Benign acute childhood myositis following human parainfluenza virus type‐1 infection

Benign acute childhood myositis (BACM) is a post‐respiratory tract infection condition of school‐age children. Presentation is typically with acute onset calf pain and tenderness and refusal to walk or altered gait during the convalescent period of an influenza A or B infection. We describe a unique cluster of children with BACM following infection with human parainfluenza 1 virus, with no evidence of influenza A or B infection. BACM is a commonly missed diagnosis of altered gait in children presenting to the emergency department. This is the first report to describe a cluster of human parainfluenza virus type‐1 associated BACM. We discuss the presentation, clinical examination and investigation results of the children identified. Furthermore, we review the current research surrounding BACM, overview the clinical presentation to healthcare professionals, and present an interesting case of a child presenting for the second time with BACM.     

儿童急性巨核细胞白血病

本研究通过实例分析儿童急性巨核细胞白血病（AMKL）的临床、病理和生物学特征。用骨髓细胞涂片观察细胞的形态;用流式细胞技术和免疫组织化学方法检测肿瘤细胞的免疫表型。结果表明：本例以发热、出血、肝脾淋巴结肿大为主要临床症状,白细胞增多伴两系血细胞减少,骨髓干抽,骨髓原始巨核细胞异常增生超过30%,原始细胞的免疫分型为CD41＋CD61＋。骨髓活检显示,髓内增生的单个核大细胞为CD42b＋。最后诊断为急性巨核细胞白血病。结论儿童急性巨核细胞白血病较为少见,容易误诊,预后不好;免疫分型和免疫组织化学检测有助于该病的早期诊断和预后评估。     

成人与儿童急性淋巴细胞白血病细胞遗传学大样本对比分析

本研究对566例成人急性淋巴细胞白血病（aALL）和586例儿童急性淋巴细胞白血病（cALL）的细胞遗传学进行对比分析。对所有患者均进行了细胞遗传学分析,部分患者进行了FISH检测。结果表明：aALL与cALL的染色体异常存在明显的差异。aALL中异常核型占62．0％,染色体异常较多见的为t（9;22）（q34;q11）、亚二倍体、超二倍体（47—50）、abn（6q）、abn（9p）、-7等,预后不良的占多数。cALL中异常核型占39．2％,染色体异常较多见的为高超二倍体、亚二倍体、TEL／AML1（＋）、＋8、超二倍体（47—50）、＋21等,预后良好的占多数。其中异常核型、总亚二倍体、总超二倍体（47—50）、t（9;22）（q34;q11）、-7、abn（7q）、abn（14q32）、＋Ph在aALL中的发生率明显高于eALL;cAu．正常核型（N）、高超二倍体、＋8、＋21＊2、TEL／AML1（＋）的发生率明显高于aALL。Ph（＋）在aALL中的检出率为20．5％,伴有附加异常的占63．8％。Ph（＋）aALL附加异常中＋Ph、-7、i（9q＋）、9p-、＋8、＋21、＋x、6q-、abn（14q32）、＋14出现的比例较高。Ph（＋）在cALL中的检出率为4．4％,明显低于aALL（P：0．000）,伴有附加异常的占42．3％,附加异常中abn（9p）、abn（7p）、-7、17p-、＋21出现的比例较高。结论：本组病例染色体数目异常及结构异常几乎涉及到每一条染色体,复杂核型多见。aALL与cALL的染色体异常存在明显的差异。     

儿童急性淋巴细胞白血病的预后评估

目前危险指向性治疗使儿童急性淋巴细胞白血病的治愈率接近80％。白血病细胞的分子基因分析和宿主的遗传药理研究是进一步改善预后的基础。评估微小残留病的早期治疗效应以综合反映白血病细胞的药物反应和宿主的遗传药理学，是调整治疗强度最可靠的指标。基因组和蛋白质组学技术有望鉴定个体化治疗的分子靶向。本文综述目前用于儿童ALL预后评估的危险因素及评估系统。     

