先天性肺囊性疾病52例北大核心CSCD

目的探讨儿童先天性肺囊性疾病的诊断、手术治疗方法及预后。方法回顾性分析2003年1月至2012年6月在复旦大学附属儿科医院住院手术治疗的先天性肺囊性疾病儿童52例。对患儿临床表现、病变部位、影像学、病理学、漏诊误诊、手术治疗方法和短期肺功能进行统计分析。结果52例先天性肺囊性疾病患儿中,男33例,女19例;年龄1d-159个月,中位数为3．67个月;新生儿5例。病种：支气管源性肺囊肿42例,肺隔离症6例,大叶性肺气肿3例,先天性囊性腺瘤样畸形1例。临床表现以肺部感染为主,其中61．5％的患儿首次肺部感染后确诊,21．1％的患儿在反复呼吸道感染后确诊,约17．3％的患儿无症状。病变部位以右下肺最多见,约占36．5％。影像学以多发气囊肿最多见,约占42．3％。病理结果均提示支气管源性。漏诊率为17．3％,误诊率为24．9％,其中支气管源性肺囊肿误诊率为19．2％。治疗以肺叶切除术为主,无死亡病例。结论4,JI,先天性肺囊性疾病的术前诊断主要依靠影像学检查,临床表现有助于早期识别,术前易漏诊误诊,术后病理有助于确诊,手术疗效肯定,预后好。     

先天性肺囊性病变28例的诊断与治疗

目的 探讨儿童先天性肺囊性病变的诊断、手术时机和方式.方法 对儿童先天性肺囊性病变28例的临床资料进行回顾性分析.男19例,女9例;年龄1～12岁,平均5.4岁,其中1～5岁11例.先天性肺囊肿21例,其中支气管源性囊肿13例;肺源性囊肿6例;混合型2例.肺隔离症7例.术前诊断主要依靠胸部X线片和CT,术前确诊21例,其中先天性肺囊肿17例,肺隔离症4例.术前误诊7例,误诊率25%,其中误诊为肺脓肿、纵隔占位各2例,气胸、肺大疱、支气管扩张症各1例.行肺囊肿摘除术 3例,肺段切除术2例,肺叶切除17例,其中2例为急诊手术,隔离肺切除4例,双肺叶、肺叶加肺段切除术各1例.结果 本组无死亡病例,术后恢复良好,均痊愈出院,随访1～5 a,患儿生长发育及活动正常.结论 儿童先天性肺囊性病变误诊率高,诊断主要依靠影像学检查,确诊后应尽早手术,手术疗效满意,预后良好.     

先天性肺囊性疾病96例临床分析

目的总结先天性肺囊性疾病的诊断、临床表现、治疗及预后。方法回顾分析在天津市儿童医院2010年1月至2019年3月住院治疗的96例先天性肺囊性疾病患儿(男50例、女46例)的临床表现、影像学检查、病理、治疗及预后。结果 96例先天性肺囊性疾病患儿年龄4 d～13岁, 86例(90%)患儿因咳嗽发热就诊时发现。先天性囊性腺瘤样畸形40例(42%), 手术治疗30例, 急症手术2例, 死亡1例。肺隔离症12例(13%), 手术治疗4例。先天性肺囊肿12例(13%), 手术治疗4例。先天性大叶性肺气肿3例(3%), 手术治疗1例, 1例气胸患儿出院后2个月于外院手术治疗。未明确分类的先天性肺囊性疾病29例(30%), 通过肺CT检查不能确诊, 考虑"先天性肺囊性疾病", 部分病例与坏死性肺炎很难鉴别, 有2例经6、10个月随访肺部病变消失诊断为坏死性肺炎。39例手术治疗患儿中37例恢复良好, 无反复下呼吸道感染。结论先天性肺囊性疾病的确诊需依靠影像学及病理检查, 多数因呼吸道感染就诊时发现, 以咳嗽发热为主要临床表现。诊断后尽早手术治疗, 手术预后较好。     

儿童先天性肺囊性疾病诊断和治疗进展

儿童先天性肺囊性疾病属临床少见肺部疾病,是肺组织胚胎发育异常所形成的畸形.根据其起源可以分为支气管源性肺囊肿、囊性腺瘤样畸形、先天性大叶性肺气肿和肺隔离症.该病临床表现无特异性,易与多种疾病相混淆,造成误诊.现对其主要发病机制、临床表现、诊断与鉴别诊断、治疗及干预措施进行阐述.     

