B超筛查疑似儿童髋关节脱位

目的探讨B超检查儿童髋关节脱位的功效和应用范围。方法2005年4月～2006年2月筛查疑似髋关节脱位儿童720例，予以出生史、家族史、其他畸形等危险因素评估，双侧髋关节B超检查。结果13个髋关节Ortolani和Barlow试验检查阳性，占0．9％。5例有家族史的儿童中1例出现髋关节异常。16例儿童至少有一侧异常髋关节超声表现，占2．2％。α角值在8周前后差异有统计学意义（t=5．438，P〈0．001）。结论选择性髋关节超声筛查是一种安全、有效的诊断方法，8周以后进行髋关节超声检查的准确率会增高，假阳性率也会逐步减小。     

急性肾炎患儿超声心动图动态观察

目的 动态观察急性肾炎患儿早期超声心动图，了解循环充血对心脏影响及该俭查在急性。肾炎时的应用价值。方法对36例急性。肾炎患儿早期同步作超声心动图动态观察，首次检查平均病程4．6d，并观察利尿消肿情况。结果超声心动图21例左心房增大，11例伴二尖瓣反流。分别于5d和6.2d后消失，左心室功能均正常，以上改变和临床利尿消肿相平行。结论急性肾炎早期心血管容量的超负荷导致了超声心动图的改变，此可作为急性肾炎早期循环充血状态的常规监护手段。     

儿童髋关节滑膜炎合并滑膜嵌顿的B超诊断

目的 探讨B超检查辅助诊断儿童髋关节滑膜嵌顿症的方法。方法 18例患儿中男11例，女7例，年龄4－12岁，平均9岁，均为单髋病变，左8例，右10例。应用Diasonic Synergy彩色超声仪检查，探头频率10MHz。髋关节中立位，探头置于患侧和正常侧髋关节的前方、大转子的上方以及髋关节的内下方分别观察A、B、C型皱襞，可见关节间隙内团状强回声。结果 18例中3例行走或下蹲时出现弹响，疼痛和典型体体征随即消失，双下肢等长，行走自如；6例腰麻下手法复位，听到或感到弹响后关节活动自如；6例手法复位失败，行患肢皮牵引，适应应用抗生素，辅助微波理疗，骨盆倾斜逐渐消失，双下肢逐渐恢复等长而治愈；3例尚无明显效果，经手术治疗，症状体征消失，关节积液作细菌培养未见细菌生长，切除的滑膜组织病理检查报告为慢性非特异性炎症。B超复查，关节间隙团状回声消失。18髋B超可见髋关节间隙增宽，内见液性暗区，深约8－13mm不等，关节腔可探测团状强回声，内部回声不均匀。正常侧髋关节间隙3－5mm，腔内无团状回声。A型皱襞致病4例、B型2例、C型12例，分别占22.2%(4/18)、11.1%(2/18)和66.7%(12/18)。B超发现1例C型皱襞嵌顿病例，保守治疗无效，经手术治疗痊愈并被证实。结论 除磁共振成像（MRI）外，B超检查能清楚观察到嵌顿的滑膜组织。除C型皱襞嵌顿可致病外，B和C型皱襞嵌顿同样可以致病。     

急性肾炎患儿超声心动图动态观察

目的动态观察急性肾炎患儿早期超声心动图,了解循环充血对心脏影响及该检查在急性肾炎时的应用价值。方法对36例急性肾炎患儿早期同步作超声心动图动态观察,首次检查平均病程4.6d,并观察利尿消肿情况。结果超声心动图21例左心房增大,11例伴二尖瓣反流,分别于5d和6.2d后消失,左心室功能均正常,以上改变和临床利尿消肿相平行。结论急性肾炎早期心血管容量的超负荷导致了超声心动图的改变,此可作为急性肾炎早期循环充血状态的常规监护手段。     

多发性骨骺发育不良髋关节病变的诊治

目的探讨多发性骨骺发育不良（multiple epiphyseal dysplasia，MED）髋关节病变的临床特点和治疗方法。方法本组6例MED患儿，均有髋关节受累。根据股骨近端的放射学表现，分为三型，Ⅰ型为股骨近端轻度发育不良，髋关节形态基本正常；Ⅱ型为股骨近端明显发育不良，股骨颈短粗，股骨头骨骺小、碎裂呈斑点状，伴不同程度的髋内翻；Ⅲ型为股骨近端严重发育不良，在骨盆前后位片上见不到股骨头影像，仅见短粗三角形的股骨颈残端，大转子高位，有严重的髋内翻畸形。本组Ⅰ、Ⅱ、Ⅲ型患儿各2例。Ⅰ型股骨近端不需手术矫形，Ⅱ型行转子间外展截骨术，Ⅲ型行转子关节成形术。如伴有髋臼发育不良，可同时行髋臼成形术。结果所有病例随访2～5．5年，平均3．5年，髋关节功能、步态及影像学表现较术前均有明显的改善，Trendelenburg征阴性。结论MED髋关节病变，预后不良。畸形轻微可保守治疗，畸形严重可采用外展截骨术、转子关节成形术矫正，短期取得良好的效果，但需长期随访。     

