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Background Descriptions of cutaneous findings associated with COVID-19 have not been consistently accompanied by histopathology or confirmatory testing for SARS-CoV-2. Objective To describe and classify the cutaneous findings with supporting histopathology of confirmed COVID-19 inpatients. Methods We included consecutive inpatients with a confirmed diagnosis of COVID-19 for whom a dermatology consult was requested. A skin biopsy was performed in all cases. Skin findings were classified as being compatible with a cutaneous manifestation of COVID-19 or as representing a distinct clinical entity. Results Twenty-eight patients were studied in whom thirty-one dermatologic diagnoses were made. Twenty-two of the dermatoses were compatible with a cutaneous manifestation of COVID-19; nine entities were not associated with infection by SARS-CoV-2. The most common COVID-19-associated pattern was an exanthematous presentation. In four patients, a new pattern was observed, characterized by discrete papules with varied histopathological findings including a case of neutrophilic eccrine hidradenitis. No cases of pernio-like lesions were identified. Skin findings not associated with COVID-19 represented 29% of diagnoses and included Malassezia folliculitis, tinea, miliaria, and contact dermatitis. Limitations There is no gold-standard test to distinguish between viral exanthems and drug reactions. Conclusion A histopathological study is critical before attributing skin findings to a manifestation of COVID-19.

To evaluate the impact in maternity hospitals of using an embroidered "I sleep on my back" sleeping bag on observing the sleeping recommendations at 1 month after birth.This was a multicentere prospective study, exposed/unexposed type, in which mothers answered questionnaires (by telephone and online) 1 month after giving birth. The exposed group consisted of mothers who had given birth in a maternity hospital of the ELENA network in which the embroidered sleeping bag was used as a preventive action; the unexposed group of mothers gave birth in a maternity hospital of the RP2S network, without this specific preventive action.A total of 540 mothers participated in the study: 245 in the exposed group and 295 in the unexposed group. In the exposed group, 87.3% of infants slept exclusively on their back versus 75.9% in the unexposed group (P0.001); 91% of the mothers reported having actually used the sleeping bag. Except for room-sharing, compliance with the other sleeping recommendations was higher in the exposed group.Sleeping practices when infants were 1 month old were not optimal in our study population. A simple preventive initiative in maternity hospitals, using the embroidered "I sleep on my back" sleeping bags, is relevant and effective in improving compliance with the sleeping recommendations for infants at home.

Aim Primary hyperparathyroidism (PHPT) is a common endocrine disorder, with an estimated incidence of 1/500 women and 1/2000 men older than 40 years. Several factors influence the positivity of preoperative scans including serum calcium, PTH and vitamin D levels, and gland size. The aim of our study is to examine the effect of biochemical adversity of primary hyperparathyroidism on preoperative imaging (SPECT-CT and US parathyroids). Method Retrospective study of 176 parathyroidectomies (2017-2020) in a tertiary referral centre with biochemistry, SPECT-CT, US parathyroids and histology outcomes being recorded. Failed parathyroidectomies were excluded. Results Patients were divided into 4 groups based on the preoperative calcium levels (normocalcemia 3.0 mmol/l, 14.2% of patients). Age (p-0.0297), preoperative vitamin D (p-0.03) and PTH levels (p-0.0001) were different while SPECT-CT positivity (p-0.29) weight of gland (p-0.015) and US positivity (p-0.09) were similar within the subgroups. Looking at the whole group, patients with positive SPECT-CT have a larger weight (p Conclusions Serum PTH levels and weight of the gland are directly correlated with positivity of preoperative imaging. Preoperative calcium and vitamin D levels correlates indirectly with the severity of the disease but did not influence the preoperative imaging positivity.

Objective This study was undertaken to describe the spectrum of lung computed-tomography (CT) findings in children with pulmonary Langerhans cell histiocytosis (PLCH) and to evaluate for this population the CT-scan nodule and cyst scores proposed by adult pulmonologists at diagnosis and during follow-up. Methods Among 175 children with PLCH identified in the French national population-based Langerhans cell histiocytosis cohort, 60 were retrospectively selected by the availability of CT for a central review by three pediatric radiologists. These 60 patients are representative of childhood PLCH for almost all clinical aspects, except a lower percentage of risk organ involvement (38% vs 54%; P = 0.05). Results The 60 children's chest CT scans (n = 218) were reviewed. At diagnosis, 63% of them had nodules, 53% had cysts, and 29% had both. The percentages of patients with nodules or cysts increased from diagnosis to peak disease activity, respectively, from 63% to 73% and from 53% to 66%. The costophrenic angle was involved in 71%. Patients with pneumothorax (25%) had a higher median cyst score. Alveolar consolidation was observed in 34%. Patients with low CT-scan nodule and cyst scores had no long-term pulmonary sequelae. Conclusions Well-known characteristics of adult PLCH (nodules and cysts) were observed in children. The chest CT scores proposed by adult pulmonologists could easily be applied to childhood PLCH. Lesions in children, unlike those in adults, are frequently located near the costophrenic angles. Alveolar consolidation might be considered an atypical feature of childhood PLCH.

Nasal glial heterotopia (NGH) is a rare benign tumor of the median line. We describe the case of a child presenting a lateral nasal mass. The characteristics of the prenatal ultrasound and the postnatal clinical examination argued in favor of a congenital hemangioma (CH). The MRI performed at 6 weeks of life suggested glial heterotopia. This diagnosis was confirmed by the pathological analysis. Congenital hemangiomas and nasal glial heterotopies have similar clinical presentations. Prenatal ultrasound diagnosis between NGH and CH is difficult. Fetal MRI is not yet highly specific for these two lesions, but it can eliminate an intracerebral connection in cases of NGH. Postnatal exams are more specific. Flow on the Doppler exam is rapid for CH and slow for NGH. On MRI, these two lesions appear as a hypersignal on T2-weighted sequences, but less intense for NGH than for CH. Distinguishing between NGH and CH can be difficult. This does not have a direct incidence on treatment because it is surgical in both cases.

