Causes of death in hyper-IgE syndrome
Causes of death in hyper-IgE syndrome
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DOI：
10.1016/j.jaci.2006.12.666
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发表时间：
2007-05-01
影响因子：
14.2
通讯作者：
Holland, Steven M.
中科院分区：
文献类型：
--
作者：
Freeman, Alexandra F.;Kleiner, David E.;Holland, Steven M.
背景资料：高IgE综合征（HIES）的特征是反复化脓性感染、湿疹、血清IgE水平升高以及各种结缔组织和骨骼系统异常。很少有关于这些患者的死亡原因或病理findings.Objective：为了确定死亡的原因与HIES患者，并描述在致命的HIES的病理结果。研究方法：我们回顾了6例HIES患者的医疗记录和尸检切片，尸检在我们institution.Results：所有6例HIES患者均为女性，年龄从24岁到40岁不等。所有患者均有囊性肺病病史，死亡时有肺炎，以铜绿假单胞菌和真菌为主。3例患者出现肺真菌血管侵犯伴致死性出血，2例患者出现由烟曲霉和多产赛多孢子菌引起的脑转移性真菌病。4例患者有肾小管损伤的证据，这可能是从阿替霉素B毒性; 3例患者有肾小球硬化症;和1例患者有2肾angiomyolipoma.Conclusions：我们的系列突出的重要作用，假单胞菌属和曲霉菌属发挥在HIES与囊性肺疾病的患者。强化抗真菌和革兰氏阴性细菌的预防需要评估，作为可能的战略，以防止这些感染并发症的患者与囊性肺diseases.Clinical影响：真菌和假单胞菌感染的囊性肺疾病在HIES可能危及生命，这些感染的适当管理和预防需要继续调查。
Background: Hyper-IgE syndrome (HIES) is characterized by recurrent pyogenic infections, eczema, increased serum IgE levels, and a variety of connective tissue and skeletal system abnormalities. Little has been published regarding the causes of death in these patients or pathologic findings.Objective: To identify the cause of death in patients with HIES and to describe pathologic findings in fatal HIES. Methods: We reviewed the medical records and autopsy slides of 6 patients with HIES with autopsies performed at our institution.Results: All 6 patients with HIES were women and ranged in age from 24 to 40 years. All patients had a history of cystic lung disease and had pneumonia at the time of death, with Pseudomonas aeruginosa and fungal organisms predominating. Pulmonary fungal vascular invasion with fatal hemorrhage was observed in 3 patients, and metastatic fungal disease to the brain was observed in 2 patients caused by Aspergillus fumigatus and Scedosporium prolificans. Four patients had evidence of renal tubular injury, which was likely from amphotericin B toxicity; 3 patients had glomerulosclerosis; and I patient had 2 kidney angiomyolipomas.Conclusions: Our series highlights the important role Pseudomonas and Aspergillus species play in patients with HIES with cystic lung disease. Intensified antifungal and gram-negative bacterial prophylaxis need evaluation as possible strategies to prevent these infectious complications in patients with cystic lung disease.Clinical implications: Fungal and Pseudomonas infection of cystic lung disease in HIES may be life threatening, and the proper management and prevention of these infections need continued investigation.