不可切除型肝母细胞瘤的术前介入治疗临床研究

目的探讨术前介入性动脉栓塞化疗（TACE）在不可切除型肝母细胞瘤治疗中的可行性。方法分析对8例经检查诊断为不可切除型肝母细胞瘤行TACE的治疗结果。结果8例患儿TACE后均有不同程度呕吐、发热、一过性肝功能损害，1例有骨髓抑制，无心、肾功能损害。有6例复查B超检查，肿瘤体积较前均有明显缩小，缩小比例为22．5％～57．5％；5例手术完整切除，均已无瘤存活1～4年，3例复诊检查发现有肺转移或多发转移，家属放弃进一步治疗后死亡。结论TACE具有化疗药物剂量小、全身副作用少、化疗效果好的优点，可作为一种术前治疗方法应用于不可切除型肝母细胞瘤。     

围产期缺氧性脑损伤的B超影象学特点及其与临床的关系

对有明确围产期缺氧史的163例新生儿进行了颅脑B超动态观察，有脑损伤影象改变32例（19．63％），以产前、产时各种缺氧因素为致病的主要原因。影象改变表现为局部缺氧缺血，合并脑水肿及出血，93．75％在生后24小时内即有异常改变，1周内好转者96％，恢复者76％。B超影象改变的严重程度与临床症状及远期预后明显相关。提示，颅脑B超的动态观测可作为围产期缺氧性脑损的有效检查手段。     

Graf Ⅱa型髋关节异常病例的回顾性分析

目的通过回顾GrafⅡa型髋关节异常婴儿的髋关节发育情况,分析其与高危因素、伴发畸形和髋关节稳定性等的关系,初步探讨其与髋关节发育不良的关系。方法回顾性分析2012年1月至2013年6月疑似髋关节异常的筛查病例5 816例,均采用标准化髋关节超声波检查,统计GrafⅡa型髋关节异常的发生率和髋关节发育的恶化率,评估其与髋关节异常的高危因素、伴发畸形、髋关节稳定性、侧别和性别的关系。结果筛查发现GrafⅡa型髋关节异常有112例(6.4%),172髋,平均随访10周后超声检查髋关节发育恶化病例为19例(18.6%),24髋,其中GrafⅡb型为14例,18髋;Ⅱc型为2例,3髋;D型为2例,2髋;Ⅲ型为1例,1髋。统计发现存在伴发畸形和单侧GrafⅡa型髋关节异常病例有更高的髋关节发育不良发生率。结论 GrafⅡa型髋关节异常病例中,18.6%的病例有髋关节发育不良的可能,临床需密切随访观察,尤其是有伴发畸形和单侧发病的病例,但与临床隐匿性髋关节发育不良的关系有待进一步研究。     

B超在先天性直肠肛管畸形诊断中的应用

目的探讨B超在先天性直肠肛管畸形分型诊断中的可行性及意义。方法应用百胜AU-4型彩色超声仪对30例先天性直肠肛管畸形进行分型诊断。结果本组30例患儿超声检查直肠盲端距肛(皮肤间的距离与手术检测结果对照,超声定位诊断符合率为90%。结论B超检查可以明确先天性直肠肛管畸形合理的临床分型。     

左旋门冬酰胺酶引起高血糖一例

患男，14岁，因双下肢痛伴发热、乏力3天入院。其父有糖尿病。查体：T：36．8＞C精神差，锁骨上、腹股沟淋巴结绿豆及黄豆大小。肝右肋下3．5cm、剑突下5cm，质中边钝。脾左肋下4．5cm、质硬。全身共有5处陈旧性淤斑。辅助检查：血WBC：31．7X10'／I－，白细胞分类：原幼细胞76％。PLT：62X10'／L。骨髓检查为急性淋巴细胞白血病，L；型。免疫分型为B－ALL。尿、便常规正常，超声心动图、心电图正常。腹部B超示肝、脾肿大。肝肾功能、血糖及电解质均正常。诊断为急性淋巴细胞白血病。给予长春新碱、强的松、柔红霉素及左旋门冬酷胺…     

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.     

�¶�֢��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.     

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.