Background RF ablation is a standard practice approach for the symptomatic AF. The interest of substrate ablation, completing pulmonary vein isolation is controversial. The current ablation strategy for persistent AF ablation is a step-by step guided approach: PVI is most often followed by a large ablation of fragmented electrograms identified in AF or completed by linear ablation, but this current ablation strategies have shown a limited success rate with frequent arrhythmia recurrences. Recent studies suggest that the atrial arrhythmogenic sites are related to regions with heterogeneities and increased fibrosis, which is detected by reduced bipolar voltage areas. Objective Defragmentation low voltage areas (LVA) in sinus rhythm (SR) in addition to PVI may represent an efficient strategy for the ablation of persistent AF. Material and methods This is a prospective multicentric interventional study in France. Inclusion criteria: minimum age 18 years, patients with persistent AF ablation, no specific indication restrictions or equipment used regardless of the outcome of the procedure. Number of patients: 210 patients included in 9 centres. Three groups will be constituted: Group 1: PVI without LVA identified, group 2: PVI only, with LVA identified but defragmentation will not be carried out and group 3: PVI with LVA identified and defragmentation will be carried out. Definition of LVA in SR: all potentials 55 ms. Inducibility of AF is realised at the end of the procedure. Usual blanking period of 3 months. Follow-up: clinical evaluation, ECG, and 24h-holter ECG at 6, 12 and 18 months. Outcomes Primary outcome: recurrence of AF at 18 months. Secondary outcomes: comparison of efficiency and safety of the procedures, procedural data (X-ray data, RF duration, AF inducibility), organised atrial tachycaria occurring during FU.

Resume Le cancer medullaire de la thyroide (CMT) est rare chez l’enfant, presque toujours hereditaire, associe a une neoplasie endocrinienne multiple (NEM) 2A ou B. La survenue d’un CMT dans une NEM est quasi-systematique, ce qui conduit a proposer une thyroidectomie prophylactique en cas de diagnostic presymptomatique. En l’absence d’un diagnostic precoce, l’evolution metastatique, ganglionnaire et pulmonaire est ineluctable. Le traitement de ces formes disseminees n’est pas codifie. L’apparition recente de therapeutiques ciblees pour le gene RET , les inhibiteurs de tyrosine-kinase, a ouvert de nouvelles perspectives pour ces patients jusque-la non curables. Nous rapportons l’observation d’une enfant traitee par vandetanib a l’âge de 11 ans, sans chirurgie thyroidienne, pour laquelle l’efficacite therapeutique et la tolerance du traitement ont ete satisfaisantes sur 5 ans.

Postvaccination osteo-articular mycobacterial infectious disease is a rare and potentially serious complication after Bacillus Calmette-Guerin (BCG) vaccine. We report on a case of a former preterm baby born at 30 weeks of gestation who was vaccinated with BCG Copenhagen strain at 2 months of age. He presented 6 months later with a painful limp, which was found to be a mono-articular osteitis of the right ankle. Histology of the biopsy showed signs of mycobacterial infection and molecular analysis confirmed a BCG infection. Blood tests did not reveal any immunodeficiency associated with the disease (IFN-gamma levels were normal). The course of the disease was favorable with 9 months of antibiotic therapy against mycobacteria. BCG complications should lead to screening for immunodeficiency. The prognosis of BCG osteitis is excellent if the disease is localized. No link between prematurity and BCG complications has been found to date. BCG vaccination of premature infant should be the same as for the general population.

Resume Dix a quinze pour cent des enfants porteurs d’un neuroblastome presentent une extension de leur tumeur primitive dans le canal rachidien, a travers un ou plusieurs foramen intervertebraux. Soixante pour cent d’entre eux ont des signes de compression medullaire lors du diagnostic (douleurs dorsales, deficit moteur, troubles de l’equilibre, faiblesse des membres inferieurs, parapaparesie ou paraplegie, cyphoscoliose, troubles sphincteriens vesicorectaux, troubles sensoriels, incluant des douleurs neuropathiques). Il s’agit le plus souvent d’enfants jeunes, au cours des deux premieres annees de vie, qui sont porteurs de tumeurs plus souvent localisees et biologiquement favorables, justifiant un traitement peu agressif sur le plan oncologique, du fait d’un pronostic vital favorable. Il faut souligner la difficulte du diagnostic des signes neurologiques chez les jeunes nourrissons. Bien que les traitements de decompression (chimiotherapie, neurochirurgie) offrent des chances d’amelioration, voire de recuperation complete, ces patients sont globalement exposes a d’importants risques de sequelles neurologiques a long terme (deficit moteur, trouble sphincterien) ainsi qu’a des sequelles orthopediques ; ceci souligne l’importance d’etudes sur ce groupe de patients pour mieux connaitre les facteurs lies a la maladie et a son traitement qui permettraient d’ameliorer leur prise en charge. C’est dans cet objectif que le reseau SIOPEN (European Neuroblastoma Research Network) a mis en place un registre international, multicentrique prospectif des tumeurs neuroblastiques avec extension intra-rachidienne, dont l’objectif principal est de collecter prospectivement les donnees afin d’evaluer les facteurs de risque sur l’evolution neurologique et orthopedique a court et long terme. Ceci devrait permettre d’ameliorer les recommandations de prise en charge de tels patients.

Despite significant progress in understanding anxiety disorders in youth, affected children are often unrecognized and never receive adequate treatment recognition. Although common among children and adolescents, many parents and health care providers do not realize anxiety disorders in youth predict anxiety disorders in adulthood. The history of anxiety disorders in childhood and their continuity into adolescence and adulthood are discussed. Treatment options and best practices for psychiatric nurses are also explored. [ Journal of Psychosocial Nursing and Mental Health Services , 54 (6), 25–28.]

Giant cell tumors (GCT) are benign primary bone tumors, locally aggressive, affecting in long bones in young adults during the third decade. It is rare to experience this lesion in skeletally immature patients. GCT are related to a risk of local recurrence and malignant transformation. We report a rare case of a giant cell tumor of the thoracic spine in a skeletally immature girl presenting with a painful right scoliosis. MRI, CT scan, and bone scintigraphy were discordant and the percutaneous biopsy non-contributive. A marginal “en bloc” resection was performed and revealed the GCT. Based on a literature review, the diagnosis and the surgical management of this case are discussed.