甲型H1N1流感合并急性呼吸窘迫综合征产妇一例的护理

目的总结1例甲型H1N1流感合并急性呼吸窘迫综合征(acute respiratory distress syndrome,ARDS)产妇的护理工作。方法采用有效的隔离防护措施,结合肺保护性通气治疗和护理,注重患者的心理护理。结果该患者救治成功,康复出院。护理过程中无医护人员受到病毒感染。结论对此类患者采取有效肺部物理治疗、监测呼吸系统功能、做好消毒隔离、加强各方面的护理、重视心理疏导是行之有效的方法。     

儿童急性髓系白血病DNMT3a基因突变的分析

随着新一代基因测序技术的发展,越来越多与肿瘤发生发展有关的基因被发现,比如最近发现的DNMT3a基因。此基因为急性髓系白血病预后判断提供了新的分子生物学标记。本研究探讨儿童急性髓系白血病患儿骨髓中DNMT3a基因的突变情况。选取就诊于中国医学科学院血液病医院儿童血液病诊治中心的57例儿童急性髓系白血病患儿,通过PCR直接扩增其骨髓细胞中DNMT3a基因全部23个外显子,并通过直接测序方法与野生型DNMT3a基因比对,检测DNMT3a基因的突变情况。结果表明,在57例患儿中未发现DNMT3a突变热点R882位点的突变,进一步分析其它位点也未发现任何突变。而在这些患儿中AML1/ETO突变10例,CBFB/MYH11突变3例,PML/RARa突变13例,FLT3/ITD突变5例,FLT3/TKD突变1例,既含有PML/RARa又含有FLT3/TKD突变2例。结论:DNMT3a基因虽然在成人急性髓系白血病患者中有着较高的突变率,且是预后不良的一个独立因素,但其突变率在儿童患者中很低,甚至没有突变。这提示儿童和成人急性髓系白血病的发生可能存在不同机制。     

Electrocardiogram Differentiation of Benign Early Repolarization Versus Acute Myocardial Infarction by Emergency Physicians and Cardiologists

Objectives: ST‐segment elevation (STE) related to benign early repolarization (BER), a common normal variant, can be difficult to distinguish from acute myocardial infarction (AMI). The authors compared the electrocardiogram (ECG) interpretations of these two entities by emergency physicians (EPs) and cardiologists. Methods: Twenty‐five cases (13 BER, 12 AMI) of patients presenting to the emergency department with chest pain were identified. Criteria for BER required four of the following: 1) widespread STE (precordial greater than limb leads), 2) J‐point elevation, 3) concavity of initial up‐sloping portion of ST segment, 4) notching or irregular contour of J point, and 5) prominent, concordant T waves. Additional BER criteria were 1) stable ECG pattern, 2) negative cardiac injury markers, and 3) normal cardiac stress test or angiography. AMI criteria were 1) regional STE, 2) positive cardiac injury markers, and 3) identification of culprit coronary artery by angiography in less than eight hours of presentation. The 25 ECGs were distributed to 12 EPs and 12 cardiologists (four in academic medicine, four in community practice, and four in community academics [health maintenance organization] in each physician group). The physicians were informed of the patients' age, gender, and race, and they then interpreted the ECGs as BER or AMI. Undercalls (AMI misdiagnosed as BER) and overcalls (BER misdiagnosed as AMI) were calculated for each physician group. Results: Cardiologists correctly interpreted 90% of ECGs, and EPs correctly interpreted 81% of ECGs. The proportion of undercalls (missed AMI/total AMI) was 2.8% for cardiologists (95% confidence interval [CI] = 0.09% to 5.5%) compared with 9.7% for EPs (95% CI = 4.8% to 14.6%) (p = 0.02). The proportion of overcalls (missed BER/total BER) was 17.3% for cardiologists (95% CI = 11.4% to 23.3%) versus 27.6% for EPs (95% CI = 20.6% to 34.6%) (p = 0.03). The mean number of years in practice was 19.8 for cardiologists (95% CI = 19 to 20.5) and 11 years for EPs (95% CI = 10.5 to 12.0) (p < 0.001). Conclusions: Although correct interpretation was high in both groups, cardiologists, who had significantly more years of practice, had fewer misinterpretations than EPs in distinguishing BER from AMI electrocardiographically.     

