[Imaging strategy for children after a first episode of pyelonephritis]. / Explorations morphologiques après un premier épisode de pyélonéphrite chez l'enfant.

Pyelonephritis is a common bacterial disease in young children and is a serious infection because of its potential to produce renal scarring. One of the concerns of physicians is therefore the diagnosis of uropathy at risk for recurrence of pyelonephritis, especially high-grade reflux. There are no French recommendations on imaging evaluation after a first episode of pyelonephritis. Voiding cystography was systematically proposed years ago and recommended by the American Academy of Pediatrics until 1999. This systematic strategy exposed all children to a painful, irradiating exam, and exposed them to urinary tract infection. The American recommendations changed in 2011 and cystography is now only proposed to children with recurrence of pyelonephritis or with ultrasound abnormalities. A collaborative review of the literature involving the Pediatric Emergency, Nephrology and Surgery Departments at Necker-Enfants-Malades Hospital led us to propose an algorithm for imaging after the first episode of pyelonephritis in children. This algorithm was based on data from the past medical history (results of prenatal ultrasonography or recurrence of pyelonephritis), the results of the ultrasound exam at the time of diagnosis, and the procalcitonin concentration, to limit the indications for voiding cystography, limiting risk for delaying high-grade reflux diagnosis. Children with low risk for high-grade reflux can be followed up with an ultrasound exam 6 months after acute infection.

[Metered-dose inhaler with spacer vs nebulization for severe and potentially severe acute asthma treatment in the pediatric emergency department]. / Traitement aux urgences des crises d'asthme par nébulisations vs chambres d'inhalation.

OBJECTIVE: To compare treatment with beta 2 agonist delivered either by a spacer device or a nebulizer in children with severe or potentially severe acute asthma. METHODS: In this randomized trial, children 4 to 15 years, cared for in the emergency department for severe or potentially severe acute asthma, received 6 times either nebulizations of salbutamol (0.15mg/kg) or puffs of a beta 2 agonist (salbutamol 50 microg/kg or terbutaline 125 microg/kg). The primary outcome was the hospitalization rate. Secondary outcomes included percentage improvement in Bishop score, in PEF, SaO(2), respiratory and heart rates, side effects, length of stay and relapses 10 and 30 days later. RESULTS: Groups did not differ for baseline data. There were no significant differences between the 2 groups (nebulizer N=40, spacer N=39) for baseline characteristics before emergency department consultation except for length of acute asthma in the spacer group. Clinical evolution after treatment, hospitalization rate, relapse were similar including the more severe subgroup. In the spacer group, tachycardia was less frequent (P<0.02). The overall length of stay in the emergency department was significantly shorter (148+/-20 vs 108+/-13 min, P<10(-9)). CONCLUSIONS: The administration of beta 2 agonist using a metered-dose inhaler with spacer is an effective alternative to nebulizers for the treatment of children with severe or potentially severe acute asthma in the emergency department. Time gained can be used for asthma education.

[Hematemesis in an 11-month-old child: an unusual symptom revealing a foreign body in the stomach]. / Hématémèse chez un enfant de 11 mois: un mode de révélation rare d'un corps étranger intragastrique.

Ingestion of foreign body has often no consequence. We report on a case in an 11-month-old girl who was referred for mild hematemesis and anorexia. Upper digestive tract endoscopy found a small metallic foreign body in the gastric antrum. After its removal, all symptoms disappeared. It is usually recommended to remove foreign bodies by endoscopy when they are in esophageal position, or are more than 3 to 5 cm long, or have a shape that may hurt the gut mucosa. Although rare, a gastric foreign body should be searched for in face of an upper gastrointestinal bleeding in an infant.

[Oral morphine administration for children's traumatic pain]. / Utilisation de la morphine orale pour les douleurs post-traumatiques de l'enfant.

