早产儿脑室周围白质软化研究的进展

脑室周围白质软化（periventricular leucomalacia,PVL）是一种较常见的脑损伤形式,早产儿发生率高。1867年Parrot和Virchow等20首先记载了本症。这种脑白质的发育损伤可导致脑瘫,主要是痉挛性双下肢瘫、四肢瘫（尤以下肢为重）,智力落后,抽搐,以及眼的各种异常（如眼球震颤、斜视、视力降低等）,对新生儿尤其早产儿的远期预后产生不良影响,已被围生、新生儿学领域多项研究结果所证实。在发达国家,随着新生儿重症监护病房（NICU）的建立和发展,早产儿存活率增加,PVL引起的脑瘫发病率明显增高,严重影响了人类生活质量。故不同程度的早产儿脑白质病变日益受到重视。     

Early echographic findings in non-hemorrhagic periventricular leukomalacia of the premature infant

The ultrasonic detection of periventricular leukomalacia is described in two neonatal patients. Confirmation of this condition by CT and post mortem was obtained in one case. CT scan and further echography was positive in the other. Neither patient had evidence of an intracranial hemorrhage. It is suggested that echography is of value in the early diagnosis of this lesion which may be similar to intraparenchymal hemorrhage in its mechanism and prognosis.     

早产儿脑室周围白质软化的研究进展

在新生儿医学所有问题中 ,早产儿脑损伤及其预防尤为重要。早产儿脑损伤包括脑室周围白质软化 (PVL)、胚胎生发层基质 脑室内出血 (IVH)、出血后脑积水及其他一些脑损伤 ,如选择性神经元坏死及基底核丘脑损伤等 ,其中PVL和IVH最为重要。但IVH发生率近年来渐呈下降趋势 ,因而PVL已上升为早产儿脑损伤的最主要类型。PVL在早产儿的发生率约为 8%～ 2 6% ,在应用呼吸机的早产儿中 ,其发生率可高达 3 8%～ 60 % [1]。美国每年约出生 <50 0g的早产儿 570 0 0例 ,因受益于NICU的精心救治和护理 ,90 %早产儿可在新生儿期存活。在存活的早…     

Ultrasonic diagnosis of periventricular leukomalacia in premature infants

The ultrasonographic study through the fontanelle of 192 children below 33 weeks of age and surviving after the neonatal period led to the diagnosis of leukomalacia by identifying anechogenic cavities in the periventricular area in 7 cases. Clinical pictures were not characteristic. Important electro-encephalographic changes were constantly found, even before the occurrence of sonographic signs. Although non specific they are still suggestive when associated hemorrhage is not present. With these criteria, diagnosis seems to be correlated with severe neurologic prognosis. Its interest and chronology warrant the extension of the ultrasonographic supervision of severely immature infants up to the end of the second month.     

Hypocarbia and cystic periventricular leukomalacia in premature infants

One hundred sixty seven survivors among very low birthweight infants with a gestational age of less than 35 weeks have been studied prospectively. The purpose of this study was to clarify the relationship of severe prenatal and perinatal complications and hypocarbic alkalosis, defined as a carbon dioxide tension (PaCO2) of less than or equal to 2.67 kPa and a pH of 7.50 or greater during the first 24 hours of life, to cystic periventricular leukomalacia (PVL) depicted by serial cranial ultrasonographic examinations. Complications occurred in 16 infants, five of whom presented with PVL, while eight of 151 infants without complications had PVL. Twenty six of the infants had hypocarbic alkalosis, six with evidence of PVL, and seven of the 136 infants without hypocarbic alkalosis had PVL. These results suggest a significant relationship of complications and hypocarbic alkalosis to PVL. Mechanical ventilation should be managed carefully in premature infants to avoid PaCO2 of lower than 2.67 kPa.     