应用MALDI-TOF-MS法筛选儿童急性淋巴细胞白血病潜在标记物

本研究旨在分析儿童急性淋巴细胞白血病（c-ALL）的肿瘤特异性蛋白,寻找新的肿瘤蛋白分子标志物。应用双向凝胶电泳（2-DE）及基质辅助激光解吸电离飞行时间质谱分析（MALDI-TOF-MS）及联网检索数据库,鉴定表达上调的差异蛋白,并应用细胞免疫组织化学法验证相关蛋白。结果表明,获得了4个表达上调的蛋白点,经质谱分析初步鉴定出其中2个表达上调的差异蛋白为谷胱甘肽S转移酶P和阻殖蛋白;细胞免疫组织化学法检测发现,二者在儿童ALL细胞内的表达阳性率明显高于对照组,差异具有统计学意义（P〈0．05）。结论：谷胱甘肽S转移酶P和阻殖蛋白有望成为儿童ALL新的诊断标志物和药物靶点。     

儿童急性淋巴细胞白血病个体化治疗现状

急性淋巴细胞白血病（ALL）是儿童时期发病率最高的恶性肿瘤,是由于造血干细胞增殖分化异常而引起的恶性血液病。目前,儿童ALL的治疗效果明显改善,行之有效的方法主要是个体化治疗。对于标危ALL,降低了化疗强度从而减轻化疗药物的副作用,只有对真正属于高危型的ALL才进一步加强化疗,且应根据其不同的生物学特征采用不同的治疗手段。对于复发型ALL,即使加强化疗或换用新药,其预后仍旧较差,可采取分子靶向治疗或造血干细胞移植。此外,近年来微小残留病检测技术及药物基因组学研究的发展在一定程度上有助于评价化疗药物敏感性并判断预后,也为ALL个体化治疗提供了可靠的客观依据。本文就目前儿童ALL个体化治疗现状进行综述。     

人感染H7N9禽流感病毒危重症患者的救治与护理

目的探讨人感染H7N9禽流感病毒危重症患者的救治与护理方法。方法回顾性分析并总结2013年3-4月南京大学医学院附属鼓楼医院重症医学科收治的6例人感染H7N9禽流感病毒危重症患者的临床资料。结果 6例人感染H7N9禽流感病毒危重症患者中,4例病情稳定已出院,其余2例生命体征平稳进入康复期;在6例患者的治疗和护理过程中,无医护人员受到H7N9禽流感病毒感染。结论高质量的专科护理是人感染H7N9禽流感病毒危重症患者抢救成功的保证。     

重型甲型H1N1流感患者T淋巴细胞亚群变化分析

[目的]探讨重型甲型H1N1流感患者T淋巴细胞亚群的动态变化.[方法]采用流式细胞仪对21例重型甲型H1N1流感患者(重症组病例12例,危重组病例9例)病程初期(入院24 h)、病程中期(入院后10 d)、恢复期(入院后30 d)外周血T淋巴细胞亚群CD3+、CD4+、CD8+进行检测和统计分析.[结果]两组患者病程初...     

甲型流感病毒肺炎的影像学特征分析

[目的]探讨甲型流感病毒肺炎的高分辨率CT 影像特征.[方法]回顾性分析临床诊断甲型流感病毒肺炎患者10例CT图像,分析CT表现形态（实变、磨玻璃影、结节、网格状改变）、病变分布、淋巴结肿大、胸腔积液、病变转归等.[结果]10例患者均为双侧肺部受累,其中周围型8例（80%）、周围＋中央型2例（20%）;双侧对称性分布7例（70%）,双侧不对称分布3例（30%）;磨玻璃影10例（100%）,同时合并实变影5例（50%）,合并网格状改变3例（30%）,4 例（40%）病人出现胸腔积液;本组病例均未见明显肿大的淋巴结;10例患者经治疗8例（80%）病变范围缩小,2例（20%）病变范围进展.[结论]与既往流感相关的病毒性肺炎对比,本组甲型流感病毒肺炎起病急、病程进展迅速和严重,首发CT表现以双肺弥漫分布的磨玻璃影为主,恢复过程中出现了不同程度的纤维化,对抗病毒药物和糖皮质激素治疗有较好反应.     

