血浆置换治疗小儿危重病的临床观察

目的 探讨对危重患儿进行血浆置换治疗的安全性和效果.方法 回顾性分析应用BM25连续肾脏替代治疗机配合血浆分离器对14例患儿进行27次血浆置换治疗的临床资料.结果 血浆置换量为50～100 ml/(kg·次),血流速度为3.5～8.5 ml/(kg·min),每次治疗时间约2～3 h.26例次治疗顺利完成,1例次因患儿血流缓慢堵塞滤器滤过膜后未能顺利完成.血浆置换治疗期间无1例出血.经血浆置换和综合治疗,1例急性脊髓炎、1例吉兰-巴雷综合征、2例脓毒症并多器官功能障碍顺利脱离呼吸机,治愈出院;1例毒草中毒并严重肝损害治愈出院;5例重症系统性红斑狼疮、1例抗中性粒细胞胞浆抗体相关性肾炎、1例紫癜性肾炎并急性肾功能衰竭和1例溶血尿毒综合征患儿肾功能恢复正常,尿蛋白减少,临床症状好转;1例肝功能衰竭、肝豆状核变性并溶血性贫血死亡.结论 血浆置换是治疗扎童危重病有效和安全的方法 .     

血浆置换在儿童非典型溶血尿毒综合征中的应用

目的观察血浆置换(PE)对儿童非典型溶血尿毒综合征(aHUS)急性期的疗效。方法回顾分析2014年5月至2017年5月收治的16例急性期采用PE治疗的aHUS患儿的临床资料。结果 16例aHUS患儿中,男10例、女6例,年龄1～14岁。PE采用Prismaflex TPE-1000或2000膜式血浆分离器,每次的血浆置换量约40～60mL/kg;每例2～5次。除1例经过4次PE后放弃治疗外,另15例患儿经治疗1月内病情均好转。结论 PE可以迅速缓解儿童aHUS的病情,可作为aHUS急性期的重要治疗手段之一。     

血浆置换联合血液滤过辅助救治剥脱性皮炎

目的 探讨床旁血浆置换(plasma exchange,PE)联合连续性静-静脉血液滤过 (continuous veno-venous hemofiltration,CVVH)在辅助救治儿童剥脱性皮炎中的作用.方法 对2例肾上腺皮质激素和静脉丙种球蛋白(intravenous immunoglobulins,IVIG)救治无效的重症剥脱性皮炎患儿采用床旁PE联合CVVH治疗,观察病情演变与转归情况.结果 2016年4月至2016年12月,本院PICU收治2例重症剥脱性皮炎患儿,年龄分别为3岁8个月及1岁11个月.两例患儿均予甲泼尼龙、IVIG、抗感染及烧伤单元式皮肤护理等积极综合救治,病情加重,高热不退,皮疹、水疱急剧增多,皮肤大片剥脱,予PE联合CVVH序贯式血液净化治疗.PE采用Prisma TPE 2000膜式血浆分离器,每次置换血浆量60~80ml/kg,间隔48h;CVVH应用AN69 M60滤器,置换液量50ml/(kg·h).2例剥脱性皮炎患儿经PE 2~3次联合CVVH治疗72~96h后,体温开始恢复正常,呼吸、循环、肝、肾功能好转;治疗7d 后,皮疹颜色变淡,水疱开始收敛结痂,新生皮肤出现.监测血液净化治疗前后C反应蛋白、肿瘤坏死因子α、白细胞介素(IL)系列(IL-2、IL-6、IL-8、IL-10)均明显下降;自然杀伤细胞百分比上升.例1住院32d治愈出院.例2入PICU第13天(入院第26天)再次高热,血、皮肤及尿培养显示多重耐药菌感染(血培养:鲍曼不动杆菌,皮肤渗出液:铜绿假单胞菌,尿培养:热带假丝酵母菌),于入PICU第20天(入院第33天)因多脏器功能衰竭(循环、血液、胃肠、呼吸、肾脏)经抢救无效死亡.结论 PE联合CVVH可减轻炎性反应、保护受损器官,可能是剥脱性皮炎患儿的辅助救治措施.     

