儿童嗜酸性粒细胞增多症15例临床分析

目的 探讨嗜酸性粒细胞增多症的病因、临床表现、诊断和转归,提高对儿童嗜酸性粒细胞增多症的认识.方法 回顾性分析2001年4月-2007年4月住院的15例嗜酸性粒细胞增多症患儿的临床资料.结果 病因主要为变态反应性疾病(4例)、恶性肿瘤(4例)、感染性疾病(寄生虫感染3例,病毒感染1例)、药物反应(2例),原因不明1例;临床表现多为发热(86.7%)、乏力(46.7%)、咳嗽(40.0%)、浅表淋巴结及肝脾肿大(53.3%);除恶性肿瘤外,经治疗多数预后良好.结论 儿童嗜酸性粒细胞增多症以继发性为主,多见于变态反应性疾病、恶性肿瘤、寄生虫感染;对嗜酸性粒细胞增多患儿需进行全身各系统评估,寻找病因,警惕肿瘤,予以积极病因治疗.     

痰液细胞在哮喘患儿病程中的动态变化及意义

目的　观察哮喘患儿痰液细胞在病程不同时相的动态变化 ,探讨其在哮喘发病中的作用。方法　收集 2 5例哮喘患儿发作之初、病情好转以及病情缓解时期的痰液 ,比较哮喘病程不同时相之间以及不同时相与 10例正常儿童之间痰液细胞成分的差异。结果　哮喘急性发作初期痰液脱落上皮细胞明显增多 ,嗜酸性粒细胞、中性粒细胞和淋巴细胞与正常对照无明显差别 ;随着病情好转 ,脱落上皮细胞数显著下降 ,而嗜酸性粒细胞数和(或 )中性粒细胞数显著升高 ,淋巴细胞与正常对照仍无明显差别 ;当病情缓解时 ,脱落上皮细胞的数量接近正常对照 ,嗜酸性粒细胞和 (或 )中性粒细胞数也显著下降 ,而淋巴细胞明显增多。结论　上皮细胞脱落可能与哮喘急性发作有关 ;嗜酸性粒细胞或中性粒细胞除造成上皮细胞脱落外 ,可能还发挥促上皮细胞再生的有益作用 ,有助于病情的恢复 ;淋巴细胞可能在维持哮喘慢性气道炎症中发挥作用。     

嗜酸细胞性胃肠道疾病的发病机制研究进展

嗜酸细胞性胃肠道疾病是指反复或持续存在胃肠道症状, 伴有消化道黏膜内嗜酸性粒细胞病理性升高的一组疾病。病理学特征性表现为胃肠道黏膜内嗜酸性粒细胞数量增多, 嗜酸细胞性食管炎患者食道黏膜固有层可见纤维化。趋化嗜酸性粒细胞聚集的细胞因子有多种, 包括Th2细胞因子、嗜酸细胞趋化因子、胸腺基质淋巴细胞生成素、巨噬细胞移动抑制因子、唾液酸结合免疫球蛋白样凝集素、整合素和细胞外基质蛋白。嗜酸细胞性胃肠道疾病的肠道组织损伤可能与嗜酸性粒细胞脱颗粒分泌特异性产物、炎症反应及氧化损伤、纤维化和组织重塑以及屏障功能受损有关。     

新生儿重症监护病房中低出生体重儿嗜酸性粒细胞增多危险因素分析

目的分析新生儿重症监护病房(NICU)中低出生体重儿嗜酸性粒细胞增多的危险因素。方法回顾性分析2019年1月1日至2020年3月31日在北京大学第三医院儿科NICU住院的496例低出生体重儿的病例资料,因各种原因剔除196例,根据嗜酸性粒细胞是否增多分为嗜酸性粒细胞增多组和对照组,各150例。通过单因素分析进行筛选,再通过非条件Logistic回归分析低出生体重儿嗜酸性粒细胞增多的影响因素。结果 (1)两组患儿嗜酸性粒细胞均在生后第3周达到高峰,然后逐渐下降,嗜酸性粒细胞增多组嗜酸性粒细胞升高持续时间更长。(2)母亲有妊娠期高血压、胎膜早破、儿童出生后患食物过敏、感染、贫血、输血、支气管肺发育不良(BPD)、白细胞增多、血小板增多在两组患儿分别为59例(39.3%)、33例(22.0%); 47例(31.3%)、31例(20.7%);10例(6.7%)、1例(0.7%); 54例(36.0%)、15例(10.0%); 133例(88.7%)、88例(58.7%); 67例(44.7%)、26例(17.3%); 75例(50.0%)、23例(15.3%); 61例(40.6%)、43例(...     

