Chronic Granulomatous Disease: the Experience of Diagnosis and Treatment in Children

• Main Authors: L.I. Chernyshova, A.P. Volokha, A.V. Bondarenko, A.M. Gilfanova, V.P. Chernyshov
• Format: Article
• Language: English
• Published: Publishing House Zaslavsky 2013-05-01
• Series: Zdorovʹe Rebenka
• Subjects: chronic granulomatous disease; primary immune deficiency; children
• Online Access: http://childshealth.zaslavsky.com.ua/article/view/89801
• ISSN: 2224-0551; 2307-1168

Chronic granulomatous disease — primary immunodeficiency with X-linked and autosomal recessive inheritance, characterized by impaired bactericidal function of phagocytic immune system. Chronic granulomatous disease is accompanied by the development of severe bacterial and fungal infections of the skin, lymph nodes, lungs, liver, and excessive inflammation with granuloma formation. For the first time in Ukraine the paper describes cases of chronic granulomatous disease in children with a characteristic clinical phenotype in the form of severe bacterial infections with the lesion of the different organs and systems, the manifestation in the first months of life. We should note patients’ predisposition to mycobacterial infection, caused both by typical Mycobacterium tuberculosis, and atypical mycobacteria, including Mycobacterium bovis, BCG strain. Now the question of early diagnosis and optimization of treatment of chronic granulomatous disease in Ukraine remaines extremely topical.