影响左向右分流型先心病新生儿撤机成功的多因素分析

目的研究不同因素对左向右分流型先心病新生儿呼吸机撤离（简称撤机）成功率的影响。方法收集左向右分流型先心病机械通气新生儿95例，定义48h内无需重新插管者属撤机成功。以撤机成功率为因变量，以新生儿胎龄、体重、肺动脉压力水平、肺血流量、缺损大小、缺损类型、合并心衰、肺炎为自变量进行多元线性逐步回归，筛选出可能的影响因素，进一步研究不同影响因素对撤机成功率的影响。结果肺动脉压力、肺血流量大小和缺损类型是影响撤机成功率的重要因素（P均〈0．05）。中重度肺动脉高压组的撤机成功率低于轻度肺动脉高压组（P均〈0．05），差异有统计学意义。高肺血流量组的撤机成功率低于无高肺血流量组（P均〈0．05），差异有统计学意义。联合缺损组的撤机成功率低于单纯缺损组（P均〈0．05），差异有统计学意义。结论肺动脉压、肺血流量、缺损类型是影响撤机成功率的重要因素。     

儿科重症监护病房呼吸机撤离失败的危险因素

目的分析PICU机械通气（MV）患儿撤机失败率及其相关危险因素。方法对2005年1月-2007年6月本院PICU收治的317例MV患儿临床资料行回顾性分析。撤离呼吸机48h内再次予以辅助通气者判定为撤机失败。结果183例按计划撤机。其中166例撤机成功，17例失败，撤机失败率9．29％（95％CI为5．08％～13．50％）。≤6个月患儿撤机失败率较〉6月龄明显升高（13．4％vs4．7％P〈0．05）。撤机前MV时间≤24h者撤机失败风险明显低于〉24h者（3．2％vs12．5％P〈0．05）。撤机失败者总MV时间[（17．8±15．2）dvs（6．0±8．7）d]、住PICU时间[（32．7±22．0）dvs（14．5±17．0）d]及总住院时间[（41．7±23．8）dvs（28．7±25．3）d]与撤机成功者比较均明显延长（Pa〈0．05），死亡率增高（23．5％vs6．0％P〈0．05）。造成撤机失败是多因素的，其中撤机后喉水肿所致最常见。多因素Logistic回归分析显示，并呼吸道狭窄（OR为4．7，95％CI为1．37～16．25）、撤机后吸气性喘鸣发生（OR为5．64，95％CI为1．69～18．84）是预示撤机失败的独立危险因素。中枢神经系统疾病患者易发生撤机延误。结论儿科医师应重视患儿自主呼吸能力的全面评估，对撤机困难者应积极查找潜在的合并症，进一步开展呼吸力学监测、新的智能通气模式，将有助于呼吸机平稳及时撤离。     

有创-无创序贯性通气治疗新生儿呼吸衰竭的临床研究

目的研究新生儿呼吸衰竭患儿的撤机过程中，采用有创一无创序贯性通气能提高撤机的成功率。方法将34例新生儿呼吸衰竭患儿随机分成两组，17例患儿接受有创一无创序贯通气治疗为治疗组，17例患儿接受传统脱机治疗为对照组，比较两组的撤机成功率、总用氧时间。并同时观察两组患儿的呼吸暂停及漏斗胸的患病率。结果治疗组与对照组的撤机成功率分别为94．1％和70．6％（P〈O．05）；总用氧时间分别为（15±6）d和（21±8）d（P〈0．05）；呼吸暂停的患病率分别为5．9％和23．5％（P〈0．05）；漏斗胸的患病率分别为0和17．6％（P〈0．05）。结论使用有创-无创序贯性通气檬吉治疗新生儿呼吸衰竭较传统脱机模式更有效，提高了撤机成功率，并降低了并发症的发生率。     

