Long-term follow-up and outcome of 39 patients with chronic granulomatous disease
Long-term follow-up and outcome of 39 patients with chronic granulomatous disease
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DOI：
10.1067/mpd.2000.109112
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发表时间：
2000-11-01
影响因子：
5.1
通讯作者：
Belobradsky, BH
中科院分区：
文献类型：
--
作者：
Liese, J;Kloos, S;Belobradsky, BH
目的：为了评估慢性肉芽肿病（CGD）患者的临床长期病程与不同的CGD亚型和目前使用的抗菌药物的预防措施。研究设计：39例CCD患者的记录进行了监测，在22年的时间进行了审查。所有的感染，感染性并发症，和临床结果被记录为610患者年的总观察期，并分层相对于不同的CGD subtypes.Results：淋巴结炎，皮肤脓肿，肺炎发生在87%，72%，和59%的患者，分别。在151株微生物分离株中，沙门氏菌和沙门氏菌是最常见的微生物。有167例重度感染需要住院和静脉内抗菌治疗，导致每100患者月（SI/100 PM）发生率为3.7例重度感染。长期抗生素预防治疗显著降低了严重细菌感染的发生率，从4.8 SI/100 PM降至1.6 SI/100 PM（P =.0035）。相比之下，抗生素预防下真菌感染的平均发病率从0.2 SI/100 PM增加到1.9 SI/100 PM（P =.04）。我们发现，在生命的第四个十年中，存活率为50%，在生命的第三个十年后达到平台。与细胞色素B（558）完全缺失的患者表现出更早的疾病表现和更高的感染发生率，并有显着降低生存率比患者只有减少细胞色素B（558）或常染色体隐性cCD.Conclusions：曲霉菌感染已成为感染并发症和死亡的主要原因CGD患者。需要采取预防和治疗措施，以进一步提高CGD患者的预期寿命和质量。
Objectives: To evaluate the clinical long-term course in patients with chronic granulomatous disease (CGD) with respect to different CGD subtypes and currently used antimicrobial prophylactic measures.Study design: The records of 39 patients with CCD who were monitored during a period of 22 years were reviewed. All infections, infectious complications, and clinical outcomes were documented for a total observation period of 610 patient-years and were stratified with respect to different CGD subtypes.Results: Lymphadenitis, skin abscesses, and pneumonia occurred in 87%, 72%, and 59% of the patients, respectively. In 151 microbiologic isolates species, and Salmonella species were the most frequently detected microorganisms. There were 167 severe infections requiring hospitalization and intravenous antimicrobial treatment, resulting in an incidence of 3.7 severe infections per 100 patient months (SI/100 PM). Long-term antibiotic prophylaxis significantly reduced the incidence of severe bacterial infections from 4.8 SI/100 PM to 1.6 SI/100 PM (P = .0035). In contrast, fungal infections increased under antibiotic prophylaxis from a mean incidence of 0.2 SI/100 PM to 1.9 SI/100 PM (P = .04). We found a 50% survival rate through the fourth decade of life, with a plateau after the third decade of life. Patients with a complete absence of cytochrome b(558) Showed an earlier manifestation of their disease and a higher incidence of infections and had significant lower survival than patients with only diminished cytochrome b(558) or autosomal recessive CCD.Conclusions: Infections with Aspergillus species have become the major cause of infectious complications and death in patients with CGD. Prophylactic and therapeutic measures are needed to further increase life expectancy and quality for patients with CGD.