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1.
Rev Med Interne ; 44(9): 479-486, 2023 Sep.
Artigo em Francês | MEDLINE | ID: mdl-36841717

RESUMO

Admission to hospital is a critical transition point for the continuity of care in medication management. Medication reconciliation can identify and resolve errors due to inaccurate medication histories. The practice of medication reconciliation is securing for the patient because of the medication errors detected with significant clinical impact. Its implementation must comply with the recommendations of the French National Authority for Health (HAS) and its deployment is now integrated into the contract for improving the quality and efficiency of care (CAQES). However, although it allows to intercept medication errors, its impact on the length of hospitalization, the rate of readmission and/or death following discharge seems limited. Given the limited human resources to carry out this time-consuming activity, patient prioritization should be considered. Studies on the fate of patients and on the medico-economic issues are also necessary in order to make this activity sustainable.


Assuntos
Reconciliação de Medicamentos , Farmacêuticos , Humanos , Erros de Medicação/prevenção & controle , Hospitalização , Alta do Paciente , Admissão do Paciente
2.
Arthritis Res Ther ; 19(1): 109, 2017 05 25.
Artigo em Inglês | MEDLINE | ID: mdl-28545499

RESUMO

BACKGROUND: In the current context of personalized medicine, one of the major challenges in the management of rheumatoid arthritis (RA) is to identify biomarkers that predict drug responsiveness. From the European APPRAISE trial, our main objective was to identify a gene expression profile associated with responsiveness to abatacept (ABA) + methotrexate (MTX) and to understand the involvement of this signature in the pathophysiology of RA. METHODS: Whole human genome microarrays (4 × 44 K) were performed from a first subset of 36 patients with RA. Data validation by quantitative reverse-transcription (qRT)-PCR was performed from a second independent subset of 32 patients with RA. Gene Ontology and WikiPathways database allowed us to highlight the specific biological mechanisms involved in predicting response to ABA/MTX. RESULTS: From the first subset of 36 patients with RA, a combination including 87 transcripts allowed almost perfect separation between responders and non-responders to ABA/MTX. Next, the second subset of patients 32 with RA allowed validation by qRT-PCR of a minimal signature with only four genes. This latter signature categorized 81% of patients with RA with 75% sensitivity, 85% specificity and 85% negative predictive value. This combination showed a significant enrichment of genes involved in electron transport chain (ETC) pathways. Seven transcripts from ETC pathways (NDUFA6, NDUFA4, UQCRQ, ATP5J, COX7A2, COX7B, COX6A1) were significantly downregulated in responders versus non-responders to ABA/MTX. Moreover, dysregulation of these genes was independent of inflammation and was specific to ABA response. CONCLUSION: Pre-silencing of ETC genes is associated with future response to ABA/MTX and might be a crucial key to susceptibility to ABA response.


Assuntos
Abatacepte/uso terapêutico , Antirreumáticos/uso terapêutico , Artrite Reumatoide/genética , Resistência a Medicamentos/genética , Complexo de Proteínas da Cadeia de Transporte de Elétrons/genética , Transcriptoma , Adulto , Idoso , Artrite Reumatoide/tratamento farmacológico , Biomarcadores/análise , Feminino , Perfilação da Expressão Gênica , Humanos , Masculino , Metotrexato/uso terapêutico , Pessoa de Meia-Idade
3.
Arch Pediatr ; 23(6): 561-9, 2016 Jun.
Artigo em Francês | MEDLINE | ID: mdl-27117994

