CRRT机血浆置换预防儿童ABO血型不合骨髓输注的溶血反应

目的观察血浆置换技术在ABO血型主侧不合的非亲缘异基因供髓骨髓移植中的应用价值。方法对3例ABO血型主侧不合非亲缘供髓骨髓移植的患儿进行血浆置换,均先置换6%羟乙基淀粉注射液500 ml,再根据体重大小置换不同剂量的AB血型血浆,以去除血浆抗红细胞抗体,防止异型骨髓输注ABO血型不合的溶血反应,检测血浆置换前后受者血浆抗红细胞抗体浓度并观察溶血反应。结果血浆置换后,3例患儿血浆抗红细胞抗体IgM抗体滴度分别由最高1:1 024、1:32、1:256降为1:8、1:0、1:0,IgG抗体滴度分别由1:256、1:32、1:128降为1:16、1:0、1:4,分别输注ABO血型主侧不合骨髓680 ml8、00 ml1、000 ml,仅1例发生轻微急性溶血反应,未出现迟发性溶血反应,3例患儿均植入成功,其中1例发生Ⅰ度、2例发生II度急性移植物抗宿主病(皮肤型)。结论血浆置换是预防ABO血型主侧不合骨髓移植溶血反应的安全有效的方法。     

减停免疫抑制剂诱发移植物抗宿主病治疗儿童ABO血型不合移植后纯红细胞再生障碍性贫血1例及文献复习

目的探讨ABO血型不合移植后合并纯红细胞再生障碍性贫血(PRCA)的发生机制及治疗对策。方法报道1例重型地中海贫血患儿接受ABO血型不合的无关供者外周血造血干细胞移植(URD-PBSCT)后合并PRCA病例并进行文献复习。结果患儿接受ABO血型不合的URD-PBSCT获得成功后出现PRCA,依赖红细胞输注伴ABO血型抗体滴度显著升高,通过减停免疫抑制剂诱发移植物抗宿主病(GVHD)后,ABO血型抗体滴度显著下降,网织红细胞、血红蛋白和骨髓象随之恢复正常。结论免疫紊乱及ABO血型抗体是ABO血型不合移植后PRCA的最可能原因,减停免疫抑制剂诱发GVHD是治疗ABO血型不合移植后PRCA的一种优先考虑的选择,但需要注意GVHD的有效控制。     

儿童异基因造血干细胞移植后纯红再障1例并文献复习

目的探讨ABO血型不合的异基因造血干细胞移植(allo-HSCT)后发生纯红细胞再生障碍性贫血(PRCA)的发病机制和治疗对策。方法报道1例极重型再生障碍性贫血患儿行同胞HSCT后发生PRCA的病例及成功治疗经过,并复习相关文献。结果 13岁女性患儿,接受ABO血型主要不合的同胞HSCT后不能脱离红细胞输注,并发PRCA,经调整免疫抑制剂、DLI、应用利妥昔单抗、血浆置换等治疗后,PRCA得到纠正,血常规恢复正常。结论 HSCT后PRCA的发生与ABO血型抗体、非清髓预处理带来的免疫紊乱有关。主要治疗方法有使用单克隆抗体、血浆置换等,对于难治性患者,达雷木单抗、硼替佐米和艾曲波帕可作为新的靶向治疗药物。     

血浆置换及换血在治疗新生儿重症Rh不合溶血症1例报告

新生儿溶血症是由于母儿血型不合引起的被动同种免疫性疾病。Rh血型不相容所致此症，虽发病率不高，但一旦发病症状重，若不及时处理，预后极差。我科采用血浆置换及换血疗法抢救成功1例，报告如下。患儿母亲29岁，14岁时有过输血史。第一、二胎足月顺产均于三天内死于重度黄疽。母血型O。。cdee，血清抗D效价为1：512，菠萝酶法为1：256。此次孕前我们即以手工血浆置换法对该母进行血浆置换，每次换浆量为400～600ml，间隔1～4周。经过10次换浆后，母血清抗D效价降至1：64。菠萝酶法降至1：32。此时母受孕，继续每隔2～3周换浆400～600ml…     

孕妇产前抗体检测预测新生儿溶血病

目的探讨血型不合妊娠产前抗体检测在新生儿溶血病预测中的意义。方法对400例先天性ABo或Rh血型不合孕妇产前抗体效价检测，以抗体效价≥1：64为阳性，药物干预后检测效价，分娩后统计新生儿溶血病（mN）发病率。结果抗体效价≥1：64并发生HDN者占25．7％。结论血型不合妊娠夫妇进行ABO、Rh血型鉴定，检测孕妇血清中血型特异性抗体及抗体效价对预测新生儿溶血有重要意义。     

