Search

Purpose To elucidate the education needs of patients who have undergone lung transplant for cystic fibrosis while participating in the development of therapeutic education programs in French transplant centers. Methods From January 2009 to March 2012, in-depth educational diagnosis interviews were conducted with 42 adult transplant recipients with cystic fibrosis who were being followed up at 7 French transplant centers. Several areas were explored: health status, social and occupational outcomes, knowledge about the disease and treatments, and experience of the disease. The interviews combined open- and closed-ended questions for more systematic exploration of knowledge about the disease and treatments. Results After receiving the transplant, the patients' health had improved and their lives were returning to normal. They had acquired much usable knowledge about managing their disease. Educational needs regarding transplant-related complications and management of immunosuppressive drugs were evident. The analysis also demonstrated the need to better inform patients about pregnancy and new social rights and to offer them psychological support in adjusting to their new health status. Conclusion Therapeutic education of patients should continue well after transplant to maintain safety knowledge and meet patients' new needs. A comprehensive therapeutic education program for transplant candidates that includes the preparatory, immediate posttransplant, and late posttransplant phases is needed.

Introduction: Life expectancy in patients suffering from cystic fibrosis has very much increased. It can now be compared to diabetes, requiring a lot of time spent with treatments and special skills that need to be acquired by patients and families. Objectives: To promote structured therapeutic education in cystic fibrosis. Methods: Our Cystic Fibrosis (CF) center has proposed education since 2006, alternating individual and group sessions, organized from the initial diagnosis to transition from paediatric to adult care. During the group sessions, hygiene precautions are strictly enforced to avoid cross infection. Since 2006, in our cohort of 92 CF children, 24 parents of children under 6 and 37 children accompanied by one or two of their parents, attended group sessions. Results: Families appreciate this approach. Skills on self-management and anticipation are therefore increased. It has produced a good response from children and meets parents’ and caregivers’ expectations. Discussion: Health care givers have to develop structured therapeutic education to improve quality of life for patients and families. Conclusion: Our center organizes the training the other 48 centers for cystic fibrosis in France to help them implement individual or collectives educative sessions.

In France patients with cystic fibrosis benefit from a multidisciplinary follow-up in Cystic Fibrosis Centres. In this follow-up, despite the numerous therapeutic benefits of exercise in this disease, little emphasis is placed on the promotion of physical activity. The aim of this article is to improve this aspect of management, giving advice from a working group of experts, based on the medical literature and clinical experience. These proposals include quantification of physical activity, evaluation of exercise, training and rehabilitation programs and finally, modification of behaviour to include physical activity in the overall cystic fibrosis treatment strategy. It is intended to set up multicentre studies to evaluate the impact of these proposals.

Introduction La prescription de traitements par voie respiratoire est particulierement seduisante dans la mucoviscidose, malgre un depot pulmonaire tres heterogene et variable. Etat des connaissances Les traitements proposes sont essentiellement ceux ayant pour but d’ameliorer la qualite du mucus (serum sale hypertonique ou RhDNase en nebulisation, bientot mannitol en poudre seche) ou de traiter la colonisation chronique et la primo-infection a Pseudomonas aeruginosa (tobramycine et colistine nebulisees, bientot lysate d’aztreonam, ciprofloxacine, amikacine liposomale). Les bronchodilatateurs meritent d’etre testes. Les corticoides n‘ont pas d’utilite demontree. D’autres molecules (therapie genique, L-arginine, antiproteases, etc.) sont actuellement en developpement ou a l’essai. En pratique, les melanges medicamenteux sont a eviter et un certain ordre chronologique d’administration merite d’etre respecte. Perspectives Les progres pharmaceutiques recents et technologiques laissent entrevoir de nouvelles perspectives therapeutiques dans la mucoviscidose. Conclusion La mucoviscidose est une maladie pour laquelle de plus en plus de traitements, efficaces mais dispendieux, se developpent pour une administration par voie inhalee. L’education a la technique d’inhalation et a l’hygiene des dispositifs d’inhalation est donc un preambule imperatif a toute prescription.