RESUMO

INTRODUCTION: As part of our antimicrobial stewardship program, we were interested in the use of anti-infectious drugs and the prevalence of medication errors associated with the use of these drugs. METHODS: The retrospective and descriptive study was conducted over a 1-year-period between 1 April 2012 and 31 March 2013 in a teaching mother-child hospital. The aim of the study was to determine the number and the type of medication errors related to anti-infectious drugs. We evaluated the importance, the significance, and the severity of medication errors and at the same time we evaluated antimicrobial consumption. Therefore, we determined the ratio of medication errors per 10,000 defined daily doses (DDD) or 10,000 days of therapy (DOT). The secondary objective of the study was to compare the medication errors related to anti-infectious drugs with the data published in the Quebec Registry of Incidents and Accidents. RESULTS: We found a total of 2164 medication errors including 301 (14%) medication errors related to anti-infectious drugs during the 2012-2013 fiscal year. The majority (95%) of the medication errors related to anti-infectious drugs was not harmful, with no consequences for the patient. Seventy-four percent of medication errors related to anti-infectious drugs were part of the C class on the severity scale. Omission (26%) and wrong dose administration were the two most frequently reported events. Eighty percent (n=242/301) of medication errors occurred during the administration step while only 8% (n=24/301) occurred during the prescribing step. Three anti-infectious drugs had the highest ratio of medication errors per 10,000 DDD or DOT: linezolid (376 medication errors per 10,000 DDD), doxycycline (357 medication errors per 10,000 DDD), and acyclovir (202 medication errors per 10,000 DDD). The nonparametric tests showed no significant differences between frequently used and infrequently used anti-infectious drugs for the ratio of medication errors per 10,000 DOT. This pilot study showed a ratio of 65.4 medication errors/10,000 DDD of anti-infectious drugs and a ratio of 41.9 medication errors/10,000 DOT of anti-infectious drugs. CONCLUSION: The use of these ratios could contribute to focusing on monitoring anti-infectious drugs and improving the risk management system associated with this class of drugs. It also shows that less frequently used anti-infectious drugs should be managed with greater caution. Anti-infectious stewardship programs should also consider ratios of medication errors for anti-infectious drugs used in order to improve patient safety and optimize use of drugs.


Assuntos
Anti-Infecciosos/administração & dosagem , Erros de Medicação/estatística & dados numéricos , Hospitais de Ensino , Humanos , Segurança do Paciente , Projetos Piloto , Quebeque , Estudos Retrospectivos
4.
Ann Pharm Fr ; 74(2): 165-72, 2016 Mar.
Artigo em Francês | MEDLINE | ID: mdl-26365553

RESUMO

OBJECTIVES: As part of our antimicrobials stewardship program, we were interested in the use of antimicrobials and prevalence of adverse drug reactions associated with the use of these drugs. METHODS: The retrospective and descriptive study was conducted over a one year-period between April 1st 2012 and March 31st 2013 in a mother-child Hospital. We determined the ratio: number of adverse drug reactions over 10,000 defined daily dose or 10,000days of therapy. We identified the ratios higher than average for which the confidence interval did not cross the calculated average. The severity of the adverse drug reactions was codified using the Common Terminology Criteria for Adverse Events. RESULTS: We found 570 adverse drug reactions including 100 (17.5%) adverse drug reactions related to antimicrobials during the financial year 2012-2013. It represented 96 patients. Thus, five antimicrobials, for which the confidence interval does not cross the calculated average value, may be targeted in risk management because they have a higher ratio than average: piperacillin (290 [113-722]), valganciclovir (244 [43-1260]), ceftriaxone (114 [56-234]), acyclovir (76 [26-220]) and liposomal amphotericin B (72 [20-258]). CONCLUSION: In a mother-child university hospital, we calculated a ratios of 19 [15-23] and 13 [10-15], it allows us targeting some antimicrobials in our approach to prevention and management of adverse drug reactions.


Assuntos
Anti-Infecciosos/efeitos adversos , Adolescente , Sistemas de Notificação de Reações Adversas a Medicamentos , Criança , Pré-Escolar , Efeitos Colaterais e Reações Adversas Relacionados a Medicamentos/epidemiologia , Feminino , França/epidemiologia , Humanos , Masculino , Projetos Piloto , Prevalência , Estudos Retrospectivos , Gestão de Riscos
5.
Ann Phys Rehabil Med ; 57(9-10): 587-99, 2014 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-25311851

RESUMO

OBJECTIVE: To study the applicability and responsiveness of the motor function measure (total score and sub-scores D1, D2 and D3) in patients with Charcot-Marie-Tooth disease. PATIENTS AND METHODS: Two hundred and thirty-three patients aged 4-86 years were included in the descriptive study. Scores and sub-scores were analyzed by age and by disease subtypes. Sensitivity to change (responsiveness) was estimated in patients having had at least two evaluations with at least six months between the first and the second. RESULTS: Motor function measure scores decrease with age, especially sub-scores D1 and D3. There were no significant differences between the scores according to type of Charcot-Marie-Tooth disease. The scores were significantly higher for ambulatory than for non-ambulatory patients. Significant responsiveness was demonstrated only in type 2 Charcot-Marie-Tooth disease. DISCUSSION/CONCLUSIONS: Our results suggest that, especially for D1 and D3 sub-scores, the motor function measure is a reliable and valid outcome measure that can be usefully applied in longitudinal follow-up. Studies of longer duration could demonstrate its responsiveness in other Charcot-Marie-Tooth disease subtypes.