新生儿嗜酸性粒细胞增多

目的　探讨新生儿嗜酸性粒细胞增多的原因 ,评价新生儿嗜酸性粒细胞增多的临床特征。方法　对 2 4例新生儿嗜酸性粒细胞增多患儿 ,监测血嗜酸性粒细胞计数 ,检测Ig系列 ,详细记录患儿入院后情况及患儿家属情况 ,并对所有患儿进行随访。结果　在同期住院的 10 5 2例新生儿中 ,2 4例 (2 .3% )患儿存在嗜酸性粒细胞增多。嗜酸性粒细胞轻度增高占 2 9.2 % ,中度增高 5 4 .2 % ,重度增高 16 .7%。嗜酸性粒细胞增多的时间为入院后 0～ 2 0d不等 ,其中 2 5 .0 %患儿入院当时即发现有嗜酸性粒细胞增多 ,2 9.2 %患儿为入院后 1d发现有嗜酸性粒细胞增多 ,2例化脓性脑膜炎患儿在恢复期出现嗜酸性粒细胞增多。在Ig系列中 ,IgA增高 6例 (2 5 .0 % ) ,IgE增高 2例 (8.3% )。嗜酸性粒细胞增多患儿的恢复时间为 2～ 2 1d。患儿出院后 ,对所有患儿进行随访 ,其中 3例(12 .5 % )患儿有湿疹存在。截止至随访日期 ,尚未发现有过敏及哮喘发作。结论　嗜酸性粒细胞增多在新生儿期并不少见 ,常见的原因与新生儿感染有关。     

变态反应性疾病中嗜酸性粒细胞特异性激活标志物

变态反应性疾病是严重影响人类健康的常见病、多发病,包括哮喘、过敏性鼻炎、过敏性紫癜、荨麻疹、过敏性休克等。多种炎症性细胞参与了变态反应的发生发展,例如哮喘患者气道存在激活的TH2淋巴细胞、嗜酸性粒细胞(Eos)、肥大细胞、嗜碱性粒细胞及中性粒细胞浸润的特征性改变。因此测定炎症激活标记物在判断变态反应性疾病炎症反应程度及诊断治疗上有着重要作用。     

高分辨CT在评价儿童哮喘气道重塑中的应用

<正>支气管哮喘是由嗜酸性粒细胞、肥大细胞和T淋巴细胞等多种炎性细胞参与的气道慢性炎症性疾病。气道炎症和气道重塑是支气管哮喘的两个主要病理学特征。1922     

嗜酸性粒细胞性支气管炎的诊断与治疗

嗜酸性粒细胞性支气管炎是儿童慢性咳嗽常见原因之一,但在临床上易漏诊、误诊,必须引起高度重视.嗜酸性粒细胞性支气管炎有着与支气管哮喘相似的嗜酸性粒细胞增多性炎症,但却无哮喘的可逆性气流阻塞或呼吸道高反应性,现归纳总结嗜酸性粒细胞性支气管炎的定义、病因学、临床表现、诊断及治疗方法.     

非嗜酸细胞性哮喘研究进展

支气管哮喘（哮喘）是由多种细胞[嗜酸性粒细胞（EOS）、肥大细胞、T淋巴细胞、中性粒细胞及呼吸道上皮细胞等]和细胞组分共同参与的呼吸道慢性炎性疾病。以往研究多认为哮喘呼吸道局部改变是以EOS为主的炎性反应,近年来一些诱导痰检查发现部分哮喘呼吸道局部炎性反应并非以EOS为主,而是以其他细胞如中性粒细胞、淋巴细胞、肥大细胞等为主,因此有研究将哮喘分为嗜酸细胞性哮喘（EA）和非嗜酸细胞性哮喘（NEA）两个亚型,前者呼吸道局部以EOS炎性反应为主,后者呼吸道局部则以非EOS炎性反应为主。本文就NEA发病机制及可能病因等进行综述。     