血浆置换在儿童危重症中的应用

目的 探讨血浆置换在儿童危重症中应用的疗效及安全性.方法 经股静脉或颈内静脉建立血管通路,应用Prisma血液净化机,对10例PICU危重患儿进行28次血浆置换治疗.患者病种分别为脓毒症合并多脏器功能不全、急性肝功能衰竭、肾移植后急性排斥反应,溶血尿毒综合征、格林-巴利综合征、狼疮性肾炎,所有病例均存在2～5个脏器功能衰竭.结果 每次血浆置换量为(63.5±19.5)ml/kg,血流速度为5～10 ml/(kg·min),置换速度为血流速度的20%～25%.经过血浆置换及综合治疗,10例患儿临床症状及生化指标均有好转,2例分别进行4次血浆置换治疗病情才得到缓解.结果8例存活,2例死亡(急性肝功能衰竭及狼疮性肾炎各1例).28例次均顺利完成血浆置换治疗.治疗中有3例次发生一过性荨麻疹,未见休克、严重出血倾向、感染等严重并发症.结论 血浆置换是治疗儿童严重脓毒症及免疫性疾病的一种有效措施.     

血液净化联合贝利尤单抗治疗儿童系统性红斑狼疮合并严重感染导致多器官功能障碍综合征一例并文献复习

目的探讨血液净化(连续性肾脏替代治疗和双滤过血浆置换)联合贝利尤单抗治疗儿童系统性红斑狼疮(cSLE)合并严重感染导致多器官功能障碍综合征的诊疗经过, 提高对cSLE合并严重感染救治的认识。方法回顾性分析深圳市儿童医院2021年9月收治的1例cSLE合并严重感染诱导的多器官功能障碍综合征患儿的临床资料及15个月的诊疗随访记录, 对其临床表现、影像学检查、实验室检查、治疗及随访结果进行分析, 并复习国内外相关文献。结果患儿, 女, 14岁, cSLE合并严重脓毒症诱导的多器官功能障碍综合征, 除常规激素及抗感染治疗外, 立即予连续性肾脏替代治疗(CRRT)联合双滤过血浆置换(DFPP)进行血液净化, 患儿右腿软组织感染形成大面积的坏死性焦痂, 病程第45天时开始进行清创(先后5种清创术和2次植皮术)。病程50 d时予第1次贝利尤单抗静滴。患儿无尿状态持续60 d, 予隔日血液透析, 第61天尿量开始慢慢增加, 病程第63天尿量为560 mL/d, 病程70 d时>1 000 mL/d, 水肿消退, 肾功能完全恢复正常, 抗ds-DNA抗体滴度水平下降、补体C3升高, 24 h尿蛋白...     

血浆置换联合连续性血液净化治疗儿童重症溶血尿毒综合征

目的：探讨床旁血浆置换（ plasma exchange,PE）联合连续性静-静脉血液滤过透析（ continuous veno-venous hemodiafiltration,CVVHDF）在抢救儿童重症溶血尿毒综合征（ hemolytic uremic syndrome,HUS）中的作用。方法总结本院PICU收治的HUS患儿的临床特点,采用儿童危重病例评分和儿童死亡危险评分Ⅲ评估病情的危重程度。对4例重症HUS患儿采用床旁PE联合CVVHDF治疗,观察病情演变与转归情况。结果2012年6月至2015年5月我院PICU收治重症HUS8例,经利尿剂、输血等保守治疗12～24 h后,病情加重的4例危重患儿进行PE＋CVVHDF序贯式血液净化治疗。 PE采用Prisma TPE 2000膜式血浆分离器,CVVHDF应用AN69 M60滤器,PE每次新鲜冰冻血浆50～70 ml／kg,连续3～6次,CVVHDF置换液50 ml／（ kg· h）。4例重症患儿治疗后均存活,血生化指标和肾功能指标改善。治疗前后血肌酐平均值（318μmol／L vs．162μmol／L）、乳酸脱氢酶（1963 U／L vs．407 U／L）明显下降,血小板计数明显升高（40×109／L vs．97×109／L）。结论 PE和CVVHDF治疗重症HUS,可以迅速改善患儿血生化指标,稳定内环境,阻断溶血和改善肾功能,可作为重症HUS抢救的重要手段。     

血浆置换治疗皮肌炎3例

3例中男2例,女1例;年龄8~11岁;均符合Bohan提出诊断标准[1]。病程4个月~1年。3例均表现为进行性双下肢无力,双下肢瘫痪1例,下蹲困难,眼睑呈红色伴水肿各2例;颜面红斑1例。实验室检查:肌酸激酶(CK)、LDH及门冬氨酸氨基转移酶(AST)明显升高。肌电图及三角肌活检示呈肌源性损伤。采用泼尼松2mg/(kg·d)口服,配合血浆置换疗法,血浆置换量20~30mL/(kg·次),并予等量706代血浆输入,2次/周,连用2周后,改为1次/月;连用2次2例,连用3次1例。血浆置换2次后面部皮疹消退,肌力明显好转;置换4次后3例肌力均恢复正常;2例CK恢复正常,1例明显下降。门诊…     

