病毒相关性噬血细胞综合征1例报告

患儿 ,男 ,14岁 ,因发热 10ｄ ,浅表淋巴结肿大 3ｄ入院。 10ｄ前无明显诱因出现发热 ,呈稽留高热 ,最高达 4 0℃ ,无寒颤、头痛、抽搐、皮疹等症状。当时查血常规ＷＢＣ 2 .9× 10 9/Ｌ ,Ｎ 0 .2 3,Ｌ 0 .6 0 ,Ｍ0 .17,ＰＬＴ 16 1× 10 9/Ｌ ,门诊给予病毒唑静脉点滴 ,疗效欠佳 ,3ｄ前发现浅表淋巴结肿大。入院查体 :Ｔ 38.5℃ ,急性病容 ,全身皮肤未见黄染及皮疹 ,腋窝及腹股沟均可触及数个肿大淋巴结 ,压痛明显 ,活动度可 ,咽部充血 ,扁桃体Ⅱ度肿大 ,心肺检查未见异常 ,腹平软 ,肝肋下未触及 ,脾肋下约 1ｃｍ ,质中 ,无压痛 ,神经系统…     

EB病毒相关性噬血细胞综合征1例

患儿,女,2岁3个月,因发热1.5个月,面色苍白1个月,牙龈出血2次入院。1.5个月前患儿首次在我院查EBV—IgM( );骨髓报告为增生性贫血骨髓象,予对症处理,无效,近3周出现弛张热,面色苍白进行性加重,自动出院。1周前牙龈出血2次入我院。既往史、个人史、家族史无异常。查体:血压10.5/7.0kPa,神志模糊,面色苍黄,全身轻度水肿,     

小儿EB病毒相关噬血细胞综合征3例报告及文献复习

EB病毒相关噬血细胞综合征 (EBV -AHS)是EB病毒感染诱发组织细胞异常增生并大量吞噬血细胞而引起的一系列临床综合征。其临床特点主要表现为高热、肝脾淋巴结肿大、全血细胞减少和肝功能异常 ,其显著的特征是骨髓涂片或组织中出现体积较大的噬血组织细胞 ,并吞噬血细胞。该文报告 3例并结合文献对本病的发病机制、临床表现、诊断及治疗进行复习。     

噬血细胞综合征1例

患儿 ,女 ,10月 ,发热 4ｄ。 4ｄ前开始无明原因发热 ,体温 37～ 40℃ ,有时呕吐 ,余无异常。既往史、个人史及家族史无异常。查体 :体温 38℃ ,一般状况好 ,面色苍黄 ,巩膜黄染不明显 ,无皮疹 ,双侧耳后、颈部及腹股沟区均可扪及数个淋巴结 ,直径 2～ 6ｍｍ ,活动度好 ,无触痛 ,咽稍红 ,心肺无异常 ,腹软 ,肝肋下 3～ 4ｃｍ ,剑下 2ｃｍ ,质韧 ,表面光滑 ,脾肋下3ｃｍ ,质韧 ,均无触痛 ,余无异常。予对症、支持疗法 ,仍持续发热 ,肝脾进行性增大 ,呕吐加重。血ＷＢＣ(1.0～ 1.5 )×10 9/Ｌ ,Ｎ 0 .41～ 0 .47,Ｌ 0 .34～ 0 .42 ,Ｍ 0 .11～…     

病毒相关性噬血细胞综合征的诊断与治疗

目的　提高对病毒相关性噬血细胞综合征的认识。方法　回顾性分析 1 6例病毒相关性噬血细胞综合征患儿的临床资料。结果　临床表现为持续性高热 ,肝脾大 ,血常规二系或三系减少 ;高三酰甘油血症、血清铁蛋白 (SF)增高 ;纤维蛋白原降低 ,凝血功能障碍 ;CD4/CD8下降 ,非杀伤细胞比例降低 ,高细胞因子血症。骨髓中找到噬血细胞。大剂量人血丙种球蛋白、甲泼尼龙治疗后 ,好转 7例 ,死亡 5例 ,4例放弃治疗。结论　早期诊治是提高病毒相关性噬血细胞综合征患儿生存率的关键。     

