Uğur ÖZÇELİK, İlhan TEZCAN, Nural KİPER, Deniz DOĞRU, Ebru YALÇIN

Tuberculosis in children with congenital immunodeficiency syndromes

Konjenital immünyetmezlik sendromları olan hastalar, birçok mikroorganizma ile infeksiyona yatkındırlar. Fakat, bu hastalarda nadiren mikobakteriyel hastalık meydana gelir. Bu yazıda, konjenital immünyetmezliği olan ve tüberküloz hastalığı gelişen çocuklardaki klinik, laboratuvar bulgular ve tedavi sonuçları verilmiştir. On hastanın dosyası gözden geçirildi. Üç hastada kronik granülomatöz hastalık, iki hastada sık değişken immünyetmezlik, diğerlerinde siklik nötropeni, kombine immünyetmezlik, hiperimmünglobulin E sendromu, selektif IgA eksikliği ve X’e bağlı agamaglobulinemi vardı. Sekiz hastada pulmoner tüberküloz, birinde tüberküloz artrit, birinde tüberküloz osteomiyelit saptandı. Aside dirençli basil, iki balgam, bir kemik iliği aspirasyonu ve bir postmortem akciğer nekropsi örneğinde tespit edildi. Bir balgam ve bir eklem sıvısı aspirasyonunda Mycobacterium tuberculosis üretildi. Bir hastaya kemik biyopsisi ile tanı konuldu. Diğer üç hasta, tüberkülin deri testi pozitifliğiyle antibiyotik tedavisine yanıt vermeyen klinik ve radyolojik bulgular nedeniyle tanı aldı. Tüm hastalar antitüberküloz tedavi aldı. Sonuç olarak, Mycobacterium türleri, özellikle endemik bölgelerde yaşayan konjenital immünyetmezliği olan çocuklarda hastalığa yol açan önemli patojenler olabilir.

Konjenital immünyetmezlik sendromu olan çocuklarda tüberküloz

Patients with congenital immunodeficiency (CID) syndromes are susceptible to various microorganisms. However, relatively few CID disorders develop mycobacterial disease. We describe clinical features, laboratory findings and therapeutic outcome of children with CID who had tuberculosis disease. Medical reports of 10 patients were reviewed. Three patients had chronic granulomatous disease, two had common variable immuno deficiency, the others had cyclic neutropenia, combined immunodeficiency, hyperimmunoglobulin E syndrome, selective IgA deficiency and X-linked agammaglobulinemia. Eight patients presented with pulmonary tuberculosis, one had tuberculosis arthritis, one had tuberculosis osteomyelitis. There was acid fast bacilli in sputum of two, bone marrow aspiration in one and postmortem lung biopsy specimen in one patient. Mycobacterium tuberculosis grew in sputum of one and articular fluid aspirate of one patient. One patient was diagnosed with bone biopsy specimens characteristic for tuberculosis. The remaining three patients were diagnosed to have tuberculosis disease as they had positive tuberculin skin test and clinical and radiologic findings unresponsive to non-specific treatment. All patients were treated with antituberculous drugs. Mycobacterium species may be important pathogenes in children with CID, especially in endemic regions.

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