小儿先天性气管支气管畸形46例诊治分析

目的 探讨先天性气管支气管畸形的临床特点,以提高对该病的认识,减少误诊漏诊.方法 2004年2月至2008年6月在温州育英儿童医院呼吸科诊断为先天性气管支气管畸形的患儿46例,其中男29例,女17例.分析其临床、影像学、纤维支气管镜等特点.结果 46例患儿年龄3d至14岁,其中<3个月8例;3个月至1岁20例,>1～3岁15例,>3～14岁3例.均诊断为先天性气管支气管畸形,其中气管支气管软化10例,支气管起源异常15例,支气管缺如5例,气管支气管狭窄21例.复合畸形10例.发病年龄3d至14岁,均表现为反复咳嗽、反复或持续喘息,喘息多在吃奶、哭闹或运动后加重.X线胸片和CT平扫可见弥漫性或局限性透明度增强或肺不张,10例气管支气管软化通过纤维支气管镜确诊,螺旋CT三维重建诊断气管支气管狭窄21例,诊断支气管起源异常14例.结论 (1)对有下列临床特点者应该考虑到气管支气管畸形的可能:持续咳嗽经常规脸查不能明确病因;反复或持续喘息,经抗炎或支气管扩张剂治疗无效;反复或持续喉鸣;X线胸片同一部位反复或持续肺炎或肺不张;X线胸片提示局限性肺气肿、肺不张、纵隔移位而原因不明.(2)纤支镜对先天性气管支气管软化的诊断具有重要价值.(3)螺旋CT三维重建对诊断气管支气管起源异常和狭窄的诊断价值和纤支镜相当,尤其适用于年幼儿和危重儿.     

先天性呼吸系统畸形234例临床分析

目的 探讨先天性呼吸系统畸形的类型和临床特点,提高对该类疾病的早期诊断率.方法 对温州医学院附属育英儿童医院呼吸科2003年7月至2008年6月收治的234例先天性呼吸系统畸形的类型、临床和影像学资料等进行总结分析.结果 234例先天性呼吸系统畸形患儿诊断时年龄1 d～14岁,平均1.12岁,主要症状为持续喉喘鸣、反复喘息、反复呼吸道感染、呼吸困难等.通过胸片、螺旋CT三维重建、纤维支气管镜和喉镜等检查,确诊复合畸形21例,单发畸形213例.单发畸形213例中,喉部畸形(97例)包括先天性喉软骨软化症90例,先天性喉蹼5例,先天性喉囊肿2例;气管支气管畸形(35例)包括先天性(支)气管狭窄17例,先天性支气管起源异常7例,气管支气管软化10例,气管食管瘘1例;肺部畸形(43例)包括肺隔离症5例,先天性肺囊肿22例,先天性大叶性肺气肿1例,肺不发育和发育不全8例,先天性肺囊性腺瘤样畸形7例;膈肌畸形(38例)包括先天性膈疝20例,先天性膈肌膨出症18例.经手术确诊的37例肺部畸形和36例膈肌畸形中,术前经临床和影像学做出初步诊断分别占83.78%和91.67%.28例先天性(支)气管狭窄(含复合畸形11例)经螺旋CT三维蕈建联合纤维支气管镜检查确诊;10例先天性支气管起源异常(含复合畸形3例)经螺旋CT三维重建诊断.喉软化和(支)气管软化的诊断依靠喉镜和纤维支气管镜检查.结论 对于持续喉喘鸣、反复喘息、反复呼吸道感染、呼吸困难的患儿应考虑到先天性呼吸系统畸形的诊断,其类型分为喉部畸形、气管支气管畸形、肺部畸形和膈肌畸形.影像学检查和呼吸内镜对先天性呼吸系统畸形的诊断有着十分重要的作用.     

婴幼儿反复或持续喘息病因谱分析及诊断程序探讨

目的 分析婴幼儿反复或持续喘息的病因分布,并探讨病因诊断程序.方法 对临床以持续喘息≥4周或反复喘息≥3次、年龄≤3岁的185例住院患儿进行病史询间和查体,并进行肺功能、X线胸片、胸部CT或气管三维重建、纤维支气管镜、24 h食管pH值监测等检查,结合治疗效果,最后确定病因诊断.结果 婴幼儿反复或持续喘息病因比例依次为:支气管哮喘123例次(62.12%),气管支气管软化症22例次(11.11%),气管支气管狭窄18例次(9.09%),吸入因素10例次(5.05%,其中异物5例、胃食管反流2例、腭裂2例、气管食管瘘1例),支气管肺发育不良5例次(2.53%),闭塞性毛细支气管炎4例次(2.02%),支气管淋巴结结核4例次(2.02%),先天性心脏病4例次(2.02%),其他病因7例次(3.54%).单一病因171例(92.4%),复合病因14例(7.6%).结论 支气管哮喘是婴幼儿出现反复或持续喘息的首要病因,支气管淋巴结结核、闭塞性毛细支气管炎等与感染相关的喘息性疾病亦不容忽视,6个月以内婴儿最多见的病因为先天性气道发育异常;反复或持续喘息、对常规治疗无效或不敏感者,应作纤维支气管镜、胸部CT三维重建检查,以排除其他原因所致喘息;应根据病因分布和临床特征,制定婴幼儿反复或持续喘息病因诊断程序.     