血浆置换治疗儿科危重症87例分析

目的观察血浆置换在治疗儿童危重症中的效果。方法首都医科大学附属北京儿童医院2003年8月至2014年2月先后收治危重症患儿87例并接受血浆置换治疗,对其适应证、治疗效果、并发症等进行回顾性分析。结果 87例危重症患儿共进行189例次血浆置换,其中全血浆置换185次(97.9%)、双重血浆置换4次(2.1%),治疗均顺利完成,未见严重并发症。好转及治愈78例(89.7%),4例(4.6%)放弃治疗并失访,5例(5.7%)因原发病死亡。结论近十年效果分析表明,血浆置换治疗为肾脏、神经、血液、感染等多系统疾病提供了有效的治疗或辅助治疗方法。     

血浆置换治疗儿童溶血尿毒综合征(附四例报告)

目的 探讨血浆置换治疗在重症溶血尿毒综合征患儿的应用价值和治疗方案.方法 应用金宝-PRISMA床旁血滤机和TPE2000膜式血浆分离器对4例重症溶血尿毒综合征患儿进行13次血浆置换治疗;以新鲜冰冻血浆作置换液,置换量40 ml/(kg次),血泵速度为50～120 ml/min,治疗时间2～3 h/次.结果 13次血浆置换治疗均顺利成功实施,无明显并发症出现;4例患儿在治疗后临床症状及生化指标明显好转,其中3例痊愈,1例好转出院.结论 血浆置换可以应用于重症溶血尿毒综合征患儿,疗效显著.     

环磷酰胺连续冲击治疗壮族儿童难治性肾病综合征疗效及远期随访

目的 探讨大剂量环磷酰胺(CTX)冲击治疗壮族儿童难治性肾病综合征(肾病)的疗效及远期对性腺的损害.方法 对38例壮族难治性肾病综合征患儿予CTX 8～12 mg/(kg·d)加入生理盐水或10%葡萄糖注射液100 mL,静滴1 h左右,1次/d,连用8～10次,累积量＜80～120 mg/kg.同时应用泼尼松2 mg/(kg·d).结果 1.临床治愈28例(73.6%),完全缓解7例(18.42%),部分缓解2例(5.26%),死亡1例(2.63%),疗效与临床分类、病理类型关系不明显(P均＞0.05);2.冲击治疗后d2血浆总蛋白、清蛋白较治疗前明显提高(P＜0.01),第4周已达正常水平(P＜0.01);24 h尿蛋白明显降低(P＜0.01);血胆固醇冲击结束后4周明显降低(P＜0.01),血肌苷、Hb、WBC、PLT治疗前后均无明显改变(P均＞0.05).3.不良反应20例(52.63%)有胃肠道反应,2例(5.26%)WBC减少,38例(100.0%)脱发,1例(2.63%)有心脏损害.CTX冲击治疗时年龄≥8岁、临床治愈、末次随访年龄≥17岁的18例精液检查或月经来潮正常,已婚7例男女均已生育.结论 CTX连续冲击联合泼尼松治疗壮族儿童难治性肾病疗效好,可快速提高血浆总蛋白、清蛋白水平,尿蛋白转阴快.CTX累积总量小于80 mg/kg,近期安全,远期未发现性腺损害.     

Allergic factors associated with the development of asthma and the influence of cetirizine in a double-blind, randomised, placebo-controlled trial: First results of ETA®