Resume Introduction En France, depuis 2002, deux changements majeurs sont intervenus dans la prise en charge des patients atteints de Mucoviscidose : le depistage neonatal systematique de la mucoviscidose et la creation des Centre de Ressources et de Competences de la Mucoviscidose (CRCM). Une des missions des CRCM est de developper l’education therapeutique du patient et de sa famille. Methodes La demarche educative proposee comprend 4 etapes : Le diagnostic d’education qui permet de faire connaissance avec son patient. Le contrat d’education propose au patient a partir des competences et objectifs clarifies auparavant en equipe. La mise en œuvre de l’education qui organise les apprentissages du patient et enfin l’evaluation. Dans le but de faciliter la mise en place de l’education therapeutique dans les CRCM, nous presentons, a partir des competences que le patient doit acquerir au cours de l’education therapeutique, les objectifs d’apprentissage des patients atteints de mucoviscidose. Conclusion En prenant appui sur les competences et objectifs d’apprentissage une reflexion s’est engagee sur l’elaboration de conducteurs, veritables instruments d’aide pour l’educateur/soignant. Ils presentent le deroulement des seances, les principes pedagogiques a mettre en œuvre et le role respectif du patient et du soignant.

Resume Objectif. – Determiner le nombre d’admissions dues directement aux infections a rotavirus dans un service de pediatrie generale par une surveillance exhaustive de quatre ans pour definir la place du rotavirus dans les gastroenterites aigues communautaires severes necessitant une hospitalisation. Resultats. – De janvier 1997 a decembre 2000, 725 patients ont ete admis pour gastroenterite aigue communautaire (infections nosocomiales et diarrhees associees a une autre maladie exclues) et 706 (97,5 %) ont eu une recherche etiologique. Un rotavirus etait a l’origine de la diarrhee dans 359 cas (50,89 %) et une salmonelle dans 61 cas (8,6 %). Les enfants hospitalises pour rotavirus etaient plus jeunes (26 % avaient trois mois ou moins et 50,3 % six mois ou moins) que dans les autres series europeennes. Conclusion. – Cette etude est la premiere rapportant une surveillance systematique en France. Les rotavirus ont ete a l’origine de plus de la moitie des gastroenterites aigues communautaires hospitalisees. Le jeune âge des patients invite a prolonger ces enquetes dans d’autres regions pour determiner l’âge des enfants hospitalises et a rechercher les souches de rotavirus en cause avant la generalisation d’un vaccin.

Resume Les epidemies virales hivernales a virus respiratoire syncytial et a rotavirus sont a l'origine de nombreuses hospitalisations, mais la date des pics epidemiques peut varier selon les regions. Patients et methodes Une etude retrospective a ete menee pour rechercher la periodicite des epidemies. De 1993 a 1998, 4 738 enfants de moins de 5 ans ont eu un examen des secretions pharyngees : dans 26,3 % des cas, le resultat etait positif pour le viras respiratoire syncytial. Un rotavirus a ete retrouve dans les selles de 23,7 % des 8 537 enfants de moins de 5 ans testes. Resultats Le pic epidemique de virus respiratoire syncytial est observe tous les ans en decembre en region parisienne, et celui de rotavirus est decale de 2 a 3 semaines avec une constance remarquable dans le temps pendant les 5 ans observes. Soixantedix-sept a 92 % des infections a viras respiratoire syncytial et 50 a 61 % des infections a rotavirus sont observees en novembre, decembre et janvier. Cette coincidence des pics epidemiques provoque un afflux brutal de malades posant de graves problemes d'organisation hospitaliere et de prevention des infections nosocomiales. Conclusion Cette simultaneite des pics epidemiques semble n'etre observee qu'en region parisienne, le decalage etant plus net dans d'autres zones europeennes et plus important aux Etats-Unis. Des etudes de ce type sont necessaires dans les differentes regions francaises et europeennes pour preciser les modalites epidemiques avant l'introduction de vaccins.

