A 9-month-old girl with a history of recurrent pulmonary infections is found to have a congenital deficiency of adenosine deaminase, which is associated with a viual absence of lymphocytes in her peripheral lymphoid organs. What is the appropriate diagnosis?
Correct Answer: Severe combined immunodeficiency
Description: SCID Group of disorders of T and B lymphocytes Characterized by recurrent viral, bacterial, fungal, and protozoal infections. Many infants with SCID have severely reduced volumes of lymphoid tissue and an immature thymus that lacks lymphocytes. In some patients, lymphocytes fail to develop beyond pre-B cells and pre-T cells. ADA deficiency causes the accumulation of intermediate products, which inhibit DNA synthesis and are toxic to lymphocytes. These children cannot survive beyond early infancy unless they are raised in a sterile environment ("bubble children"). None of the other choices are associated with ADA deficiency. Diagnosis: Severe combined immunodeficiency
Category:
Pathology
Get More
Subject Mock Tests
Practice with over 200,000 questions from various medical subjects and improve your knowledge.
Attempt a mock test nowMock Exam
Take an exam with 100 random questions selected from all subjects to test your knowledge.
Coming SoonGet More
Subject Mock Tests
Try practicing mock tests with over 200,000 questions from various medical subjects.
Attempt a mock test now