Les malaises du nourrisson d’origine neurologique

Whenever abnormalities are found during the neurological examination of an infant who presented an apparent life-threatening event, traumatic or metabolic etiologies must be searched for in priority. Indeed, these etiologies require emergency diagnosis and treatment due to the major risk of recurrence, and consequent worsening of the neurological and functional prognosis or death.     

Sudden death from fulminant acute cerebellitis

Acute cerebellitis is postulated to result from viral and/or autoimmune etiologies. This disease has been reported to have a variable course. We report a case of sudden death from acute fulminant cerebellitis in a 13-year-old ballet dancer. Serial CT and MRI demonstrated rapid progression of the disease. Histopathologic correlation is provided. The etiologies, clinical course, therapeutic interventions and postmortem evaluation of this potentially life-threatening condition are briefly reviewed.     

Perinatal hemochromatosis]

Neonatal hemochromatosis is responsible for death in utero or severe hepatic insufficiency in the newborn, generally with rapid progression to death. Recurrence in sibships is frequent. Necropsy findings are characterized by siderosis in the liver, pancreas, heart, thyroid, but not in the reticuloendothelial system. Urinary cytology, serum iron concentration, iron-binding capacity, ferritin, and magnetic resonance imaging allow a diagnosis to be made before death. Liver transplantation probably offers the best chance of survival. The pathophysiology is unknown; there may be a variety of etiologies.     

Supraventricular tachyarrhythmias,congenital heart disease,and sudden cardiac death

Summary The etiologies of sudden cardiac death following the surgical treatment of congenital heart defects remain uncertain. A young patient with prior repair of partial anomalous plumonary venous return is presented, in whom brief episodes of a supraventricular tachyarrhythmia (rate 170/min) were documented to result in ventricular fibrillation. This unusual sequence may represent a basis for unexplained sudden cardiac death in other patients following atrial surgical procedures for the treatment of congenital heart disease.     

Aborted sudden cardiac death in adolescent

Sudden cardiac death is rare in children and adolescents but accounts for 19% to 30% of sudden deaths until 21 years of age. Fatal ventricular arrhythmias are usual common pathways in such tragic events, and underlying etiologies include cardiac ion channelopathies in majority of cases. We present a case of aborted sudden cardiac death in field, resuscitated successfully, and a clinical event in the pediatric emergency department that led to the diagnosis of the underlying rare condition.     

Sudden cardiac death in children and adolescents: introduction and overview

Sudden unexpected cardiac death (SCD) in a child or adolescent is a devastating event with serious impact on the family, the school, and the community. This article reviews the epidemiology of SCD in children and adolescents and includes a discussion of its incidence and etiologies. It also discusses strategies for prevention.     

Acute bleomycin toxicity

A case of acute pulmonary toxicity due to bleomycin leading to death in 60 hours is described in an 11-year-old male with Hodgkin's disease. This acute presentation in an immunologically comprised host caused consideration of etiologies other than drugs. Previous reports in the literature have not documented such an acute fatal course as a result of bleomycin toxicity.     

Neonatal long QT syndrome and sudden cardiac death

Neonatal sudden cardiac death most often results from cardiac electrical diseases, cardiomyopathies, or sudden infant death syndrome. In infants without a known premortem diagnosis or abnormalities identified at autopsy, sudden infant death syndrome accounts for the vast majority of sudden deaths. Potential cardiac causes of some sudden infant death syndrome cases may include malignant brady- or tachyarrhythmias and congenital long QT syndrome. The possible mechanisms include abnormal brain stem respiratory control of arousal, dysautonomia and malignant cardiac bradyarrhythmias or tachyarrhythmias. Screening for neonatal sudden cardiac death may not be feasible, but hopefully through careful review of history, physical examination, and family health history, and judicious diagnostic testing, can the risk of cardiac sudden death be reduced. Further comprehension of the genetic basis of inherited arrhythmia disorders may help elucidate the mechanisms of arrhythmogenesis and etiologies of sudden infant death. Prevention and treatment of these disorders may also be improved through more detailed understanding of the molecular basis of cardiac electrical pathophysiology.     