Hypocarbia and cystic periventricular leukomalacia in premature infants

One hundred sixty seven survivors among very low birthweight infants with a gestational age of less than 35 weeks have been studied prospectively. The purpose of this study was to clarify the relationship of severe prenatal and perinatal complications and hypocarbic alkalosis, defined as a carbon dioxide tension (Paco₂) of less than or equal to 2.67 kPa and a pH of 7.50 or greater during the first 24 hours of life, to cystic periventricular leukomalacia (PVL) depicted by serial cranial ultrasonographic examinations. Complications occurred in 16 infants, five of whom presented with PVL, while eight of 151 infants without complications had PVL. Twenty six of the infants had hypocarbic alkalosis, six with evidence of PVL, and seven of the 136 infants without hypocarbic alkalosis had PVL. These results suggest a significant relationship of complications and hypocarbic alkalosis to PVL. Mechanical ventilation should be managed carefully in premature infants to avoid Paco₂ of lower than 2.67 kPa.     

Markers of oxidative injury in the cerebrospinal fluid of a premature infant with meningitis and periventricular leukomalacia

Free radicals have been hypothesized to play a key role in the evolution of periventricular leukomalacia, although direct evidence of oxidative injury in the human infant is lacking. This case report is the first to demonstrate a marked elevation in the levels of lipid and protein oxidative products in the cerebrospinal fluid during the evolution of periventricular leukomalacia in a premature infant with meningitis.     

早产儿脑室周围白质软化危险因素分析

目的 调查6年来早产新生儿发生脑室周围白质软化(PVL)的危险因素.方法 以2002年8月-2008年11月经头颅B超或MRI诊断为PVL的患儿为研究组,按1:2数量配对抽取入院日期最接近,入院日龄、胎龄、出生体质量相近、诊断为早产儿脑室周围强回声(PVE)的早产儿(PVE组)和无相应神经系统检查异常发现的早产儿(正常对照组)为研究对象,对PVL危险因素进行分析.结果 单因素分析显示,与正常对照组相比,PVL组和PVE组新牛儿呼吸窘迫综合征(NRDS)(50%比12.5%,X2=10.000,P=0.002;32.5%比12.5%,X2=4.588,P=0.032)和肺部合并症(30.0%比5.0%,P=0.013;22.5%比5.0%,X2=5.165,P=0.023)发生率较高;使用呼吸支持频率高(85.0%比27.5%,X2=17.712,P<0.001;52.5%比27.5%,X2=5.208,P=0.022);PVL组呼吸支持频率高于PVE组(85.0%比52.5%,X2=6.065,P=0.014).PVL组24 h内最低PaCO2低于正常对照组[(33.5±7.5)mmHg比(42.0±9.8)mmHg,LSD=8.578,P=0.0031,24 h内最低pH值高于PVE组(7.326±0.129比7.254±0.092,LSD=0.072,P=0.008),颅内出血发生率较PVE组和正常对照组高(30%比5%,P=0.013;30%比2.5%,P=0.004).多因素分析发现,围生期/出生早期感染、呼吸支持、24 h内最低PaCO2和颅内出血是PVL发生的主要高危因素,其OR值(95%CI)分别为5.745(0.778,42.430)、9.370(2.168,40.504)、0.937(0.872,1.007)、1.826(1.083,3.077).结论 早产儿PVL致病因素复杂,围生期/出生早期感染、呼吸支持、24 h内最低PaCO2和颅内出血是重要原因,有待进一步加强围产期保健,提高危重症患儿治疗水平,以减少PVL的发生.     