Assuntos
Doença de Charcot-Marie-Tooth/fisiopatologia , Atividade Motora/fisiologia , Análise e Desempenho de Tarefas , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Exame Neurológico , Adulto Jovem
6.
Child Care Health Dev ; 40(4): 525-32, 2014 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-23905548

RESUMO

BACKGROUND: To estimate the prevalence of undernutrition among children with profound intellectual and multiple disabilities (PIMD) and to explore its influence on quality of life. METHODS: Seventy-two children with PIMD (47 male; 25 female; age range 2 to 15 years 4 months; mean age 8.6, SD 3.6) underwent an anthropometric assessment, including body weight, triceps skinfold thickness, segmental measures and recumbent length. Undernutrition was determined using tricipital skinfold percentile and z-scores of weight-for-height and height-for-age. The quality of life of each child was evaluated using the QUALIN questionnaire adapted for profoundly disabled children. RESULTS: Twenty-five children (34.7%) were undernourished and seven (9.7%) were obese. Among undernourished children only eight (32 %) were receiving food supplements and two (8%) had a gastrostomy, of which one was still on a refeeding programme. On multivariate analysis, undernutrition was one of the independent predictors of lower quality of life. CONCLUSION: Undernutrition remains a matter of concern in children with PIMD. There is a need to better train professionals in systematically assessing the nutritional status of profoundly disabled children in order to start nutritional management when necessary.


Assuntos
Crianças com Deficiência/estatística & dados numéricos , Deficiência Intelectual/epidemiologia , Desnutrição/epidemiologia , Transtornos das Habilidades Motoras/epidemiologia , Obesidade/epidemiologia , Adolescente , Criança , Fenômenos Fisiológicos da Nutrição Infantil , Pré-Escolar , Estudos Transversais , Crianças com Deficiência/psicologia , Feminino , França/epidemiologia , Humanos , Deficiência Intelectual/psicologia , Masculino , Desnutrição/psicologia , Transtornos das Habilidades Motoras/psicologia , Obesidade/psicologia , Prevalência , Qualidade de Vida/psicologia , Índice de Gravidade de Doença , Inquéritos e Questionários , Suíça/epidemiologia
7.
Ann Phys Rehabil Med ; 56(9-10): 673-86, 2013 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-24231198

RESUMO

OBJECTIVE: To develop a classification for neuromuscular disease patients in each of the three motor function domains (D1: standing and transfers; D2: axial and proximal function; D3: distal function). MATERIALS AND METHODS: A draft classification was developed by a study group and then improved by qualitative validation studies (according to the Delphi method) and quantitative validation studies (content validity, criterion validity and inter-rater reliability). A total of 448 patients with genetic neuromuscular diseases participated in the studies. RESULTS: On average, it took 6.3minutes to rate a patient. The inter-rater agreement was good when the classification was based on patient observation or an interview with the patient (Cohen's kappa=0.770, 0.690 and 0.642 for NM-Score D1, D2 and D3 domains, respectively). Stronger correlations (according to Spearman's coefficient) with the respective "gold standard" classifications were found for NM-Score D1 (0.86 vs. the Vignos Scale and -0.88 vs. the Motor Function Measure [MFM]-D1) and NM-Score D2 (-0.7 vs. the Brooke Scale and 0.64 vs. MFM D2) than for NM-Score D3 (0.49 vs. the Brooke scale and -0.49 vs. MFM D3). DISCUSSION/CONCLUSIONS: The NM-Score is a reliable, reproducible outcome measure with value in clinical practice and in clinical research for the description of patients and the constitution of uniform patient groups (in terms of motor function).