OBJECTIVES: To study the compliance of prescription, the efficacity and the adverse events of oral morphine used in the pediatric emergency department (ED) in traumatic pains. METHOD: This prospective study was conducted in the ED from october 2002 to september 2003. Children aged six months to 16 years with a visual analogic scale (VAS) score higher than 70 or with a traumatic member deformation received oral morphine (0,5 mg/kg). Pain was assessed every 30 minutes using two scales: behavioral observation by the faces scale and objective pain scale (OPS) for children less than five years, behavioral observation by the faces scale and self-report by VAS for children older than five years. The compliance of prescription, the pain scores and the adverse events were studied. RESULTS: Ninety-one children received oral morphine and seventy-four children were studied. Seventy per cent of prescriptions were in accordance with the recommendations. For patients younger than five years a rapid decrease of pain was observed in thirty minutes. The pain's reduction was respectively 79 and 84% with faces scale and OPS when they left ED. For children older than five years, pain's reduction was more important and more rapid when pain assessment was made by nurses than when it was self-reported in the first hour (pain reduction 58,2 and 36,1%). When leaving, pain reduction was the same with the two different assessments. No major adverse event was noted. CONCLUSION: Use of oral morphine in ED is simple, with a few numbers of adverse events. None was severe. Efficiency is correct after 30 to 60 minutes.

[Analgesia in the pediatric emergency department]. / Analgésie aux urgences pédiatriques.

Pain concerns more than 50% of the children cared in the emergency unit. After evaluation, it has to be cured with drugs adapted to its level and its origin. Residual pain needs therapeutic adjustment. The goal is to allow the child to restart his activities. A preventive sedation analgesia is necessary when a painful exam is to be performed, either for diagnosis or therapeutic purpose. For this goal ideal analgesics, either for monotherapy or associated, are easily and painlessly administered. They have rapid onset of action, brief half-life, predictable, effective analgesic properties without side effects and they are quickly reversible. These drugs do not exist and every sedation procedure has a risk of hypoxemia. With the human and equipment's investment an emergency department should be able to ensure that procedures are performed in children under sedation with a standard of safety that is similar to general anaesthesia. The main drawback in a well-organised system should be a significant children's rate for which general anaesthesia is preferred.

[Effect of racecadotril in the management of acute diarrhea in infants and children]. / Effet du racécadotril sur le recours aux soins dans le traitement des diarrhées aiguës du nourrisson et de l'enfant.

BACKGROUND: The treatment of diarrhoea relies on the maintenance or restoration of hydration with maintenance of an adequate nutritional intake. Racecadotril has been shown to reduce the stools output during acute diarrhoea. The present work was aimed at measuring the number of emergency department visits for acute diarrhoea either the children received racecadotril or not. METHOD: Racecadotril and rehydration were compared with rehydration alone in children aged three months to three years who had acute diarrhoea and were evaluated in the emergency department (ED). The primary end point was the number of medical exams during the week after starting treatment. Secondary end points were the number of stools during the first 48 hours, the duration of the diarrhoea and the weight on day 7. RESULTS: One hundred and sixty-six children were alternatively randomized to the treated and the control groups. There was no difference for age, degree of dehydration and length of illness before the first visit between the groups. Whatever type of rehydration (oral or i.v.), the treated group had a significant lower number of stools (p < 0.001) and a faster recovery (p < 10(-9)). The children receiving racecadotril needed less additional ED visits for the same episode (p < 0.05). There was no difference for the weight-gain on day 7. CONCLUSIONS: This study demonstrates the efficacy of racecadotril as adjuvant therapy to oral and i.v. rehydration in the treatment of acute diarrhoea and a fewer emergency department second visit before recovery.

[Benefits of ipratropium bromide in the management of asthmatic crises in the emergency department]. / Apport du bromure d'ipratropium dans la prise en charge des crises d'asthme aux urgences.