Mangiarotti P, Moulin F, Palmer P, Ravilly S, Raymond J, Gendrel D. Interferon-alpha in viral and bacterial gastroenteritis: a comparison with C-reactive protein and interleukin-6. Acta Paediatr 1999; 88: 592-4. Stockholm. ISSN 0803-5253 The aim of the study was to identify serum markers able to differentiate bacterial and viral origin in acute diarrhoea. Interferon-alpha (INF-α), C-reactive protein (CRP) and interleukin-6 were determined on admission in the sera of 119 children aged between 1 mo and 14 y who were hospitalized for rotavirus (n = 60) or bacterial diarrhoea (Salmonella spp. 39 cases, Shigella spp. 15 cases, Campylobacter jejuni 5 cases). CRP concentration was >10mg/l in 48.3% of children with viral gastroenteritis and 86.4% of children with bacterial gastroenteritis. IL6 concentration was >100pg/ml in 11.7% and 26.3% of cases, respectively. INF-α was detected in 79.1% of children with rotavirus (sens 79%) and in 3.5% (spec 93%) with bacterial gastroenteritis. However the INF-α assay takes 48 h and pathogens are often identified from stools before interferon results are available. We found that serum markers are not discriminating enough to differentiate between viral and bacterial gastroenteritis in emergency cases. □C-reactive protein, interferon-alpha, interleukin-6, viral gastroenteritis, bacterial gastroenteritis

The aims of this study are to describe qualitatively the perceptions of three groups involved in the cystic fibrosis transplant decision, looking for similarities and differences between groups, and to identify those that act as motivations or barriers to transplantation.Thirty patients, 26 patient relatives and 27 physicians were interviewed, and concept maps were constructed from those interviews. Their degree of transplant acceptance at the time of the interview was measured.There were motivations and barriers in the pre-, peri- and post-transplant period. Analysis revealed similar perceptions regarding the risks and benefits of transplantation, but also different perceptions in the specific concerns of each group. Patients and patient relatives expressed many questions and fears in their concept maps, and physicians expressed difficulties.This study highlights the value of better understanding the perceptions of patients, relatives and physicians, in order to remove some of the barriers to transplantation. It also demonstrates the benefits of education and support activities for patients and patient relatives prior to transplantation, and continuing education and supervision for physicians.

In France patients with cystic fibrosis benefit from a multidisciplinary follow-up in Cystic Fibrosis Centres. In this follow-up, despite the numerous therapeutic benefits of exercise in this disease, little emphasis is placed on the promotion of physical activity. The aim of this article is to improve this aspect of management, giving advice from a working group of experts, based on the medical literature and clinical experience. These proposals include quantification of physical activity, evaluation of exercise, training and rehabilitation programs and finally, modification of behaviour to include physical activity in the overall cystic fibrosis treatment strategy. It is intended to set up multicentre studies to evaluate the impact of these proposals.

Delivery of drugs by the inhaled route is particularly attractive in cystic fibrosis despite heterogeneous and variable deposition in the lungs.Proposed therapies include drugs aimed at improving the mucus quality (nebulised hypertonic saline or RhDNase and, in the future, mannitol as a dry powder) or treating chronic colonization and early infection with Pseudomonas aeruginosa (nebulised tobramycine and colistine and, soon, aztreonam lysate, ciprofloxacin and liposomal amikacine). Bronchodilators need to be tested. Corticosteroids have no proven benefit. Other drugs (gene therapy, L-arginine, anti-proteases, etc...) are under development or on trial. In practice, mixtures of drugs should be avoided and the chronological order between drugs observed to achieve correct delivery.Developments in pharmaceutics and device technology allow us to consider new therapeutic approaches in cystic fibrosis.More and more efficacious but expensive inhaled treatments are now, and will in the next future, be available for cystic fibrosis patients. Teaching of good inhalational technique and proper hygiene concerning the devices is an absolute necessity before any inhaled treatment is prescribed.

In France since 2002 two major changes have occurred in the management of patients with cystic fibrosis: systematic neonatal screening and the establishment of specialised CF centres. One of the roles of the CF centres is therapeutic education of the patients and their families.The proposed educational approach consists of 4 steps: an educational diagnosis to assess the patient; an educational contract based on the capabilities and objectives previously identified by the team; implementation of the education programme which organises the teaching of the patient and, finally, evaluation.With the goal of facilitating the establishment of patient education in the CF centres we present the learning objectives for patients suffering from cystic fibrosis, based on the capabilities that the patients need to acquire in the course of the education programme.Based on the capabilities and learning objectives of the patients the working party are developing valid tools for the instructor/carer. They present the outcomes of the meetings, the educational principles of implementation and the respective roles of the patient and the carer.

