新生儿真菌败血症22例临床分析

目的分析新生儿真菌败血症的临床特点、病原菌情况及相关因素,探讨其防治措施。方法对2005年7月至2009年9月中南大学湘雅二医院22例新生儿真菌败血症的临床资料、病原菌及药敏结果、相关因素及治疗预后情况进行回顾性研究。结果 (1)新生儿真菌败血症发生率为0.64%,占新生儿败血症的13.17%,且以医院获得性感染为主。(2)病原菌主要为念珠菌(86.4%),包括白色念珠菌、近平滑念珠菌和热带念珠菌等,药敏结果显示除1株对伊曲康唑耐药,其余对5-氟胞嘧啶、两性霉素B、氟康唑、伊曲康唑和伏立康唑均敏感。(3)早产、低出生体重、长期留置中心静脉导管、气管插管、激素使用及长疗程广谱抗生素联合使用为真菌感染的高危因素,真菌感染与院内感染具有较高的相关性。结论新生儿真菌败血症病原菌以念珠菌为主,应加强护理、严格无菌操作、合理使用激素和抗生素以及实施预防性和早期经验性抗真菌治疗,减少真菌败血症及其并发症的发生。     

儿童急性白血病合并中枢神经系统真菌感染5例并文献复习

目的通过5例儿童急性白血病合并中枢神经系统(CNS)真菌感染的临床总结及相关文献复习,提高对本病的认识。方法回顾性分析北京儿童医院收治的5例急性白血病合并CNS真菌感染患儿的临床特点及诊治经过并文献复习。结果(1)4例急性淋巴细胞白血病(ALL)患儿均接受强化疗,1例为急性非淋巴细胞白血病(AML-M2)半相合造血干细胞移植术后,发生CNS真菌感染前均有肺部真菌感染病史,其中2例合并肝、脾、肾真菌感染,1例合并下肢皮肤真菌感染(2)实验室检查3例有病原学依据,1例烟曲霉菌,2例念珠菌,2例无病原学依据。(3)影像学5例患儿均作头颅CTMRI,表现脓肿、梗塞、颅内出血。(4)治疗及预后1例患儿伊曲康唑+脂溶性两性霉素B治疗,2例患儿伏立康唑治疗,2例患儿伏立康唑和两性霉素B联合抗真菌治疗,抗真菌治疗均有效,4例存活,1例死于严重移植物抗宿主病(GVHD)。结论儿童急性白血病合并CNS真菌感染为侵袭性真菌感染的表现形式之一,多半有其他脏器感染,临床表现不典型,可有脑膜炎或脑脓肿症状和体征,诊断困难,CT、MRI影像学检查重要,伏立康唑、两性霉素B抗真菌治疗有效。     

童急性白血病合并中枢神经系统真菌感染5例并文献复习

目的 通过5例儿童急性白血病合并中枢神经系统(CNS)真菌感染的临床总结及相关文献复习,提高对本病的认识.方法 回顾性分析北京儿童医院收治的5例急性白血病合并CNS真菌感染患儿的临床特点及诊治经过并文献复习.结果 (1)4例急性淋巴细胞白血病(ALL)患儿均接受强化疗,1例为急性非淋巴细胞白血病(AML-M2)半相合造血干细胞移植术后,发生CNS真菌感染前均有肺部真菌感染病史,其中2例合并肝、脾、肾真菌感染,1例合并下肢皮肤真菌感染(2)实验室检查3例有病原学依据,1例烟曲霉菌,2例念珠菌,2例无病原学依据.(3)影像学5例患儿均作头颅CT MRI,表现脓肿、梗塞、颅内出血.(4)治疗及预后1例患儿伊曲康唑+脂溶性两性霉素B治疗,2例患儿伏立康唑治疗,2例患儿伏立康唑和两性霉素B联合抗真菌治疗,抗真菌治疗均有效,4例存活,1例死于严重移植物抗宿主病(GVHD).结论 儿童急性白血病合并CNS真菌感染为侵袭性真菌感染的表现形式之一,多半有其他脏器感染,临床表现不典型,可有脑膜炎或脑脓肿症状和体征,诊断困难,CT、MRI影像学检查重要,伏立康唑、两性霉素B抗真菌治疗有效.     

