Thoracic Surgery in Chronic Granulomatous Disease: a 25-Year Single-Institution Experience.
Thoracic Surgery in Chronic Granulomatous Disease: a 25-Year Single-Institution Experience.
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DOI：
10.1007/s10875-016-0319-9
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发表时间：
2016-10
影响因子：
9.1
通讯作者：
Ripley RT
中科院分区：
文献类型：
--
作者：
Feingold PL;Quadri HS;Steinberg SM;Malech HL;Gallin JI;Zerbe CS;Zarember KA;Marciano BE;Holland SM;Schrump DS;Ripley RT
慢性肉芽肿病（CGD）是一种遗传性疾病，其中吞噬细胞功能障碍导致反复感染。持续性肺部感染有时需要胸外科手术干预。我们回顾了我们25年的经验，以确定与这些患者胸外科手术相关的结局和预后因素。对1990年至2015年的所有CGD患者进行了回顾性单机构审查。单变量分析确定预后变量，包括在一个考克斯模型。采用Kaplan-Meier法估计总生存期。我们确定了258例患者，共2221例入院（包括计划和紧急）。在检查期间，13.6%（35/258）的患者和2.3%（35/2221）的总入院人数进行了51次胸部手术。与不需要手术的患者相比，接受手术的患者在疾病基因型方面没有统计学显著差异。从67%（34/51）的标本中鉴定出病原体。并发症发生率为27%（14/51），其中伤口并发症10%（5/51），肺部感染并发症12%（6/51）。30天和90天时的死亡率分别为0和6%（3/51）。5年和10年随访时的总生存概率分别为75%和62%（中位潜在随访时间：16.5年）。接受胸外科手术与死亡风险比增加相关，为3.71（p < 0.0001）。胸壁切除和EBL > 500 mL均为预后不良因素（p < 0.05）。少数CGD患者因抗生素或抗真菌治疗难治性感染需要胸外科手术。接受这些手术的患者有显著的发病率和相对较差的长期生存率，特别是在胸壁切除或大量失血的情况下。
Chronic granulomatous disease (CGD) is a genetic disorder in which phagocyte dysfunction leads to recurrent infection. Persistent pulmonary infections sometimes require thoracic surgical intervention. We reviewed our 25-year experience to identify outcomes and prognostic factors associated with thoracic surgery in these patients. A retrospective single-institution review of all patients with CGD from 1990 through 2015 was performed. Univariate analysis identified prognostic variables to include in a Cox model. Overall survival was estimated by the Kaplan-Meier method. We identified 258 patients who had 2221 admissions (both scheduled and emergent). During the period examined, 51 thoracic operations were performed in 13.6 % (35/258) of patients and 2.3 % (35/2221) of overall admissions. Patients undergoing surgery did not have statistically significant differences in disease genotype compared to those that did not require surgery. Pathogens were identified from 67 % (34/51) of specimens. Complications occurred in 27 % (14/51), including 10 % (5/51) with wound and 12 % (6/51) with pulmonary infections. Mortality at 30 and 90 days was 0 and 6 % (3/51), respectively. Overall survival probabilities were 75 and 62 % at 5- and 10-year follow-up (median potential follow-up: 16.5 years), respectively. Undergoing thoracic surgery was associated with an increased hazard ratio for death of 3.71 (p < 0.0001). Both chest wall resection and EBL > 500 mL were negative prognostic factors (p < 0.05). A minority of CGD patients required thoracic surgery for infections refractory to antibiotic or antifungal therapy. Patients who had these operations had significant morbidity and relatively poor long-term survival, particularly in the cases of chest wall resection or significant blood loss.
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影响因子：
3
作者：
ECKERT, JW;ABRAMSON, SL;BRANDT, ML
通讯作者：
BRANDT, ML
影响因子：
3.5
作者：
MOSKALUK, CA;POGREBNIAK, HW;TRAVIS, WD
通讯作者：
TRAVIS, WD
影响因子：
11.8
作者：
Marciano, Beatriz E.;Spalding, Christine;Holland, Steven M.
通讯作者：
Holland, Steven M.
影响因子：
20.3
作者：
Matute, Juan D.;Arias, Andres A.;Dinauer, Mary C.
通讯作者：
Dinauer, Mary C.
影响因子：
3.7
作者：
van den Berg JM;van Koppen E;Ahlin A;Belohradsky BH;Bernatowska E;Corbeel L;Español T;Fischer A;Kurenko-Deptuch M;Mouy R;Petropoulou T;Roesler J;Seger R;Stasia MJ;Valerius NH;Weening RS;Wolach B;Roos D;Kuijpers TW
通讯作者：
Kuijpers TW