EB病毒相关噬血细胞综合征并重症肝炎1例报告

目的探讨EB病毒相关噬血细胞综合征的临床与病理特征。方法回顾性分析1例EB病毒相关噬血细胞综合征并重症肝炎死亡病例的临床资料,复习相关文献。结果患儿1岁2个月,有发热、血细胞减少、纤维蛋白原、自然杀伤细胞下降、血清铁蛋白及三酰甘油升高;血涂片异常淋巴细胞占5%;EBV-DNA荧光定量检测1.39×108拷贝/ml;尸检结果提示死亡原因为多脏器功能衰竭;肝脏组织行EB病毒编码RNAs(EBERs)原位杂交检测,约30%淋巴细胞中EBERs阳性,肝细胞EBERs阴性。结论该例患儿确认EB病毒相关噬血细胞综合征,累及多器官,其中肝损伤并非EB病毒直接感染所致。     

细小病毒B19相关性噬血细胞综合征一例

细小病毒Ｂ１９相关性噬血细胞综合征一例焦西英钱新宏张国成李佐华患儿男，４岁。因不规则发热３周，伴进行性面色苍白１０天入院。体检：一般情况较差，体温３９．２℃，双下肢散在瘀点，无皮疹及黄染。颈部、腋下、腹股沟等处均可触及２～３个肿大的淋巴结（２ｃｍ×３...     

Outcomes of Pneumocystis jiroveci pneumonia infections in pediatric heart transplant recipients

PJP is known to cause significant morbidity and rarely death in immunosuppressed patients. The prevalence and outcomes of PJP in pediatric solid-organ transplant patients are not well established. This study utilizes data from the PHTS to establish the prevalence and outcome of PJP in pediatric heart transplant recipients. We conducted a retrospective cohort study using data from the PHTS, including data from 24 institutions between January 1, 1993, and December 31, 2004. Infections that occur in PHTS subjects are recorded in a standardized data collection form. The prevalence and outcomes of PJP in pediatric heart transplant recipients were determined. There were a total of 18 patients (1%) with PJP out of the 1854 pediatric heart transplant recipients in the PHTS database. A majority of PJP occurred two months to two yr post-transplant, and patients with PJP had a significantly decreased mortality compared with other fungal infections. PJP is an infrequent complication experienced by pediatric heart transplant recipients. Patients that have experienced PJP have an increased survival compared to patients with other fungal infections, and most PJP occurred within two yr of transplant.     

The use of intravenous pentamidine for the prophylaxis of Pneumocystis pneumonia in pediatric patients

Pneumocystis jiroveci pneumonia was common in the immunocompromised host before the widespread use of prophylaxis. When trimethoprim–sulfamethoxazole is not tolerated, prophylaxis with intravenous pentamidine (IVP) may be initiated. We performed a retrospective analysis of all pediatric patients who received IVP regarding efficacy, safety, and reason for initiation. Of 106 patients included in our analysis, one patient tested positive for Pneumocystis DNA. Adverse events were reported in 18% of IVP courses, and main reason for initiation was cytopenia (59%). We found IVP to be effective and safe, and recommend the use of IVP in pediatric patients in whom first‐line prophylaxis is contraindicated.     

Hemophagocytic syndrome and acute liver failure associated with ethylene glycol ingestion: a case report

The authors describe a case of accidental ethylene glycol poisoning in an 18-month-old boy who developed hemophagocytic syndrome (HPS). Ethylene glycol is a common substance in various antifreeze preparations. Acute ethylene glycol intoxication is a medical emergency that, if not diagnosed correctly and treated aggressively, will lead to serious neurological, cardiopulmonary, and renal dysfunction, and may result in death. The taking of a detailed history, physical examination, and laboratory testing are essential for diagnosis. To the best of the authors' knowledge this is the first case in the literature of a subject who developed HPS after ethylene glycol intoxication.     