Spinal cord compression in Wilms’ tumor (WT) is an extremely rare event that can have a very poor prognosis if not taken care of rapidly. Most cases reported in the literature involve widely metastatic patient with bone or paraspinal metastases or occasionally intradural metastasis. Here, we present the case of a 3-year-old girl of WT confirmed by biopsy, with spinal cord compression due to the direct contiguous spread of a tumor through 2 vertebral foramina. Abdominal ultrasonography and magnetic resonance imaging performed for an abdominal mass revealed a large heterogeneous tumor near the upper pole of the left kidney. A nodular infiltration extended through the T11-L1 and L1-L2 neural foramina, forming an intraspinal mass that compressed the spinal cord. Major paresthesia subsequently occurred, requiring urgent treatment with corticosteroids and chemotherapy. The evolution was rapidly satisfying. After six courses of chemotherapy, a left nephrectomy was performed. Macroscopic examination identified a large tumor attached to the kidney without renal infiltration. Microscopical examination concluded to a nephroblastoma with regressive changes, of intermediate risk. Evolution at 6 months is satisfactory, with no neurological deficit. The histological aspect of the tumor and the clinical outcome suggest that she had an extrarenal WT that spread through the vertebral foramina and was secondarily attached to the kidney.

Children and adolescents exposed to violence and upheaval of war and relocation are at high risk of developing posttraumatic stress disorder (PTSD) and depression. Rates of PTSD among refugee children may exceed 50%. Additional stressors encountered while adjusting to host cultures add another layer of difficulty. Most refugee children struggling with symptoms of PTSD or depression are never linked with appropriate mental health care resources. Psychiatric nurses can serve a critical function in the identification and treatment of refugee children experiencing PTSD and depression. [ Journal of Psychosocial Nursing and Mental Health Services , 53 (12), 16–18.]

International audience; Neonatal arterial ischemic stroke (NAIS) is a rare event that occurs in approximately one in 5000 term or close-to-term infants. Most affected infants will present with seizures. Although a well-recognized clinical entity, many questions remain regarding diagnosis, risk factors, treatment, and follow-up modalities. In the absence of a known pathophysiological mechanism and lack of evidence-based guidelines, only supportive care is currently provided. To address these issues, a French national committee set up by the French Neonatal Society (Société française de néonatologie) and the national referral center (Centre national de référence) for arterial ischemic stroke in children drew up guidelines based on an HAS (Haute Autorité de santé [HAS]; French national authority for health) methodology. The main findings and recommendations established by the study group are: (1) among the risk factors, male sex, primiparity, caesarean section, perinatal hypoxia, and fetal/neonatal infection (mainly bacterial meningitis) seem to be the most frequent. As for guidelines, the study group recommends the following: (1) the transfer of neonates with suspected NAIS to a neonatal intensive care unit with available equipment to establish a reliable diagnosis with MRI imaging and neurophysiological monitoring, preferably by continuous video EEG; (2) acute treatment of suspected infection or other life-threatening processes should be addressed immediately by the primary medical team. Persistent seizures should be treated with a loading dose of phenobarbital 20mg/kg i.v.; (3) MRI of the brain is considered optimal for the diagnosis of NAIS. Diffusion-weighted imaging with apparent diffusion coefficient is considered the most sensitive measure for identifying infarct in the neonatal brain. The location and extent of the lesions are best assessed between 2 and 4 days after the onset of stroke; (4) routine testing for thrombophilia (AT, PC PS deficiency, FV Leiden or FII20210A) or for detecting other biological risk factors such as antiphospholipid antibodies, high FVIII, homocysteinemia, the Lp(a) test, the MTHFR thermolabile variant should not be considered in neonates with NAIS. Testing for FV Leiden can be performed only in case of a documented family history of venous thromboembolic disease. Testing neonates for the presence of antiphospholipid antibodies should be considered only in case of clinical events arguing in favor of antiphospholipid syndrome in the mother; (5) unlike childhood arterial ischemic stroke, NAIS has a low 5-year recurrence rate (approximately 1 %), except in those children with congenital heart disease or multiple genetic thrombophilia. Therefore, initiation of anticoagulation or antithrombotic agents, including heparin products, is not recommended in the newborn without identifiable risk factors; (6) the study group recommends that in case of delayed motor milestones or early handedness, multidisciplinary rehabilitation is recommended as early as possible. Newborns should have physical therapy evaluation and ongoing outpatient follow-up. Given the risk of later-onset cognitive, language, and behavioral disabilities, neuropsychological testing in preschool and at school age is highly recommended.; L’accident vasculaire cérébral ischémique artériel néonatal (AVCian) est une pathologie rare. Afin d’actualiser les connaissances sur ce sujet, un groupe de travail multidisciplinaire s’est constitué sous l’égide de la Société française de néonatologie et le Centre national de référence de l’AVC de l’enfant afin de proposer des recommandations sur les facteurs de risque, les modalités de transfert et de prise en charge pré-hospitalière, les modalités diagnostiques et thérapeutiques, le traitement, le pronostic et la prise en charge à court et moyen terme. Ces recommandations ont été réalisées selon la méthodologie de la Haute autorité de santé et en fonctions des thématiques proposées par un comité d’experts. Les principales recommandations issues de ce travail sont : (1) l’orientation du patient vers une unité de réanimation ou de soins intensifs néonatals disposant d’une imagerie par résonance magnétique (IRM) facilement accessible et de la possibilité de réaliser une surveillance continue par électro-encéphalogramme ; (2) le phénobarbital est le médicament de première ligne pour le traitement des crises convulsives ; (3) l’IRM réalisée entre j2 et j4 après la survenue de l’AVCian est la meilleure technique pour confirmer le diagnostic et préciser son extension ; (4) un facteur biologique de risque thrombotique ne doit pas être systématiquement recherché après un AVCian, sauf en cas d’antécédent thrombotique veineux familial ; (5) un traitement thrombolytique n’est pas recommandé ; (6) une prise en charge rééducative précoce est recommandée en cas de déficience motrice évidente.

