nasiri kalmarzy R, sedaghat A, sedaghat B, irandoost G, sayfmanesh M, fattahi N. A 2 months old infant with severe combined immunodeficiency. Zanko J Med Sci 2015; 15 (47) :52-57
URL:
http://zanko.muk.ac.ir/article-1-62-en.html
kurdistan university of medical sciences
Abstract: (4846 Views)
Background: Severe combined immunodeficiency (SCID) is a primary Immunodeficiency syndrome which is created by diverse genetic mutations. It will lead to the absence of all adaptive immune functions in all affected patients and a lack of Natural Killer cells in some cases. Affected infants will almost always die before their second birthday, due to serious and intractable infections. The reasons of these infections are the opportunistic microorganisms which cause mild or self-limited diseases in immunocompetent hosts. If population-based newborn screening programs has not been established in a community (which is the case in many parts of the world), the best test for early diagnosis is the determination of Absolute Lymphocyte Count (ALC) in peripheral blood. These patients are uniformly lymphopenic because normally T-cells constitute 70% of peripheral blood lymphocytes and patients lack or have extremely low number of them. Case report: We present a two month-old male infant with chronic diarrhea, failure to thrive and lymphopenia in which the result of examinations showed a rare type of immunodeficiency in which flow-cytometry of peripheral blood was consistent with T – B+ NK- variant of SCID
Type of Study:
case report |
Subject:
Special Received: 2015/03/9 | Accepted: 2015/03/26 | ePublished: 2015/03/29