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特发性嗜酸粒细胞增多综合征(idiopathichypereosino-philicsyndrome,IHES)又称高嗜酸粒细胞综合征(idiopath-iceosinophilia),由Hardy和Anderson于1968年首先提出,是一种病因不明,以外周血嗜酸粒细胞增多、多脏器受累为特征的临床综合征,多发生于中年以上男性,预后较差,而儿童和青少年罕见。儿童IHES临床表现不典型,合并多器官受损者少见。现报告1例以消化道为首发症状并合并多器官受损的IHES患儿。     

High-Risk ALK Negative Anaplastic Large-Cell Lymphoma Presenting with Hypereosinophilic Syndrome in a 2.5-Year-Old Child

The hypereosinophilic syndromes (HES) are characterized by prolonged nonreactive peripheral blood hypereosinophilia with tissue damage. The lymphocytic HES variant can precede malignant clonal T-cell disease in adults but it is extremely rare to be the presenting feature of lymphomas in children. Here we present a 2.5-year-old boy with HES and mediastinal T-cell anaplastic lymphoma kinase (ALK) negative systemic anaplastic large-cell lymphoma. Mature and immature eosinophils without blasts were shown on bone marrow aspiration while biopsy revealed malignant infiltration. The patient responded well to initial corticosteroid therapy, but high-risk features make a challenge of finding the cure in this extremely rare case.     

Childhood post-transplant lymphoproliferative disorder

Post-transplant lymphoproliferative disorders (PTLDs) are a heterogeneous group of neoplasms that occur as a serious complication of haematopoetic stem cell or solid organ transplantation. They are Epstein Barr virus (EBV) driven in over 90% of the cases. Incidence of PTLD varies from 1 to 20 % depending on the organ transplanted, the intensity of immunosuppression and the recipient's pre transplant EBV status. The pathology of PTLD varies from polymorphic to monomorphic/monoclonal disease which is indistinguishable from a high-grade lymphoma. The clinical course varies from indolent to rapidly progressive, fulminant disease. Over two thirds to 90% of PTLDs are of B cell origin. The current standard of treatment involves reduction of immunosuppressive drugs and if that is not sufficient, use of anti CD20 monoclonal antibody (Rituximab) alone or in combination with conventional chemotherapy. This review offers an overview for healthcare professionals looking after children and young people who may be at risk of or have PTLD. It discusses the pathophysiology, the risk factors, classification and diagnosis of PTLD. It also summarises the current approach to management and monitoring strategies.     

Zespół hemofagocytarny u dzieci

Hemophagocytic lymphohistiocytosis (HLH) also known as hemophagocytic syndrome is a severe, life threatening inammation, caused by ineffective, prolonged immune response. It may occur as a primary (genetic) or secondary (acquired) disease. Primary HLH is divided into familiar HLH (FHLH) and other genetically determined conditions. In familiar form, HLH is the only manifestation of the disease. In other genetic forms, HLH is one of clinical manifestations. Secondary HLH may arise in a course of severe infections, autoimmune diseases, neoplasms, metabolic diseases, immunosuppressive therapy and after organ transplantation. The prognosis in HLH is inferior. Lack of proper treatment leads to death in primary HLH and in most cases of secondary HLH.     

严重脓毒症血制品应用和免疫支持治疗

严重脓毒症和脓毒性休克是在脓毒症基础上并发器官功能衰竭或者组织灌注不足为特征的临床综合征,其病死率高.虽然早期足量快速的液体复苏是严重脓毒症和脓毒性休克治疗的关键,但白蛋白、血浆、红细胞、血小板、丙种球蛋白等血制品输注,以及血液净化、乌司他丁联合胸腺肽和抗CD14单克隆抗体等免疫支持也起着一定作用.     

Update on therapeutic monoclonal antibodies

Monoclonal antibodies are among the most important class of drugs introduced into the therapeutic armamentarium since the introduction of antimicrobials in the 1930s. The first therapeutic monoclonal antibody, the anti T-cell monoclonal antibody OKT4, was licensed in 1986. Since then, 18 additional antibodies have been licensed in the US, with many more in the pipeline. Before 1986, many monoclonal antibodies were available for laboratory studies, notably to identify specific cells in the blood and tissues. This is best illustrated by the cluster designation (CD) system for antigens present on hematopoietic cells, now numbering over 200.     