Ⅱ型呼吸衰竭新生儿高频通气时呼吸力学动态监测的意义

目的探讨Ⅱ型呼吸衰竭新生儿高频通气时呼吸力学特征及其临床意义。方法选择Ⅱ型呼吸衰竭新生儿31例，随机分为观察组16例，对照组15例。观察组采用高频通气治疗并行呼吸力学指标监测，对照组采用同步间歇指令通气进行治疗。观察在高频通气治疗前及治疗后2、12、24、48h及撤机前患儿呼吸系统动态顺应性（C）、呼吸道阻力（R）、肺过度膨胀系数（C20／C）、每分通气量（MV）、平均呼吸道压（MAP）等指标并进行比较。对二组患儿治愈率及病死率进行比较。结果观察组患儿C随疾病的好转逐渐增加，撤机前与治疗前相比有显著性差异（P〈0．01）；R在撤机前明显降低，与治疗前相比有显著性差异（P〈0．01）；MV在撤机前明显升高，与治疗前相比有显著性差异（P〈0．01）；MAP逐渐下降，C、MV及R均与肺部病变的严重程度有关。与对照组相比，观察组治愈率（87．5％）较对照组（80％）高，病死率（6．25％）较对照组（13．3％）低，但统计学分析均无明显差别（P。〉0．05）。结论Ⅱ型呼吸衰竭新生儿高频通气时呼吸力学动态监测可判断肺部病变的严重程度，评估通气策略，掌握撤机时机。高频通气是治疗Ⅱ型呼吸衰竭新生儿故重要手段之一。     

肺保护性通气策略对呼吸窘迫综合征新生儿肺氧合功能的影响

目的探讨肺保护性通气策略在新生儿呼吸窘迫综合征（NRDS）机械通气治疗中对肺氧合功能的影响及其临床应用评价。方法将需进行机械通气治疗的40例NRDS患儿随机分成两组，即保护通气组（PV组）和传统通气组（CV组），每组20例。对两组呼吸机参数、血气分析结果、肺氧合功能指标、并发症及其他临床资料进行比较分析。结果（1）PV组呼吸机参数吸气峰压、平均气道压和氧浓度显著低于CV组（P均〈0．05）；呼气末正压高于CV组（P〈0．01）；两组通气频率（RR）差异无显著性（P〉0．05）。（2）Pv组PaCCh高于CV组（P〈0．01）；pH低于CV组（P〈0．01）；两组Pa02差异无显著性（P〉0．05）。（3）Pv组上机后48h与72h氧合指数明显低于CV组（P〈0．01），动脉血氧分压与肺泡氧分压的比率在机械通气后72h也高于CV组（P〈0．01），PaO2／FiO2在各监测时间段差异无显著性（P〉0．05）。（4）Pv组呼吸机相关性肺损伤的发病率低于CV组（P〈0．05）。（5）两组脑室内出血及动脉导管开放的发病率差异无显著性（P〉0．05）；PV组上机时间均短于CV组（P〈0．01），两组病死率比较差异亦无显著性（P〉0．05）。结论实施肺保护性通气策略可改善肺部气体氧合，维持适当血氧分压，减少并发症，缩短上机时间，降低病死率，提高患儿的生存质量，不增加脑室内出血、动脉导管开放的发病率。     

允许性高碳酸血症通气法治疗急性呼吸窘迫综合征的临床研究

目的 探讨允许性高碳酸血症通气法（PHV）治疗急性呼吸窘迫综合征（ARDS）中的价值。方法 1996年3月～2005年12月收住PICU，符合1994年欧美制订的ARDS诊断标准确诊为ARDS的患儿27例，分为治疗组12例，采用PHV通气，维持PaCO2在45～55mmHg（1mmHg=0．133kPa）之间；对照组15例，常规通气方式。监测机械通气24、48h的呼吸机参数、血气值和氧合指数（PaO2／FiO2，OI），比较两组机械通气时间、并发症、死亡例数。结果 （1）治疗24、48h后，治疗组PIP、PEEP、MAP值显著低于对照组（P〈0．05）；OI、pH及PaO2差异无显著性（P〉0．05），治疗组PaCO2较对照组显著高（P〈0．01）。（2）治疗组机械通气时间较对照组显著减少（P〈0．01）；对照组5例发生气漏较治疗组差异有显著性（P〈0．05）；两组病死率差异无显著性（25％比26．7％，P〉0．05）。结论 PIV法治疗ARDS较传统通气方式可减低并发症、缩短通气时间，病死率无明显降低。     

危重患儿呼吸衰竭机械通气时呼吸力学动态监测及血气分析评价

目的探讨危重患儿呼吸衰竭机械通气时呼吸力学及血气分析指标动态监测的临床意义。方法将2005年1月-2008年5月本院PICU收治的机械通气〉24h的64例危重患儿分为肺源性疾病组（44例）及非肺源性疾病组（20例）。应用Evita 4呼吸机进行呼吸系统动态顺应性（Crs）、呼吸道阻力（Raw）、平均呼吸道压（MAP）、每分钟通气量（MV）、氧合指数（OI）、肺内分流量与心排出量的比值（Qs／Qt）、呼吸指数（RI）等呼吸力学动态监测及血气分析评价，分析其特征及临床意义。结果肺源性疾病组与非肺源性疾病组患儿Crs、Raw、MAP、MV、OI、Qs／Qt、RI等比较均有显著性差异（Pa〈0．05，0．01）。随着肺源性疾病的恢复，上述指标均发生不同程度的变化，差异有显著性意义（Pa〈0．05，0．01）。Crs与OI呈显著负相关（r=-0．872P〈0．01）。肺源性疾病组患儿机械通气时间较非肺源性疾病组长，差异有显著性意义（P〈0．05）。结论机械通气时动态监测患儿呼吸力学指标变化，可进一步反映危重患儿疾病的病理生理过程，血气分析与呼吸衰竭的严重程度一致，可以判断肺部病变的严重程度和呼吸循环系统的并发症，评估通气策略，确定撤机时机，指导治疗，增加机械通气的安全性，减少呼吸机治疗并发症。     