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目的探讨血液病患儿侵袭性真菌感染联合药物治疗组合及疗效。方法 2006年1月至2011年4月在中山大学孙逸仙纪念医院就诊的血液病合并侵袭性真菌感染并予以联合治疗患儿39例,对患儿的临床资料及治疗效果进行回顾性分析。结果联合抗真菌药物治疗者共48例次,其中拟诊11例次,临床诊断32例次,确诊5例次。两性霉素B联合卡泊芬净18次,无效3次;两性霉素B联合伊曲康唑静脉13次,口服4次,均有效;两性霉素B联合伏立康唑9次,无效1次;卡泊芬净联合伊曲康唑6次,无效1次;卡泊芬净联合伏立康唑5次,均有效;伊曲康唑联合制霉菌素1次,有效。完全缓解27例,部分缓解7例,放弃1例,恶化死亡4例。三唑类联合两性霉素B或卡泊芬净与两性霉素B联合卡泊芬净相比,效果差异无统计学意义(P>0.05)。结论 免疫功能明显抑制、既往侵袭性真菌感染且再次接受强化疗或造血干细胞移植、严重或突破性真菌感染、单药治疗失败或毒副反应大、合并移植物抗宿主病患儿的联合治疗疗效较满意。三唑类联合两性霉素B或卡泊芬净与两性霉素B联合卡泊芬净均可作为联合用药选择。     

ICU患儿肺部真菌感染的危险因素分析及对策

目的 分析重症监护病房患儿肺部真菌感染的危险因素,探讨控制对策及治疗措施.方法 采用回顾性调查方法对2003年1月--2007年2月PICU收治的1 057例危重症患儿进行调查,对肺部真菌感染率、感染相关危险因素、病原学特点及预防控制、治疗效果进行分析.结果 发生肺部真菌感染48例,PICU肺部真菌感染发生率为4.54%,肺部真菌感染的相关危险因素主要有住院时间长、短期内反复住院、使用广谱抗生素、肾上腺皮质激素及免疫抑制剂的应用、机械通气及溺粪.病原菌主要有白色念珠菌、光滑念珠菌、热带念珠菌、克柔念珠菌、曲霉菌、毛霉菌等.经抗真菌治疗治愈28例(58.3%),显效9例(18.8%),无效10例(20.8%),放弃治疗1例(2.1%).结论 PICU患儿肺部真菌感染的发生与多种易感因素有关;合理使用抗生素、慎用.肾上腺皮质激素、加强消毒隔离措施等是降低PICU患儿发生肺部真菌感染的关键;认识患儿发生真菌感染的易感因素、早期诊断、合理使用抗真菌药,是治愈儿童肺部真菌感染的关键.     

儿童急性白血病化疗合并真菌感染探讨

目的探讨儿童急性白血病化疗合并真菌感染的因素及其感染病原学特点。方法白血病并发真菌感染按EORTC诊断标准。分析真菌感染率、感染部位、感染相关危险因素、病原菌特点。结果共17例白血病患儿21次并发真菌感染,急性淋巴细胞性白血病(ALL)13例,急性非淋巴细胞性白血病(AML)4例;合并真菌感染率为27.6%。感染部位主要位于上呼吸道(61.9%)。病原菌培养均示浅表真菌,如白色念珠菌、酵母样菌。化疗阶段是白血病患儿合并真菌感染的重要因素(P<0.05),免疫功能和中性粒细胞绝对值在临床上亦是主要的影响因素。结论白血病患儿合并真菌感染与疾病本身、机体免疫功能低下、中性粒细胞绝对值和化疗阶段相关。因此,免疫支持、早期诊断、及时治疗颇为重要。     

