重型颅脑损伤的诊断与治疗

目的　探讨重型颅脑损伤的诊断与治疗。方法　回顾性分析我院 1 995年 1月～ 2 0 0 3年 1 2月收治 93例重型颅脑损伤 (GCS≤ 8分 )的临床资料。结果　 93例 (其中GCS≤ 5分 2 2例 )中并颅内血肿 41例 ,脑室出血 3例 ,占 46 .2 4 %。手术治疗55例 ,非手术治疗 38例。 68例存活 ,随访 1～ 3年 ,头颅CT复查 40例恢复正常 ,1 8例有软化灶 ;轻中残 1 0例 ,死亡 2 5例 ,死亡率 2 6 .88%。结论　应尽早确诊重型颅脑损伤 ,抢救生命。选择及时、恰当的综合治疗方案 ,是提高疗效的关键。     

重型颅脑损伤126例

目的探讨小儿重型颅脑损伤的诊断及治疗方法。方法小儿重型颅脑损伤(GCS≤8分)126例,45例行颅内血肿清除术,36例行严重脑挫裂伤破碎脑组织清除术,26例行开放性颅脑损伤清除术,22例行颅骨凹陷骨折整复术。余病例予保持呼吸道通畅、呼吸机辅助呼吸等保守治疗。结果126例小儿重型颅脑损伤中并颅内血肿66例,占52%。手术治疗91例,非手术治疗35例。110例存活,随访6个月~3年,头颅CT复查86例恢复正常,26例有脑软化灶;轻度偏瘫5例,智力障碍3例,6例有外伤性癫。死亡16例,死亡率12.7%。结论小儿重型颅脑损伤应尽早确诊,及时选择恰当的治疗方案,是提高存活率和生活质量的关键。     

小儿重型颅脑损伤后脑性盐耗综合征

目的探讨小儿重型颅脑损伤后并发脑性盐耗综合征的发病机制、临床诊断及治疗。方法回顾性分析我院1990年1月~2003年12月收治18例小儿重型颅脑损伤(Glasgow昏迷分级标准评分3~8分)后并发脑性盐耗综合征的临床资料。结果18例据临床表现和实验室检查被确诊。经补钠和输液治疗,低钠血症和低血容量均得以纠正。结论低血钠、高尿钠、低血容量及意识状态改变是脑性盐耗综合征的临床诊断依据,补钠和补足血容量治疗安全有效,对预后至关重要。     

影响小儿重型颅脑损伤预后的危险因素分析

目的分析各种危险因素对小儿重型颅脑损伤预后的影响。方法用寿命表分析，单变量和多变量分析等统计学方法分析年龄、损伤类型、颅骨骨折、颅内血肿、反射消失、癫痫、脑肿胀／脑水肿、格拉斯哥昏迷计分（GCS）、低血压、低氧血症等因素对预后的影响。结果91例患儿，死亡31例（34．1％），死亡大多发生在伤后1周以内，预后与原发性损伤的严重程度有关，GCS记分是影响预后的最主要因素，GCS记分5～8分者88．9％存活，而GCS小于5分者仅14．3％存活；伤后6个月时的致残率为23．1％。单变量分析发现癫痫大发作（P〈0．0001）、多发性损伤（P=0．0112）、反射消失（P〈0．0001）、外伤后急性弥漫性脑水肿／脑肿胀（P〈0．0001）是影响预后的因素，年龄、颅骨骨折和颅内血肿对预后的影响无统计学意义。用Cox模型进一步分析发现治疗前的反射消失、外伤后急性弥漫性脑水肿／脑肿胀是影响预后的最主要因素（P〈0．0009），年龄、颅骨骨折、颅内血肿对预后无影响。结论原发性反射消失、GCS记分是预测预后的最好指标，脑水肿／脑肿胀是影响预后的最主要因素。所以，为了改善患儿的预后，应将颅脑损伤患儿的治疗重点放在预防和减低脑肿胀／脑水肿上。     

