儿童脾脓肿1 例报告及文献复习

目的总结儿童脓毒症并发脾脓肿的临床特点及治疗。方法回顾分析1例脓毒症并发脾脓肿患儿的临床资料,检索中国知网、万方数据知识服务平台、PubMed中的相关文献并进行总结分析。结果男性患儿,11月龄,以反复发热为主要表现。血培养示屎肠球菌感染,抗感染治疗效果不理想。腹部超声及磁共振成像示脾脏多发脓肿。经超声引导下脓肿抽液后患儿仍发热,最终行脾脓肿切除术。手术1周后患儿体温恢复正常。共检索到国内外相关文献15篇,共59例儿童脓毒症并发脾脓肿病例。临床主要表现为发热、咳嗽、腹痛或腹胀,4例有基础疾病;其中血培养阳性12例,4例为球菌阳性,8例为杆菌阳性。59例患儿均接受抗感染治疗,2例同时行抗结核治疗,1例行脾穿刺,5例行脾切除术。55例患儿预后良好,2例好转,2例病情反复。结论儿童脓毒症并发脾脓肿以发热为主要表现,部分患儿有基础疾病,以足量、足疗程抗感染治疗为主,必要时需行脾脓肿切除术。     

儿童急性淋巴细胞白血病化疗后并发脓毒症临床分析

目的探讨儿童急性淋巴细胞白血病(ALL)化疗后并发脓毒症的临床及病原学特点。方法回顾性分析2008年4月至2014年12月诊断为ALL并以CCLG-ALL 2008方案化疗后并发血培养阳性脓毒症患儿的临床及病原学资料。结果 545例诊断ALL并以CCLG-ALL2008方案治疗的患儿中112例(145例次)化疗后并发脓毒症,发生率为20.55%。标危和中危组患儿脓毒症高发于诱导缓解治疗阶段,高危组患儿脓毒症高发于巩固治疗阶段。最常见感染部位是呼吸道。血培养病原菌以革兰阳性菌居多。革兰阳性菌对万古霉素、利奈唑胺、替考拉宁敏感率高,革兰阴性菌对美罗培南、亚胺培南敏感率高。高危型、中心静脉置管、中性粒细胞计数0.1×109/L及中性粒细胞缺乏持续时间7 d者脓毒症发生率显著升高(P0.001)。112例患儿治疗有效率95.17%,脓毒症相关病死率1.28%。结论脓毒症是ALL患儿化疗后的重要并发症及死亡原因之一。     

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。     

15例血液肿瘤患儿化疗后感染合并急性呼吸窘迫综合征回顾性分析

目的分析探讨血液肿瘤患儿化疗后感染合并急性呼吸窘迫综合征(ARDS)的临床特点、治疗及预后情况。方法回顾性分析2014年1月—2018年12月的15例化疗后感染合并ARDS患儿的病例资料、实验室检查、治疗效果及预后情况。结果 (1) 15例患儿化疗后感染:肺炎11例,脓毒症2例,脓毒症伴肺炎2例;(2)实验室检查:病原学阳性7例(细菌感染2例,真菌感染2例,病毒感染3例),CRP> 30 mg/L的患儿10例;(3) 15例患儿确诊ARDS时均在粒细胞上升期,其中13例粒细胞缺乏;(4)患儿轻、中、重度ARDS分别为5例、7例、3例; 1例行无创机械通气,2例行有创机械通气,死亡4例。结论化疗后骨髓抑制合并ARDS患儿均有肺炎或脓毒症的诱因,ARDS在中性粒细胞开始恢复时发生,停用G-CSF并积极治疗原发病,大多数患儿ARDS症状很快得到改善。     

儿童急性白血病合并脓毒症69例分析

目的探讨儿童白血病治疗中出现脓毒症(败血症)的原因及治疗对策。方法对白血病患儿化疗后出现血培养阳性的病原学、临床表现、相关因素、治疗方法与疗效进行分析。结果824例白血病患儿全身炎症反应综合征的总发生率为13.6%(69/824)。确诊脓毒症8.37(69/824)。结论急性淋巴细胞白血病化疗中脓毒症的好发阶段是诱导缓解及再诱导缓解阶段;病原菌以革兰氏阴性杆菌多见。常见的感染途径是消化道与呼吸道。脓毒症的发生与粒细胞绝对值负相关,与粒细胞缺乏,持续时间及化疗强度呈正相关。早期对粒细胞缺乏伴发热的治疗可降低脓毒症的并发症及死亡率。     

