Shigella sonnei meningitis due to interleukin-1 receptor-associated kinase-4 deficiency:: First association with a primary immune deficiency
Shigella sonnei meningitis due to interleukin-1 receptor-associated kinase-4 deficiency:: First association with a primary immune deficiency
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DOI：
10.1086/428733
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发表时间：
2005-05-01
影响因子：
11.8
通讯作者：
Casanova, JL
中科院分区：
文献类型：
--
作者：
Chapel, H;Puel, A;Casanova, JL
背景遗传性白细胞介素-1受体相关激酶-4（IRAK-4）缺乏症是最近描述的一种与化脓性细菌感染和不良炎症反应相关的免疫缺陷。宋内志贺菌通常与发达国家的直肠结肠炎暴发有关，但偶尔也有系统性疾病的报道。在此之前，在此类病例中尚未发现潜在的原发性免疫缺陷。我们报告了一个IRAK-4缺乏症患者的临床和免疫学特征，该患者除了其他感染外，还有全身性志贺菌病病史。该患者在儿童时期有金黄色葡萄球菌、肺炎链球菌和铜绿假单胞菌感染史，并有一次S。在10岁时患索内氏败血症和脑膜炎。该患者的病史与同时感染同一病原体的其他患者的病史形成对照。值得注意的是，在我们的患者中，这些发作并不伴有急性期反应。随后，患者出现更多葡萄球菌疾病发作，但无全身性疾病。患者现年30岁，自4年前停止预防性抗生素治疗以来一直表现良好。据我们所知，这是第一例原发性免疫缺陷患者发生系统性志贺菌病的报告，扩大了与IRAK-4缺陷相关的感染谱。因此，由IRAK-4介导的免疫似乎对S.肠粘膜中的宋内氏感染。系统性志贺菌病患者应考虑IRAK-4缺乏及相关疾病。
Background. Inherited interleukin-1-receptor-associated kinase-4 (IRAK-4) deficiency is a recently described immunodeficiency associated with pyogenic bacterial infections and a poor inflammatory response. Shigella sonnei is generally associated with outbreaks of rectocolitis in developed countries, but systemic illnesses have occasionally been reported. An underlying primary immunodeficiency has not been found in such cases before now.Methods. We report the clinical and immunological features of a patient with IRAK-4 deficiency who has a history of systemic shigellosis in addition to other infections.Results. The patient has a history of Staphylococcus aureus, Streptococcus pneumoniae, and Pseudomonas aeruginosa infections during childhood and an episode of S. sonnei septicemia and meningitis at 10 years of age. This patient's history contrasted with that of other individuals infected concurrently by the same organism. Of note, these episodes were not accompanied by acute phase responses in our patient. Subsequently, the patient has had more episodes of staphylococcal disease, but no systemic illnesses. The patient is now 30 years old and has been doing well since prophylactic antibiotic treatment was stopped 4 years ago.Discussion. To our knowledge, this is the first report of a case of systemic shigellosis in a person with a primary immunodeficiency, expanding the spectrum of infections associated with IRAK-4 deficiency. Thus, immunity mediated by IRAK-4 seems to be crucial for both the containment of and the inflammatory response to S. sonnei infection in the intestinal mucosa. IRAK-4 deficiency and related disorders should be considered in patients with systemic shigellosis.