Assuntos
Atividades Cotidianas , Destreza Motora/classificação , Doenças Neuromusculares/fisiopatologia , Índice de Gravidade de Doença , Adolescente , Adulto , Criança , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Movimento , Variações Dependentes do Observador , Reprodutibilidade dos Testes , Fatores de Tempo , Adulto Jovem
8.
Rev Neurol (Paris) ; 169(8-9): 583-94, 2013.
Artigo em Francês | MEDLINE | ID: mdl-23954141

RESUMO

The objective of this work was to study the natural history of dystrophinopathies and the genotype-phenotype correlations made possible by the development of the clinical part of the French DMD database. The collection of 70,000 clinical data for 600 patients with an average longitudinal follow-up of 12years enabled clarification of the natural history of Duchenne and Becker muscular dystrophies and clinical presentations in symptomatic females. We were able to specify the phenotypic heterogeneity of motor, orthopedic and respiratory involvements (severe, standard and intermediary form), of the cardiac disorder (severe, standard or absent cardiomyopathy, absence of correlation between motor and cardiac involvements), and of brain function (mental deficiency in the patients with Becker muscular dystrophy, psychopathological disorders in dystrophinopathies). Phenotypic variability did not correlate with a specific mutational spectrum. We propose a model of phenotypic analysis based on the presence or not of muscular and cardiac involvements (described by age at onset and rate of progression) and brain involvement (described by the type and the severity of the cognitive impairment and of the psychological disorders). The methodology developed for the DMD gene can be generalized and used for other databases dedicated to genetic diseases. Application of this model of phenotypic analysis for each patient and further development of the database should contribute substantially to clinical research providing useful tools for future clinical trials.


Assuntos
Distrofina/genética , Estudos de Associação Genética , Heterogeneidade Genética , Distrofia Muscular de Duchenne/genética , Adolescente , Idade de Início , Criança , Pré-Escolar , Estudos de Coortes , Bases de Dados Factuais , Feminino , França/epidemiologia , Técnicas Genéticas , Humanos , Masculino , Atividade Motora , Distrofia Muscular de Duchenne/epidemiologia , Fenótipo
9.
Ann Phys Rehabil Med ; 54(6): 348-58, 2011 Sep.
Artigo em Inglês, Francês | MEDLINE | ID: mdl-21840280

RESUMO

UNLABELLED: Osteoporosis is a common complication in children with motor impairments. They have a higher risk of fractures (20% during their lifetime) mostly at femoral level. Furthermore, these children have pain yet no clear relation has been established between osteoporosis and pain. The efficacy of bisphosphonates has been validated in adults and for children with osteogenesis imperfecta (OI). However, its use in children with motor impairments has not yet been validated. PATIENTS AND METHODS: Retrospective study on the medical charts of children presenting neurological diseases and motor impairments associated to secondary symptomatic osteoporosis. These children underwent treatment with Pamidronate(®) intravenous infusions (I.V.) in Lyon and Valence between 2002 and 2008. Data were collected on pain control, incidence and frequency of fractures and bone mass density (BMD). Data on adverse events were also collected to evaluate treatment's tolerance. RESULTS: Twelve children's charts were studied for a total of 50 Pamidronate(®) I.V. infusions. Regarding treatment's efficacy, we observed a clear decrease and even total relief of the pain with improvement reported after 98% of perfusions. Regarding BMD, there was a real improvement after the treatment (e.g., lumbar BMD measures, -46.5% before treatment and -27% after treatment). The adverse events, flu-like syndrome, muscle pain and asymptomatic hypocalcemia, were minor and quickly reversible. CONCLUSION: It seems quite essential to screen for osteoporosis-related pain in these children and treat them quickly to avoid a negative impact on their quality of life. Treatment with I.V. bisphosphonates has shown its relevance, yet practical modalities still need to be defined. It would be interesting but quite difficult to implement, in light of the positive effect of this study, a prospective, randomized, controlled, double-blind vs. placebo study on a large enough sample of patients.


Assuntos
Conservadores da Densidade Óssea/uso terapêutico , Difosfonatos/uso terapêutico , Dor Musculoesquelética/prevenção & controle , Osteoporose/tratamento farmacológico , Adolescente , Analgésicos/uso terapêutico , Conservadores da Densidade Óssea/administração & dosagem , Conservadores da Densidade Óssea/efeitos adversos , Criança , Difosfonatos/administração & dosagem , Difosfonatos/efeitos adversos , Avaliação de Medicamentos , Feminino , Fraturas Espontâneas/etiologia , Fraturas Espontâneas/fisiopatologia , Fraturas Espontâneas/prevenção & controle , Humanos , Infusões Intravenosas , Masculino , Dor Musculoesquelética/induzido quimicamente , Dor Musculoesquelética/etiologia , Doenças Neuromusculares/complicações , Doenças Neuromusculares/terapia , Osteoporose/etiologia , Osteoporose/fisiopatologia , Pamidronato , Modalidades de Fisioterapia , Estudos Retrospectivos , Vômito/induzido quimicamente
10.
Eur J Paediatr Neurol ; 15(5): 439-48, 2011 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-21745754