Background Anticholinergic drugs constitute the first-line pharmacologic treatment for overactive bladder (OAB). Of the various anticholinergic agents available, trospium chloride appears to have potentially favorable chemical and pharmacologic properties leading to reduce the incidence of central nervous system (CNS) adverse reactions, since their ability to cross the blood-brain barrier is reduced (relative to other drugs in this therapeutic class). Objective The objective of the present survey was to establish whether or not CNS adverse reactions may be associated with trospium exposure by evaluating spontaneous reports made to the French pharmacovigilance database (FPVD). Methods All serious adverse drug reactions, specifically confusion, hallucinations, agitation and cognitive impairment, associated with trospium (the immediate-release form only) recorded in the FPVD between September 2005 to September 2011 were identified and analyzed. Results We identified 15 cases of CNS adverse events, according to at least one of the following terms: confusion (n = 9), hallucinations (n = 6), cognitive impairment (n = 4) and agitation (n = 2) and in which trospium chloride was suspected to have a causal link according to their imputability evaluation. The reports concerned 10 women and 5 men, with a mean age of 81 years (range: 62 to 96 years). The symptoms varied from relatively acute states of confusion and/or hallucinations to more progressive cognitive changes. In all these cases, CNS symptoms resolved rapidly after treatment discontinuation. Conclusion Despite the drug's favorable physiochemical properties, we have found pharmacovigilance data indicating that trospium chloride prescribed for OAB can induce CNS adverse reactions, such as confusion, hallucinations, agitation and/or cognitive impairment. Physicians should consider withdrawing trospium chloride if CNS symptoms suggestive of anticholinergic adverse effects occur in patients treated with this drug.

International audience

Irritability is common among children 5 and younger and is defined as a marked predisposition to anger. Irritability in childhood is associated with later psychopathology, suicide, and financial instability. Its persistence through later childhood may be associated with mood, anxiety, and substance use disorders. Assessment of irritability and psychiatric nursing implications are discussed. [ Journal of Psychosocial Nursing and Mental Health Services, 54 (12), 28–31.]

Suicide by children younger than 11 is a sad and tragic occurrence. Indeed, suicidal ideation and behavior has been documented in children as young as ages 3 to 7. The current overview provides a discussion of the frequency of suicide in children and associated predisposing conditions, such as depression and abuse. In response to these tragic events, nursing interventions are also proposed. [ Journal of Psychosocial Nursing and Mental Health Services , 54 (10), 27–30.]

With alarming frequency, an individual's first substance use occurs in childhood or adolescence. However, the use of many types of substances among individuals younger than 18 has been gradually declining over the past 6 years, and our understanding of risk factors for youth substance use has improved. Risk factors identified as possibly contributing to a young individual's first encounter with cigarettes or alcohol include parents' own substance use or mental health problems. Mental disorders of children have been implicated in substance use as well. Screening and interventions are available to reduce the frequency and intensity of adolescent substance use and are suggested in the current article. Nurses are in a position to identify adolescents who are at risk for substance use disorders and link the family system to effective intervention. [ Journal of Psychosocial Nursing and Mental Health Services, 54 (8), 24–27.]

Resume Les salmonelloses non typhiques peuvent etre responsables d’infections invasives severes chez les enfants drepanocytaires, avec notamment des localisations osteo-articulaires, mettant en jeu le pronostic vital et fonctionnel des patients. Nous decrivons les cas de 2 enfants drepanocytaires ayant presente des tableaux d’infections osteo-articulaires pauci-symptomatiques a Salmonella au retour de voyages en Afrique du Nord. L’evolution a ete favorable dans les 2 observations apres un traitement antibiotique approprie, malgre le caractere multiresistant de la salmonelle identifiee chez l’un des enfants. Les salmonelloses invasives restent des affections rares en France mais qui, du fait de leur gravite, doivent etre evoquees chez tout patient drepanocytaire febrile, notamment dans un contexte de retour d’Afrique. Un diagnostic clinique precoce est indispensable afin de mettre en route, sans attendre les resultats des prelevements, une therapeutique empirique appropriee.

Resume Objectif L’objectif principal est de decrire l’incidence des effets indesirables medicamenteux (EIM) codes par les archivistes medicales a partir des informations contenues dans le dossier medical des patients. L’objectif secondaire est de determiner les classes therapeutiques causant les EIM en fonction de l’âge et de comparer l’incidence selon la revision de la classification internationale des maladies (CIM) utilisee. Methode Il s’agit d’une etude non experimentale et descriptive des EIM dans un centre hospitalier universitaire. Ont ete inclus dans l’etude tous les EIM (i.e. codes E930 a E949 de la CIM-9 et codes Y40 a Y59 de la CIM-10) survenus chez des patients âges de 18 ans et moins. Le protocole de recherche a ete accepte par le comite d’ethique de la recherche du CHU Sainte-Justine. Resultats On a denombre en moyenne 225,7 ± 65,1 EIM codes par annee pour une incidence moyenne annuelle des EIM en pediatrie de 1,64 % ± 0,41 % ( n = 4740 EIM, en moyenne 1,3 EIM par patient) pour 294 480 admissions survenues du 1 er avril 1989 au 31 mars 2010. Les EIM codes surviennent chez les 0–2 ans et les 12–18 ans dans une proportion de 30,5 % chacun et chez les 3–11 ans dans une proportion de 39 %. Les EIM causes par les antibiotiques (28 %), les anticancereux (18 %), les opiaces (6 %) et les corticoides (6 %) representent plus de 50 % des EIM totaux. Conclusion On note une augmentation de l’incidence des EIM au fil des annees, particulierement a cause de l’importance accordee a la codification complete des dossiers par les archivistes medicales.