早产儿有创机械通气初次撤机失败相关危险因素分析

目的 研究早产儿有创机械通气初次撤机失败相关危险因素及不良预后。方法 回顾性纳入北京大学第三医院新生儿重症监护室收治的生后72 h内有创机械通气的早产儿,根据初次撤机后72 h内是否需要再插管分为撤机成功组和撤机失败组,分析初次撤机失败相关危险因素及不良预后。结果 共纳入282例早产儿,其中撤机失败组43例（15.2%）。撤机失败组胎龄、出生体重均低于撤机成功组（P < 0.05）,撤机失败组产房内插管率、动脉导管未闭（patent ductus arteriosus,PDA;内径≥2.5 mm）比例高于撤机成功组（P < 0.05）。撤机前应用≥2种血管活性药物（OR=2.48,95%CI：1.22~5.03）、PDA（≥2.5 mm）（OR=4.54,95%CI：2.02~10.24）为撤机失败的危险因素（P < 0.05）。撤机失败组患儿呼吸机相关性肺炎、中-重度支气管肺发育不良、败血症的发生率均高于撤机成功组（P < 0.05）,撤机失败组吸氧时间、住院时间长于撤机成功组（P < 0.05）。结论 撤机前使用≥2种血管活性药物、PDA（≥2.5 mm）是撤机失败的主要危险因素,撤机失败可能与住院早产儿不良结局有关。     

影响左向右分流型先心病新生儿撤机成功的多因素分析

目的研究不同因素对左向右分流型先心病新生儿呼吸机撤离(简称撤机)成功率的影响。方法收集左向右分流型先心病机械通气新生儿95例,定义48 h内无需重新插管者属撤机成功。以撤机成功率为因变量,以新生儿胎龄、体重、肺动脉压力水平、肺血流量、缺损大小、缺损类型、合并心衰、肺炎为自变量进行多元线性逐步回归,筛选出可能的影响因素,进一步研究不同影响因素对撤机成功率的影响。结果肺动脉压力、肺血流量大小和缺损类型是影响撤机成功率的重要因素(P均<0．05)。中重度肺动脉高压组的撤机成功率低于轻度肺动脉高压组(P均<0．05),差异有统计学意义。高肺血流量组的撤机成功率低于无高肺血流量组(P均<0．05),差异有统计学意义。联合缺损组的撤机成功率低于单纯缺损组(P均<0．05),差异有统计学意义。结论肺动脉压、肺血流量、缺损类型是影响撤机成功率的重要因素。     

儿童呼吸机撤离失败相关因素分析

目的 分析机械通气患儿呼吸机撤离失败率及失败的相关因素.方法 回顾分析我院2005年1月至2008年12月内科重症监护室收治的机械通气患儿214例,统计撤机失败率,分析撤机失败的相关因素.结果 141例患儿按计划撤机,成功122例,失败19例,撤机失败率13.5%.年龄＜6个月患儿撤机失败率较年龄＞6个月患儿高,但差异无显著性(15.0% vs9.8%,P＞0.05).因肺炎进行机械通气的患儿撤机成功率(78/86,90.7%)最高.机械通气时间对撤机成功与否无影响.撤机后皮质激素应用时间成功组短于失败组,差异有显著性[(12.35±9.69)h vs(18.63±12.17)h,P＜0.05).多元线性逐步回归(R2=0.093)分析提示呼吸道梗阻是撤机失败的高危因素(F=14.256,P＜0.001).结论 目前PICU中机械通气患儿的撤机问题还依赖于临床医生的经验和客观指标相结合,探索合理可行的撤机方案,尽早发现和去除引起撤机失败的因素是提高撤机成功率的关键.     