儿童脐血移植后血型及血型抗体的转变

目的脐带血移植是治疗儿童高危白血病最有效的方法之一。脐血与患儿的血型不同可能对脐血移植有影响 ,了解脐血移植后ABO血型抗体的转变 ,对选择成分输血具有重要指导意义。方法4例白血病患儿行ABO血型不合的脐血移植 ,次要不合2例 ,4例均存在主要不合。1例同胞脐血移植 ,3例非血缘脐血移植。移植前后动态观察ABO血型及血型抗体的转变。结果4例患儿均成功植入 ,移植后受者的血清凝集素1个月左右消失 ,植入后没有出现针对受者原有血型抗原的抗体。输血过程中未发生临床溶血情况。结论供受者脐血ABO血型不合可以进行移植 ,按患儿当时的情况进行输血是安全的。     

高效价抗A(B)引起新生儿溶血病二例

新生儿溶血病（HDN）指母婴血型不合引起胎儿或新生儿的免疫溶血性疾病。ABO新生儿溶血病是由于母婴ABO血型不合，母体的IgG抗A（B）经胎盘进入胎儿血液循环，破坏胎儿红细胞引起。高效价（≥1024）IgG抗A（B）引起的极少见。现报道2例如下。     

孕妇IgG血型抗体与新生儿溶血病的关系

目的 探讨孕妇IgG血型抗体与新生儿溶血病(HDN)的关系.方法 对617例夫妇血型不合的孕妇进行产前血型血清学检查:包括孕妇ABO正反定型和Rh(D)定型;IgG抗-A(B)效价测定;不规则抗体筛查及抗体特异性和效价测定.对其中分娩的388例新生儿进行HDN确认试验:包括患儿ABO和Rh(D)定型;红细胞直接抗人球蛋白试验;血清游离抗体测定;红细胞抗体放散试验及血清胆红素测定.结果 1.孕妇617例中ABO血型不合者606例(98.2%),Rh(D)不合者15例(2.4%),ABO和Rh(D)同时不合者4例(0.6%).606例ABO血型不合孕妇中,IgG抗-A(B)效价≥64(定为抗体阳性)者250例(41.3%),其中O型204例(33.7%);A型32例(5.3%);B型14例(2.3%);O型孕妇与A、B型孕妇抗体阳性率分别比较,差异均有统计学意义(P<,a><0.01).617例孕妇不规则抗体筛查阳性者共10例(1.6%),经特异性鉴定均为Rh系统IgG抗体,效价8～256.2.新生儿388例共确认HDN 107例(27.6%).其中ABO-HDN 96例(24.7%),Rh-HDN 11例(2.8%);孕妇IgG抗-A(B)效价,低值组(≤64)与中值组(128～256)、高值组(≥512)ABO-HDN发病率分别比较,差异均有统计学意义(P<,a><0.01);11例Rh-HDN由抗-D(含抗-DC、抗-DE)引起6例(54.5%),抗-E(含抗-cE)引起的4例(36.4%),抗-c引起的1例(9.1%).结论 与A、B型孕妇比较,O型孕妇更易引起ABO-HDN,且ABO-HDN发病率随其母亲体内IgG抗体效价的升高而升高;Rh系统不规则抗体极易引起Rh-HDN,且与孕妇输血史和(或)不良妊娠史有关.     

非抗D抗体致新生儿RH溶血病2例报告

新生儿溶血病（hemolytic disease of the newborn）是指母、婴血型不合引起的新生儿同种免疫性溶血性疾病。临床上以ABO血型不合引起的新生儿溶血病最常见,其次为RH血型系统,后者又以RHD血型不合引起的新生儿溶血病常见,而非抗D抗体引起的新生儿溶血病报道较少[1-3]。本文报道我院收治的2例非抗D抗体引起的新生儿RH溶血病,提高临床医师对本病在预防及治疗方面的认识。     

新生儿溶血性疾病

1病因 在新生儿期有多种疾病可引起溶血,包括:1.1 同族免疫溶血性贫血 即新生儿溶血病,是因母婴血型不合,母亲的血型抗体通过胎盘进入胎儿血循环,引起胎儿、新生儿红细胞破坏。在我国常见的是ABO血型不合,其次为Rh血型不合,其他血型(MN、Kell等)不合则少见。新生儿溶血病是新生儿期主要的溶血性疾病,且本病仅见于胎儿、新生儿期。     

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.