儿童气管性支气管诊断、分类和气道管理的临床研究

目的探讨儿童气管性支气管的诊断、分类和气道管理。方法对住院临床有喘息的9例患儿进行了胸部CT气道三维重建检查，其中5例进行了纤维支气管镜检查。结果胸部CT气道三维重建9例中发现7例存在气管性支气管，纤维支气管镜检查5例全部发现存在气管性支气管，包括2例胸部CT气道三维重建结果阴性的患儿。同时还发现了伴有的其他气道异常，包括气管或支气管狭窄、会厌软化和声门麻痹。结论儿童气管性支气管的发现带有偶然性，支气管镜检查是诊断的“金标准”。若同时伴有先天性心脏病，应加强监护，早期手术根治可提高存活率。在对有气管性支气管的患儿进行气管插管和单肺通气时，应加强气道管理。     

支气管桥21例临床分析

目的探讨儿童支气管桥畸形及其合并肺动脉吊带的临床诊治。方法回顾性分析21例确诊为支气管桥畸形或同时合并肺动脉吊带畸形患儿的临床资料和超声心动图,多层螺旋CT胸部平扫、增强及气道重组图像影像学表现。结果 21例患儿中有10例为单纯的支气管桥,其中3例合并其他类型先天性心脏病;11例为支气管桥合并肺动脉吊带,其中8例合并其他类型先天性心脏病。年龄除1例外均2岁。大多以反复呼吸道感染及阵发性呼吸困难起病。根据Wells法分为I型15例、Ⅱ型6例。其中单纯支气管桥10例均为I型,5例合并肺动脉吊带;Ⅱ型6例均合并肺动脉吊带。结论对于临床上有反复呼吸道感染的婴幼儿,应警惕支气管桥畸形可能,并注意合并肺动脉吊带。多层螺旋CT胸部平扫、增强及气道重建图像是诊断该疾病的重要方法。     

儿童气管性支气管3例报道并文献复习

目的 提高对气管性支气管的认识和诊治水平.方法 对3例儿童气管性支气管的临床资料进行回顾性分析,分析其临床特征及纤维支气管镜检查在诊断和治疗方面的作用.结果 3例患儿的主要临床表现为反复发生肺炎、喘鸣和喘息,初次发病年龄均在1岁前,并均有2次以上的住院治疗经历;2例经纤维支气管镜检查直接诊断为气管性支气管,1例纤维支气管镜检查怀疑该病后经CT三维重建证实;3例气管性支气管均发生在气管右侧壁;3例患儿经过非手术治疗(药物治疗)后均好转出院.结论 气管性支气管是一种罕见的先天性气管支气管畸形,支气管镜检查有助于提高该病的检出率,可明确反复发生肺炎、喘鸣和喘息的病因,对指导临床治疗具有重要意义.     

以反复喘息为表现的肺动脉吊带畸形2例报告并文献复习

目的 提高对肺动脉吊带畸形(PAS)的认识和早期诊疗水平,减少漏诊、误诊.方法 分析2例婴幼儿PAS的临床资料,评价超声心动图、支气管镜、螺旋CT、MRI等方法在早期诊断方面的作用.结果 2例患儿主要临床表现为反复喘息、肺部感染.初次发病年龄＜3个月,且多次住院,纤支镜检查示气管狭窄,1例经螺旋CT及超声心动图确诊,1例经手术诊断.结论 婴儿早期出现反复喘息、肺部感染及气道梗阻表现时,应考虑PAS可能.超声心动图、螺旋CT、MRI足确诊的最佳方法.     

