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.     

Pulmonary vascular sling: Report of seven cases and review of the literature

Summary A total of 130 cases of pulmonary vascular sling, including seven new cases in our collection, were studied. The sex distribution was 60% male and 40% female. The age at presentation was in the first year of life in 90% of cases. Barium-esophagraphy showing anterior indentation was diagnostic in most of the cases. Bronchoscopy and tracheobronchography were useful in detecting associated tracheobronchial anomalies preoperatively. The analysis of 68 autopsied cases revealed associated tracheobronchial anomalies in 40% of the cases. Anomalies of the tracheobronchial tree took three major forms: abnormal distribution of cartilage in the walls of the trachea and major bronchi, intrinsic stenosis, and abnormal branching, the latter being that ofbronchus suis. Acquired changes secondary in the sling resulted in compression of the major respiratory pathway by the anomalous left pulmonary artery. Major associated cardiovascular anomalies were present in 30% of the cases. These were represented by ventricular septal defect, atrial septal defect, patent ductus arteriosus, tetralogy of Fallot, common ventricle, and coarctation of the aorta.     

Malignant mesothelioma in children: report of seven cases and review of the literature

Seven children with malignant mesothelioma have been seen at Memorial Hospital since 1953. In six, the origin was at the pleura and in one at the peritoneum. None of the patients related a history of exposure to asbestos. Two patients lived more than five years. The other five patients died within two years of the diagnosis. Distant metastases were seen in four of the patients, including three who had metastases to brain. Surgery or radiotherapy were not effective in controlling the disease in most of the patients. One patient had a complete response to a combination of Adriamycin, cyclophosphamide, and vincristine and has remained free of disease for 5 1/2 years. The seven cases are reviewed, as are the other 42 cases of malignant mesothelioma in children reported in the literature.     

Preleukemic syndrome in children. Report of four cases and review of literature

Childhood preleukemia, known as a rare condition, was evaluated in four of the authors' cases and in 24 cases from the literature. The required condition was evolution into overt acute leukemia. The children were 5 months to 15 years of age, and the preleukemia period ranged from 2 to 42 months. The symptoms and physical signs were nonspecific. Different kinds of cytopenia were found in the peripheral blood. Twelve children developed ALL and 16 developed AML. The analysis revealed that in childhood there exist two different types of preleukemia: pre-ALL and pre-AML. The age and sex distribution were different, as were the hematological changes. The marrow was usually hypoplastic in pre-ALL but hyperplastic in pre-AML. True hypoplasia in any of the three cell lines was more common in pre-ALL, whereas ineffective thrombopoiesis and normal or increased myelopoiesis were specific for pre-AML. Ineffective erythropoiesis was characteristic of both types. A typical chromosomal change in marrow, seen in pre-AML only, was a missing group C chromosome. The childhood pre-AML resembled adult preleukemia (also pre-AML) in many aspects, whereas the childhood pre-ALL seemed to be a different entity. It might be assumed that all preleukemic conditions do not evolve to overt malignancy. The incidence and true prognosis therefore remain unknown.     

小儿颅脑外伤合并胸腹部复合伤16例

目的通过对16例颅脑外伤合并严重复合伤病例的回顾性分析,探讨提高小儿颅脑外伤合并胸腹部复合伤救治成功率的方法。方法回顾性分析本院收治的16例小儿颅脑外伤合并胸腹部复合伤患儿临床资料。判别严重休克的病因,准确发现胸腹部合并伤等重要损伤,快速纠正休克和分别处置。结果16例颅脑外伤合并胸腹部损伤的严重复合伤患者,经上述处理,均抢救成功,治愈13例,平均格拉斯哥（GCS）评分13．7分;致残3例,平均GCS评分6．67分。无一例死亡。结论准确判断病情,在颅脑外伤影响患儿意识时及时准确地判断可能合并的胸腹部外伤,迅速纠正失血性休克,处置合并的胸腹部外伤,可挽救大部分危重病例,提高救治效果。     

Radiation-induced meningioma in children: Report of two cases and review of the literature

Meningioma developed in two children who had received high-dose cranial radiation for malignant brain tumors. Meningioma as a radiation-induced neoplasm has received little notice in the radiologic literature. Thirty-three cases have been reported since 1953, primarily in the neurosurgical literature. The current cases differ from those previously reported in having a much shorter latency period between irradiation and the development of meningioma.     