伊曲康唑预防急性白血病患儿侵袭性真菌感染的临床研究

目的：评价伊曲康唑口服液在急性白血病（AL）患儿化疗后粒细胞缺乏伴感染时侵袭性真菌感染（IFI）预防性治疗的效果。方法回顾性分析2009年6月-2013年6月我院收集的AL化疗后伴粒细胞缺乏的213例患儿,将其分为伊曲康唑早期预防组和晚期预防组。伊曲康唑早期预防组（330例次）在粒细胞缺乏伴发热应用广谱抗生素同时服用伊曲康唑口服液;对照组（191例次）在粒细胞缺乏出现发热、经广谱抗生素应用72 h 后发热无改善者加用伊曲康唑口服液,比较两组患儿IFI发生率。结果伊曲康唑早期预防组和对照组各有7例和18例发生IFI,发生率分别为2.12％和9.42％,两组IFI发生率差异有显著性（P＜0．01）。结论 AL化疗后粒细胞缺乏患儿在发热早期进行预防性口服伊曲康唑可以有效降低IFI的发生率。     

急性淋巴细胞白血病患儿首次诱导缓解化疗合并肠梗阻的临床分析

目的探讨儿童急性淋巴细胞白血病(ALL)首次VDLP诱导缓解化疗合并肠梗阻的发生情况、影响因素。方法收集2009年5月-2017年3月在安徽医科大学第二附属医院儿科住院并完成首次诱导缓解化疗的新发ALL患儿共246例,比较合并肠梗阻组与非肠梗阻组患儿的感染发生情况、伊曲康唑使用情况,并进行卡方检验及相关性分析,讨论肠梗阻发生的影响因素。结果246例ALL中,合并肠梗阻者共43例(17.48%),其中39例梗阻前1周内合并感染并静脉使用广谱抗菌药,有34例使用伊曲康唑口服液预防真菌感染;未合并肠梗阻者共203例,其中合并感染者133例,有89例采用伊曲康唑口服液预防真菌感染,经统计学分析,梗阻前合并感染及应用伊曲康唑与肠梗阻发生存在相关性(P0.05)。结论 VDLP方案化疗期间,合并感染组的肠梗阻发生率(22.67%)高于非感染组(5.41%),如需应用预防性抗真菌治疗,因伊曲康唑口服液可增加肠梗阻发生风险,应尽量避免使用。     

伏立康唑治疗白血病患儿肺部真菌感染10例临床分析

目的探讨伏立康唑治疗白血病患儿肺部真菌感染的疗效及安全性。方法对10例急性白血病合并肺部真菌感染的患儿给予伏立康唑治疗,静脉滴注负荷量6 mg/kg,1次/12 h,1 d后改为维持量4 mg/kg,2次/d,疗程6～8周。观察治疗效果,总结相关临床资料。结果痊愈3例,显效4例,进步3例;转氨酶升高2例,低钾血症1例。结论伏立康唑治疗白血病患儿肺部真菌感染疗效较满意、安全,值得临床应用。     

伊曲康唑治疗儿童肺部真菌感染临床疗效及安全性观察

目的评价伊曲康唑注射液治疗儿童肺部真菌感染的有效性和安全性。方法以2004年5月~2005年10月武汉市儿童医院ICU病房13例确诊为肺部真菌感染患儿为研究对象,所有患儿在停用抗生素或改用窄谱抗生素、停用肾上腺皮质激素、加强对症和支持治疗基础上,给予伊曲康唑注射液治疗。治疗期间观察患儿临床症状体征变化,反复监测血、尿常规和肝、肾功能;反复取痰液标本作真菌学检查(涂片 培养);定期复查胸片;并记录药物不良反应。结果13例患儿中,治愈7例,有效4例,有效率为84.62%;6例出现轻度不良反应,经停药或对症支持治疗后均恢复正常。结论在我们治疗与观察的病例中,通过对伊曲康唑不良反应用药过程进行合理地预防干预,未见严重不良反应,且抗真菌疗效明显,说明伊曲康唑治疗儿童肺部真菌感染具有良好应用前景。     

Resilience in Families with a Child with Cancer

The aim of this study was to identify and explore resilience factors associated with family adaption after a child had been diagnosed with cancer. Using a cross-sectional survey research design, parents (n = 26), and children (n = 25) from the same families independently completed six self-report questionnaires, as well as responded to an open-ended question about those qualities that helped their family through the period following the diagnosis. The most significant results came from the children's data. According to these results, connectedness within the family, the experience of control over life events, family routines, positive, and supportive communication, redefinition of crisis situations, and lastly, a passive appraisal of crisis situations, were positively linked to better family adaptation. The identified factors should be strengthened and developed in families finding themselves in a similar situation.     