Mixed epithelial and stromal tumour of the kidney (MESTK) is a rare kidney neoplasm that occurs almost exclusively in perimenopausal women. Long-term oestrogen replacement appears to play a major role in its pathogenesis. Around 70 cases have been described in the international literature, none of which involve male children. Herein, we describe an atypical case of MESTK diagnosed in a 12-year-old prepubertal boy who presented with hematuria. Pathology and immunohistochemistry revealed a typical MESTK. The child was free of disease at 2-year follow up after a partial nephrectomy and tumour excision.

Carcinoid tumors are the most common endobronchial tumor in the pediatric population, and represent a rare cause of airway obstruction. The authors report two cases of boys aged 10 and 11 years old, who presented with a 12-month history of recurrent pneumonia. Bronchial endoscopy showed an endobronchial tumor. Chest CT-scan identified local extension and lung-associated lesions; octreoscan was performed to detect distant metastases. Histopathological study concluded in typical carcinoid tumor. The outcome after surgical conservative resection is uneventful with a follow-up of 7 and 26 months. Bronchial tumors must be considered in children with recurrent pneumonia or persistant respiratory symptoms, and require CT scan and bronchial endoscopy for their diagnosis.

Objectives To investigate the correlation between fetal lung volume (FLV), measured with magnetic resonance imaging (MRI), and postnatal mortality in newborns with prenatally diagnosed isolated congenital diaphragmatic hernia (CDH). Methods In a 4-year prospective multicenter study, 77 fetuses with isolated CDH diagnosed between 20 and 33 weeks' gestation underwent fast spin-echo T2-weighted lung MRI. These MRI-FLV measurements were compared with a previously published normative curve obtained in 215 fetuses without thoracoabdominal malformations and with normal ultrasound biometric findings. FLV measurements were correlated with postnatal survival. The mean gestational age at MRI was 31.3 weeks. Results The measured/expected FLV ratio was significantly lower in the newborns with CDH who died compared with those who survived (23.6 ± 12.2 vs. 36.1 ± 13.0, P < 0.001). When the ratio was below 25%, there was a significant decrease in postnatal survival (19% vs. 40.3%, P = 0.008). Survival was significantly lower for neonates when one lung could not be seen by fetal MRI compared with those fetuses with two visible lungs on MRI (17.9% vs. 62.1%, P < 0.001). Conclusion In isolated CDH, FLV measurement by MRI is a good predictor of postnatal mortality due to pulmonary hypoplasia. Copyright © 2005 ISUOG. Published by John Wiley & Sons, Ltd.

Reaching an accurate diagnosis in children with mental retardation associated or not with dysmorphic signs is important to make precise diagnosis of a syndrome and for genetic counseling. A female case with severe growth and development delay, dysmorphic features and feeding disorder is presented. Antenataly, the fetus was observed to have increased nuchal translucency and a slight hypoplastic cerebellum. A standard karyotype was normal. RES and a submicroscopic unbalanced subtelomeric translocation t(2p; 10q) were demonstrated after birth. We show that within the framework of a collaborative approach, a concerted research of submicroscopic subtelomeric rearrangements should be performed in case of mental retardation associated with facial dysmorphic features, and when other etiologies or non-genetic factors (iatrogenic, toxic, infectious, metabolic...) have been ruled out.

Background The aim of this study was to evaluate the impact of an intervention designed to improve postoperative pain assessment and recording by nurses. Methods This cluster randomized clinical trial included two intervals of 3 months each: a preintervention observational study (period 1) followed by a period of intervention (period 2). Six surgical wards were randomly assigned to receive either an experimental intervention, including educational seminars for nurses on pain, pain assessment and the use of a visual analogue scale (n = 3), or standard care (control group; n = 3). The main outcome measures were the percentage of patients in whom pain was assessed, number of pain assessments per patient and postoperative pain intensity scores. Results A total of 2278 patients were included. Significant improvements were found in the percentage of patients in whom pain was assessed and the number of daily pain assessments per patient in the intervention group between periods 1 and 2. In contrast, these outcomes were unchanged between the two intervals in wards randomized to standard care. During period 2, pain scores at 48 h were significantly decreased in the intervention wards compared with those in the control group. Conclusion An educational programme dedicated to nurses strongly increased the use of regular pain assessment, and may have contributed to a modest improvement in postoperative analgesia.

Resume Objectifs. – L’invagination intestinale sur lymphome reste, pour le chirurgien pediatre, une situation delicate, rare et source de pieges aux consequences parfois lourdes. L’analyse retrospective de sept observations associee a une revue de la litterature permet de degager une conduite a tenir pratique. Observations. – Six garcons et une fille, âges de trois a quinze ans, ont presente un lymphome revele par une invagination intestinale. Tous les enfants ont ete operes par laparotomie : biopsie d’une volumineuse masse abdominale six et huit semaines apres la resection d’un boudin d’invagination (2 cas), resection ileale segmentaire d’un boudin d’invagination necrose (1 cas), hemicolectomie droite realisee devant la constatation d’une tumeur appendiculaire (1 cas), resection cuneiforme d’une lesion tumorale parietale hemicirconferentielle (2 cas), biopsie d’une adenopathie mesenterique lymphomateuse avec analyse extemporanee sans geste sur la lesion tumorale ileale dans un cas. Une polychimiotherapie a ete initiee des le diagnostic. Les suites ont toujours ete simples. Tous les enfants sont suivis sans recidive avec un recul de 15 mois a 13 ans. Commentaires. – L’aspect peroperatoire des lymphomes digestifs doit etre connu de tout chirurgien. Le diagnostic peut etre difficile a porter lors d’un episode d’invagination imposant, en cas d’intervention, des biopsies sur toute zone suspecte. La resection intestinale permet d’alleger la chimiotherapie mais doit etre limitee : resection segmentaire en cas d’invagination irreductible ou de complication, simple tumorectomie en zone saine en cas de lesion parietale isolee. Si le diagnostic de lymphome peut etre obtenu par des prelevements peripheriques (liquide peritoneal, liquide pleural, adenopathie mesenterique, myelogramme, …), le chirurgien peut s’abstenir de tout geste sur le tube digestif en dehors d’une desinvagination manuelle.