解鸟氨酸拉乌尔菌致儿童感染5例报告并文献复习

目的：探讨儿童感染解鸟氨酸拉乌尔菌的临床特征和治疗。方法：回顾性分析5例感染解鸟氨酸拉乌尔菌患儿的临床资料,在PubMed、Web of Science、中国知网、维普数据库和万方数据库中检索解鸟氨酸拉乌尔菌感染的儿童病例,检索时间均为建库至2018年6月15日,复习相关文献。 结果：男3例。2例合并先天性发育畸形,分别为3、6月龄;免疫相关性疾病2例,分别为11和16岁;颅脑恶性肿瘤1例,11岁。4例有呼吸机辅助通气史;4例有发热;3例血培养阳性,2例痰培养阳性;3例WBC升高明显,1例WBC降低。2例予环丙沙星,1例予阿米卡星,2例予头孢类抗生素,5例均病情好转出院。共检索到8篇英文文献,报告了9例解鸟氨酸拉乌尔菌感染患儿,与本文报告的5例合并后共14例。年长患儿的原发病以肿瘤性和免疫系统疾病为主,婴幼儿感染者多合并先天性发育畸形;发热为主要症状,新生儿感染以呼吸困难和低氧血症为主要表现,多伴有皮肤潮红和全身红斑;多伴有WBC明显升高;6例行呼吸机辅助通气,5例有留置导尿管,3例行手术治疗或化疗,1例多次行血液净化;血培养为主要鉴定方法,该菌对头孢三代、美罗培南、阿米卡星、环丙沙星和左氧氟沙星敏感度较高;抗感染治疗预后大多良好,合并脓毒症患者可并发多功能脏器衰竭而死亡。结论：儿童感染解鸟氨酸拉乌尔菌多以血源性感染为主,先天性多发畸形、肿瘤和免疫功能低下者易感,有创操作提高感染该菌风险,抗生素优先选择头孢三代、碳青霉烯类、喹诺酮类和氨基糖苷类,选用敏感抗生素治疗预后较好,合并脓毒症者预后较差。     

Huntington's chorea. Report of 3 cases and review of the literature.

Three cases of Huntington''s chorea with onset before age 10 years are reported. Each child presented with rigidity and indistinct speech, and there was progressive deterioration. Necropsy examination confirmed the diagnosis in 2 of them. A review of reports showed a further 43 cases with onset before 10 years. The rigid variety of disease was seen most often, but isolated chorea and isolated progressive mental deterioration occurred. Fits were common but occurred late and were often difficult to control. Dysarthria was common and occurred early. The duration of illness was very variable and ranged from 2 to 38 years. Symptoms can occur in a child before appearing in the affected parent who is most likely to be the father. Affected siblings develop the disease early, often in the first decade. Siblings of patients with onset before age 10 years who are unaffected by age 25 years had only an 8% chance of developing the disease, compared with a 50% chance in unselected at risk individuals of the same age.     

Reflex sympathetic dystrophy syndrome in children and adolescents. Report of 18 cases and review of the literature

Reflex sympathetic dystrophy syndrome is a well-recognized disorder in adults, but it is rarely diagnosed in the pediatric age group. This report summarizes our experience with this condition from 1975 to 1985. We diagnosed, treated, and followed up this condition in 18 children and adolescents. The condition usually followed trauma. The most prominent feature in all patients was a constant limb pain with episodes of paroxysmal exacerbation. The pain was associated with two or more of the following: edema, hyperhidrosis or anhidrosis, cyanosis or erythema, and, in severe cases, dystrophic skin changes and muscle atrophy. Roentgenograms were normal. Bone scans were helpful to exclude other possible causes of bone and joint pain. Reflex sympathetic dystrophy syndrome in children probably often goes unrecognized, sometimes being confused with psychiatric conditions such as conversion reaction and malingering. Reflex sympathetic dystrophy syndrome should always be considered in the differential diagnosis of unexplained persistent limb pain in children: early recognition and proper management may result in the prevention of potentially crippling sequelae.     

Lyme neuroborreliosis in children: Report of nine cases and a review of the literature

Lyme neuroborreliosis is a bacterial infection caused by the dissemination and proliferation of a Borrelia species in the central nervous system. Neuroborreliosis occurs after transmission of the pathogen from an infected tick to a human host during a tick bite. We report nine cases of pediatric neuroborreliosis collected by the National Observatory of Pediatric Bacterial Meningitis in France between 2001 and 2012. The nine children, aged 4–13 years, were identified in northern and eastern France and had the following clinical features: meningeal irritation alone or with facial palsy, or isolated facial palsy. All cases showed anti-Borrelia antibodies in cerebrospinal fluid or serum, or with a positive Borrelia PCR in the CSF. The outcome was favorable in all cases after a 2- to 3-week course of third-generation cephalosporin. On the basis of these nine pediatric cases, this study provides an update on the epidemiology, pathophysiology, diagnostic strategy, and treatment of neuroborreliosis, with insight into the specific features of pediatric neuroborreliosis and the difficulties encountered in the diagnosis of this infection.