RESUMO

BACKGROUND: Dystonia and spasticity are common symptoms in children with Cerebral Palsy (CP), whose management is a challenge to overcome in order to enable the harmonized development of motor function during growth. AIM: To describe botulinum toxin A (BTX-A) use and efficacy as a treatment of focal spasticity in CP children in France. METHODS: This prospective observational study included 282 CP children mostly administered according to French standards with BTX-A in lower limbs. Realistic therapeutic objectives were set with parents and children together before treatment initiation and assessed using the Visual Analogue Scale (VAS). Child management was recorded and the efficacy of injections was assessed during a 12-month follow-up period by physicians (Modified Ashworth Scale, joint range of motion, Physician Rating Scale, Gillette Functional Assessment Questionnaire and Gross Motor Function Measure-66) and by patients/parents (Visual Analogue Scale). RESULTS: BTX-A treatment was administered in different muscle localizations at once and at doses higher than those recommended by the French Health Authorities. Children were treated in parallel by physiotherapy, casts and ortheses. Injections reduced spasticity and improved joint range of motion, gait pattern and movement capacity. Pain was reduced after injections. BTX-A administration was safe: no botulism-like case was reported. The log of injected children who were not included in the study suggested that a large population could benefit from BTX-A management. CONCLUSIONS: We showed here the major input of BTX-A injections in the management of spasticity in CP children. The results are in favor of the use of BTX-A as conservative safe and efficient treatment of spasticity in children, which enables functional improvement as well as pain relief.


Assuntos
Toxinas Botulínicas Tipo A/administração & dosagem , Paralisia Cerebral/tratamento farmacológico , Espasticidade Muscular/tratamento farmacológico , Fármacos Neuromusculares/administração & dosagem , Adolescente , Toxinas Botulínicas Tipo A/efeitos adversos , Paralisia Cerebral/complicações , Paralisia Cerebral/fisiopatologia , Criança , Pré-Escolar , Feminino , Seguimentos , França , Humanos , Injeções Intramusculares/métodos , Masculino , Espasticidade Muscular/etiologia , Espasticidade Muscular/fisiopatologia , Fármacos Neuromusculares/efeitos adversos , Estudos Prospectivos
11.
J Nutr Health Aging ; 14(7): 595-600, 2010 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-20818476

RESUMO

BACKGROUND: Physical performance may predict survival independently of other current predictors in non selected elderly subjects. We determined if poor balance and decreased gait speed may predict mortality after adjustment for both baseline and follow-up confounders in well-functioning elderly women. METHODS: A subgroup of participants in the Epidemiology of osteoporosis (EPIDOS) study (N = 1,300) was followed for 8 years. Participants were community-dwelling women aged 75 or older able to go outside home without assistance. The baseline examination included a questionnaire and a clinical and functional examination. Participants were contacted every year thereafter by mail. RESULTS: Poor balance, defined by the inability to stand in a tandem position or to complete ten foot taps in less than 4.6 seconds, and poor mobility, defined by a gait speed of less than 0.80 m/s or a stride length of less than 0.5 m were significant predictors of low 8-year survival, independently of other predictors of death at baseline (educational level, social network, number of drugs, fear of falling, visual acuity, perceived health, IADL score, physical activity, and comorbidities) and during follow-up (falls, IADL score, the need to be accompanied to go outside, weight loss, hospitalization, and the report of new comorbidities). CONCLUSION: The current study shows that poor balance and mobility are significant predictors of 8-year mortality independently of baseline and intermediate events in pre-disabled women aged 75 years and older, suggesting that they may reflect a certain failure to respond adequately in the face of present and future medical and non-medical events.