Detection of antibodies to human parvovirus in erythema infectiosum (fifth disease).

Two Japanese outbreaks of erythema infectiosum were investigated for evidence of human parvovirus infection by a solid phase antibody capture radioimmunoassay based on a monoclonal antibody to human parvovirus. Specific IgM and high concentrations of specific IgG were detected in 37 sera from 27 children with erythema infectiosum. No anti human parvovirus IgM was detected in a remaining case of erythema infectiosum, in five patients with Kawasaki disease, or in the 17 control children. Seven of the controls were also anti human parvovirus IgG negative, and the 10 who were seropositive had lower concentrations of anti human parvovirus IgG than the patients with erythema infectiosum. These data indicate that human parvovirus is a cause of erythema infectiosum.     

Albendazole therapy for coagulation abnormality in children with eosinophilia

Hemorrhage may be associated with eosinophilia. Here, 2 pediatric cases of coagulation abnormality were reported. The 2 children presented ecchymoses and petechiae in the skin. Laboratory investigations revealed hypereosinophilia in the peripheral blood. Moreover, the activity of coagulation factor IX significantly decreased. The serum immunoglobulin G (IgG) antibody to cysticercus was positive in patient 1, and IgG antibodies to cysticercus and plerocercoid were positive in patient 2. These 2 patients received 2 courses of albendazole therapy, at 15 mg/kg/day in divided doses for 10 days for each course. They responded well to the therapy. This report indicates that patients with hypereosinophilia and bleeding abnormalities should undergo evaluation of coagulation pathways, including measurements of coagulation factors.     

Albendazole Therapy for Coagulation Abnormality in Children with Eosinophilia

Hemorrhage may be associated with eosinophilia. Here, 2 pediatric cases of coagulation abnormality were reported. The 2 children presented ecchymoses and petechiae in the skin. Laboratory investigations revealed hypereosinophilia in the peripheral blood. Moreover, the activity of coagulation factor IX significantly decreased. The serum immunoglobulin G (IgG) antibody to cysticercus was positive in patient 1, and IgG antibodies to cysticercus and plerocercoid were positive in patient 2. These 2 patients received 2 courses of albendazole therapy, at 15 mg/kg/day in divided doses for 10 days for each course. They responded well to the therapy. This report indicates that patients with hypereosinophilia and bleeding abnormalities should undergo evaluation of coagulation pathways, including measurements of coagulation factors.     

Hypereosinophilic syndrome in a child presenting as eosinophilic pharyngitis

A 3-year-old child with idiopathic hypereosinophilic syndrome (IHES) presented with sore throat and pharyngeal exudate. Recurrent throat cultures were negative and microscopic section of the exudate revealed an extensive eosinophilic infiltration. Fourteen months later, the child still has marked hypereosinophilia and pharyngeal involvement without other organ involvement. Eosinophilic pharyngitis may be a target organ in IHES. The benign clinical course and the laboratory characteristics are described.     

Immunophenotyping to Detect and Characterize Acute Lymphocytic Leukemia in Testicular Biopsies

This study establishes the utility of immunophenotyping testicular biopsy specimens in patients with acute lymphoid leukemia. The value of immunophenotyping in detecting or excluding leukemic testicular infiltration is demonstrated in six children with acute lymphocytic leukemia. A panel of monoclonal antibodies was employed on snap-frozen testicular biopsies, allowing both detection and immunologic characterization of four neoplastic lymphocytic infiltrates. Two samples were proven both histologically and phenotypically negative for leukemic infiltration. One of the four leukemic cases was clinically silent and might have escaped detection except for phenotyping. One leukemic infiltrate was also suspected to possess a multidrug-resistant phenotype (-glycoprotein +); the latter possibility was excluded by an absence of reactivity with anti-p-glycoprotein monoclonal antibody. Thus, three clinically useful applications are demonstrated: (1) confirmation of testicular leukemic relapse, gaining assertion in histologically uncertain cases; (2) exclusion of clinically suspected disease relevant to cessation of therapy, and (3) detection/exclusion of drug-resistant phenotypes. Unexpectedly, we found expression of plasma cell-associated antigen in testicular germ cells, which may prove to be diagnostically useful in the future evaluation of germ cell tumors.