Objectives: The prenatal finding of a large cystic adrenal mass raises the dilemma of the differential diagnosis between adrenal hemorrhage and cystic neuroblastoma. The possibility of a neuroblastoma usually leads to surgical excision of such tumors. Nevertheless, an adrenal hemorrhage has to be recognized, so that unnecessary surgery may be avoided. Methods: Three cases of large prenatally detected adrenal masses managed nonoperatively are reported. Data studied were: age at the diagnosis, prenatal and postnatal ultrasonographic consistency, and tumor marker levels. Size and sonographic evolution were also studied. Results: In all 3 cases, a cystic mass, measuring more than 40 mm in size, was detected during the 3rd trimester of pregnancy. The sonographic appearance evolved from a sonolucent tumor to a heterogeneous mass with hyperechoic areas. The tumor marker levels were normal. All infants had a documented decrease in mass size at birth and were managed nonoperatively. All these tumors were considered adrenal hemorrhages. Conclusions: Prenatal ultrasonography rarely permits to distinguish an adrenal hemorrhage from a cystic neuroblastoma. The differential diagnosis, even in large masses, is based on close postnatal follow-up with serial sonography. Surgery is not mandatory, unless the size does not decrease. However, without pathologic proof, it is not possible to differentiate an adrenal hemorrhage from a spontaneously resolved neuroblastoma.

Neuroimaging is critical for the diagnosis of neonatal arterial ischemic stroke (NAIS) and for prognosis estimation. The purpose of this work is to define guidelines of clinical neuroimaging for the diagnosis of NAIS, for the optimization of the imaging timing and for the assessment of the prognostic value of each imaging technique. A systematic search of electronic databases (Medline via Pubmed) for studies whose title and abstract were focused on NAIS has been conducted. One hundred and ten articles were selected and their results were analyzed by three Senior Practitioners of pediatric radiology using common methodology for guidelines elaboration within the group of experts gathered by Scientific Societies in the field. MRI with a diffu si on-weighted sequence (DWI) and T1, T2, and T2*-weighted sequences must be performed in the case of suspected NAIS (no sedation is required). In the first hours after the injury, an acute ischemic lesion is characterized by a hypersignal on the diffusion-weighted sequence, with a decrease of the apparent coefficient of diffusion (ADC). The best time to evaluate the extent of the ischemic lesion is between day 2 and day 4 after injury, when the ADC decrease reaches its nadir. In the acute phase, US may be useful as first imaging at the bedside to exclude other pathologies like large space-occupying hemorrhages, but its specific added value on NAIS diagnosis or prognosis assessment is very low. CT scan has no added value in NAIS, compared to MRI. Motor outcome is correlated with the extent of the lesion and with the presence of a definite injury of the corticospinal tract, which is well seen on the diffusion sequence at the acute stage. A secondary atrophy within the mesencephalon (cerebral peduncles) is tied in with a high risk of hemiplegia. Visual outcome is more often compromised in the case of lesions of the posterior cerebral artery territory.

European Annals of Otorhinolaryngology, Head and Neck Diseases - Vol. 128 - N° 3 - p. 159-161

Background The conventional approach for MRI procedures in very young children is to use general anesthesia which comes with inherent risks. Non-pharmacological strategies to reduce anxiety in children have also been described, but they all require patient cooperation. The purpose of the study was to evaluate the ability to complete diagnosis using temporary spica cast immobilization (TSCI) in children less than 3 years old undergoing MRI procedures for lower limb disorders. Materials and methods A retrospective review identified 14 children under 3 years old that had required an MRI for a lower limb disorder, using TSCI. The MRI procedure was performed for evaluation of hip dysplasia, bone infections, limping, evaluation of soft tissue tumor and femoral head osteonecrosis. A spica cast was fitted by the pediatric orthopedic team. The MRI procedure was subsequently performed. Results Diagnosis was achieved in all cases. The radiologist identified movement artifacts (14 %) that did not impair the image quality enough to prevent interpretation. Conclusion TSCI is a safe, effective and costless procedure avoiding general anesthesia for young patients under 3 years old who require MRI for pelvis or lower limb disorders. Level of evidence IV.

Holoprosencephaly (HPE) is the most common forebrain defect in humans. It results from incomplete midline cleavage of the prosencephalon and can be caused by environmental and genetic factors. HPE is usually described as a continuum of brain malformations from the most severe alobar HPE to the middle interhemispheric fusion variant or syntelencephaly. A microform of HPE is limited to craniofacial features such as congenital nasal pyriform aperture stenosis and single central maxillary incisor, without brain malformation. Among the heterogeneous causes of HPE, point mutations and deletions in the SHH gene at 7q36 have been identified as well as extremely rare chromosomal rearrangements in the long-range enhancers of this gene. Here, we report a boy with an HPE microform associated with a Currarino syndrome. Array CGH detected a de novo 2.7-Mb deletion in the 7q36.3 region including the MNX1 gene, usually responsible for the Currarino triad but excluding SHH, which is just outside the deletion. This new case provides further evidence of the importance of the SHH long-range enhancers in the HPE spectrum.

We carried out a cross-sectional study in 12 rural villages in order to identify the risk factors for Kashin-Beck disease in Tibet. Children aged 5-15 years (n=575) were examined and their corresponding houses were visited. Samples were collected in order to study fungal contamination of stored grain and the organic matter content of drinking water. Multivariate analysis was performed using logistic regression and population attributable fractions were computed to estimate the impact of each factor. The following variables were independently associated with the disease: age, gender, low socio-economic status, indicators of a poorly diversified diet, iodine deficiency and small water container size (with higher organic matter levels in small containers). Selenium deficiency was severe in all study subjects. The degree of fungal contamination of barley grain was related to the highest percentage of cases (65%) in a sample of the study population. Higher urinary iodine levels were not associated with decreasing prevalence rates when Alternaria sp. was isolated. The data that we report supports the hypothesis that Kashin-Beck disease occurs as a consequence of oxidative damage to cartilage and bone cells when associated with decreased antioxidant defence. Another mechanism that may coexist is bone remodelling stimulated by thyroid hormones whose actions can be blocked by certain mycotoxins.