BACKGROUND: To determine if the addition of ipratropium bromide in the emergency department (ED) for the treatment of childhood asthma reduces rates of hospitalization and relapses for moderate and severe exacerbations. METHODS: Patients were given an oral corticosteroid treatment (2 mg/kg) and received every 20 minutes either three nebulizations with albuterol (0.15 mg/kg) and ipratropium bromide (250 micrograms) or six nebulizations with albuterol alone (control group). The primary end point was the need for hospitalization, additional nebulizations or a relapse during the following week. Secondary end point included the effect of age. RESULTS: One hundred and forty three children, two to 15 years old, were randomized to ipratropium or control groups and 121 were evaluated on day seven. As a whole, the control group was less often hospitalized or in relapse than those treated with three nebulizations of albuterol and ipratropium (17.5% vs 37.9%, p < 0.02). The ipratropium group reached the same result after three additional albuterol nebulizations. The benefit of anticholinergic therapy was observed for children less than six years of age who had a similar rate of success (73.5 vs 75.7%). CONCLUSION: The association of ipratropium bromide to the first three doses of the albuterol protocol for acute asthma did not act as well as six nebulizations of albuterol alone. The effect was age dependent and two to six years old children needed more attention. Nevertheless the hospitalization rate did not support the use of ipratropium compared with repeated albuterol nebulizations.

[Assessing the cost of the first episode of bronchiolitis]. / Evaluation du coût du premier épisode de bronchiolite.

BACKGROUND: Each year, a quarter of the children younger than 24 months has respiratory syncytial virus bronchiolitis. The morbidity among high-risk infants and the possible association with the development of asthma lead to propose preventive measures whose cost-effectiveness relationship is unknown. The present work was aimed at measuring costs of a first attack of bronchiolitis. METHOD: For children less than two years visited in the emergency department, direct and indirect costs were measured according to the 'Sécurité Sociale' prices. Associated morbidity, the management of care (inpatient versus outpatient), outpatients' outcome two weeks after the visit, socioeconomic data were recorded. RESULTS: One hundred eighty three children have been studied. The length of stay for 40 hospitalizations was 7.6 +/- 4.3 days. Direct costs were 37,200 +/- 22,000 FF for inpatients, and 1286 +/- 633 F for outpatients. For 113 outpatients' families, indirect costs were 49 working days lost. The way the child was looked after and the unemployment rate in the study were similar to data provided by the National Institute of Statistics and Economic Studies. CONCLUSION: Because of the variability of the hospitalization rate from one setting to another, overall costs of the epidemic cannot be evaluated. For the policymaker, the greatest costs come from the outpatient care. Others studies will be necessary to evaluate the price of future preventive measures.

[Drugs for pediatric emergencies]. / Médicaments de l'urgence en pédiatrie.

Drugs for pediatric emergencies are useful for respiratory (croup, asthma), cardiologic (hypertensive crisis, acute congestive heart failure, arrhythmias, hypoxic spells), neurologic (seizures), metabolic (dehydration, hypoglycaemia), infectious (meningococcemia) or allergic (anaphylaxis) distresses. Pain management is always important whether to relieve or to prevent the discomfort which would happen during diagnosis or therapeutic procedures.

WT1 splice-site mutations are rarely associated with primary steroid-resistant focal and segmental glomerulosclerosis.

