婴儿捂热综合征与多脏器功能障碍的关系

为探讨婴儿捂热综合征治疗干预时间早晚与多脏器功能障碍 (ＭＯＤＳ)的发生率、病死率的关系 ,对婴儿捂热综合征患儿入院后根据ＭＯＤＳ的诊断标准进行各方面检查并及时进行治疗干预。结果婴儿捂热综合征患儿入院越迟 ,ＭＯＤＳ发生率越高 ,病死率越高。认为对婴儿捂热综合征患儿在临床治疗中要注意心、肺、肝、肾、脑等重要器官的保护 ,及早发现、干预ＭＯＤＳ ,降低死亡率婴儿捂热综合征与多脏器功能障碍的关系$武汉市儿童医院!430016@孙继民     

婴儿捂热综合征血糖与血浆渗透压增高的临床意义

婴儿捂热综合征是儿科的急危重病,可引起机体代谢紊乱和多脏器功能损害。对我院1995年2月~2002年10月收治的17例婴儿捂热综合征患儿血糖及血渗透压的情况进行分析如下。1资料及方法1·1临床资料17例患儿中,男11例、女6例;25d~1个月4例,~3个月9例,~6个月4例。存活13例,死亡4例。     

小儿多器官功能障碍综合征的死亡因素分析

目的探讨小儿多器官功能障碍(MODS)的病因、死亡因素与临床对策。方法统计分析我院135例MODS患儿的原发病、脏器衰竭数及各器官功能障碍分布与病死率的关系。结果135例MODS患儿均存在全身炎症反应综合征(SIRS),以婴儿多见(68.1%),且原发性MODS多于继发性MODS,与成年人不同,病死率为33.3%,以2~3个器官功能障碍占多数(82.2%),其病死率与年龄、性别、感染和非感染因素无关(P>0.05),原发病因以感染性疾病和捂热综合征多见(44.4%、36.3%)。累及最多的脏器是脑、心、肺(84.4%、72.6%、69.6%),而且随着器官功能障碍数目增加,发生率逐渐减少,病死率却逐渐升高。结论SIRS与MODS关系密切,积极控制过度的炎症反应,阻止SIRS向MODS发展,重视对脑、心、肺等重要脏器的维护和支持,减少器官功能衰竭数量,对降低MODS的病死率有着重要的临床意义。     

新生儿细菌性脑膜炎合并低钠血症20例临床探讨

目的探讨新生儿细菌性脑膜炎合并低钠血症的原因及干预方法。方法回顾性分析20例新生儿细菌性脑膜炎合并低钠血症的临床资料。结果患儿均表现为血钠下降，尿钠升高，血浆平均渗透压下降。其中12例有脱水、低血容量表现者，在综合治疗的基础上输液、补钠治疗；8例无脱水、低血容量表现者，予限制液体入量、限钠治疗。全部病例1～3d血钠恢复正常，治愈12例，好转6例，死亡2例。结论新生儿细菌性脑膜炎合并低钠血症系脑性耗盐综合征和抗利尿激素分泌异常综合征所致，治疗应具有针对性。早期纠正低钠血症在脑水肿的治疗中具有重要意义。     

新生儿术后低钠血症与抗利尿激素异常分泌

对１６例新生儿手术前后血钠、血渗透压、尿渗透压及血浆抗利尿激素（ＡＤＨ）监测：①大手术组患儿术后血钠、血渗透压平均值下降，尿钠、尿渗透压平均值升高，７／１０例发生低钠血症。②４／１６例术后出现抗利尿激素异常分泌综合征，其中隔疝２例，膈膨升１例，肠闭锁１例。③大手术组术后血浆ＡＤＨ明显升高。并且结合文献对新生儿术后低钠血症及ＡＤＨ分泌异常进行讨论。     

重型肺炎并发抗利尿激素分泌异常与循环充血的研究

目的　探讨重肺型炎并发抗利尿激素分泌异常对血循环的影响。方法　对４８ 例重型肺炎小儿血、尿电解质、渗透压、抗利尿激素（ＡＤＨ） 及心功能进行了对比研究。结果　１３ 例在肺炎极期出现尿少、体重增加、急性肺水肿表现，同时血钠、血浆渗透压下降，尿钠、尿渗透压升高、血浆ＡＤＨ 增高，而心功能测定正常，为并发抗利尿激素异常分泌综合征（ＳＩＡＤＨ）。经过限水、利尿、补钠治疗，临床症状改善，血、尿指标恢复正常。结论　重型肺炎并发ＳＩＡＤＨ可引起非心源性循环充血症状，易与心力衰竭混淆，临床医师应注意识别。     