小儿重型颅脑损伤后脑性盐耗综合征

目的探讨小儿重型颅脑损伤后并发脑性盐耗综合征的发病机制、临床诊断及治疗。方法回颐性分析我院1990年1月～2003年12月收治18例小儿重型颅脑损伤（Glasgow昏迷分级标准评分3～8分）后并发脑性盐耗综合征的临床资料。结果18例据临床表现和实验室检查被确诊。经补钠和输液治疗，低钠血症和低血容量均得以纠正。结论低血钠、高尿钠、低血容量及意识状态改变是脑性盐耗综合征的临床诊断依据，补钠和补足血容量治疗安全有效，对预后至关重要。     

小儿弥漫性轴索损伤治疗体会

目的探讨小儿弥漫性轴索损伤最佳治疗方法。方法分析总结1993年10月～2002年12月间的42例弥漫性轴索损伤患儿的临床表现及治疗方法。行多参数监测，维持体温、血氧分压及脑灌注压正常，保持水、电解质、酸碱平衡，给予综合治疗。结果随访1年，GOS1分5例，GOS2分3例，GOS3分2例，GOS4分4例，GOS5分28例。结论小儿弥漫性轴索损伤在小儿重型颅脑损伤中发病率较高，一般病情凶险，但小儿修复能力较强，通过积极治疗预后较好。大多无任何后遗症。     

小儿弥漫性轴索损伤49例

目的：探讨小儿弥漫性轴索损伤（DAI）的临床特征、早期诊断和治疗方法。方法：对49例小儿DAI病因、临床表现、CT结果、诊断和治疗方法进行分析。结果：其中伤后持续昏迷45例，有中间清醒期4例；CT显示弥漫性脑白质肿胀、点状出血，无脑中线移位。根据以上临床特征进行诊断，并采取相应的治疗措施。本组生存28例（59．1％），死亡21例（40．9％）。结论：小儿DAI伤情重、预后差，是重型颅脑损伤致死和致残的重要原因。     

去骨瓣减压术在儿童重型颅脑损伤中的应用及研究进展

儿童颅脑损伤是引起儿童创伤死亡和致残的首位危险因素.多数颅脑损伤为轻中度损伤,预后相对良好.重型颅脑损伤(severe TBI,sTBI)是指心肺复苏后格拉斯哥昏迷指数(glasgow coma scale,GCS)评分≤8分的颅脑外伤,常常会导致患儿死亡或残疾.外科常采用去颅瓣减压术(decompressive craniectomy,DC)治疗TBI患儿,通过去除部分颅骨,使脑实质不再受限于骨腔,通过增加脑容积来降低颅内压(intracranial pressure,ICP),从而改善脑灌注压力(cerebral perfusion pressure,CPP),并降低发生大脑中线移位、脑干压缩和脑疝的概率:然而DC通常不是首选策略而是作为辅助治疗手段用于临床.本文就DC的优缺点及其在儿童重症颅脑外伤中的应用和研究进展进行探讨.     

小儿外伤性基底节梗塞42例临床分析

目的 探讨小儿外伤性基底节梗塞的发生机制和临床特点。方法总结并分析1989年7月至2002年12月收治的42例小儿外伤性基底节梗塞。结果 按格拉斯哥预后分级(Glasgow Outcome Scale,GOSI)标准评定：治愈37例(88．1％)，轻残3例(7．1％)，重残2例(4．8％)，无植物生存和死亡。结论颅脑损伤后微循环障碍、基底节血供特点和血管损伤、小儿自身特点是小儿外伤性基底节梗塞的主要发生机制。临床特点：(1)头外伤较轻，多无意识障碍；(2)神经功能缺失征明显，以运动性功能障碍为主；(3)头颅CT和(或)MRI可明确诊断；(4)早期针对发病机制的系统治疗，多数预后良好。     

小儿弥漫性轴索损伤治疗体会

目的探讨小儿弥漫性轴索损伤最佳治疗方法。方法分析总结1993年10月～2002年12月间的42例弥漫性轴索损伤患儿的临床表现及治疗方法。行多参数监测,维持体温、血氧分压及脑灌注压正常,保持水、电解质、酸碱平衡,给予综合治疗。结果随访1年,GOS1分5例,GOS2分3例,GOS3分2例,GOS4分4例,GOS5分28例。结论小儿弥漫性轴索损伤在小儿重型颅脑损伤中发病率较高,一般病情凶险,但小儿修复能力较强,通过积极治疗预后较好,大多无任何后遗症。     

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.