Children with enteric fever or severe salmonella infections are usually treated with beta-lactam antibiotics, particularly ceftriaxone. Due to their poor penetration into cells, beta-lactam antibiotics, even if active in vitro, are sometimes clinically ineffective because they cannot reach the intracellular sites of Salmonella multiplication.To evaluate in a retrospective study usefulness, efficacy and safety of oral ciprofloxacin in patients with severe salmonellosis and clinical failure of ceftriaxone or beta-lactam antibiotics.From July 1, 1995 to 2000, the bacteriology laboratory of a French pediatric hospital had identified 215 patients aged between 1 month and 15 years with positive blood or stools for Salmonella sp, 113 of them requiring hospitalization due to their clinical symptoms. Three were excluded for sickle-cell disease or poor nutritional status. None of the 110 strains (including 4 S. typhi, 51 S. typhimurium, 25 S. enteritidis, 6 S. hadar and 5 S. heidelberg) isolated was resistant to ceftriaxone or ciprofloxacin. Forty-one of the 110 strains (37.3%) produced a beta-lactamase. Twelve patients had a rapid recovery without antibiotic treatment, and 98 (mean age 3.9 years) were given antibiotics (ceftriaxone in 91 and amoxicillin in 7) for dysentery (43%), shock (15%) or persistent high fever and severe diarrhea (42%).In 72 children (mean age = 3.6 years) ceftriaxone treatment (amoxicillin in 5) for 5 or 7 days was rapidly effective: apyrexia was obtained in 1.5 day after the start of treatment and the number of stools per day was 4 or less in 2.2 days. Two to 3 weeks after clinical recovery, asymptomatic carriage was present in 22/38 patients. In the 26 other patients ceftriaxone (amoxicillin in 2) treatment was clinically ineffective, despite good in vitro activity, and was switch for oral ciprofloxacin (20 mg kg(-1) d(-1), 5 days) after 2 to 7 days of lasted fever and/or severe diarrhea. Clinical improvement with ciprofloxacin was obtained in less than 48 h. The strains involved in these 26 patients included the 4 S. typhi and 15 S. typhimurium (P0.05), 13/15 (P0.01) producing beta-lactamase. Asymptomatic carriage was found in 5/22 patients (P0.05) after recovery. None of the patient treated with ciprofloxacin had side effect.In severe salmonellosis, the clinical failure of treatment with ceftriaxone is not rare, particularly in S. typhimurium producing beta-lactamase infection and short treatment with oral ciprofloxacin is safe and allows to obtain a rapid recovery.

To evaluate the number of hospitalizations due to community-acquired rotavirus acute gastroenteritis in a general pediatric unit during a four-year survey.From January 1997 to December 2000, 725 patients were admitted for acute gastro-enteritis to the general paediatric unit of a Parisian children hospital (nosocomial diarrhoea excluded) and 706 (97.5%) of these patients had had a stool microbiologic examination. Diarrhoea was caused by rotavirus in 359 patients (50.89%) and Salmonella sp in 61 (8.6%). Children and infants hospitalized for rotavirus acute gastroenteritis were younger (26% had three months or less, and 50.03% had six months or less) than in other European studies.This study is the first in France reporting a systematic survey of hospitalized gastroenteritis during four years. More than half of hospitalized community-acquired gastroenteritis were due to rotavirus in this Parisian area. The young age of patients should be investigated in other French areas, searching for risk factors and rotavirus strains.