BACKGROUND: Donor splice-site de novo heterozygous mutations in intron 9 of the Wilms' tumor gene (WT1) have been reported in Frasier syndrome, which is defined by the association of focal and segmental glomerulosclerosis (FSGS), male pseudohermaphroditism, and gonadoblastoma. These splice-site mutations alter the WT1 alternative splicing leading to two WT1 isoforms, with (+) or without (-) three amino acids, lysine-threonine-serine (KTS), between zinc fingers 3 and 4. The aim of this work was to investigate the possibility that some cases of primary steroid-resistant nephrotic syndrome associated with FSGS may be caused by WT1 splice-site mutations. METHODS: We analyzed WT1 exons 8 and 9 and the surrounding exon/intron boundary DNA sequences in 37 children with nonfamilial primary steroid-resistant nephrotic syndrome. Semiquantitative reverse transcription-polymerase chain reaction (RT-PCR) was used to determine the relative ratio of +KTS/-KTS transcripts from immortalized lymphocyte RNA. RESULTS: One boy with FSGS and associated pathologies (diaphragmatic hernia, proximal hypospadias, and unilateral testicular ectopia) was found to carry the heterozygous 1228 +4 C-->T splice-site mutation. RT-PCR quantitation of the +KTS/-KTS transcripts from immortalized lymphocyte RNA of this patient showed a diminution of the +KTS/-KTS isoform ratio (0.43), which is identical to that reported in patients with Frasier syndrome. Using the same approach, healthy control subjects have +KTS/-KTS ratios ranging from 1.50 to 2.00. CONCLUSIONS: This study expands the range of the phenotypic presentation of the intron 9 splice-site WT1 mutations and adds to the already reported heterogeneity of primary steroid-resistant nephrotic syndromes. We suggest that these mutations are not likely to be a common cause of isolated steroid-resistant nephrotic syndrome, and recommend a WT1 exon 9/intron 9 splice-site study in children with primary steroid-resistant nephrotic syndrome if genital or diaphragmatic anomalies are associated. The identification of such WT1 mutations has practical implications for the management of these patients.

Mother-to-child transmitted WT1 splice-site mutation is responsible for distinct glomerular diseases.

Mutations in the Wilms' tumor suppressor gene (WT1) are linked with Denys-Drash syndrome (DDS), a rare childhood disease characterized by diffuse mesangial sclerosis and renal failure of early onset, XY pseudohermaphroditism, and high risk of Wilms' tumor. KTS (lysine-threonine-serine) splice site mutations in WT1 intron 9 have been described in patients with Frasier syndrome, another rare syndrome defined by focal and segmental glomerulosclerosis (FSGS), XY pseudohermaphroditism, and frequent occurrence of gonadoblastoma. Cases of Frasier syndrome raise the question whether splice site mutations may also be found in XX females with isolated FSGS. A girl (index case) presented with the nephrotic syndrome at 9 mo of age. The diagnosis of DDS was based on the finding of diffuse mesangial sclerosis in the kidney biopsy and of a XY karyotype. The index case's mother had had proteinuria since she was 6 years of age. A renal biopsy was performed when she was 28 and disclosed FSGS. The same splice site mutation in intron 9 (WT1 1228+5 G-->A) involving one allele was found in the child and in her mother, but not in other members of the kindred (including the parents, the two brothers, and the two sisters of the index case's mother) who were free of renal symptoms. Quantification of WT1 +KTS/-KTS isoforms in the index case's father and one index case's maternal uncle showed a normal +KTS/-KTS ratio of 1.50. In contrast, the index case and her mother had a low ratio (0.40 and 0.34, respectively), within the range reported in Frasier syndrome. In conclusion, this study shows that the KTS splice site mutation is not specific for Frasier syndrome, but that it can also be found in DDS and in a normal female (XX) with FSGS, a woman who achieved normal pregnancy. It is suggested that WT1 splice site mutations should be sought in phenotypically normal females who present with FSGS or with related glomerulopathies of early onset.

[Munchausen syndrome by proxy]. / Le syndrome de Munchausen par procuration.

Munchausen syndrome by proxy is a form of child abuse presenting as a disease produced or simulated by a parent, the mother in most cases. Its diagnosis is uneasy because of its miscellaneous and unusual clinical presentation and of the misleading apparently normal attitude of the parents. Physicians may participate in the abuse by insistently looking for diagnostic and therapeutic measures, therefore contributing to the significant mortality of the syndrome. It is therefore important that physicians consider Munchausen syndrome in any ambiguous situation in order to protect the child by an early diagnosis.