Pneumocystis carinii pneumonia: Pitfalls of prophylaxis

Pneumocystis carinii pneumonia (PCP) occurs commonly in immunocompromised patients. Sulfamethoxazole-trimethoprim (SMX-TMP) is effective prophylaxis, although PCP may still occur despite apparently adequate use. We report three cases of PCP which highlight some of the pitfalls of prophylaxis.     

婴幼儿巨细胞病毒性肺炎49例临床分析

目的探讨小儿巨细胞病毒（CMV）肺炎临床特点及更昔洛韦疗效。方法采用ELISA方法检测血清CMV-IgM,采用荧光定量PCR法检测咽分泌物中的CMV-DNA,对49例CMV肺炎临床特点及应用更昔洛韦治疗效果进行分析。结果49例患儿中CMV-IgM阳性41例,CMV-DNA阳性43例,定量值为（3.203±0.12）×10^4copy/ml;40例正常对照组CMV-IgM阳性3例,CMV-DNA阳性0例。CMV肺炎发病年龄以〈6个月多见（55.1%）,病程多在2周左右,以咳嗽为主要表现,伴或不伴发热,肺部体征可不明显,多为间质性肺炎（77.6%）,有消化系统及神经系统的肺外表现。更昔洛韦治疗总有效率达93.9%。结论对临床表现不典型而又有多系统受累的婴幼儿肺炎应注意CMV肺炎,荧光定量PCR检测CMV-DNA简便易行,特异性强。更昔洛韦治疗CMV肺炎疗效好。     

儿童肾病合并肺孢子菌肺炎3例临床分析

目的 提高对肺孢子菌肺炎(PCP)的认识.方法 回顾分析3 例肾病并发PCP患儿的临床资料.结果 3例患儿的共同特点是,PCP起病急,临床以发热、咳嗽起病,伴有低氧血症,症状与体征不平行;早期临床表现无特异性,易漏诊,并发PCP前患儿均长期或大量应用免疫抑制剂、足量泼尼松口服疗程达8周及以上;免疫功能检查均有不同程度的CD4细胞比例下降.结论 提高对PCP的认识,做到早期诊断;适当控制免疫抑制剂应用、改善患儿生活环境、针对高危人群定期检测CD4细胞水平以及酌情应用复方磺胺甲基异噁唑预防均有利于降低肺孢子菌肺炎的发病率.     

An outbreak of Pneumocystis carinii pneumonia in children with malignancy

An outbreak of Pneumocystis carinii pneumonia (PCP) in three patients within a 6 week period was reported. Two patients had acute lymphoblastic leukaemia and one had brain-stem glioma. They shared common features of immuno-suppression and absence of cotrimoxazole prophylaxis and had been nursed in the same room. The severity of PCP and its response to treatment may be related to the degree of immunosuppression. Because of the morbidity and mortality of PCP, chemoprophylaxis should be given to all at-risk cases. Furthermore, isolation of patients with PCP should be considered in view of increasing evidence of nosocomial transmission.     

Hemophagocytic lymphohistiocytosis associated with visceral leishmaniasis: a case report

Leishmania-associated hemophagocytic lymphohistiocytosis is a rare clinicopathological entity. This condition is often difficult to diagnose, so treatment is often delayed. This report describes the case of a 5-year-old boy who was admitted with fever of 1 month's duration, hepatosplenomegaly, and pancytopenia. Serum testing showed elevated transaminase levels, hypertriglyceridemia, hyperferritinemia, and normal fibrinogen level. Hemophagocytic lymphohistiocytosis was diagnosed on bone marrow examination. The patient was tested for various infectious agents. He was negative for all except Leishmania, which was detected by indirect fluorescent antibody testing. Treatment with amphotericin B resulted in a dramatic resolution of all signs and symptoms within 1 week.