Procalcitonin concentration increases in bacterial infections but remains low in viral infections and inflammatory diseases. The change is rapid and the molecule is stable making it a potentially useful marker for distinguishing between bacterial and viral infections.Procalcitonin (PCT) was determined with an immunoluminometric assay on plasma collected at admission in 436 infants and children hospitalized for bacterial or viral infection. It was compared with C reactive protein, interleukin-6 and interferon-alpha measured on the same sample.PCT was 41.3 +/- 77.4 micrograms/l in children with septicemia or bacterial meningitis (n = 53), 0.39 +/- 0.57 microgram/l in children with viral infection (n = 274) and 3.9 +/- 5.9 micrograms/l in children with a localized bacterial infection who had a negative blood culture (n = 109). PCT was1 microgram/l in 126 children with a localized or systemic bacterial infection (sensitivity 78%). PCT was1 microgram/l in 258 children with a viral infection (specificity 94%). For differenciation between viral and bacterial infections, CRP valueor = 20 mg/l, IL-6100 pg/ml and interferon-alpha0 Ul/ml have 85, 48 and 76% sensitivity and 73, 85 and 92% specificity respectively.In this study, a PCT value of 1 microgram/l or greater had better specificity, sensitivity and predictive value than CRP, IL-6 and interferon-alpha in children for distinguishing between viral and bacterial infections. PCT may be useful in pediatric emergency room for making decision about antibiotic treatments.

Thoracoabdominal asynchrony (TAA) and the ratio of time to peak tidal expiratory flow over total expiratory time (TME/TE) have been used to assess airway obstruction in infants and adults. We obtained these measurements using calibrated respiratory inductance plethysmography (RIP) on 15 adolescents and young adults with cystic fibrosis (CF) and varying disease severity. The measurements were then compared to 15 normal age-matched controls. TAA was expressed as a phase angle (phi) calculated from the abdominal (AB) and ribcage (RC) signals acquired from scalar strip chart recordings. Using CODAS (DATAQ Instruments, Akron, OH) software, the analog signals were digitized, and the differentiated sum (AB + RC) signal was used to calculate TME/TE. Forced vital capacity (FVC) and forced expiratory volume in 1 sec (FEV1) were obtained using RIP in all subjects. Subjects with CF had a significantly higher mean phi than the control subjects (15 degrees vs. 8 degrees, respectively, P = 0.01). In the CF patients the specificity of a high phi as an indicator of abnormality was 80%, while the sensitivity was 65%. There was no correlation in the magnitude of phi and disease severity as assessed by FVC or FEV1. There was no significant difference in TME/TE between the groups. We conclude that RIP-acquired phi, but not TME/TE, is a simple and useful method to detect the presence of airway obstructive disease. We speculate that the sensitivity of this method will increase in younger patients with more compliant chest walls and less air trapping. Longitudinal studies of phi in infants and young children with lung disease could help in assessing disease severity and progression in this population, in whom repeated measures are few and complex.

In a pediatric hospital of Paris, from 1993 to 1998, respiratory secretions were positive for respiratory syncytial virus (RSV) in 26.3% of 4,738 children (0-5 years) examined or hospitalized for lower respiratory tract infections. Rotavirus detection was positive in stools of 23.7% of the 8,537 children of the same age with acute diarrhea.The RSV epidemic peak occurred annually in Paris in December and the rotavirus outbreak peaks were observed in December/January. The winter seasonal peaks remained constant for both pathogens and the temporal appearance of these peaks was constant from 1993 to 1998. Fifty to sixty-one percent of rotavirus and 77 to 92% of RSV infections were observed in November, December or January. These simultaneous outbreaks provoked important problems in hospital organization and prevention of nosocomial infections.The coincidence of RSV and rotavirus peaks is not found in all countries. The epidemic patterns have to be checked in other parts of France and Europe because this could be important when active immunization programs will be available for these two pathogens.

