Rapid Resolution of Multiple Liver Abscesses in a Chronic Granulomatous Disease Patient with Granulocyte Transfusions

Abstract

Chronic granulomatous disease (CGD) is a genetically heterogeneous primary immune deficiency of phagocyte function characterized by recurrent, life-threatening bacterial and fungal infections that lead to granuloma formation. Early diagnosis is possible by the awareness of the clinician about early infectious clues of the disease. Aggressive treatment of infectious complications is very important in CGD patients and subsequently antimicrobial (antibiotic and antifungal) and immunomodulatory (interferon-gamma) prophylaxis until hematopoietic stem cell transplantation. Despite improved mortality, morbidities due to complications associated with CGD remain significant. One of these is a hepatic abscess in CGD patients which is seen in more than one-quarter of patients and also very refractory and frequently requires multiple surgeries with frequent morbidities. Therefore, the most optimal and beneficial treatments are still being investigated in the world. We present a 3 y old CGD patient with multiple liver abscesses due to S.aureus and Aspergillus spp who treated by several percutaneous liver-directed interventional radiological treatment along with granulocyte transfusions

Keywords

• chronic granulomatous disease
• child
• hepatic abcess

Çoklu Karaciğer Apsesi Olan Bir Kronik Granülomatöz Hastasının Granülosit Transfüzyonu İle Hızlı Tedavisi

Abstract

Kronik granülomatöz hastalık (CGD), tekrarlayan, yaşamı tehdit eden bakteri ve mantar enfeksiyonları ile karakterize olan, granülom oluşumuna yol açan genetik olarak heterojen bir primer immün yetmezlik olan fagosit fonksiyon yetersizliğidir. Erken teşhis, klinisyenin hastalığın erken enfeksiyon ipuçları konusunda bilinçlenmesi ile mümkündür. CGD hastalarında Enfeksiyöz komplikasyonların agresif tedavisi çok önemlidir ve hematopoietik kök hücre nakline kadar antimikrobiyal (antibiyotik ve antifungal) ve immünomodülatör (interferon-gama) profilaksileri de çok önemlidir. Mortalitenin azalmasına rağmen, CGD ile ilişkili komplikasyonlara bağlı morbiditeler önemini korumaktadır. Bunlardan biri, CGD hastasında, hastaların dörtte birinden fazlasında görülen ve aynı zamanda çok dirençli ve sıklıkla sık morbiditeli birden fazla ameliyat gerektiren karaciğer apsesidir. Bu nedenle dünyada halen en uygun ve faydalı tedaviler araştırılmaktadır. Granülosit transfüzyonları ile birlikte perkütan karaciğere yönelik girişimsel radyolojik tedavi ile tedavi edilen S.aureus ve Aspergillus spp’ye bağlı çoklu karaciğer apsesi olan 3 yaşında bir CGD hastasını sunuyoruz.

Keywords

• Kronik granulomatöz hastalık
• çocuk
• hepatik abse

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