Influenza A in Young Children with Suspected Respiratory Syncytial Virus Infection

OBJECTIVES: To determine the prevalence of influenza A in young children suspected of having respiratory syncytial virus (RSV) infection and to compare the clinical presentation of these patients with those who have proven RSV infection. METHODS: Children younger than or at 36 months of age who presented to a pediatric emergency department (ED) with suspected RSV infection during the influenza A season of 2001-2002 were eligible. Eligible children had an RSV antigen test ordered as part of their initial clinical management. A consecutive sample of children was enrolled for prospective observational analysis. The main outcome measure was the prevalence of influenza A in young children with suspected RSV infection. The secondary outcome measure was a comparison of the clinical presentations, of the two groups. RESULTS: During the study period, 420 patients presented for evaluation of respiratory illness. RSV tests were ordered on 251 patients. Of 197 eligible patients, 124 (63%) tested positive for RSV and 33 (17%) for influenza A. Influenza A patients were more likely to have temperatures at or above 39 degrees C than RSV patients (36% vs. 15%; p = 0.01). RSV patients were more tachypneic (54 vs. 43 breaths/minute; p < 0.0001) and more often had wheezing (90% vs. 8%; p < 0.0001). Twenty influenza patients (61%) were hospitalized. CONCLUSIONS: This study found a high prevalence of influenza A in young children suspected of having RSV infection. Clinicians should consider influenza A in young febrile children presenting with respiratory illnesses.     

肾移植术后肺部感染致急性肺损伤/急性呼吸窘迫综合征的诊治体会

【目的】进一步提高肾移植术后肺部感染致急性肺损伤(ALI)/急性呼吸窘迫综合征患者(ARDS)的临床诊疗水平。【方法】回顾性分析总结20例肾移植术后肺部感染致ALI/ARDS患者的临床资料及诊治情况。【结果】16例患者找到病原体28株,4例未找到确切的病原体;20例患者中,16例(80%)治愈,2例死亡,2例病情严重患者因经济困难自动出院;其中6例通过高流量面罩吸氧后缺氧情况改善,8例行无创机械通气治愈,2例行有创机械通气治愈。【结论】正确应用机械通气治疗对于提高肾移植术后肺部感染致ALI/ARDS患者的治愈率有特殊重要的作用。     

多参数流式细胞术动态监测儿童B系急性淋巴细胞白血病微量残留病的临床意义

摘要本研究旨在探讨儿童B系急性淋巴细胞白血病（B-ALL）化疗过程中监测不同时间点微量残留病（MRD）水平的临床意义。回顾性分析我院2008年8月至2011年9月以流式细胞术监测3个时间点（即诱导化疗第15天、第33天和治疗第12周）的206例B-ALL患儿骨髓的MRD。结果表明：①206例B-ALL患儿中196例诱导化疗后迭完全缓解（CR）（CR率95．1％）,其1年与3年无事件生存（EFS）率分别为（92．7±1．8）％和（78．7±3．7）％,其中标危、中危、高危组的3年EFS率分别为（85．6±4．9）％、（82．1±5．8）％和（58．1±9．2）％,3组比较有明显统计学差异（P〈0．001）;②各时间点上MRD分析显示：MRD水平越高,患儿的3年EFS率越低,其中第15天MRD≥10^-2组、第33天和第12周MRD≥10^-3组的患儿预后明显不佳;多因素分析显示,第12周MRD≥10^-3为独立的不良预后因素;第12周MRD〈10^-3的3年EFS率为（86．3±4．1）％,MRD≥10^-3的3年EFS率为（55．8±9．1）％,差异有统计学意义（P〈0．05）;③在化疗第33天MRD阴性（MRD〈10^-4）的98例患儿中有8例复发（复发率为8．2％）,在这98例惠儿中有39例在第12周时转为阳性（MRD≥10^-4）,其中5例复发;在第12周时MRD仍为阴性的59例中有3例复发;在第33天MRD阳性的108例患儿中有19例复发（复发率为17．6％）,复发率在MRD阳性和阴性两组间差异有统计学意义（P〈0．05）。结论：B-ALL患儿治疗过程中以流式细胞术动态监测MRD可有效地评估治疗反应、判断预后、预测复发及指导化疗方案的调整,其中第15天、第33天及第12周的MRD分别以10^-2、10^-3、10^-3为区分危险度最佳界值,第12周MRD≥10^-3为独立的不良预后因素。     