In young children with meningitis, blood or cerebrospinal fluid (CSF) analysis cannot differentiate all cases of viral meningitis (VM) from bacterial meningitis (BM). Empirical antibiotic therapy is often given. As new markers are needed, we compared serum proCalcitonin (PCT) with CSF analysis for C-reactive protein (CRP) and interleukin-6 (IL6).PCT was measured with a chemoluminescent assay in the sera of 23 children (aged 3 months to 14 years) hospitalized for BM and in 51 patients with VM.Initial CRP (mean 143.3 mg/l, range 28-351 and mean 13.9, range 1-48), CSF proteins (mean 2.2, range 0.4-4.74 and mean 0.57, range 0.12-2.72) and white blood cell count in CSF (range 240-17500 and 20-3200) in BM and VM respectively, were not sufficiently discriminative to distinguish between BM and VM. Twenty-four of the 51 patients with VM were given antibiotics. IL6 values at admission showed an overlap zone (100 pg/ml in 7/19 patients with VM and100 pg/ml in 1/8 patients with BM. PCT was discriminative in all cases: mean PCT in BM was 61 micrograms/l (range 4.8-335) and 0.33 in VM (range 0-1.7; p0.001). No production of PCT was detected in CSF. After antibiotic therapy, PCT decreased and reached undetectable levels after recovery.PCT is a sensitive and specific marker for early diagnosis of viral meningitis versus bacterial meningitis in children.

Define a therapeutic management schema adapted to children with community-acquired pneumonia.A prospective survey was conducted in 104 children over 18 months of age with community-acquired pneumonia. The pathogen was isolated in 85% of the cases.Viral infection alone was proven in 30 children (respiratory syncytial virus in 10). Pneumococci pneumonia was found in 12 patients; the isolated strains were sensitive to penicillin. Apyrexia was obtained in 11/12 cases with amoxicillin. Mycoplasma infections occurred in 42% of the cases (41 alone and in association with pneumococci in 2 cases). Pneumococci and mycoplasma infections could not be differentiated with standard radiography and laboratory tests. Initial treatment with beta lactamines was always unsuccessful in children with mycoplasma infections. Apyrexia was achieved when antibiotics were changed to macrolides.Since lower respiratory tract infections due to pneumococci are much more severe than those due to mycoplasma, beta lactamines should be given as first intention treatment for children over 18 months with pneumonia. Macrolides should be given in case of failure because mycoplasma would then be the most probable infectious agent.

Serum and/or cerebrospinal fluid (CSF) alpha interferon (aIFN) levels were determined in 31 neonates hospitalized for suspected sepsis. Final diagnosis was bacterial sepsis in 15, viral infection in 13 and no infection in 3. Among the 13 neonates with viral infection, enterovirus was isolated 5 times and coxsackievirus 4 times. No aIFN was found in cerebro-spinal fluid and/or serum among the neonates with bacterial sepsis or without infection. By contrast, in neonates with viral infection, CSF and/or serum aIFN levels were always elevated except in one case in which serum aIFN determination was not available. We conclude that in neonates, elevated levels of CSF and serum aIFN appear to be specific of viral infection, and that this might be helpful in the differential diagnosis of suspected sepsis.

RésuméLe groupe de travail sur l’aérosolthérapie (GAT) de la Société de pneumologie de langue française (SPLF) s’est réuni pour la troisième fois en 2012 sous la forme d’un « Aérosolstorming ». En l’espace d’une journée, différents aspects des thérapeutiques inhalées ont été abordés et seront traités de manière distincte dans deux articles, celui-ci étant le premier. Les produits inhalés représentent un large volume de prescription, en ville ou à l’hôpital, et dans divers spécialités notamment en ORL et en pneumologie. Les aspects techniques du développement des ces produits, les modalités d’administration, le respect des indications sont des éléments clés pour l’utilisation de cette ressource thérapeutique. Nous verrons dans cette première partie les difficultés posées par les génériques inhalés, l’existence d’antidote par voie inhalée, les nouveaux anti-infectieux et les indications de la pentamidine en aérosols.SummaryThe working group on aerosol therapy (GAT) of the Société de pneumologie de langue française (SPLF) organized its third “Aerosolstorming” in 2012. During the course of one day, different aspects of inhaled therapy were discussed, and these will be treated separately in two articles, this one being the first. Inhaled products represent a large volume of prescriptions both in the community and in hospital settings and they involve various specialties particularly ENT and respiratory care. Technical aspects of the development of these products, their mode of administration and compliance with their indications are key elements for the effective therapeutic use of inhaled treatments. In this first article, we will review issues concerning generic inhaled products, the existence of inhaled antidotes, new anti-infective agents and indications for inhaled pentamidine.