Recent advances in the treatment of congestive heart failure

Treatment of heart failure should include correction of the underlying cause. These causes include large left to right shunts, obstructive lesions, arrhythmias, primary myocardial disease etc. The main pharmacological therapy includes inotropic agents, vasodilators and diuretics. Inotropic agents increase myocardial contractility and include digoxin, intravenous dopamine, dobutamine and isoproterenol. Vasodilators improve cardiac pump performance by decreasing the vascular resistance and/or increasing the venous capacitance. Commonly used vasodilator agents include angiotensin converting enzyme inhibitors (captopril, enalapril etc.), hydralazine, prazosin hydrochloride etc. Diuretics inhibit salt and water reabsorption promoting their excretion. Furosemide, thiazide diuretics, aldactone, are commonly used diuretics. Electrolyte and acid-base imbalance can occur on chronic diuretic therapy. Cardiac transplantation is considered for patients where all medical management has failed.     

Children with chronic organ failure possibly ending in organ transplantation: a survey in an Italian region of 5,000,000 inhabitants

Aim: The Italian Piedmont region sponsored in 2005 a population-based registry to assess the epidemiology of childhood chronic organ failure involving kidneys, liver, heart or lungs.
Methods: Patients in chronic organ failure who were younger than 18 years were selected, and entered the registry when accomplishing the standard failure criteria for each organ. The cases were reported by the general paediatricians of the region and integrated with the data gathered by the Children University Hospital, a tertiary care centre.
Results: In Piedmont (647 727 inhabitants < 18 years), a total of 146 children (217 cases per million of paediatric population) were found to be affected by chronic organ failure (mean age 10 years; range 0–17). The organ failure involved kidneys in 68 subjects (48%), liver in 24 (17%), heart in 21 (15%) and lungs in 28 (20%), and was severe in 32 subjects (6 on transplantation waiting list). The most represented disease leading to chronic renal failure was renal hypodysplasia (79%). Chronic liver failure was mostly caused by biliary atresia (30%), autoimmune hepatitis (25%) and Wilson's disease (21%). Dilated cardiomyopathy (62%) and surgically treated congenital cardiopathy were the two leading causes of chronic heart failure. The most represented disease leading to chronic lung failure was cystic fibrosis (89%).
Conclusion: This is the first report of the literature focusing on the epidemiology of chronic organ failure in children encompassing a region of 4 000 000 inhabitants. This clinical condition is rare, but medically and socially very demanding not only in childhood but the life along, as most of these patients will need solid organ transplantation decades later.     

苯那普利用于儿童抗心力衰竭治疗的临床研究

目的 探讨心力衰竭（简称心衰）患儿血浆内皮素（ＥＴ）和血管紧张素Ⅱ（ＡｎｇⅡ）的变化。并观察苯那普利对心衰串儿的疗效。方法 ４０例充血性心力衰竭（ＣＨＦ）患儿中坚持药物疗程的３５例，分为苯那普利组（２０例）和常规组（１５例）用放射免疫法测定血浆ＥＴ和ＡｎｇⅡ，ｐｅｒｃｏｌｌ密度梯度离心法测定循环内皮细胞（ＣＥＣ），比色法测定血管紧张素转换酶（ＡＣＥ）。常规组用常规抗心衰治疗（洋地黄，利尿剂及血管扩     

机械通气治疗新生儿呼吸衰竭相关措施探讨

为探讨改进措施改善呼吸机治疗新生儿呼吸衰竭(简称呼衰)的预后,将经呼吸机治疗的265例呼衰分为3组,以1985年～1989年57例为甲组,1990年～1995年107例为乙组,1996年～2000年101例为两组作对比。3组的基本情况(性别、胎龄、入院体重、异常分娩史)比较,差异无显著性,P均>0.05。结果显示治愈率甲乙丙3组各为28.1％、52.3％和97.0％,病死率各为63.1％、17.8％和0％(x~2各为86.2,89.4,P均<0.01);在合并症中插管不当、肺气压伤、颅内出血、酸碱紊乱、肺不张、感染加重等,丙组的合并症明显低于甲、乙组,P均<0.01。丙组除做好呼吸管理外,加强对心、脑、肾、循环、胃肠功能受损的防治,使预后得以改善。     

Subacute hepatic failure after the perinatal period with haemochromatotic siderosis at the age of 11 months: an unusual perspective on neonatal haemochromatosis

Neonatal haemochromatosis is a rare disease characterized by the early onset of liver failure, a rapidly deteriorating clinical course and high mortality. An unusual case is presented of subacute hepatic failure in which autopsy findings at the age of 11 mo showed a haemochromatotic pattern of siderosis. Despite the similarity in the pattern of iron distribution to neonatal haemochromatosis, the clinical course was highly atypical in that the infant had later and milder presentation at 1 mo of age and slow progression into liver failure. This case illustrates the heterogeneous nature of the haemochromatotic phenotype in newborns and infants. CONCLUSION: The entity "neonatal haemochromatosis" may reflect a spectrum of diseases with diverse aetiologies. Fulminant liver disease in the newborn period can also result in similar pattern of iron distribution.     