Role of congenital long-QT syndrome in unexplained sudden infant death: proposal for an electrocardiographic screening in relatives

Introduction  Congenital long-QT syndrome (LQTS) is a sporadic or familial inherited arrhythmia. It can lead to sudden death by ventricular fibrillation which occurs at any age but particularly during infancy. Recent studies of postmortem molecular analysis in infants who died of unexplained sudden infant death syndrome (SIDS) showed abnormal mutations to LQTS in 10% to 12%. Current methods of etiologic investigation of sudden infant death syndrome do not allow the diagnosis of LQTS. A targeted anamnesis together with systematic electrocardiograms of first- and second-degree relatives could be an efficient LQTS diagnostic tool. Therefore, we propose to include them in screening procedures for SIDS etiologies. Conclusion  LQTS accounts for a significant number of unexplained SIDS. We suggest adding a systematic familial electrocardiographic screening to the current etiologic investigations in order to track congenital LQTS in relatives.     

Giant Cell Myocarditis and Endomyocardial Calcification in a 2.5-Month-Old Infant Triggered by Excessive Maternal Alcohol Abuse: Case Study of an Unusual Association

This report describes an unusual case of a 2.5-month-old infant’s sudden death secondary to giant cell myocarditis and endomyocardial calcification, both unusual entities in pediatric patients. The mother had a history of excessive alcohol consumption during pregnancy and the postnatal period. No infectious etiologies, hypersensivity, or autoimmune disorders were identified. Therefore, the authors assume that alcohol exposure might be responsible for the inflammatory giant cell process complicated with endomyocardial calcification in susceptible infants. This report is the first to describe the rare form of noninfectious myocarditis complicated with endomyocardial calcification possibly triggered by a toxic agent. The authors discuss the possible interaction between these processes that led to the infant’s sudden death.     

Sudden death in children and adolescents

This article focuses on sudden unexpected cardiac death (SCD) in children and adolescents. The authors discuss the epidemiology of SCD in children and adolescents, its incidence and etiologies, and strategies for prevention. Because most of the episodes of SCD or sudden cardiac arrest in children and adolescents occur in asymptomatic individuals unknown to have an underlying abnormality before their "event," the issues in this article primarily focus on this group of individuals.     

严重高钙血症发生机制及救治

钙是人体中必不可少的元素,在骨形成、激素释放和肌肉收缩神经传导方面具有重要作用。高钙危象属于内分泌急症,需要进行紧急救治,以免产生严重后果。儿童高钙危象发生虽然远远低于成人,但由于其临床症状重,更应引起临床医生警惕。本文对严重高钙血症的诊断、鉴别诊断及治疗原则等方面进行归纳总结。     

Sudden infant death syndrome, child sexual abuse, and child development

Since the introduction of the Back to Sleep Campaigns, there has been a dramatic reduction in sudden infant death syndrome in this country. Steven Blatt and Victoria Meguid review the literature surrounding sleep position. Investigators have continued efforts to find other modifiable risk factors of sudden infant death syndrome. A prospective study of more than 33,000 neonates found a link between a prolonged QT electrocardiogram interval and sudden infant death syndrome. Also discussed are investigations seeking to explain the relationship between smoking and sudden infant death syndrome. Ann Botash, Florence Jean-Louis and Mongkae Ploy Siripornsawan review the latest thinking on genital warts and their relation to specific viral etiologies and child sexual abuse. Other symptoms and signs of sexual abuse are the focus of a number of articles that can help the practitioner care for these unfortunate children. Catherine Church reviews medication options for children diagnosed with pervasive developmental disorders or autism spectrum disorders. Finally, in this article, risperidone, fluoxetine and naltrexone are reviewed.     

Immune-mediated coagulopathy associated with topical bovine thrombin: review of the pediatric literature

Postoperative bleeding may occur from a number of etiologies. An uncommon cause of postoperative bleeding is immune-mediated coagulopathy resulting from reexposure to a topical hemostat containing bovine thrombin. Some patients may develop antibodies to bovine thrombin (and other bovine coagulation proteins present in the product) which cross-react with human coagulation proteins, resulting in a coagulopathy, and occasionally, in serious bleeding and death. Most of the clinical information on this coagulopathy is in the adult medical literature. This article reviews the literature on pediatric cases with this coagulopathy and summarizes clinical outcomes and effective therapies.     

