儿童骨嗜酸性肉芽肿

目的 提高对骨嗜酸性肉芽肿的诊断及治疗水平，减少误诊。方法回顾性分析了1997年～2001年间收治的29例骨嗜酸性肉芽肿的影像学资料、临床资料及随访结果。行手术刮除病灶及植骨术治疗17例，仅作病灶活检术的5例，非手术治疗7例。结果29例中初诊仅9例为骨嗜酸性肉芽肿，8例为骨病变待诊人院，有12例均诊断为其它疾病。手术治疗的17例中11例痊愈，随访2～5年无复发，3例有残余低密度灶，2例有骨骼的畸形愈合，1例疗效差并已失随访；作病灶活检术的5例中3例痊愈，2例病灶仍有待观察后必要时手术治疗。非手术治疗的7例中2例治愈，4例病灶明显缩小，症状消失，1例放弃治疗。结论 儿童骨嗜酸性肉芽肿的X线表现多样，容易与其它病变混淆而引致误诊或漏诊，X线平片是发现骨破坏病灶和随访嗜酸性肉芽肿的首选和主要的检查方法，强调与临床、病理结合才能提高本病的诊断准确率。治疗上以手术刮除病灶及植骨为佳，在作活检术时尽可能刮除所有病变组织，避免二次手术。     

儿童骨嗜酸性肉芽肿34例诊疗分析

目的讨论儿童骨嗜酸性肉芽肿的影像学特点及临床表现,提高对骨嗜酸性肉芽肿的诊断与治疗水平。方法回顾性分析2003年至2013年我们收治的34例儿童骨嗜酸性肉芽肿患儿的影像学、临床、病理资料及随访结果,所有病例均经病理检查证实为嗜酸性肉芽肿,患儿平均年龄6.3岁。结果全部病例均获随访,随访时间6个月至10年,平均随访6年8个月,其中孤立性病变21例,多骨病变5例,多系统病变8例,免疫组化检查结果显示CD1a、S-100 protein和CD68阳性率分别为100%(34/34)、94.1%(32/34)和85.2%(29/34);保守治疗18例,手术治疗16例,其中复发1例,予加强化疗后治愈,所有患儿在最后一次随访时均获得良好的结果。结论儿童骨嗜酸性肉芽肿的临床及影像学表现多样,容易与其它病变混淆而致误诊,综合临床及影像学检查资料可以提高诊断准确率,但确诊需要病理学检查,治疗多选择保守治疗。     

儿童颅骨膜血窦27例的诊断与治疗

目的 探讨儿童颅骨膜血窦的临床特点,影像学诊断、治疗与疗效.方法 回顾性分析本科2007年7月-2011年7月收治的27例颅骨膜血窦病例的临床资料.本组病例术前均行头颅CT三维成像或头颅CT血管造影三维成像,清楚显示病灶内异常血管团与颅内静脉窦相通,病灶下方存在不同程度的骨质缺损.治疗方法以手术为主,目的在于切除异常血管团,离断交通静脉,封堵缺损骨孔.结果 本组病例共手术治疗23例,切除病灶25处,手术均获成功,未出现术中大出血、颅内静脉栓摩等并发症.术后21例症状消失,2例好转.术后随访6个月～3 a,无复发,复查CT大部分骨孔愈合.结论颅骨膜血窦是一种少见的静脉异常,是沟通颅外静脉和颅内静脉的无肌层静脉血管团,多见于儿童.临床常表现为与体位改变有关的非搏动性可回复的头皮软组织肿块.诊断主要依靠CT或头颅CT血管造影三维成像.治疗以手术切除为主,亦可血管内栓塞.术后效果满意.     

24例婴幼儿颅骨生长性骨折的早期诊治

目的 探讨儿童颅骨生长性骨折的临床特点和早期诊断及治疗的意义.方法 回顾性分析本院2007年1月至2013年4月收治的24例经手术确诊为婴幼儿颅骨生长性骨折的患儿临床资料.结果 24例患儿均有颅脑外伤史,CT或MRI检查显示脑挫裂伤及脑软化,头颅3D-CT检查显示颅骨骨折.24例患儿均在就诊后16 d内明确诊断并手术治疗,术中均发现硬脑膜破裂,其中22例行硬脑膜修补和颅骨复位固定手术;1例行硬脑膜修补及颅骨修补手术,1例仅行颅骨修补手术;2例合并脑积水,先行V-P手术.随访1个月至6年,颅骨生长良好,无手术并发症.结论 颅骨骨折和硬脑膜破裂是发生生长性骨折的重要因素;头颅3D-CT及MRI检查对婴幼儿生长性骨折的早期诊断有重要价值,一旦确诊应尽早手术治疗;早期硬脑膜修补和颅骨成形治疗可防止产生进一步的脑损害和更大的颅骨缺损.     

