小儿消化道大出血的救治

目的 讨论小儿消化道大出血的病因诊断及救治。方法 通过对49例消化道大出血患儿的临床救治资料作回顾性分析。结果 48例消化道大出血患儿的获救，1例死亡。结论 小儿消化道大出血患儿的救治除对症及全身止血治疗外。尽早找出病因，积极去除病因及内镜下止血治疗是获得救治成功的重要手段。     

经颅多普勒超声在深昏迷患儿预后判断中的应用

为了评价经颅多普勒超声（ＴＣＤ）在估计昏迷患儿预后及诊断小儿脑死亡中的价值，使用ＭａｄｅｓｏｎｉｃｓＴＣＤ仪，动态监测了５８例Ｇｌａｓｇｏｗ评分≤７分昏迷患儿双侧大脑中动脉（ＭＣＡ）的频谱形态和血流速度。其中脑死亡组１７例，普通死亡组２１例，存活组２０例。结果：深昏迷患儿双侧ＭＣＡ呈全舒张期反向血流或尖小收缩波频谱，血流方向指数（ＤＦＩ）＜０．８，持续２小时以上无恢复，是预示或证实小儿脑死亡较可靠的指标；本组１７例脑死亡患儿ＴＣＤ均出现上述改变，如出现舒张期部分反流，ＤＦＩ＞０．８，积极治疗有恢复可能；周期血流出现是预后不良的先兆；两侧ＴＣＤ频谱明显不对称与局限性脑损伤有关。提示：ＴＣＤ监测对正确估计昏迷患儿预后、指导治疗有较重要的临床意义。     

小儿外伤性小肠损伤的诊断和治疗分析

目的总结小儿外伤性小肠损伤的诊断和治疗经验。方法对38例外伤性小肠损伤患儿的病因、损伤部位、诊断和治疗进行回顾性分析。结果38例均行手术治疗．行小肠修补术及小肠切除吻合术，同时处理其它脏器损伤，注意防治感染．加强支持治疗。36例治愈，2例死亡。结论小儿外伤性小肠损伤经早期发现，正确处理，预后良好。     

小儿消化道大出血的救治

目的 讨论小儿消化道大出血的病因诊断及救治。方法通过对49例消化道大出血患儿的临床救治资料作回 顾性分析。结果48例消化道大出血患儿的获救,1例死亡。结论 小儿消化道大出血患儿的救治除对症及全身止血治疗外, 尽早找出病因,积极去除病因及内镜下止血治疗是获得救治成功的重要手段。     

小儿肥厚型心肌病病因学研究现状

小儿肥厚型心肌病系一组多病因疾病,以不能解释的心室肥厚为特征。由于不同病因的肥厚型心脏病危重程度及治疗方法不尽相同,故能否早期做出正确诊断对患儿预后和生存率有很大影响。该文就小儿肥厚型心肌病的病因分类及特点进行综述。     

小儿晕厥的紧急处理、预后和预防

小儿晕厥病因多、检查复杂。现就小儿晕厥的紧急处理、预后和预防作一介绍。1　紧急处理小儿晕厥紧急处理包括 3方面 ,即确定是否晕厥 (即与晕厥相似症状的鉴别 )、晕厥病因的鉴别、紧急治疗。以上3方面是相互交叉的。不能明确是否晕厥、晕厥的病因是什么 ,就不能正确处理晕厥 ,但晕厥病情紧急又不可能在明确病因后采取治疗措施 ,因此只能边治疗边研究其病因。随着检查病因逐步明确 ,随时调整治疗措施。为了便于叙述分以下 3方面介绍。1.1　与晕厥相似症状的鉴别　有些症状和晕厥有相似之处 ,如休克、昏迷、惊厥、癔病等。其鉴别点如下。1.1…     

小儿遗传性高氨血症三例临床和头颅MRI影像学特点

目的：探讨小儿遗传性高氨血症的病因、临床及影像学特征。方法对3例临床诊断为高氨血症患儿的临床表现、头MRI影像学、治疗等进行分析。结果3例高氨血症患儿均行遗传代谢病筛查明确病因为鸟氨酸氨甲酰基转移酶缺陷、瓜氨酸血症、甲基丙二酸血症,3例患儿临床表现差异较大,其中2例患儿早期行头颅MRI检查,弥散加权成像均提示高信号。早期积极对症治疗,1例患儿治疗效果较好。另2例患儿治疗效果差。结论对高氨血症患儿早期寻找病因,早期干预,对预后的判断至关重要。头MRI对高氨血症患儿的病因学判断有重要意义。     

小儿外伤性小肠损伤的诊断和治疗分析

目的总结小儿外伤性小肠损伤的诊断和治疗经验。方法对38例外伤性小肠损伤患儿的病因、损伤部位、诊断和治疗进行回顾性分析。结果38例均行手术治疗,行小肠修补术及小肠切除吻合术,同时处理其它脏器损伤,注意防治感染,加强支持治疗。36例治愈,2例死亡。结论小儿外伤性小肠损伤经早期发现,正确处理,预后良好。     

小儿急性肝功能衰竭的特征与评估

小儿急性肝功能衰竭发病率低,但病死率高,可在较短时间内发生多脏器功能障碍甚至死亡.小儿急性肝功能衰竭在病因、临床表现、诊断及预后等方面均与成人有所区别,且具有年龄特点和地域特点.因此,掌握小儿急性肝功能衰竭的病因及临床表现等特征,早期识别、早期诊断、全面评估、早期治疗有助于改善患儿的预后,提高存活率和生存质量,为可进行肝移植的患儿争取更多的生存机会.     

先天性胸腹裂孔疝手术的麻醉及围术期管理

目的 提高对小儿先天性胸腹裂孔疝的麻醉和围术期特点的认识，并探讨影响患儿预后的因素。方法 回顾性分析9例小儿先天性胸腹裂孔疝病例资料，总结 其麻醉和围术期处理及治疗效果。结果 9例患儿中，死亡3例，均为生后48h内的新生儿。结论 小儿先天性胸腹裂孔疝手术和麻醉死亡率很高，合理的麻醉和围术期处理可以降低死亡率。     

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   

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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.     

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.

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Untersuchungen mit Unterstützung durch die Deutsche Forschungsgemeinschaft.