Under fives mortality in the urban slums of Lucknow

The main objective of this study was to elicit proportional cause specific mortality in the underfives in the urban slums of Lucknow in North India. The families with under five mortality in the 28 randomly selected slums in 1993 were located from the records of the slum health workers and verbal autopsy was conducted to assign a cause of death. There were 71 deaths among 2796 children. The annual under five mortality was 25.4 and the under five mortality rate was 126.7. After the neonatal period, “high fever” that could not be classified into any other disease incorporated in the verbal autopsy instrument, was the most common symptom associated with death, seen in 21.1% cases (95% C.I.: 15.5–34.4%) followed by these diseases: pheumonia in 19.7%, diarrhea in 18.3% and measles in 11.4%. “High fever” as the leading symptom associated with death is being reported for the first time from the urban slums of India. There is an urgent need to identify the underlying etiologies of death due to “high fever” and the policy implications are that children with fever must receive immediate and continued medical attention till the symptom persists.     

Surgical therapy for sudden cardiac death in children

Sudden cardiac death (SCD) in children is the result of multiple etiologies and treatment (prophylaxis) must be tailored accordingly. In children who do not have congenital heart disease, surgical therapy of SCD typically consists of implantation of an internal defibrillator, with specific attention to the small size of the patient. In children who have unrepaired congenital heart disease, therapy of SCD is primarily repair of the congenital anomaly. In children or young adults who have previously undergone surgery for congenital heart disease, SCD therapy consists of repair of any residual or acquired structural defect, often in combination with antiarrhythmia surgery or defibrillator implantation.     

Sudden death by arrythmia in a 4-year-old boy

Cardiac arrhythmia with sudden death is rare in children but mainly due to ventricular tachycardia. In case of pulseless ventricular tachycardia, prehospital treatment is crucial with immediate cardiopulmonary resuscitation and external electrical cardioversion. We report the case of pulseless ventricular tachycardia in a child with no past medical history. Sinus rhythm was obtained after 12 min of cardiorespiratory resuscitation and three external electrical shocks. An exhaustive diagnostic approach allow us to find its origin. The clinical progression was marked by a severe encephalopathy. The authors discuss different etiologies and treatment of arrhythmia in children, reviewing the pediatric algorithm for shockable rhythm.     

新生儿惊厥84例临床分析

目的 探索新生儿惊厥的病因、临床表现和急救治疗方法.方法 对我院84例新生儿惊厥患儿的临床资料、急救治疗措施的有效性进行分析总结.结果 新生儿惊厥的发病原因依次为新生儿缺氧缺血性脑病41例,颅内出血16例,低钙9例,此三种病因占全部病例的2/3以上.结论 减少新生儿惊厥的关键是做好围生期保健,针对病因及时诊治,快速止惊.     

儿童全血细胞减少症354例临床分析

目的 探讨引起儿童全血细胞减少的病因学及临床特征.方法 对354例在我科就诊的初发全血细胞减少症的患儿临床资料进行回顾性分析.结果 急性白血病和再生障碍性贫血为儿童全血细胞减少的主要病因,两者共占本组病例的71.2％;其次为噬血细胞综合征（9.9％）、感染（5.1％）、巨幼细胞性贫血（4.5％）以及免疫相关性全血细胞减少症（3.1％）,累积共占据了本组患儿的22.6％;其余病因比较少见,包括脾功能亢进、朗格罕细胞组织细胞增生症、神经母细胞瘤、脂质代谢异常、骨髓增生异常综合征、系统性红斑狼疮等,共占本组病例6.5％.中性粒细胞≤0.5×109/L者发热几率90％;血小板≤20×109/L者出血倾向严重,8.5％伴有内部脏器出血;血液系统疾病患者血红蛋白多数都波动在60 ～ 90 g/L,而非造血系统疾病血红蛋白＞90 g/L.结论 引起儿童全血细胞减少症的病因复杂,病种繁多,除急性白血病和再障外,噬血细胞综合征、感染、巨幼细胞性贫血是常见病因,其余少见病最初较难确诊,应引起儿科医生重视,就诊时血红蛋白水平可能对是否为血液系统疾病有提示意义.