儿童急性白血病合并败血症56例分析

目的：探讨儿童急性白血病（AL）治疗中合并败血症的原因及治疗方法。方法2011年1月至2014年7月入住安徽医科大学第二附属医院儿科的血培养阳性的AL患儿56例，分析其发病因素、病原学、耐药情况、治疗方法及疗效。结果56例患儿中，急性淋巴细胞白血病32例，急性非淋巴细胞白血病22例，混合表型急性白血病（淋髓混合型）2例。常见感染途径依次是消化道、呼吸道、口腔，高发时间为诱导化疗阶段。中性粒细胞计数＜0.5×109／L持续时间一周以上患儿败血症发生率显著升高。血培养病原菌以革兰阴性杆菌多见，前三位分别为大肠埃希氏菌、肺炎克雷伯杆菌、铜绿假单胞菌，其中产ESBL（超广谱β-内酰胺酶）大肠埃希氏菌9例（16％），产ESBL肺炎克雷伯杆菌2例（4％），对美罗培南、亚胺培南等碳青酶烯类药物均敏感。结论 AL患儿败血症的发生与化疗阶段、中性粒细胞缺乏时间等诸多因素相关。病原菌以革兰阴性杆菌多见，常见的感染途径为消化道和呼吸道。     

肝窦阻塞综合征12例临床分析

目的 总结肝窦阻塞综合征(SOS)的临床特点,以提高对该病的认识。方法 回顾性分析12 例SOS患儿的临床表现、实验室检查、影像学表现、治疗及预后等情况。结果 12 例患儿中,8 例患儿原发病为白血病,4 例为造血干细胞移植患儿。临床表现主要有腹胀(4例)、肝肿大伴触痛(9例)、脾大(6例)、体重增加(10例)。12 例患儿均有不同程度的肝损害;凝血功能检测中活化部分凝血活酶时间延长7 例;7 例患儿行血D-二聚体检查,其中4 例升高。血常规检测中,4 例白细胞、粒细胞均降低;9 例有不同程度的血小板降低。8 例患儿行彩色多普勒超声检查,可见弥漫性肝肿大、实质非均质改变,肝静脉变细、显示不清或胸腹水、脾大。2 例行肝活检,示肝窦内细胞浸润或肝窦扩张。治疗以对症支持为主,均预后良好。结论 白血病及干细胞移植患儿治疗后如果发生肝肿大、体重突然增加、肝功能异常、凝血功能异常等表现,需警惕SOS 的发生。     

39例儿童白血病合并败血症临床特点分析

目的探讨儿童急性白血病(AL)化疗后合并败血症的临床特点及降钙素原(PCT)在判断细菌类别中的作用。方法 2014年6月-2016年6月入住安徽医科大学第二附属医院儿科AL患儿39例,回顾性分析患儿化疗期间合并败血症的的发病因素、感染部位、病原学及不同PCT水平时的细菌类别。结果 39例患儿中急性淋巴细胞白血病28例,急性髓细胞白血病11例。15例患儿有明确感染部位,依次为呼吸道、口腔、肛周及皮肤。病原菌中革兰阳性球菌占53.8%(21/39),革兰阴性杆菌占43.6%(17/39),真菌占2.6%(1/39)。中性粒细胞计数0.5×109/L时败血症发病率显著提高(P0.05)。PCT高水平组革兰阴性杆菌检出率高,PCT低水平组革兰阳性球菌检出率高,不同菌种之间PCT水平存在显著性差异(P0.01)。结论 AL患儿合并败血症与粒细胞计数密切相关;PCT的结果对不同的细菌感染有提示作用,高水平的PCT提示革兰阴性杆菌感染可能性大。     

急性白血病患儿中性粒细胞减少合并感染诊治分析

中性粒细胞减少症合并感染是儿童急性白血病(A L)化疗的主要并发症之一,尽管有效的抗感染药物、粒细胞集落刺激因子(G C SF)及其他支持疗法,大大降低了死亡率,但仍严重危害患儿的生命犤1～2犦。临床上大约30%的患儿可发现明确的病原体,主要是细菌、真菌和病毒。但大部分患儿找不到病原体,因此以经验用药为主。现对我院小儿血液科急性白血病(A L)患儿中,发生中性粒细胞减少合并感染353例次的诊治问题进行分析探讨如下。1对象及方法1.1对象自2003年1月～2004年12月共收治A L105例,其中急性淋巴细胞性白血病(A LL)82例,急性非淋巴细胞性…     

儿童急性淋巴细胞白血病合并真菌性食管炎3例临床分析

目的总结急性淋巴细胞白血病(ALL)患儿合并真菌性食管炎的诊断与治疗经验。方法回顾分析3例ALL患儿于化疗过程中合并真菌性食管炎的临床特点及诊治经过,与同期住院的ALL不同部位感染作比较。结果 3例ALL患儿于化疗期间和化疗后出现上腹痛、呕吐或心前区不适,经胃镜检查诊断为真菌性食管炎。1例患儿伴发热和粒细胞缺乏,3例患儿均接受包含地塞米松的化疗。3例患儿分别经氟康唑、米卡芬净、伊曲康唑治疗2~4周,胃镜复查均治愈。结论 ALL患儿化疗过程中出现上腹部疼痛或心前区不适等症状时应考虑行胃镜检查以及时发现真菌性食管炎,胃镜检查可作为粒细胞缺乏伴感染时寻找真菌感染的手段,真菌性食管炎的治疗效果佳。     

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.