危重患儿并高渗血症的病因及处理对策

1998年 9月～ 2 0 0 1年 12月我院ＩＣＵ病房通过检测住院患儿的血渗透压 ,发现 4 6例危重患儿并高渗血症 (ＨＢＳ) ,男 2 9例 ,女 17例。～ 2 8ｄ 13例 ,～ 1岁 18例 ,～ 3岁 11例 ,～ 10岁 4例。原发病为肠炎并Ⅱ～Ⅲ度脱水 16例 ,捂热综合征 10例 ,病毒性脑炎 7例 ,缺氧缺血性脑病 9例 ,糖尿病并急性胃扩张、先天性心脏病术后各 1例 ,脓毒败血症 2例。按全国小儿危重病例评分标准 ,<70分 12例 ,～ 80分 34例。均检测血浆渗透压或一次采血同步测定血电解质离子浓度、血糖和尿素氮 ,计算血渗透压 =1.86× [Ｎａ+ ]+Ｇｌｕ +ＢＵＮ ,血浆渗…     

蒙被缺氧综合征的内环境变化与分析

通过对 1998年 1月～ 2 0 0 1年 12月收治的 4 5例蒙被缺氧综合征患儿检查血气、血生化、血清酶、二氧化碳结合力等发现 ,大多数患儿血钠、钾、糖、血清酶、尿素氮升高 ,血钙、ＣＯ2 ＣＰ降低。其发生机制主要是由于新生儿、小婴儿具有体表面积大、散热快等特点。捂热或保暖过度后 ,机体处于高热状态 ,代偿性的扩张末梢血管 ,通过皮肤蒸发、出汗、呼吸加快促进散热 ,由于代谢加快 ,耗氧增加 ,加上捂闷致呼吸不畅 ,肺呼吸换气功能降低引起低氧和高碳酸血症 ,机体可出现一系列的内环境失衡 ,并发多脏器功能障碍 ,尤其心、脑、肾、血液系统受损。发现此类情况应给予吸氧、降温、保证内环境稳定等治疗。加强预防 ,普及卫生宣教。医务人员应提高对本病的认识 ,及时诊断 ,及时处理 ,提高治愈率 ,减少后遗症。蒙被缺氧综合征的内环境变化与分析$郑州市儿童医院!450053@王群思 $郑州市儿童医院!450053@成怡冰 $郑州市儿童医院!450053@侯新光     

儿童肺炎支原体感染肺外脏器受累56例临床分析

分析肺炎支原体(MP)感染肺外脏器受累的临床特征和治疗结果，采用SERODIA-MYCOⅡ明胶凝集法检测血MP—IgM，对MP-IgM≥1：80且鼻咽部洗液呼吸道病毒检测和呼吸道分泌物细菌培养均为阴性、确诊为MP感染的110例患儿进行回顾性临床分析。结果：①110例患儿均有呼吸道表现，56例(50．9％)肺外脏器受累，其中年长儿51例；受累脏器中以泌尿、消化、血液系统多见，分别为37．5％、35．7％、21．4％，皮肤损害(1．8％)少见；全部病例经大环内酯类抗生素治疗14d左右好转。②实验室检查中：血MP-IgM均≥1：80，痰MP-PCR阳性率28．2％，痰MP培养阳性率20．0％，冷凝集试验阳性率5％。提示MP感染临床特征及胸部X线表现缺乏特异性，且易有肺外脏器受累，尤以肺外并发症为首发症状时，更易造成误诊；对于治疗不理想及多脏器受累的呼吸道感染，应注意有无MP感染可能，及时进行相关检测，以及早确诊，对因治疗。     

婴儿捂热综合征患儿脑血流监护探讨

婴儿捂热综合征多见于1岁以下小儿,是一种人为原因造成的严重水、电解质平衡失调的综合征。本综合征可累及各个脏器,中枢神经系统最易受损,不易恢复,直接影响预后。近年来,随着经颅多普勒(TranscranialDoppler Ultrasound,TCD)在临床的广泛应用,笔者注意对婴儿捂热综合征进行其颅脑血液循环的动态观察,其结果不仅能够协助医生及时判断中枢神经系统受损程度,指导临床治疗,而且对指导其护理有一定意义。     