早产儿脑室周围白质软化20例B超表现及分析

目的　分析早产儿脑室周围白质软化 (PVL)的超声表现以及预后。方法　对入住福建省妇幼保健院新生儿科所有早产儿进行常规床边头颅B超检查,对确诊为PVL的早产儿进行PVL超声图像分析并定期随访。结果　1996年底至 2004年 7月间经常规床边B超检查确诊为PVL的早产儿共 20例,其中 19例初次B超检查时间为(4 20±0 85)d,均在超声中表现为双侧脑室前角、体部或三角部外上方对称性强回声区。1例因故迟至 29日龄进行初次B超检查,显示双侧侧脑室三角部外上方已呈多发小囊腔改变。10例于 (23 6±6 1)d在原回声增强区呈现多个低回声或无回声囊腔,直径范围在 2~19 5mm,其中 2例显示囊腔分别于 41日龄和 67日龄消失。除早期死亡、自动出院及失访 12例外,余 8例患儿中,发生脑瘫 4例,疑似脑瘫 1例,其中 2例伴有智测评分低下;仅 3例 3~7个月龄随访时暂未发现异常。结论　PVL的超声表现十分典型。由于PVL患儿预后不良,对早产儿在生后早期进行常规床边头颅B超检查很有必要。     

Late-onset circulatory dysfunction of premature infants and late-onset periventricular leukomalacia

Background: The sudden appearance of hypotension and oliguria without obvious cause following stable circulation and respiration in preterm infants is frequent in Japan. Such episodes are referred to as late‐onset circulatory dysfunction of premature infants (LCD). Volume expanders and inotropic agents are often ineffective against this condition, whereas i.v. steroids are significantly effective. A major problem is that cystic periventricular leukomalacia (PVL) often develops a few weeks after an episode. The aim of the present study was to clarify the risk factors, including LCD, related to cystic PVL. Methods: A case–control study was performed for preterm infants who were delivered at <33 weeks of gestation and admitted to seven neonatal intensive care units in Japan. Cystic PVL infants were stratified into early‐onset PVL diagnosed within 28 days of age and late‐onset PVL diagnosed after more than 28 days of age. The reported and new risk factors for PVL, for each group of PVL infants, and for all PVL infants, were compared with controls. Results: Thirty‐two infants were diagnosed with cystic PVL (17 early‐onset and 15 late‐onset). All PVL infants significantly differed from controls on Apgar score, number of abortions and pregnancies, intraventricular hemorrhage, and LCD. LCD was diagnosed in 28.1% of both PVL groups compared with 6.3% of controls (P = 0.02). Multivariate analysis demonstrated significant association between late‐onset PVL and LCD. Conclusion: LCD was significantly associated with cystic PVL, especially late‐onset PVL. Elucidating the cause of LCD might reduce the incidence of PVL and improve the neurological prognosis of preterm infants.     

机械通气早产儿脑室周围白质软化危险因素分析

随着新生儿尤其是早产儿急救技术的发展,早产儿脑室周围白质软化(perliventricular leucomalacia,PVL)在胎龄32周以下的早产儿中发生率为5％～15%,而在应用呼吸机治疗的早产儿发生率可高达38％～60％。本文分析因严重肺部疾病而接受呼吸机治疗的早产儿PVL的高危因素,进一步提高对本病的认识,有利于早期干预和及时防止,减少后遗症的发生。     

早产儿脑室周围-脑室内出血与脑室周围白质软化的诊断建议

早产儿常见脑损伤主要为脑室周围．脑室内出血（PVH-IVH）和脑室周围白质软化（PVL）,与早产儿中枢神经系统的解剖生理学和神经生物学发育不成熟密切相关。前者为出血性病变,常导致脑室内出血后脑积水和脑室周围出血性髓静脉梗死等严重并发症。后者为缺血性病变,也与官内感染有关。其中局部PVL的病理特征是白质少突胶质细胞前体的急性坏死,在后期可形成多发小囊腔。弥漫性PVL又称为弥漫性白质损伤,其病理特征是白质少突胶质细胞前体的凋亡性死亡,少见出现囊腔改变。局部和弥漫性PVL最终均导致脑白质容量减小和髓鞘化受损。PVH-IVH和PVL是引起早产儿早期死亡、脑瘫、视、听和认知障碍的主要原因。     