There is a common progression known as the allergic march from atopic dermatitis to allergic asthma. Cetirizine has several antiallergic properties that suggest a potential effect on the development of airway inflammation and asthma in infants with atopic dermatitis. Methods. Over a two year period, 817 infants aged one to two years who suffered from atopic dermatitis and with a history of atopic disease in a parent or sibling were included in the ETAC^® (Early Treatment of the Atopic Child) trial, a multi-country, double-blind, randomised, placebo-controlled trial. The infants were treated for 18 months with either cetirizine (0.25mg/ kg b.i.d.) or placebo. The number of infants who developed asthma was compared between the two groups. Clinical and biological assessments including analysis of total and specific IgE antibodies were performed. Results. In the placebo group, the relative risk (RR) for developing asthma was elevated in patients with a raised level of total IgE (≥ 30 kU/I) or specific IgE (≥ 0.35 kUA/I) for grass pollen, house dust mite or cat dander (RR between 1.4 and 1.7). Compared to placebo, cetirizine significantly reduced the incidence of asthma for patients sensitised to grass pollen (RR = 0.5) or to house dust mite (RR = 0.6). However, in the population that included all infants with normal and elevated total or specific IgE (intention-to-treat - ITT), there was no difference between the numbers of infants developing asthma while receiving cetirizine or placebo. The adverse events profile was similar in the two treatment groups. Discussion. Raised total IgE level and raised specific IgE levels to grass pollen, house dust mite or cat dander were predictive of subsequent asthma. Cetirizine halved the number of patients developing asthma in the subgroups sensitised to grass pollen or house dust mite (i.e. 20% of the study population). In view of the proven safety of the drug, we propose this treatment as a primary pharmacological intervention strategy to prevent the development of asthma in specifically sensitised infants with atopic dermatitis.     

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孤独症谱系障碍(autistic-spectrum disorders,ASDs)近年来患病率逐年攀升至1%左右,其症状往往伴随终生,成为严重威胁儿童健康和发展的神经发育性疾患;注意缺陷多动障碍(attention deficit hyperactivity disorder,ADHD)是儿童期最常见的精神障碍,国内报道患病率为4.13%～5.83%,其症状可延续至青少年期,甚至到成年期[1]。这两类精神障碍在成年期的临床表现、共患病、治疗策略和预后与儿童期有哪些不同呢?本文通过回顾相     

EXCITING NEW TREATMENT APPROACHES FOR PATHYPHYSIOLOGIC MECHANISMS OF SICKLE CELL DISEASE

During the past several decades, our understanding of the complex pathophysiology of vasoocclusion associated with sickle cell disease has improved greatly. Interaction of genes, hemoglobin molecules, red cell membrane and metabolic changes, cell-cell interactions and cell-plasma interactions, red cell adhesion to vascular endothelium, activation of coagulation, and vascular reactivity play a role in vaso occlusion. Penicillin prophylaxis of pneumococcal infections and appropriate use of blood transfusions and other supportive measures improved survival of sickle cell patients. Hydroxyurea made a major impact on sickle cell therapy when it was shown to decrease acute painful episodes, acute chest syndrome, and the need for blood transfusion in adults. Significant experience in the use of hydroxyurea has been accumulated in older children. The benefits and risks of hydroxyurea for younger children and long-term risks in all patients will be evaluated in future investigations. Other promising therapies include butyrate compounds, clotrimazole, magnesium supplementation, poloxamer 188, antiadhesion agents, anticoagulant approaches, and nitric oxide. Hemopoietic transplantation remains the only curative therapy. However, several transgenic mouse models are available for studies of gene therapy or other treatment approaches on biochemical, cellular, and pathologic effects of mutant genes.     

Infiltrations of Epstein-Barr virus-carrying cells in granular lymphocyte-proliferative disorders corresponding to chronic active Epstein-Barr virus infection

A 21-year-old man with granular lymphocyte-proliferative disorders (GLPD) associated with chronic active Epstein-Barr virus (EBV) infection is described. Chromosomal analyses revealed several clonal abnormalities and two of them were mainly repetitious. High copy numbers of monoclonal EBV genome were also detected in the proliferative large granular lymphocytes (LGLs), indicating the monoclonal expansion of EBV-infected LGLs. The patient had an indolent course for several years, and there was no evidence of infiltrations of his bone marrow until the end stage. At autopsy, microscopic studies revealed marked infiltrations of LGL in the liver and spleen, and the infiltrating cells were NK-cell immunophenotype. The infiltrated LGLs showed latency I.     