腹膜透析治疗小儿重症颅脑损伤并严重高钠血症的临床研究

目的：观察腹膜透析对小儿重症颅脑损伤合并严重高钠血症的治疗效果。方法对47例重症颅脑损伤合并严重高钠血症患儿,观察腹膜透析前后血钠浓度变化及其规律,并经有创颅内压监测颅内压变化,评估腹膜透析对颅内压的影响,测定血浆晶体渗透压、血肌酐和血气分析、血压、心率等的变化,评估腹膜透析治疗的效果与安全性。结果与治疗前相比,血钠每天均下降,以第1天下降幅度大,速度快（187．49±2．91 vs 202．48±9．31,P＜0.05）,其后降钠速度减慢。持续颅内压监测颅内压逐渐下降,第1天下降明显（164．58±5．98 vs 177．83±7．47,P＜0.05）。腹膜透析期间,血浆晶体渗透压下降,酸中毒纠正,生命体征稳定。结论腹膜透析可有效治疗重型颅脑损伤后高钠血症,与其弥散、降低颅内压等因素有关,临床安全有效。     

连续血液净化治疗在危重症患儿中的应用

目的 探讨连续血液净化治疗(CBP)在PICU危重患儿中的应用价值.方法 实施CBP治疗患儿18例,其中急性肾功能衰竭14例,行连续静脉-静脉血液滤过(CVVH)治疗;另外2例瑞氏综合征、2例吉兰-巴雷综合征患儿行血浆置换(PE)治疗.观察治疗前后患儿临床症状、血生化、血气指标及氧合的变化情况.结果 14例急性肾功能衰竭患儿CVVH治疗后血清尿素氮(BUN)、肌酐(Scr)明显下降[(48.6±14.8)mmol/L vs(28.9±5.4)mmol/L;(634.3±258.2)μmol/L vs(318.4±143.5)μmol/L],血钾、pH值基本维持正常范围,氧合也有明显的改善.14例患儿中12例肾功能恢复正常.2例吉兰-巴雷综合征患儿经PE治疗后呼吸困难、肌力明显改善,顺利撤离呼吸机.2例瑞氏综合征患儿PE治疗后ALT、AST、血氨明显下降,经包括PE在内的综合抢救治疗后好转出院.CBP治疗并发症:穿刺口渗血3例,体温不升2例.治疗过程中患儿生命体征稳定,未见血压、脉搏异常波动.结论 CBP对急性肝、肾功能衰竭及自身免疫性疾病危重患儿的治疗安全有效.     

Relationship between maternal hemodynamics and hematocrit and hemodynamic effects of isovolemic hemodilution and hemoconcentration in the awake late-pregnant guinea pig

The relationship between the spontaneous hematocrit (Hct) and maternal hemodynamics and the hemodynamic effects of experimentally induced changes in this Hct were studied in the awake late-pregnant guinea pig. In animals with a relatively low Hct, cardiac output fractions and blood flows to brain and heart determined with microspheres were higher and those to kidneys lower than in animals with a relatively high Hct. The O2 flows to the kidneys and skin in the former animals were also lower. Cardiac output, heart rate, systemic blood pressure, and blood flows to the other organs, including the placenta, were not related to the Hct. Placental blood flow was found to vary in proportion to cardiac output. Both hemodilution and hemoconcentration induced changes in the cardiac output distribution and organ flows which resembled those observed in the spontaneous relation with Hct. In addition, hemodilution decreased systemic blood pressure. Both hemodilution and hemoconcentration increased placental blood flow; the magnitude of this flow increase was twice as high after hemodilution. The changes in oxygen flows after experimentally induced hemodilution and hemoconcentration appears to be directly related to the concomitant change in whole blood oxygen capacity. It is concluded that in the awake late-pregnant guinea pig, only brain, heart, and kidney perfusion vary in relation to the arterial Hct. The higher placental blood flow after isovolemic hemodilution may be a result of better blood rheology in the porous-like intervillous space of the placentas in a state of reduced systemic blood pressure. The modest rise in placental blood flow after isovolemic hemoconcentration appears to be mostly related to the methodology employed.     