中国人群早产儿脑室周围白质软化危险因素的meta分析

目的探讨中国人群早产儿发生脑室周围白质软化的主要危险因素,为今后防治工作提供依据。方法利用meta分析方法分析国内14篇关于早产儿脑室周围白质软化危险因素的研究文献。累计病例748例,对照3 366例。根据齐性检验结果选择计算各危险因素合并比值比(OR)及其95%可信区间(95%CI)模型。结果脑室周围白质软化发生的OR值(95%CI)分别为:产前使用激素0.46(0.34～0.61),胎膜早破3.60(1.40～9.24),胎龄5.05(2.66～9.58),低出生体质量2.78(2.30～3.35),重度窒息5.35(2.09～13.68),感染4.46(3.08～6.44),机械通气3.67(1.74～7.72),低碳酸血症4.49(2.03～9.94),脑室内出血2.00(1.15～3.45),酸中毒1.58(1.19～2.08)。结论胎膜早破、胎龄、低出生体质量、重度窒息、感染、机械通气、低碳酸血症、脑室内出血和酸中毒是中国人群早产儿脑室周围白质软化发病的主要危险因素,产前使用激素可能为脑室周围白质软化的保护因素。     

我国早产儿脑室周围白质软化发生率的多中心调查报告

目的：在中华医学会儿科分会新生儿学组的发起下，国内十余家大型医院于2005年1月始进行了为期近两年的《早产儿脑损伤》多中心协作研究。该文报告我国10家三级甲等医院近两年对早产儿脑室周围白质软化（PVL）发生率的调查结果。方法：2005年1月至2006年8月期间，各参加单位对所有胎龄<37周的早产儿在生后7 d内常规进行初次床边头颅B超检查，以后每隔3～7 d复查一次，直至出院。结果：10单位共出生或收住早产儿4 933例，总PVL发生率为2.3％（112/4 933），囊性PVL发生率为0.3％（16/4 933）。分别为I级PVL 85.7％（96/112），II级PVL 12.5%（14/112），III级PVL 1.8％（2/112），无IV级PVL。4家妇婴医院的早产儿PVL总发生率非常显著低于6家综合性或儿童专科医院（1.4% vs. 2.8％）（χ2＝10.284，P<0.01）。与发生囊性PVL相关的可能高危因素为阴道分娩和机械呼吸。结论：该调查数据基本可以反映我国主要大城市早产儿PVL发生率的情况。提高对PVL尤其是非囊性脑室周围白质损伤的超声识别率，是今后临床要大力加强的重点     

神经节苷脂和脑活素治疗早产儿脑室周围白质软化的疗效观察

目的探讨应用神经节苷脂(GM-1)和脑活素治疗早产儿脑室周围白质软化的临床疗效。方法将86例确诊为脑室周围白质软化的患儿随机分为三组。Ⅰ组(对照组)27例,采用保暖、吸氧、控制感染、纠正酸碱及电解质紊乱、维持正常血糖、血压、血气等常规治疗;Ⅱ组31例,在常规治疗基础上加用脑活素静脉注射;Ⅲ组28例,在常规治疗基础上加用GM-1静脉注射。定期观察和随诊颅脑B超的变化,进行统计学分析。结果GM-1治疗组治疗有效率优于对照组,差异有统计学意义(P<0.05);脑活素治疗组治疗有效率高于对照组,但差异无统计学意义。结论神经节苷脂是治疗早产儿脑室周围白质软化的有效药物之一,脑活素治疗早产儿脑室周围白质软化的有效性尚待进一步研究。     

The diagnosis of periventricular leukomalacia

During the last trimester of pregnancy the perinatal brain undergoes rapid growth and development and premature delivery exposes the brain to complications of circulatory instability including haemorrhage and ischaemia. The type of cerebral injury sustained depends on gestational age, the nature of the insult and the timing of the event. Although compromise of the cerebral circulation is more likely to occur in the infant born premature, the fetus is also dependent on adequate cerebral perfusion and should this fail the unborn infant is liable to similar neuropathological damage as his prematurely born peer.