PICU血液肿瘤患儿粒细胞减少症伴脓毒性休克的临床特点与预后

Characteristics and outcomes of septic shock in onco-hematological malignancy children with granulocy-topenia in the pediatric intensive care unit

目的探讨PICU住院的血液肿瘤患儿化疗后骨髓抑制期或再生障碍性贫血（骨髓增生低下）状态下粒细胞减少症并发脓毒性休克的临床特点及影响预后的因素。方法对我院2002年1月至2008年5月PICU收治的4JD例血液肿瘤患儿粒细胞减少症并发脓毒性休克临床资料进行回顾性分析。结果本组40例患儿，存活12例，死亡18例，放弃治疗10例，病死率60％（剔除放弃病例）。在发生脓毒性休克时全部病例体温均显著升高（〉38．5℃），C反应蛋白也明显升高。肺部感染是其主要病因（35％），其次为胃肠道感染（30％）。本组血培养阳性结果20例，其中革兰阴性杆菌14例（14／20，70％），铜绿假单胞菌为首位（8／14，57％）。存活组与死亡组血糖、血浆白蛋白水平、血pH值、标准碱剩余、乳酸和小儿死亡危险评分比较，差异均有显著性（P〈0．05）。原发病缓解（或复发后缓解）与未缓解（或复发）患儿伴发脓毒性休克病死率分别为41％、85％，差异有显著性（P〈0．05）。脓毒性休克伴1个、2个、3个、3个以上器官功能不全，病死率分别为0、27％、89％、100％（剔除放弃病例），差异有显著性（P〈0．05）。结论血液肿瘤化疗后骨髓抑制或骨髓增生低下的粒细胞减少症患儿并发脓毒性休克病死率高。高热是患儿主要临床表现；肺和胃肠道是脓毒症重要感染来源。革兰阴性杆菌（尤其是铜绿假单胞菌）是主要致病菌；在早期经验性抗感染治疗中，需加强对革兰阴性杆菌的治疗力度。C反应蛋白增高有利于早期判断脓毒症。患儿血糖、血浆白蛋白水平、血pH值、标准碱剩余、乳酸和小儿死亡危险评分与脓毒性休克死亡相关。积极治疗原发疾病，早期及正确治疗脓毒性休克能降低血液肿瘤骨髓抑制或增生低下的粒细胞减少症伴脓毒性休克患儿的病死率。

Objective To describe the characteristics and prognostic factors of septic shock in onco- hematological malignancy children with granulocytopenia, admitted to pediatric intensive care unit （PICU）. Methods Retrospective cohort study was conducted among septic shock children with hematological cancer and granulocytopenia （ n = 40） admitted to PICU between January 2002 and May 2008. All the patients were in bone marrow inhibition phase with malignancy post-chemotherapy or had bone marrow hypoplasia. Results Eighteen out of 40 patients died （60%） and twelve survived,ten quitted. All patients were significantly fe- brile （ 〉38.5 ℃） when they had septic shock; C-reaction protein （CRP） was significantly elevated. The main causes were pulmonary infections （35%） ,gastrointestinal tract infections （30%）. Twenty patients had positive blood culture results, among whom 14 were Gram-negative bacilli infections （14/20,70%） and pseudomonas aeruginosa was the main pathogen （8/14,57%）. There were significant differences in serum glucose, serum albumin level, pH, standard base excess（ SBE）, serum lactic acid level and the Pediatric Risk Index Score for Mortality Ⅲ （PRISM Ⅲ） between the survival group and the death group（P 〈0. 05）. The mortality were 41% and 85% in patients with septic shock between primary remission or secondary remission group and relapse or non-remission group, and there was significantly difference （ P 〈 0. 05 ）. For the septic shock with dysfunction of 1,2,3 ,or more organs,mortality were 0,27% ,89%, 100% （excluded the quitted cases）, and there was significantly difference （ P 〈 0. 05 ）. Conclusion For granulocytopenia children who were in bone marrow inhibition phase with malignancy post-chemotherapy or had bone marrow hypoplasia, there was an increase in mortality if complicated with septic shock. High fever was the main clinical manifestation;pulmonary and gastrointestinal tract are the main infection foci. Gram-negative bacteria （especially pseudomonas aeruginosa） was the main pathogen; so we suggested enhance the early empirically antibiotic therapy ,especially preventive therapy for anti-gram-negative pathogens. The elevation of CRP was helpful in the early diagnosis of sepsis. Serum glucose, serum albumin level, pH, SBE, lactic acid level, and PRISM m were significantly associated with the mortality of septic shock. Aggressively enhancing the primary diseases therapies, early recognition and proper treatment of septic shock would decrease the mortality of granulocytopenia children complicated with septic shock, who were in bone marrow inhibition phase with malignancy post-chemotherapy or bone marrow hypoplasia.