儿童骨嗜酸细胞性肉芽肿的诊断与治疗

目的对我院22例儿童骨嗜酸细胞性肉芽肿的临床资料进行分析,讨论其临床病理特点及影像学改变,探讨其治疗方法。方法收集2000年以来我院22例骨嗜酸细胞性肉芽肿病例,结合文献对其临床表现、诊断、治疗等进行总结分析。结果22例临床诊断为骨嗜酸细胞性肉芽肿,15例经病理证实,予手术刮除病灶或病灶加植骨治疗;7例采取非手术治疗,全部病例随访2个月～3年,其中2例合并韩-薛-柯病的患儿检查化疗,2例手术病人于2年后复发,予再次手术治疗,其余病例复查X光片病灶愈合良好。结论骨嗜酸细胞性肉芽肿多见于儿童与青少年,以骨质破坏、组织细胞增生和嗜酸性细胞浸润为主要病理特点,临床症状不典型,X线片是主要诊断方法,CT和MRI对其诊断帮助不大,治疗上应依据病灶情况选择相应的治疗方案。     

强的松龙和小牛胸腺肽治疗嗜酸细胞肉芽肿9例

我科自1988年以来收治的9例颅骨嗜酸性细胞肉芽肿采用强的松龙、小牛胸腺肽联合治疗，效果满意。临床资料一、一般资料男5例，女4例；年龄18月～10岁，平均年龄56岁。二、临床表现9例均为颅骨多发病灶，3例反复出现颅骨病灶，表现局部疼痛及肿块高部可触及限局性肿块，轻度压痛     

颅骨和脊椎朗格汉斯组织细胞增生症

目的：探讨颅骨和脊椎朗格汉期组织细胞增生症（LCH）的诊断和治疗。方法：总结5例中经手术后病理证实的颅骨和脊椎LCH15例，男10例，女5例，年龄1．5年－10岁，颅骨损害11例（单发9例，多发2例），颅骨合并脊椎损害2例，脊椎损害伴椎旁软组织肿块2例。影像学上均表现为骨质破坏，颅骨单发和多发损害，术后化疗，不作放疗，颅骨伴脊椎损害和脊椎损害伴椎管内外软组织肿块，脊椎放疗化化疗，结果：全部病例随访0．5－4．5年，无一例死亡，2例颅多骨多发损害，术后化疗期间，颅骨有新病灶出现，改用环磷酰胺，病灶消失，无复发，结论：病变组织活检是明确诊断的依据。术后化疗，放疗应视疾病程度而定，颅骨和脊椎LCH预后较好。     

化疗治疗儿童骨嗜酸性肉芽肿的临床疗效观察

目的 探讨采用化疗治疗儿童骨嗜酸性肉芽肿(EGB)的疗效,提高该病的诊治水平,改善预后。方法 回顾性分析我院4年来22例经病理证实的儿童骨嗜酸性肉芽肿病例,其中10例采用了DAL-HX83／90化疗方案,对其疗效进行分析。结果 单发8例(36．4％),多发14例(63．6％),发病的部位不同,骨损害的影像学表现不一样。10例患儿接受了诱导和维持两个阶段的化疗,其中7例系单纯手术或局部治疗后复发或多发。6例治愈、3例病灶缩小或稳定,1例多发患者治疗不敏感,有播散和复发。结论 单纯手术治疗无法解决。EGB的“此起彼伏”现象,采用DAL-HX83／90方案化疗预防其远处播散或后期复发,目的是控制甚至治愈本病,可明显改善预后。     

小儿脑型肺吸虫病的诊治

目的 探讨小儿脑型肺吸虫病的临床特点和诊治.方法 59例患儿中生食淡水蟹或饮用疫水52例,高颅压症状49例,肢体偏瘫19例,偏盲10例,癫痫发作19例,合并肺部症状6例;外周血嗜酸性粒细胞升高45例,白细胞升高36例;肺吸虫抗原皮试阳性55例,ELISA试验阳性15例.CT或MRI检查均发现脑内病灶.结果 所有病例均口服吡喹酮治疗,其中14例手术开颅切除病灶.痊愈41例,好转18例.43例患儿随访6～12个月预后良好.结论 小儿脑型肺吸虫病临床表现多样,早期易误诊.流行病学资料、外周血嗜酸性粒细胞升高、肺吸虫免疫学检查和MRI有助于早期诊断.根据病情采用药物及手术治疗效果满意.     

小儿局灶性结节性肝脏增生临床诊治经验探讨

目的探讨小儿局灶性结节性肝脏增生的临床特点、诊断方法及治疗经验,提高其诊治水平。方法回顾性分析2006年1月至2018年1月由首都医科大学附属北京儿童医院经手术切除及病理检查证实为局灶性结节性肝脏增生的患儿临床资料,其中男童9例,女童13例,发病年龄7个月至11岁1个月,中位年龄4岁6个月,所有病灶为单发,均经不规则性肝切除术治疗。结果临床表现:22例局灶性结节性肝脏增生(focal nodular hyperplasia,FNH)患儿中腹痛7例,腹部膨隆或包块4例,体检发现11例;实验室检验:肝功异常8例,AFP升高3例,均于术后恢复正常;影像学检查:所有22例均行超声检查,10例拟诊断为FNH,拟诊断正确率为45. 5%;误诊为肝脏血管瘤5例,其中肝母细胞瘤1例,间叶错构瘤1例。20例行CT(含增强)检查,11例拟诊断为FNH,拟诊断正确率为55%。3例术前行MRI检查,1例拟诊断为FNH,2例未予明确诊断。术后肿瘤最长径线5～15 cm,中位长度8 cm,其中≥10 cm者5例,最大肿物体积为15 cm×10 cm×8 cm。所有病例为单发病灶,均行手术切除,病理提示肿物中央灰白色放射样瘢痕特征11例(50%)。术后随访0. 5～10. 8年,未见复发或严重并发症发生。结论小儿局灶性结节性肝脏增生在临床及影像学上有一定特征,联合应用CT等影像学检查和AFP水平及肝功能实验室检查指标能提高其诊断水平,最终确诊需术后病理检查,手术切除治疗能够有效解除病灶,远期预后良好。     

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.