急性早幼粒细胞白血病合并颅咽管瘤

本研究分析急性早幼粒细胞白血病（acutepromyelocyticleukernia,APL）合并颅咽管瘤的临床表现及治疗方案,提高对APL合并或继发第二肿瘤的认识。应用骨髓细胞形态学检查对APL进行确诊;应用免疫分型技术对骨髓中白血病细胞进行分析;应用分子生物学检测PML—RARa融合基因以定性及确定治疗前后定量变化;应用常规染色体核型技术及HSH技术以分析该患者细胞遗传特点。结果表明,急性早幼粒细胞白血病合并或继发第二肿瘤的临床表现多样,病情复杂。通过早期发现、及时治疗、多学科合作取得了良好的疗效,避免了误诊、漏诊延误诊治。结论：当急性早幼粒细胞白血病经过治疗缓解后,再出现其它临床症状时需考虑其他肿瘤的可能,临床上需仔细观察临床表现,早期诊断,及时治疗,以提高患者的生存率。     

Acute Human Immunodeficiency Virus (HIV) Infection Presenting with Fever,Elevated Amylase/Lipase,and Hematologic Abnormalities

Background

Many cases of acute human immunodeficiency virus (HIV) infection (AHI) present to the Emergency Department (ED). Early diagnosis allows a patient to modify their behavior and seek early treatment. The Emergency Physician should know that the antibody response may be absent.

Objective

We report a case of AHI and review its presentation to increase the awareness of this important diagnosis.

Case Report

A 20-year-old black man who had a history of sex with men initially presented to the ED with a chief complaint of blood per rectum when he passed stool, and chills for the prior few days. His work-up was normal, including a rapid HIV screen, and he was discharged. He returned 2 weeks later with constipation, fatigue, myalgias, decreased urination, chills, and a productive cough. His physical examination was unremarkable, HIV antibody test was negative, but his laboratory tests showed an elevation of creatine phosphokinase, amylase, and lipase. His blood count showed a normal hematocrit and white blood cell count, but there were juvenile and vacuolated white cells and giant platelets reported. HIV viral load was reported as > 1,000,000 copies/mL.

Conclusions/Summary

This case illustrates that AHI can present as a non-specific illness. Patients at risk should be screened for HIV. However, AHI can occur before there is a significant antibody response. In such cases, a viral load test may help make the diagnosis, allowing for early treatment and patient counseling.     

5例甲型H1N1流感危重患者救治

目的 探讨甲型H1N1流感危重症患者的抢救疗效.方法 2009年10月-2010年1月,对我院ICU收治5例甲型H1N1流感危重症患者采用集束化治疗方案进行救治,并就患者的器官功能变化进行评价.结果 5例患者平均住院天数为(11.8±5.59)d,4例存活,1例死亡.存活患者在治疗过程中急性生理和既往健康(APACHE...     

Posterior Reversible Encephalopathy Syndrome: A Rare Childhood Case with Unconsciousness

Background

Posterior reversible encephalopathy syndrome (PRES) is a condition characterized by seizures, altered consciousness, visual disturbances, and headache. Characteristic findings on neuroimaging include cerebral edema, typically involving the parieto-occipital white matter. PRES has been associated with hypertension, autoimmune disease, and Henoch-Schölein purpura (HSP), but few cases have been reported, and fewer cases of PRES have been reported in children.