To plot normal fetal lung volume (FLV) obtained with fast spin-echo magnetic resonance (MR) images against gestational age; to investigate the correlation between lung growth and fetal presentation, sex, and ultrasonographic (US) biometric measurements; and to investigate its potential application in fetuses with thoracoabdominal malformations.In a prospective multicenter study, 336 fetuses suspected of having central nervous system disorders underwent fast spin-echo T2-weighted lung MR imaging. Data obtained at 21-38 weeks gestation in 215 fetuses without thoracoabdominal malformations and with normal US biometric findings were selected for an FLV normative curve. FLV measurements obtained at pathologic examination with an immersion method were compared with MR FLV measurements in 11 fetuses. MR FLV values in 16 fetuses with thoracoabdominal malformations were compared with the normative curve.Normal FLV increased with gestational age as a power curve; the spread of values increased with age. Interobserver correlation was excellent (R(2) = 0.96). FLV measurements at MR imaging were 0.90 times those at pathologic examination. A constant ratio (0.78) between FLV on the left and right sides was observed. No significant difference in FLV was observed between fetal presentations. Normal FLV was observed in all fetuses with cystic adenomatoid malformations and in four of six with oligohydramnios. Lowest FLV values were observed in fetuses with diaphragmatic hernia.In fetuses with normal lungs, FLV distribution against gestational age is easily assessed in utero with fast spin-echo T2-weighted MR imaging. These preliminary findings illustrate the potential for comparing FLV measurements in fetuses at risk of lung hypoplasia with normative values.

Resume Objectif Nous avons voulu evaluer l'etat actuel de la sensibilite d'especes bacteriennes isolees d'infections non urinaires en pratique de ville vis-a-vis de dix antibiotiques couramment prescrits. Materiel et methodes Douze laboratoires prives ont isole, en 1997–1998, 700 souches, une majorite provenant d'infections respiratoires (57,5 %). Les resultats ont ete analyses en fonction des criteres suivants: antecedents d'antibiotherapie et/ou d'hospitalisation, existence d'une pathologie chronique ou recidivante. Resultats Concernant les enterobacteries, les pourcentages de sensibilite des souches variaient selon qu'elles avaient ete isolees chez des patients sans antecedent ou ayant au moins un antecedent: respectivement 42,4 et 29 % pour l'amoxicilline, 72,7 et 56,4 % pour l'amoxicilline-acide clavulanique, 97 et 91,1 % pour l'ofloxacine, 100 et 95,2 % pour la ciprofloxacine par exemple. La resistance aux fluoroquinolones n'etait rencontree que chez Proteus mirabilis et les Enterobacteries de type nosocomial. On notait l'absence d'evolution vers la resistance aux fluoroquinolones des isolats de Escherichia coli et de Staphylococcus aureus sensibles a la meticilline. Les souches de Haemophilus influenzae productrices de betalactamases etaient plus frequemment isolees au cours des recidives, 52 % des souches contre 29,8 % dans le groupe sans recidive. Parmi les souches de Streptococcus pneumoniae , 68,3 % etaient de sensibilite diminuee a la penicilline G. Commentaires L'analyse ou le suivi des simples pourcentages de resistance des isolats de laboratoires de ville est peu informative sur l'epidemiologie reelle des infections non urinaires, compte tenu du contexte actuel de la prescription des examens bacteriologiques en medecine de ville.

Agenesis of the corpus callosum is a frequent congenital malformation with many etiologies and clinical features. It can occur as an isolated defect which is usually sporadic, it is frequently associated with other malformations, genetic syndromes and metabolic disorders. Ante- or postnatal diagnosis must be followed by a careful search for etiology, and it raises uncertain and difficult question with regard to pregnancy continuation and infant development.

Early detection and low-risk treatment are the two main objectives of the management of developmental dislocation of the hip. The best way to evaluate neonatal hips is to perform clinical and ultrasound examinations at the same time, and to confront their results. Early diagnosis allows to restrict treatment to infants with neonatal dislocation who do not improve by 4 weeks of age. On the other hand, neonates with reductible dislocated hips must be treated at birth and followed at the joint consultation. Early diagnosis and management must not decrease later efforts to detect dislocated hip until walking age.

Through two clinical studies, tumor cells were searched for in the bone biopsies and cytapherisis of patients suffering from inflammatory tumors and who had undergone intensive therapy and autografts (Pegase 2 program). In these studies we used immunocytochemical test with two monoclonal antibodies. The results have shown the presence of tumor cells in 14% of the patients (respectively 18%), with no correlation to the appearance of metastases after 4 years in the first study. Nevertheless, the presence of these tumor cells seems to be an important factor in the number of relapses. It seems important to develop research into tumor contamination especially in the selection of grafts over the next few years.

Neonatal splenic injury is a rare but serious condition, due to the risk of haemorrhagic shock. We report on the case of a newborn infant with a neonatal respiratory distress that first evoked materno-fetal infection. Clinical deterioration, with anemia and abdominal distension, led then to the proper diagnosis. Dystocia seems to be the most likely cause of the splenic rupture in this report. Medical treatment is advocated as first line, while surgical treatment may be necessary in some cases. In the case surgery is inevitable, a conservative approach is preferable.

Intestinal perforations in preterm newborn are characterized with high morbidity and mortality rates. They often are associated with necrotizing enterocolitis and seldom correspond to idiopathic spontaneous intestinal perforation. Perforations upstream of an intestinal organic obstruction (atresia), or of a functionnal obstruction (meconium-ileus, Hirschsprung disease) have been considered to be rare in preterm newborns. We report a case of caecal perforation with a pneumoperitoneum which occurred at 5 days of life, in a 28-week gestational age infant, that was treated by cecostomy. There were no signs of necrotizing enterocolitis. At 43 days of life, the preoperative contrast enema study revealed a left colon transition zone, suggesting a Hirschsprung disease, which was confirmed by rectal biopsies. Transanal pull-through was performed. There were no postoperative complication. This case demonstrates that an isolated intestinal perforation in very preterm newborn can reveal a Hirschsprung disease, especially if it occurs in the caecum.

