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Amblyopie chez l'enfant 儿童弱视
Pub Date : 2004-08-01 DOI: 10.1016/j.emcped.2004.03.001
P Dureau (Praticien titulaire), G Caputo (Chef de service)

Amblyopia is the most frequent aetiology of unilateral poor vision in childhood. It is related to abnormal development during the first years of life. Some cases are secondary to a malformation of the globe, but most of the amblyopias are functional, related to a refractive error or strabismus. Early detection is essential to the success of the treatment. This treatment has to be undertaken before the age of seven, and the paediatrician is the first-line actor in the detection of amblyopia. As soon the first months of life, observation of a strabismus, a nystagmus, and some simple examination signs must lead to an ophthalmologic examination in order to exclude any organic disease and to begin a treatment. In older children, vision testing with optotypes is simple and efficient for the detection of amblyopia. Basic of treatment principales are the correction of any refractive trouble by spectacles, patching of the good eye and penalization methods.

弱视是儿童单侧视力不佳最常见的病因。它与生命最初几年的异常发育有关。有些病例继发于眼球畸形,但大多数弱视是功能性的,与屈光不正或斜视有关。早期发现对治疗的成功至关重要。这种治疗必须在7岁之前进行,儿科医生是发现弱视的第一线演员。在出生后的最初几个月,如果观察到斜视、眼球震颤和一些简单的检查迹象,必须进行眼科检查,以排除任何器质性疾病并开始治疗。在年龄较大的儿童中,用视型检测弱视是一种简单有效的方法。基本的治疗原则是眼镜矫正任何屈光问题,好眼补眼和处罚方法。
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引用次数: 0
Psychoses à l'adolescence 青少年精神病
Pub Date : 2004-08-01 DOI: 10.1016/j.emcped.2004.03.002
M Botbol (Psychiatre des Hôpitaux, directeur médical) , Y Barrère (Psychiatre, médecin adjoint) , M Speranza (Praticien hospitalier)

Psychosis is a major concern for adolescent-specialized psychiatrists who face great difficulties in making diagnosis in case of acute or torpid symptomatology since at this age, such symptoms are not systematically correlated with a specific mental disorder. This symptomatology is relatively frequent as a polymorph expression of psychic suffering due to adolescence process. Adolescence is a period characterized by many deep internal and external changes, and it is also the preferential age period for the onset of most chronic mental disorders. At their beginning, most of these disorders have not yet the typical recognizable form they will have latter on, allowing at that time easier diagnosis. More than at other life periods, the clinician has to go beyond the descriptive symptomatology to find out the underlying psychopathological organisation that may be more informative on the observed disorder in terms of diagnosis and prognosis, with the awareness that schizophrenia begins frequently at this age. Similarly, we should not forget that this age corresponds also with the onset of mood disorders, often associated with psychotic positive symptoms that may lead to misdiagnosis. We should also distinguish, among pre-morbid symptoms, vulnerability factors or prodromal manifestations that induce important consequences in terms of therapeutic strategies.

精神病是青少年精神病专家关注的主要问题,他们在诊断急性或迟钝症状时面临很大困难,因为在这个年龄,这些症状与特定的精神障碍没有系统的联系。这种症状是相对常见的由于青春期过程的精神痛苦的多形态表达。青春期是一个具有许多内在和外在深层变化特征的时期,也是大多数慢性精神障碍发病的优先年龄期。在一开始,这些疾病中的大多数还没有典型的可识别的形式,他们后来会有,允许在那个时候更容易诊断。与其他生命阶段相比,临床医生必须超越描述性症状学,找出潜在的精神病理组织,这可能在诊断和预后方面对观察到的障碍提供更多信息,并意识到精神分裂症通常在这个年龄段开始。同样,我们不应该忘记,这个年龄也与情绪障碍的发病相对应,通常与可能导致误诊的精神病阳性症状相关。我们还应该在病前症状、易受伤害因素或前驱症状中区分出在治疗策略方面会引起重要后果的表现。
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引用次数: 8
Syndrome infectieux fœtal 胎儿感染性综合征
Pub Date : 2004-08-01 DOI: 10.1016/j.emcped.2004.02.005
F Jacquemard