A Prospective Evaluation of Nesiritide in the Treatment of Pediatric Heart Failure

This study sought to determine the potential of recombinant B-type natriuretic peptide (nesiritide) for the treatment of pediatric decompensated heart failure. Nesiritide is a widely used and effective treatment for decompensated heart failure (HF) in adults, but its safety and efficacy in pediatric patients is unclear. Outcomes of 55 separate nesiritide infusions of varying durations in 32 patients (13 males and 19 females; mean age, 8.01 years; range, 0.01–20.4) were evaluated prospectively. All patients received nesiritide in the intensive care unit. The starting dose (0.01 μg/kg/min) was titrated to a maximum of 0.03 μg/kg/min. All patients were monitored for clinical signs and symptoms, hemodynamics, urine output, electrolytes, oxygen requirements, and oral intake. Functional status was assessed by patients and/or their parents. All patients successfully underwent initiation and titration of nesiritide infusion. No hypotension or arrhythmias were noted during 478 cumulative days of therapy. Nesiritide was given safely with vasoactive medications. Mean urine output improved from 2.35 ± 1.71 cc/kg/hr on the day before nesiritide initiation (baseline) to 3.10 ± 1.94 cc/kg/hr on day 4 of treatment (p < 0.01). Serum creatinine decreased from 1.04 to 0.92 mg/dl (p = 0.096), mean central venous pressure from 13 to 7 mmHg (p = 0.018), and mean weight from 30.4 to 29.7 kg (p < 0.001) with therapy. Thirst, as subjectively assessed by patients old enough to respond, decreased with infusion in 31 of 42 cases (74%). Mean New York Heart Association functional class improved significantly (p < 0.001). Nesiritide infusion, alone or in combination, is a safe treatment for decompensated HF in pediatric patients. It is associated with decreased thirst and improved urine output and functional status, and it may be efficacious in the treatment of pediatric HF.     

肝功能衰竭患者体外替代与支持治疗

肝功能衰竭患者病死率极高,虽然肝脏移植被认为是最有效的治疗方法,但由于供体有限和价格昂贵,使其应用受到限制.目前人工肝仍是肝功能衰竭患者除内科综合治疗外的惟一选择.本文就肝功能衰竭患者体外替代与支持治疗作简要综述.

Abstract：

The patients with liver failure have a high risk of death.Liver transplant is known as the most curative therapy for those patients,but the transplant operations are often restricted because of the donor deficiency and high medical cost.Now artificial liver is still the best choice to treat the liver failure.In the article,the research progress on the replacement and the support therapy in the patients with liver failure is reviewed.     

肺炎患儿粒细胞-巨噬细胞集落刺激因子水平的初步探讨

目的 探讨粒细胞-巨噬细胞集落刺激因子(GM-CSF)在肺炎发生发展中的作用。方法 采用放射免疫法检测30例轻症肺炎、20例重症肺炎伴心力衰竭患儿及25例正常健康儿血浆中GM-CSF水平。结果 肺炎轻症组GM-CSF浓度为12.79±2.02 pg/ml,肺炎重症组为9.14±2.39 pg/ml,均较对照组 (7.59±3.13pg/ml) 明显增高,差异有显著意义 (P＜0.01,P＜0.05＝。结论 GM-CSF在肺炎的发生发展中可能起着一种免疫防御因子的作用。     

Current status of liver transplantation

Liver transplantation is accepted therapy for acute or chronic liver failure. Survival after LT has improved significantly in developed countries and this has increased the awareness of this treatment modality in the developing world. Successful LT in both children and adults have now been reported from India. Chronic liver failure secondary to cholestatic liver disease in the most frequent indication for LT, with biliary with atresia as the single commonest cause. Innovative techniques such as reduced size, splint, and living donor liver transplantation are being applied more often to decrease long waiting times and reduce associated morbidity and mortality. Early postoperative complications include primary graft failure, venous thrombosis, rejection, biliary complications and infections. Late complication includes CMV or EBV infections, side effects of immunosuppression, post transplantation lymphoproliferative disease and late biliary strictures. Most children achieve good quality of life. There are still many lessons to learn and there are future challenges such as the ever increasing problems of donor scarcity and the search for potent but less toxic immunosuppressive agents.