Colloid osmotic pressure of umbilical cord plasma in healthy and sick newborn infants

Colloid osmotic pressure of umbilical cord plasma was measured in 242 healthy infants, in 34 infants with respiratory distress syndrome (RDS), in 18 infants with asphyxia, in 13 infants who were small for gestational age, in 15 infants born to mothers with diabetes mellitus, and in 18 infants born to mothers with pregnancy-induced hypertension. In healthy infants, colloid osmotic pressure correlated highly significantly with umbilical cord blood total protein level, gestational age, and birth weight. In infants with RDS, no correlation between colloid osmotic pressure and gestational age or birth weight was found. Infants with RDS and gestational age between 36 and 38 weeks had significantly lower colloid osmotic pressure than healthy infants, whereas colloid osmotic pressure of infants with RDS and gestational age between 32 and 35 weeks did not differ from that of healthy infants of corresponding gestational age. Healthy term infants delivered by cesarean section had significantly lower colloid osmotic pressure than infants delivered vaginally. Infants with asphyxia had significantly higher colloid osmotic pressure than healthy infants. Colloid osmotic pressure is related to the lung maturity of the near-term and term neonate. Infants with a colloid osmotic pressure greater than 16 mm Hg are unlikely to develop RDS.     

EDEMA FORMATION IN THE LUNGS AND ITS RELATIONSHIP TO NEONATAL RESPIRATORY DISTRESS

Bland, R. D. (Cardiovascular Research Institute and Department of Pediatrics, University of California, San Francisco, Ca, USA). Edema formation in the lungs and its relationship to neonatal respiratory distress. Acta Paediatr Scand, Suppl. 305: 92–99, 1983.—Pulmonary edema is an important feature of many newborn lung diseases, including respiratory distress from severe perinatal asphyxia, heart failure, hyaline membrane disease, pneumonitis from group B β–hemolytic streptococcus, and chronic lung disease (bronchopulmonary dysplasia). Neonatal pulmonary edema often results from increased filtration pressure in the microcirculation of the lungs. This occurs during sustained hypoxia, in left ventricular failure associated with congenital heart disease or myocardial dysfunction, following excessive intravascular infusions of blood, colloid, fat, or electrolyte solution, and in conditions that increase pulmonary blood flow. Low intravascular protein osmotic pressure from hypoproteinemia may predispose infants to pulmonary edema. Hypoproteinemia is common in infants who are born prematurely. Large intravascular infusions of protein-free fluid further decrease the concentration of protein in plasma and thereby facilitate edema formation. Lymphatic obstruction by air (pulmonary interstitial emphysema) or fibrosis (long-standing lung disease) also may contribute to the development of edema. Bacteremia, endotoxemia. and prolonged oxygen breathing injure the pulmonary microvascular endothelium and cause proteinrich fluid to accumulate in the lungs. The risk of neonatal pulmonary edema can be reduced by several therapeutic measures designed to lessen filtration pressure, increase plasma protein osmotic pressure, and prevent or reduce the severity of lung injury.     

儿童高血压危象的认识与处理

高血压危象指血压显著升高并伴有心脏、肾脏和中枢神经系统等靶器官损伤的重症高血压.儿童高血压危象虽较少见,但常隐匿起病,易被忽视,严重威胁儿童健康.在不同的年龄阶段,具有不同的病因构成,需要密切监测并予以针对性、个体化治疗.初始治疗可以采用动脉血压监测下静脉降压药物,以精确控制血压下降速度,后逐渐改为口服降压药物,达到最大程度的保护靶器官,避免血压波动造成的永久伤害.     

