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Feature Article Children with Congenital Disorders of The Phagocytic System 先天性吞噬系統疾病 Abstract Patients with congenital disorders of the phagocytic system have served as "nature's own experiments" demonstrating the importance of specific functions of phagocytic cells for host defense against bacterial and fungal infections. Patients with Leukocyte Adherence Deficiency (LAD) stimulated research on surface receptors involved in margination and diapedesis of leukocytes from the circulation into tissues. Patients with Chronic Granulomatous Disease (CGD), who are unusually susceptible to serious infections from catalase positive bacteria and fungi, revealed the importance of a respiratory oxidative response of phagocytes. We have learned that products of oxidative metabolism are essential for efficient intracellular killing of microbes by neutrophils and macrophages. Patients with Job's Syndrome suffer recurrent infections related to dysfunction of neutrophils which may be secondary to cytokine dysregulation. These patients have taught us the importance of immunologic "discipline" for normal function. Patients described in this review with congenital disorders of the phagocytic system may be rare, but the lessons they provide are applicable to many more patients with acquired or transitory disorders of phagocytes. We continue to be amazed by this remarkably complex and essential host-defense system. 先天性吞噬系統疾病患者就像天生的試驗系統顯示出該系統對細菌和真細菌感染的特異性防禦作用。罹患白細胞黏附功能低下的患兒激發了這方面的研究,在白細胞從血液循環進入周圍組織的過程中,表面受體與白細胞向血管壁遷移及滲出有關。慢性肉芽腫患兒對過氧化氫酶陽性細菌和真菌易感,而且病情嚴重。這顯示出呼吸氧化反應在吞噬細胞中的重要性。我們已經知道氧化代謝的產物對白細胞和單核細胞有效地去除微生物是非常必要的。Job's綜合徵病兒出現反復感染就與白細胞功能紊亂有關,這種紊亂可能是繼發於細胞因子的失調。這些病人使我們意識到正常功能的維持有賴於正常的免疫。本綜述所涉及的有關吞噬系統先天性疾病可能屬少見病,但從中獲得的經驗卻可運用於其他獲得性或暫時性吞噬細胞疾病。我們仍然被如此錯綜複雜而又非常重要的宿主防禦系統迷住了。 Keyword : Chronic granulomatous disease; Cyclic neutropenia; Leukocyte adhesion deficiency; Job's syndrome; Polymorphonuclear leukocytes
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