Colonization with Clostridium difficile in Children with Cancer

Objective

Clostridium difficile is a gram-positive, anaerobic, spore-forming bacillus. Usually it does not cause disease unless a patient who is colonized with toxin-producing strains has been treated with antibiotics, particularly those that change the anaerobic flora of the large intestine.

Methods

We investigated in a prospective study intestinal colonization of C. difficile and its toxins in children with malignancy that used different antibiotics and cytotoxic drugs.

Findings

One hundred fifty-two patients were included in this prospective study. Stool samples were obtained within the first 48 hours after admission and cultured for C. difficile; cytopathic effect of C. difficile was detected on HELA cells, also ELISA test was performed for detection of toxins A and B. 25% of patients had positive culture for C. difficile; 36/38 (92%) revealed positive cytopathic effect on HELA cells. No significant relation was found between age, gender, history of antibiotic consumption and C. difficile positive culture and cytopathic effect on HELA cells. The only relation was seen between cotrimoxazol usage and cytopathic effect on HELA cells (P=0.03).

Conclusion

Although the rate of C. difficile colonization (25.6%) and toxigenic strains (23.7%) in admitted children in hematologic ward is high, the rate of ELISA positive test for toxin A+B was not correspond with culture and cytopatic effect on HELA cell. With respect to sensitivity and specificity of ELISA test, possibility for existence of toxin C with cytopathic effect is high in this type of patients.     

Treatment with Probucol of Children with Familial Hypercholesterolemia

ABSTRACT. Nine children with familial hypercholesterolaemia, age range 2 to 12 years, were treated with a low cholesterol diet and probucol (10 mg/kg/day). The year before, the children received, as only treatment, a low fat-cholesterol diet. During this period their mean plasma total cholesterol level fell from 8.2±1.45 mmol/l to 7.17±0.84 mmol/l (12.6%). This level was further reduced to 5.92±0.63 mmol/l (17.1%) after the addition of probucol. Plasma high density lipoprotein cholesterol levels were lowered in absolute terms but not in relation to total cholesterol. No apparent side effects were observed. However, the use of probucol should be restricted for the moment to severe cases of hypercholesterolaemia as the long-term excretion of the drug in children is not yet known.     

Juvenile rheumatoid arthritis with myelofibrosis with myeloid metaplasia

Myelofibrosis with myeloid metaplasia is defined as a myeloproliferative disorder characterized by leukoerythroblastosis, tear drop erythrocytes, extramedullary hematopoesis and varying degree of myelofibrosis. It may be idiopathic or secondary to a large number of conditions. Here is a rare case of myelofibrosis occurring in a patient with juvenile rheumatoid arthritis.     

Therapeutic experience with hepatoblastoma associated with trisomy 18

Trisomy 18 is often fatal, but patients with this disease can now have longer survival due to proactive treatment intervention. However, hepatoblastomas may develop in these patients. In this study, we report four cases of hepatoblastoma associated with trisomy 18. All of the patients had congenital heart disease and three had undergone intracardiac surgical repair. Tumor growth was relatively slow in all cases, and there were no problems with chemotherapy tolerability and surgical resection. Three of the patients are currently disease‐free and the fourth is alive with remaining of the tumor. These cases suggest that combined chemotherapy and surgical resection may be an option to treat hepatoblastoma associated with trisomy 18 when cardiac pulmonary function is relatively stable.     

Tyrosinemia associated with perinatal infection with cytomegalovirus.

A premature infant presented with elevated concentrations of tyrosine in blood and urine, evidence of hepatocellular damage, demineralization of the bones, and a renal Fanconi syndrome. This is the clinical picture found in hereditary tyrosinemia. The infant also had a perinatal infection with cytomegalovirus.