After neonatal lesions of dopamine neurones, an enhanced behavioural responsiveness towards D1 agonists has been described, suggesting a D1 receptor hypersensitivity. In the present study, unilateral striatal dopamine denervation in newborn rats induced a pronounced rotational behaviour following apomorphine injection at the adult age, without any change in the density of D1 binding sites in the denervated striatum. The amount of stimulatory G(olf) alpha subunit was increased by 35% in the lesioned striatum. The large form and the short forms of Gs alpha were also increased by 26% and 9%, respectively. Since in striatal neurones, the coupling of D1 receptor to adenylate cyclase is mostly provided by G(olf) alpha, our results strongly suggest that D1 hypersensitivity described after neonatal dopamine lesions results from an increase in the levels of G(olf) alpha protein.

The authors present a study of the intrinsic anatomy of the gluteus medius m, and of its innervation through the caudal branch of the superior gluteal n. The existence of an intramuscular tendon in the thickness of the gluteus medius was constantly prooved in 40 muscles. The relations of the intrinsic fibrous structure of the muscle and its innervation were studied. The authors deduce from that the topography of a gluteus medius incision, with respect to a safety area towards its innervation, which leads to an exposure of the acetabulum that is satisfying and gives opportunities of a sound repair after the surgery of the hip joint through the transgluteal approach. They propose the "anterior hemimyotomy of the gluteus medius m" designation.

Basal ganglia of adult rats were examined for morphological and biochemical changes resulting from neonatal unilateral dopamine denervation of the striatum with increasing doses of 6-hydroxydopamine (4, 12 and 20 microg). Rotational behaviour induced by apomorphine (0.1 mg/kg) was observed in all rats injected with the high dose (20 microg) and totally absent in those injected with the low dose (4 microg). As assessed with tyrosine hydroxylase immunocytochemistry, the extent of dopamine denervation within the injected striatum was clearly related to the dose injected. In the mesencephalon, losses of tyrosine hydroxylase-immunoreactive cell bodies were proportional to the dose injected and the extent of neostriatal dopamine denervation. This retrograde cell loss predominated in the ventromedial and lateral parts of the substantia nigra pars compacta, with relative sparing of the ventral tegmental area. After the injection of the intermediate (12 microg) and the high (20 microg) doses, a network of thin tyrosine hydroxylase-immunoreactive fibres was visualized in the ventral part of the pars reticulata ipsilateral to the injected striatum, suggesting a neoinnervation of this structure by dopamine axons. After the high dose, the density of serotonin-immunoreactive fibres was enhanced in the anterior half of the lesioned striatum. Associated changes in dopamine and serotonin content and turnover were also documented on both sides, in the striatum and in two output structures of the basal ganglia, the globus pallidus and the substantia nigra. Dopamine content was decreased only on the injected side. After the low dose, equal reductions (-60%) were observed in the anterior striatum and the substantia nigra, whereas a more marked decrease was measured in the anterior striatum (-93%) than in the substantia nigra (-60% to -74%) after the intermediate and high doses. In the globus pallidus, dopamine tissue content was decreased (-51%) only after the high dose. Dopamine turnover was unchanged after the low dose in all structures examined and was increased in the striatum, on the lesioned side only, after the intermediate and high doses. Serotonin content was increased only on the injected side in the anterior striatum (+50% after the low and +92% after the high dose). Serotonin turnover was unchanged on the injected side but increased by +118% and by +81% in the contralateral anterior striatum after the low and high doses, respectively. It was also increased in both substantia nigra after the high dose. In conclusion, morphological changes similar to those described after a bilateral neonatal lesion were observed on the injected side in the model of the unilateral neonatal nigrostriatal dopamine denervation. Biochemical changes were, however, not restricted to the lesioned side. Notably, changes in serotonin turnover developed on the contralateral side. These morphological and biochemical adaptative changes need to be taken into account in considering the mechanisms implicated in the rotional behaviour measured in these animals.

Resume But de l'etude. – Notre objectif etait d'etudier la prevalence et les caracteristiques des syndromes de Guillain-Barre (SGB) associes a Campylobacter jejuni et au cytomegalovirus (CMV) dans un centre de prise en charge francais. Patients et methodes. – L'etude a porte sur 264 patients atteints de SGB hospitalises a l'hopital Raymond-Poincare (Garches) entre 1996 et 2001. Les donnees cliniques ont ete recueillies prospectivement. Les serums preleves a l'admission ont ete analyses retrospectivement pour la recherche des anticorps anti-C. jejuni, anti-CMV et antigangliosides GM1 et GM2. Resultats. – Les SGB associes a un profil serologique d'infection recente a C. jejuni etaient les plus frequents (58/264, 22 %) ; ils survenaient majoritairement chez des hommes d'âge mur (âge moyen : 51,3 ans ; sex-ratio H/F : 1,76), generalement apres des troubles gastro-intestinaux (52 %) ; ils etaient souvent purement moteurs (57 %), severes (ventilation artificielle : 40 %) et associes a une reponse IgG et/ou IgM anti-GM1 (44 %). Les SGB associes a une primo-infection par le CMV etaient moins frequents (40/264, 15 %), mais de gravite comparable (ventilation artificielle : 37,5 %) ; ils survenaient principalement chez des femmes jeunes (âge moyen : 35,9 ans ; sex-ratio H/F : 0,82), souvent apres une infection du tractus respiratoire (28 %) ou un syndrome pseudogrippal (15 %), et etaient frequemment associes a des troubles sensitifs (73 %) et a une reponse IgM anti-GM2 (47 %). Conclusion. – C. jejuni et le CMV sont deux causes majeures de SGB en France et sont associes a des tableaux distincts mais de severite comparable.

SUMMARYThe role of Campylobacter jejuni as the triggering agent of Guillain–Barré syndrome (GBS) has not been reassessed since the end of the 1990s in France. We report that the number of C. jejuni-related GBS cases increased continuously between 1996 and 2007 in the Paris region (mean annual increment: 7%, P = 0·007).