NEZELOF SYNDROME IN A TERTIARY HOSPITAL, A CASE REPORTJournal: International journal of ecosystems and ecology science (IJEES) (Vol.6, No. 2)
Publication Date: 2016-05-04
Authors : Aida Bushati; Aferdita Tako; Robert Rado; Migena Kika;
Page : 239-242
Keywords : Nezelof's syndrome; immunodeficiency disease; congenital;
Nezelof's syndrome is a congenital immunodeficiency disease. It is due to underdevelopment of thymus gland. Thymus gland is a lymphoid organ situated behind the sternum, in the superior mediastinum. It forms an important organ of adaptive immune system. Thymus produces T cells [T lymphocytes] and B cells [B lymphocytes] which form integral part of adaptive immunity. Nezelof's syndrome is caused as a result of underdevelopment of thymus gland. The condition produces several infections. Malignancies occur. Chronic pulmonary infections occur. Several other infections include oral and cutaneous candidiasis, chronic diarrhea, skin infection, urinary tract infection, gram-negative sepsis, severe progressive varicella infection. Medical history by the patient and Clinical examination by the doctor helps in diagnosis. Several blood tests are required to be done to detect T cell, B cell count. Genetic testing is recommended. Imaging studies such as X ray, USG, CT scan, and MRI may be useful for the evaluation of associated abnormalities.No specific treatment is available. Antimicrobial therapy is helpful. Immunoglobulin therapy is also sometimes helpful which consists of receiving antibodies, or immunoglobulins from a donor's plasma. Treatment involves transplant of thymus tissue from a donor. Bone marrow transplant may be required. Recurrent infections should be treated symptomatically. Complications such as pulmonary infections, malignancies, less life expectancy can occur.
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