儿童肺动脉吊带与双主动脉弓伴随的肺气管畸形比较

目的评价血管环中双主动脉弓与肺动脉吊带伴随的气管狭窄与气道畸形的异同。方法回顾性分析2010年7月至2015年7月的双主动脉弓和肺动脉吊带患儿的临床和螺旋CT影像学资料。结果双主动脉弓患儿16例,男11例、女5例,发病中位年龄3.5个月;肺动脉吊带患儿47例,男28例、女19例,发病中位年龄4个月。16例双主动脉弓患儿中14例伴随气道狭窄,累及多个分段者2例,均为累及分段Ⅱ、Ⅲ;仅累及1个分段者12例。47例肺动脉吊带患儿均发生不同程度气道狭窄,其中狭窄累及多个分段者27例,最常见的是同时累及分段Ⅱ、Ⅲ者19例;2例同时累及4个气道段,仅累及1个气道分段者20例。两者累及气道分段数量差异有统计学意义(χ~2=13.588,P=0.001)。16例双主动脉弓患儿合并气管性支气管1例,合并肺发育不良1例;47例肺动脉吊带患儿中合并支气管桥8例,合并气管性支气管3例,合并肺发育不良3例,支气管缺如(或气管憩室)5例,合并肺前肠畸形2例。两者合并气道畸形的差异有统计学意义(χ~2=5.333,P=0.021)。结论肺动脉吊带合并的气道狭窄和气道肺畸形较双主动脉弓更为突出。     

Clinical of tracheal bronchus in 20 children

目的 分析经支气管镜检查诊断的气管性支气管患儿的临床特征.方法 回顾分析20例接受支气管镜检查而确诊的气管性支气管患儿的镜下结果、临床表现、治疗与转归.结果 先天性气管性支气管患儿20例,右侧气管性支气管19例,移位型18例,其中6例为2种或2种以上气道异常;左侧气管性支气管1例,为移位型.20例患儿的临床症状包括持续或反复的喘息,持续性局限性肺炎、肺不张,局限性肺气肿.大部分患儿经临床对症治疗后症状缓解.结论 气管性支气管异常在临床上并不少见,其临床表现为治疗反应不佳的反复喘息、肺炎、肺不张以及肺气肿等,对可疑患儿应及时进行支气管镜检查以明确诊断.     

婴幼儿反复、持续吼喘58例病因分析

目的提高临床儿科医师对婴幼儿反复吼喘的鉴别诊断能力。方法对临床持续吼喘≥4周或反复吼喘≥3次、年龄≤3岁的58例住院患儿进行病因分析。结果58例中诊断为婴幼儿哮喘26例,气管、支气管软化10例,气管、支气管狭窄9例,异物4例,支气管肺发育不良2例,胃食管返流4例,其他原因3例。结论婴幼儿出现反复吼喘最多见原因为婴幼儿哮喘;小婴儿必须排除先天性因素的可能性,6个月以内的小婴儿持续或反复吼喘最多见原因为先天性气道或肺发育异常疾病。     

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.     

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   

�¶�֢��ϵ�ϰ���ע��ȱ�ݶද�ϰ������ڵ�ת��

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.     

A profile of respiratory symptoms in urban and rural South Australian school children

This report describes the cross-sectional analyses of data from the first year of a longitudinal study using questionnaire and respiratory function data over a 5 year period from a sample of rural South Australian school children. The cumulative or lifetime prevalences of respiratory symptoms were estimated in 825 rural and 1261 urban school children aged between 5 and 15 years in order to determine if the prevalence rates differed between rural and urban school children. The study found the overall cumulative prevalence of asthma and/or wheezy breathing (AWB) to be 24.1% in the rural school children compared to 27.6% in the urban school children. Most children developed AWB symptoms before the age of 7 years, with 20% reporting moderately severe symptoms and 10% having more than one attack per fortnight. The cumulative prevalence of bronchitis, loose/rattly cough (BLRC) differed significantly between the rural school children (34.1%) and urban school children (47.9%). The BLRC symptoms preceded the development of AWB in many cases. Urban school children also reported a higher prevalence of atopic conditions.     

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.     

Hauttemperaturen verschiedener Körperoberflächenareale des Rumpfes bei Säuglingen

Summary In two groups of infants (3–53 weeks old) skin temperatures were controlled in different areas of the trunk—i.e.: regions of sternum, lungs, heart, liver, spleen, kidneys—at different room-temperatures (group I: 21–25°C; group II: 29–32°C). Rectal temperatures of some probands in both groups also had been controlled simultaneously. A definite change in the reaction to heat was proofed in different periods of the first year of life. In higher environmental temperatures the skin temperature was almost constant at every controll-point of the skin, even in older infants. In lower environmental temperatures the skin temperatures lowered continuously with age till 7. to 9. moth. From 10. to 12. month the lowering of skin temperature discontinued. The rectal temperatures were relatively constant in all infants. Only in infants from 7. to 12. month, whose skin temperatures were controlled in lower as well as in higher environmental temperatures, a tendency to higher rectal temperatures was proofed in warmer environmental temperatures.The significance of these results is discussed.

---

---

Untersuchungen mit Unterstützung durch die Deutsche Forschungsgemeinschaft.