LUTEINIZING HORMONE RECEPTOR MUTATIONS IN DISORDERS OF SEXUAL DEVELOPMENT AND CANCER

Human male sexual development is regulated by chorionic gonadotropin (CG) and luteinizing hormone (LH). Aberrant sexual development caused by both activating and inactivating mutations of the human luteinizing hormone receptor (LHR) have been described. All known activating mutations of the LHR are missense mutations caused by single base substitution. The most common activating mutation is the replacement of Asp-578 by Gly due to the substitution of A by G at nucleotide position 1733. All activating mutations are present in exon 11 which encodes the transmembrane domain of the receptor. Constitutive activity of the LHR causes LH releasing hormone-independent precocious puberty in boys and the autosomal dominant disorder familial male-limited precocious puberty (FMPP). Both germline and somatic activating mutations of the LHR have been found in patients with testicular tumors. Activating mutations have no effect on females. The molecular genetics of the inactivating mutations of the LHR are more variable and include single base substitution, partial gene deletion, and insertion. These mutations are not localized and are present in both the extracellular and transmembrane domain of the receptor. Inactivation of the LHR gives rise to the autosomal recessive disorder Leydig cell hypoplasia (LCH) and male hypogonadism or male pseudohermaphroditism. Severity of the clinical phenotype in LCH patients correlates with the amount of residual activity of the mutated receptor. Females are less affected by inactivating mutation of the LHR. Symptoms caused by homozygous inactivating mutation of the LHR include polycystic ovaries and primary amenorrhea.     

Resurgence of measles in Singapore: profile of hospital cases

OBJECTIVE: To ascertain the profile of cases of measles seen at a general hospital during a recent outbreak that occurred despite a measles vaccination program. METHODOLOGY: A retrospective study from January 1991 to March 1998. All patients with measles (ICD code 055. 9) seen at the emergency unit or as inpatients were included. RESULTS: There were 87 cases identified. The diagnosis was clinical in all and proven serologically in 71%. Eighty-five per cent of the cases occurred between January 1997 and March 1998. There was a bi-modal age distribution with peaks in the very young (相似文献   

Personality profiles and heart rate variability (vagal tone) in children with recurrent abdominal pain

The aim of the study was to explore psychological factors and autonomic activity in children with recurrent abdominal pain and to compare them with those in a control group of healthy children. The Personality Inventory for Children was used for assessment of developmental, emotional and psychosocial factors in 25 children with recurrent abdominal pain (age, 7-15 y). Parasympathetic and sympathetic functions in these children and in 23 healthy control subjects (age, 7-13 y) were also investigated, non-invasively using a computerized polygraph. Vagal tone (parasympathetic function) was indexed by calculation of respiratory sinus arrhythmia in beats/min. Skin conductance (sympathetic function) was recorded by the constant current method. On the Personality Inventory for Children, 16 patients had high scores on somatic concern. Several patients had scores in the clinical range for depression, withdrawal and anxiety, but the mean scores for these personality profile scales were well within the normal range of healthy children. Interestingly, there was a spike on the L (Lie)-scale for most of the patients and 15 patients had scores above or close to the clinical cut-off value. As compared with the scores in healthy children, vagal tone and sympathetic tone were normal. Conclusion: Many children with recurrent abdominal pain have scores in the clinical range for depression, withdrawal, anxiety and L-scale indicating coping problems, denial and a trend towards somatic concern that may contribute to the evolution of abdominal pain. Autonomic nerve activity was not disturbed in these children.     

Overcoming surfactant inhibition with polymers

Inhibition of the function of pulmonary surfactant in the alveolar space is an important element of the pathophysiology of many lung diseases, including meconium aspiration syndrome, pneumonia and acute respiratory distress syndrome. The known mechanisms by which surfactant dysfunction occurs are (a) competitive inhibition of phospholipid entry into the surface monolayer (e.g. by plasma proteins), and (b) infiltration and destabilization of the surface film by extraneous lipids (e.g. meconium-derived free fatty acids). Recent data suggest that addition of non-ionic polymers such as dextran and polyethylene glycol to surfactant mixtures may significantly improve resistance to inhibition. Polymers have been found to neutralize the effects of several different inhibitors, and can produce near-complete restoration of surfactant function. The anti-inhibitory properties of polymers, and their possible role as an adjunct to surfactant therapy, deserve further exploration.     

Understanding infant formula

The World Health organisation recommends breast feeding infants for the first six months of life. When this breast feeding does not occur either through parental choice or medical need, infant formulas will be required. There is a bewildering array of formulas on the UK market for many different requirements. When faced with an unsettled infant many parents (and healthcare professionals) will experiment with the infant formula available and then attend the paediatric clinic looking for help and advice. It is therefore essential that paediatricians understand what milks are available and what the key differences between different products are. This review attempts to provide a simple guide through many of the formulations currently available in the UK; and offers advice for the dietary management of the child with extra calorie requirements, infants with cow's milk protein allergy, gastro oesophageal reflux disease, apparent unresolved hunger and infantile colic. Whatever the underlying condition, there is likely to be an infant formula that is suitable in this generation of ever expanding formulations.