Prenatal diagnosis of foetal infections has been greatly improved these last years since new molecular biology techniques became available, allowing to establish rapid and reliable diagnosis using polymerase chain reaction on amniotic fluid. In France, toxoplasmosis is the most frequently observed congenital parasitic infection. Any occurrence of maternal seroconversion (ascertained or very suspected) during pregnancy constitutes an indication for early clinical and biological check-up of the newborn. A chemo-prophylactic combination of chloroquine and proguanil (Savarine®) should be recommended in a pregnant woman going to endemic areas, in order to prevent the risk of congenital malaria and its clinical consequences. The cytomegalovirus (CMV)- induced infection is the most frequent maternal-foetal infection. It constitutes the first cause of neuro-sensorial handicaps acquired during intrauterine life. CMV-induced primary infection in a pregnant woman is therefore a situation with major pathological risk for the foetus. The foetal contamination is principally due to hematogenous trans-placental transmission, inducing viral proliferation in the foetus. The B19 parvovirus is commonly considered responsible for the megalerythema, or fifth disease. Embryo-foetopathies associated to parvovirus infections are now better understood: the rate of maternal-foetal transmission and the risk of complications are better identified. Rubella which is a benignant infection occurring during early childhood was the first infectious disease identified as responsible for embryo-foetopathies. The Australian ophthalmologist MacAlister Gregg was the first to hypothesise, in 1941, a relationship between the onset of a per gravidic rubella and congenital cataract. The serological screening for rubella which is mandatory on early pregnancy allows to identify the initial serological status of the pregnant woman. Potential embryo-foetopathy should therefore be suspected in case any delayed intrauterine growth associated to a heart abnormality is observed in the foetus. Chickenpox is an uncommon disease in women during pregnancy. For the mother, complications are mainly of pulmonary type (in about 10% of the cases), and potentially life-threatening. Before 24-week amenorrhoea, the fœtal transmission (estimated to approximate 6%) may be responsible for a congenital varicella syndrome in about 2% of cases. Perinatal chickenpox due to peripartum transmission induces in about 25% of the cases a neonatal chickenpox potentially serious and sometimes fatal; this risk is essentially associated to an eruption in the mother, occurring during the period from 5 days before delivery to 2 days after. Bacterial infections remain the major concern for paediatric obstetricians since they always constitute a frequent risk for perinatal morbidity and mortality (3rd cause of mortality). In all studies on maternal-foetal bacterial infections, the streptococcus B was

产前诊断的胎儿感染已经大大提高了这些年来,因为新的分子生物学技术成为可能,允许建立快速和可靠的诊断使用聚合酶链反应羊水。在法国,弓形虫病是最常见的先天性寄生虫感染。妊娠期间发生的任何母体血清转化(确定的或非常怀疑的)都是对新生儿进行早期临床和生物学检查的指征。应建议前往疟疾流行地区的孕妇使用氯喹和proguanil (Savarine®)联合化疗预防,以预防先天性疟疾的风险及其临床后果。巨细胞病毒(CMV)引起的感染是最常见的母婴感染。它构成了在宫内生活中获得的神经-感觉障碍的首要原因。因此,孕妇巨细胞病毒引起的原发性感染对胎儿具有重大病理风险。胎儿污染主要是由于血液经胎盘传播,诱导病毒在胎儿中增殖。B19细小病毒通常被认为是造成大红斑或第五种疾病的原因。与细小病毒感染相关的胚胎-胎儿病现在得到了更好的了解:母婴传播率和并发症的风险得到了更好的确定。风疹是一种发生在儿童早期的良性感染,是第一个被确定为导致胚胎-胎儿病的传染病。1941年,澳大利亚眼科医生麦克阿利斯特·格雷格(MacAlister Gregg)首先提出了妊娠风疹发病与先天性白内障之间的关系。风疹血清学筛查是妊娠早期的强制性检查,可用于确定孕妇的初始血清学状况。因此,如果在胎儿中观察到任何与心脏异常相关的宫内生长延迟,应怀疑潜在的胚胎-胎儿病。水痘在怀孕妇女中是一种罕见的疾病。对于母亲来说,并发症主要是肺部类型(约占病例的10%),并且可能危及生命。在24周闭经之前,fœtal传播(估计约为6%)可能导致约2%的先天性水痘综合征。围产期传播引起的围产期水痘约占25%,新生儿水痘可能严重,有时甚至致命;这种风险主要与母亲在分娩前5天至分娩后2天期间发生的皮疹有关。细菌感染仍然是儿科产科医生关注的主要问题,因为它们总是构成围产期发病和死亡的常见风险(第三大死亡原因)。在所有关于母胎细菌感染的研究中,B型链球菌被证明是主要的细菌。观察到死亡率显著下降(20世纪70年代为30-90%,目前为5-15%),这与怀孕期间的检测策略有关,例如在闭经35至37周期间进行阴道取样,筛查B型链球菌,以及对受感染妇女进行围产期预防性抗生素治疗。
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引用次数: 3
Diététique et néphropathies de l'enfant 儿童饮食与肾病
Pub Date : 2004-08-01 DOI: 10.1016/j.emcped.2004.04.002
M. Broyer , D. Folio , F. Mosser

