

IP X SCID PT JRNY PJ

Severe combined immunodeficiency (SCID) is one of the most severe forms of primary immunodeficiency disorders in children. We report additional findings in the patient of SCID reported earlier by Gupta, et al. [1]. This was a 7-month-old boy symptomatic since 3 months of age with recurrent pneumonia, failure to thrive, oral thrush and oral ulcers. There was a significant family history of death of two elder siblings during infancy due to repeated infections. The index child died in the hospital due to respiratory failure and acute respiratory distress syndrome. Investigations revealed severe lymphopenia. An autopsy performed revealed thymic dysplasia with marked lymphoid depletion in lymphoid organs and bone marrow consistent with the clinical diagnosis of SCID. He also had severe Respiratory syncytial virus pneumonia and Cytomegalovirus inclusions in the lungs and adrenals.

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