Deficiency of plasmalogens in the cerebro-hepato-renal (Zellweger) syndrome

We have analyzed the phospholipid composition of various organs of patients with the cerebro-hepato-renal (Zellweger) syndrome. The phospholipid composition of tissues from controls and patients was very similar except for their plasmalogen contents. In controls about 50% of the phosphatidylethanolamine fraction of brain, heart, kidney and skeletal muscle and about 10% of that fraction in control liver tissue was found to consist of plasmalogen. In control heart muscle, but not in other control tissues about 25% of the phosphatidylcholine fraction consist of plasmalogens. In contrast, plasmalogens were nearly absent in the corresponding tissues of Zellweger patients.The amount of phosphatidylethanolamine plasmalogens in both erythrocytes and fibroblasts of Zellweger patients is lowered significantly compared to control erythrocytes and control fibroblasts respectively, although this reduction is not as dramatic as in brain, heart, kidney, skeletal muscle and liver of patients. Phosphatidylcholine-plasmalogens are only present in low amounts in both controls, heterozygotes and patients.In recent years considerable evidence has accumulated to show that peroxisomes are involved in cellular lipid metabolism. Notably, the key enzymes of ether lipid (plasmalogen) biosynthesis in rodents were recently found to be located in peroxisomes. Since electronmicroscopic studies have shown that peroxisomes are absent in liver and kidney of patients with the cerebro-hepato-renal syndrome, our results suggest that an inability to integrate these key enzymes in a functional peroxisome leads to a severe disturbance in plasmalogen biosynthesis.We propose that the multiple clinical and biochemical defects in Zellweger patients are secondary to a deficiency in peroxisomal function.     

Posterior reversible encephalopathy syndrome after liver transplantation in children: a rare complication related to calcineurin inhibitor effects

PRES is a neuroclinical and radiological syndrome that results from treatment with calcineurin inhibitor immunosuppressives. Severe hypertension is commonly present, but some patients may be normotensive. We report herein two children who received liver transplants, as treatment for biliary atresia in the first case and for Alagille's syndrome in the second one. In the early postoperative, both patients presented hypertension and seizures. In both cases, the image findings suggested the diagnosis of PRES. The CT scan showed alterations in the posterior area of the brain, and brain MRI demonstrated parietal and occipital areas of high signal intensity. Both children were treated by switching the immunosuppressive regimen and controlling arterial blood pressure. They displayed full recuperation without any neurologic sequelae. Probably, the pathophysiology of PRES results from sparse sympathetic innervation of the vertebrobasilar circulation, which is responsible for supplying blood to the posterior areas of the brain. In conclusion, all liver-transplanted children who present with neurological symptoms PRES should be considered in the differential diagnosis, although this is a rare complication. As treatment, we recommend rigorous control of arterial blood pressure and switching the immunosuppressive regimen.     

纤维支气管镜在新生儿临床应用的安全性分析(附12例报告)

目的评价纤维支气管镜(简称纤支镜)在新生儿呼吸系统疾病临床应用的安全性。方法对12例纤支镜检查新生儿进行前瞻性研究,分析新生儿呼吸系统疾病特征,观察纤支镜检查前后生命体征的变化,评价气管内压力支持通气的作用。结果12例新生儿顺利完成纤支镜检查,其中NICU患儿10例(83%)。11例发生并发症,发生率为92%,全部为明显低氧血症及高碳酸血症,其中合并X线胸片肺部阴影扩大3例(25%),呼吸道出血2例(17%),心力衰竭1例(8%)。检查前后脉搏、血压差异无显著性(P>0.05),而检查后气促症状改善(P<0.05)。心力衰竭应用西地兰、速尿治疗后纠正,呼吸道出血、肺部阴影扩大无需处理。气管内压力支持通气可保证新生儿纤支镜检查期间正常氧合。结论纤支镜检查在新生儿呼吸系统疾病(包括部分重症新生儿)的临床应用是安全的。     

Intrarenal mechanisms of the regulation of homeostasis in children with nephrotic syndrome]

Overall 40 patients with associated glomerulonephritis and the nephrotic syndrome were examined for renal response to the induced shifts in water balance: tests with 18-hour water deprivation characterizing concentration function of the kidneys, followed by water loading (22 ml/kg bw) which allows estimation of water excretion function and osmotic dilution. Osmotic concentration defect was revealed. It manifested in a decrease of osmotic concentration of the urine, a reduction of the concentration index according to creatinine. It should be mentioned that the more preserved was the magnitude of the concentration index the more this was ensured by sodium reabsorption, decrease of its excretion. Meanwhile osmotic concentration of the urine was determined by urea excretion. Study of partial functions in water diuresis discovered a dramatic lowering of sodium-free water clearance characterizing participation of the kidney in the maintenance of effective osmotic pressure of the plasma and reflecting the intensity of distal sodium reabsorption.