儿童肺部囊性病变的诊断和治疗

目的探讨儿童肺部囊性病变的诊断、手术治疗时机和方式。方法对23例儿童肺部囊性病变进行回顾性分析，其中支气管源性肺囊肿16例，肺隔离症4例，肺大疱3例。16例行肺叶切除术，7例行囊肿切除术。5例为急诊手术。结果无手术死亡病例，全部患儿术后恢复良好，无脓胸、气胸、支气管胸膜瘘等并发症。术后随访3月-2年，无复发病例。结论儿童先天性肺囊肿确诊后应尽早手术，合并囊肿或肺部感染者待感染控制后手术，压迫症状明显则应急诊手术。肺大疱如经抗感染治疗无好转或进行性增大及合并气胸、脓胸应行手术治疗。手术中应尽量保留正常肺组织。     

支气管源性囊肿的研究进展

支气管源性囊肿( bronchogenic cysts,BC)是一种先天性肺发育畸形,好发于儿童及青少年。该病在临床上比较少见,且临床表现、体征及胸部影像学多样,缺乏特异性,故容易误诊。如何提高诊断准确率及早期确诊率目前仍是难点。该文就支气管源性囊肿的组织胚胎学、临床表现、影像学特点以及治疗等方面的研究进展作一综述。     

先天性肺囊性疾病患儿基因组拷贝数变异分析

目的探讨先天性肺囊性疾病(CCLD)相关基因的拷贝数变异。方法回顾分析16例CCLD患儿的临床资料以及全基因组拷贝数变异检测结果。结果 16例患儿中男12例,女4例,年龄2个月~12岁6个月。10例患儿为支气管源性肺囊肿,其余分别为肺隔离症2例、先天性囊性腺瘤样畸形2例、先天性大叶性肺气肿1例、临床未分型1例。临床表现以发热、咳嗽、咳痰为主,无特异性。全基因组拷贝数变异检测,2例患儿有6个临床意义暂不明确的基因拷贝数变异;4例支气管源性肺囊肿患儿有HDAC8基因部分片段的异常扩增。结论 CCLD与基因拷贝数变异相关的可能性小;HDAC8基因可能为支气管源性肺囊肿相关基因。     

小儿先天性气道畸形23例临床及影像学特点

目的 分析先天性气道畸形的临床及影像学特点,深入对先天性气道畸形的认识,指导临床诊断,减少漏诊及误诊率.方法 收集2005年至2011年中国医科大学附属盛京医院小儿呼吸内科首次就诊诊断为先天性气道畸形患儿的临床资料,回顾性分析其临床及影像学特点.结果 患儿23例,男12例,女11例;年龄1个月～10岁,平均年龄32个月;病程3d～3个月,平均18 d;临床多表现为反复咳嗽、喘息、呼吸困难、反复呼吸道感染.经螺旋CT及三维重建、联合增强CT检查,可见气管支气管狭窄6例,气管性支气管3例,左肺动静脉发育不良1例,肺隔离症3例,肺囊肿4例,肺发育不良6例.结论 临床上先天性气道畸形易发生漏诊及误诊.当患儿出现持续咳嗽;反复或持续喘息,经抗炎或支气管扩张剂治疗效果不佳;反复或持续喉鸣;反复肺内感染,呼吸困难;X线胸片同一部位反复或持续肺炎;X线胸片提示纵隔移位而原因不明时应考虑先天性气道畸形.螺旋CT三维重建及增强对诊断气道畸形具有重要价值.     

小儿先天性肺囊性疾病（附65例分析）

目的探讨先天性肺囊性疾病分类、诊断及治疗。方法回顾分析65例先天性肺囊性疾病的临床资料。结果自1980年1月～2004年12月经手术、病理证实的65例，其中先天性肺囊肿51例，先天性肺隔离症8例，先天性囊性腺瘤样畸形6例。结论先天性肺囊性疾病主要分3类，易与其他疾病混淆，引起误诊。影像学检查发现，明确手术指征者应积极采取外科治疗。     

先天性肺囊肿21例

目的：探讨先天性肺囊肿的诊断、鉴别诊断及手术治疗。方法：先天性肺囊肿21例经X线及CT检查明确有手术指征，分别行肺叶切除或囊肿摘除。结果：术前确诊19例，2例术前误诊为其他肺部疾病患儿术后病理检查证实为本病。均经手术治愈，术后随访1－4年效果良好。结论：先天性肺囊肿易与其他疾病混淆，引起误诊误治。影像学检查发现明确手术指征者应积极采取外科治疗，有感染者应在控制中毒症状后手术。     

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.