Dietary recommendations constitute a major intervention in children with renal diseases. A sodium-limited diet is prescribed in potential or overt sodium retention states, such as nephritic syndromes and, generally speaking, in glomerulo-nephritis. Practical modalities of this prescription are explained taking into account the sodium content of the different foods. Diet plays a major role in the treatment of the infant with renal failure, because related anorexia and gastrointestinal disorders frequently result in naso-gastric or gastrostomy nutrition in order to provide sufficient energy. The principles of diet in patients with chronic renal impairment have evolved these last years. The previously recommended severe protein restriction is no longer recommended. Nevertheless, according to age and degree of renal impairment, recommendations for protein intakes are proposed on the basis of standard occidental alimentary habits, generally largely above the minimum amounts as estimated by experts taking into account the age. Limited protein intakes is useful for limiting phosphorus and acid loads, but its interest in slowing the renal function degradation remains questionable. Other recommendations concern the child with renal failure, especially potassium limitation when GFR decreases to below 20 mL/mn; water and sodium intakes must also be adjusted. Examples of dietary regimens together with practical indications are provided. Diet in nephrogenic diabetes insipidus and in some metabolic lithiasis is also considered.

饮食建议是肾病儿童的一项主要干预措施。在潜在的或明显的钠潴留状态下,如肾病综合征,一般来说,肾小球肾炎,应规定限钠饮食。考虑到不同食物的钠含量,解释了这种处方的实际方式。饮食在婴儿肾衰竭的治疗中起着重要的作用,因为相关的厌食症和胃肠道疾病经常导致鼻胃或胃造口营养,以提供足够的能量。近年来,慢性肾功能损害患者的饮食原则不断发展。以前推荐的严格的蛋白质限制不再被推荐。然而,根据年龄和肾功能损害程度,建议的蛋白质摄入量是在标准的西方饮食习惯的基础上提出的,通常大大高于专家考虑年龄估计的最低摄入量。有限的蛋白质摄入有助于限制磷和酸的负荷,但其在减缓肾功能退化方面的作用仍值得怀疑。其他建议涉及肾功能衰竭的儿童,特别是当GFR降至低于20 mL/mn时的钾限制;水和钠的摄入量也必须调整。提供了饮食方案的实例以及实际适应症。饮食在肾源性尿崩症和一些代谢性结石也被考虑。
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引用次数: 0
Néphrocalcinose de l’enfant
Pub Date : 2004-05-01 DOI: 10.1016/j.emcped.2004.01.001
M.-F Gagnadoux (Praticien hospitalier)

Nephrocalcinosis, the diagnosis of which is now made easier by ultrasonography, is not exceptional in children and even infants. Its etiologies are multiple, but almost all of them involve a metabolic disorder, most often of genetic or iatrogenic origin, increasing the urinary excretion of calcium or oxalate. The most frequent causes vary according to age. Nephrocalcinosis in newborn and infants suggests three main causes : “prematurity-associated hypercalciuria”, the origin of which, multifactorial, is mostly iatrogenic, early-onset tubulopathies (distal tubular acidosis, Bartter's syndrome), and infantile-onset primary hyperoxaluria, which is fortunately very rare. In older children, the main causes are : distal tubular acidosis, primary hyperoxaluria, and hypercalciurias from known genetic origin (Dent's disease, familial hypomagnesemia-hypercalciuria) or still “idiopathic”. The prognosis depends upon the extension of calcic deposit and, above all, on the feasibility, by treating the causal disease, of preventing the calcification process.

肾钙质沉着症的诊断现在通过超声检查变得更加容易,在儿童甚至婴儿中并不罕见。其病因多种多样,但几乎所有的病因都与代谢紊乱有关,最常见的是遗传或医源性,增加尿液中钙或草酸盐的排泄。最常见的病因因年龄而异。新生儿和婴儿肾钙质沉着症有三种主要原因:“早产相关高钙尿症”,其病因多因素,主要是医源性的;早发性小管病(远端小管酸中毒、巴特氏综合征);以及婴儿发病的原发性高钙尿症,幸运的是,这种情况非常罕见。在年龄较大的儿童中,主要原因是:远端小管酸中毒、原发性高氧尿和已知遗传来源的高钙尿(邓氏病、家族性低镁血症-高钙尿)或仍然是“特发性”的。预后取决于钙沉积的扩展,最重要的是,取决于通过治疗病因来预防钙化过程的可行性。
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引用次数: 1
Pathologie neurologique malformative fœtale 胎儿畸形神经病理学
Pub Date : 2004-05-01 DOI: 10.1016/j.emcped.2004.02.006
M.L Moutard, C Fallet-Blanco

« Congenital malformation » refers to any structural abnormality of the central nervous system (CNS), present at birth, and due to interrupted or modified CNS development during intrauterine life, whatever the aetiology. The frequency of CNS malformations has been estimated to 5-10/ 1,000 births, found in 5-10% of stillborn infants, or early deceased neonates. “Neural tube defect” (NTD) refers to a large set of malformations resulting from a neural tube closure that may be defective to a variable extent, or abnormal covering tissues induced by the axis mesoderm (posterior dura mater, axis skeleton, hypoderm) and the ectoderm.

“先天性畸形”是指任何中枢神经系统(CNS)的结构异常,在出生时就存在,并且由于子宫内中枢神经系统发育中断或改变,无论病因如何。据估计,中枢神经系统畸形的发生率为5-10/ 1000,在5-10%的死产婴儿或早期死亡新生儿中发现。“神经管缺损”(Neural tube defect, NTD)是指神经管闭合后可能出现不同程度缺陷的大量畸形,或由中轴中胚层(后硬脑膜、中轴骨架、下胚层)和外胚层诱发的覆盖组织异常。
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引用次数: 5
Détresses respiratoires du nouveau-né (en dehors des malformations et des maladies génétiques ou constitutionnelles) 新生儿呼吸窘迫(畸形、遗传或体质疾病除外)
Pub Date : 2004-05-01 DOI: 10.1016/j.emcped.2004.02.002
S Lorotte-Namouni (Chef de clinique-assistante des hôpitaux de Paris) , C Clamadieu (Praticien hospitalier) , P.-H Jarreau (Professeur des Universités, praticien hospitalier)

Respiratory disorders are one of the main cause of neonatal morbidity and mortality. Their aetiology is predominantly linked to an inappropriate cardio-respiratory adaptation at birth and to infections. Recent improvements in the management of these disorders diminished dramatically the mortality. These improvements are due to a better perinatal care organization, and to substantial advances in obstetrics and paediatrics, among which a wide use of antenatal steroids, exogenous surfactants and improvements in mechanical ventilation. Nevertheless, bronchopulmonary dysplasia remains a major problem in the outcome of the most preterm infants. A better knowledge of its physiopathology may open new therapeutic advances.

呼吸系统疾病是新生儿发病和死亡的主要原因之一。其病因主要与出生时不适当的心肺适应和感染有关。最近在这些疾病管理方面的改进大大降低了死亡率。这些改善是由于更好的围产期护理组织,以及产科和儿科的实质性进展,其中产前类固醇、外源性表面活性剂的广泛使用和机械通气的改进。然而,支气管肺发育不良仍然是大多数早产儿预后的主要问题。更好地了解其生理病理可能会带来新的治疗进展。
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引用次数: 10
Erratum à l'article : « Insuffisance rénale aiguë chez l’enfant » [EMC-Pédiatrie 2004;1:73–88] 文章勘误表:“儿童急性肾衰竭”[emc - pediatrie 2004;1:73 - 88]
Pub Date : 2004-05-01 DOI: 10.1016/j.emcped.2004.04.001
M.-A Macher
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引用次数: 0
Pathologie génitale fœtale
Pub Date : 2004-05-01 DOI: 10.1016/j.emcped.2004.02.004
V Mirlesse , C Nihoul-Fekete , J Mac Aleese

An abnormality of genital organs may occur during pregnancy in 0.1 to 0.3% of the cases. This event always constitute a real tragedy. Sexuality and reproduction appear to be the first concern, even before potential associate abnormalities or a clear urinary-genital prognostic are considered.

生殖器官的异常可能发生在怀孕期间在0.1%至0.3%的情况下。这种事件总是构成真正的悲剧。性和生殖似乎是首要关注的问题,甚至在潜在的相关异常或明确的泌尿生殖预后被考虑之前。
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引用次数: 1
Rein et médicaments Rein和massdiments
Pub Date : 2004-05-01 DOI: 10.1016/j.emcped.2004.01.003
P. Cochat (Professeur des Universités, praticien hospitalier) , L. Dubourg (Maître de conférence des universités, praticien hospitalier)

The interaction between drugs and the kidney can be considered at different levels: normal drug clearance, influence of the renal function on their elimination, expression of the drug nephrotoxicity, and their elimination in case of overdosing. In the child, available information often consists in extrapolated observations from adult subjects, and sometimes from animal experiments. It is therefore important to clearly identify the paediatric features of the drugs-kidney relationship, insofar as they considerably vary from the foetal period towards adolescence.

药物与肾脏的相互作用可以从不同的层面来考虑:正常的药物清除,肾功能对其消除的影响,药物肾毒性的表达,以及过量时药物的消除。对儿童而言,可获得的信息通常来自于对成年受试者的推断性观察,有时也来自于动物实验。因此,重要的是要清楚地确定药物-肾脏关系的儿科特征,因为它们从胎儿期到青春期有很大的不同。
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引用次数: 6
期刊
EMC - Pédiatrie
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