Assuntos
Limitação da Mobilidade , Mortalidade , Aptidão Física , Equilíbrio Postural , Caminhada , Acidentes por Quedas , Atividades Cotidianas , Idoso , Idoso de 80 Anos ou mais , Comorbidade , Feminino , Seguimentos , Marcha , Avaliação Geriátrica , Hospitalização , Humanos , Fatores de Risco , Inquéritos e Questionários , Redução de Peso
12.
Arch Pediatr ; 14(9): 1076-83, 2007 Sep.
Artigo em Francês | MEDLINE | ID: mdl-17467248

RESUMO

UNLABELLED: To resort to gastrostomy feeding is a difficult decision to take in children with severe disability, the objective being better quality of life. OBJECTIVES: To describe quality of life in children with severe disability and to look for factors which influence this quality of life, in particular gastrostomy. METHOD: Descriptive study in 28 patients, aged 1 to 18 years, with severe motor and mental disability, with or without gastrostomy. The studied factors were: health and nutritional status and quality of life. The quality of life was evaluated with QUALIN questionnaire, specifically designed for infants. RESULTS: This questionnaire was pertinent and well accepted by the family. Quality of life was influenced by capacity of communication (P=0.006), quality of sleeping (P=0.004), digestive problems (P<0.05) and age (P=0.031). CONCLUSION: Gastrostomy did not impair quality of life. Its benefit was estimated as high as 8.71/10 in average by parents.


Assuntos
Crianças com Deficiência/psicologia , Gastrostomia , Nível de Saúde , Qualidade de Vida , Adolescente , Fatores Etários , Criança , Pré-Escolar , Comunicação , França , Gastroenteropatias/psicologia , Humanos , Lactente , Índice de Gravidade de Doença , Sono , Inquéritos e Questionários
13.
Ann Readapt Med Phys ; 50(4): 258-65, 2007 May.
Artigo em Francês | MEDLINE | ID: mdl-17349713

RESUMO

OBJECTIVES: To propose a standardized test of joint range of motion in paediatric patients with cerebral palsy, based on the opinion of a cerebral palsy specialist group (physicians, physiotherapists and surgeons) and literature review. MATERIALS AND METHODS: A Delphi process was adapted to elaborate a complete test of lower-limb-joint range of motion. During the preparation phase, a pilot committee selected a list of items compiled from literature search and personal experience. A first questionnaire was proposed to 16 experts, then six discussion meetings followed. From the results, the pilot committee prepared a second questionnaire for the experts and finalized the complete test of joint range of motion. RESULTS: The complete test includes 24 items related to goniometric measurements in supine and prone positions. Principal conditions necessary for an accurate exam are included. Further, a testing guide with visuals of the proposed exam techniques was developed. CONCLUSIONS: The standardized testing of lower-limb-joint range of motion will allow for better communication between clinicians and will facilitate the development of a database. Care should be taken in the interpretation of the joint range-of-motion test results and realized only after reliability analysis of the test, especially interobserver reliability. A further step would be to develop an outpatient booklet for cerebral palsy that shows growth and weight curves, joint range-of-motion curves and curves of the principal radiographic measurements during growth.


Assuntos
Paralisia Cerebral/fisiopatologia , Extremidade Inferior/fisiopatologia , Amplitude de Movimento Articular/fisiologia , Adolescente , Adulto , Artrometria Articular , Criança , Pré-Escolar , Técnica Delphi , Humanos , Projetos Piloto
14.
Ann Readapt Med Phys ; 50(4): 218-24, 2007 May.
Artigo em Francês | MEDLINE | ID: mdl-17321626

RESUMO

INTRODUCTION: The literature is controversial about how to manage scoliosis in cerebral palsy patients with total body involvement. Spinal fusion is admitted as the only means to stop the evolution of the curve but remains associated with many complications. OBJECTIVES: The aim of this descriptive study was to formulate hypotheses concerning the interest of spinal fusion in this population. METHODS: This descriptive cross-sectional study was conducted in 61 patients aged 4 to 48 years (mean 24 years, median 20 years) with severe scoliosis (Cobb score>40 degrees ). Each patient had undergone recent X-ray of the spine and hips and a clinical exam. Data were collected on orthopaedic and functional status, health level (pulmonary, digestive, nutrition, skin parameters), pain, and subjective quality of life (sQoL). RESULTS: Data were collected for all 61 patients (35 females, 26 males), 16 underwent spinal fusion at a mean age of 16.5 years (prevalence of 32.0%). No significant difference was found between patients with a spinal instrumentation and others in variables tested. A difference might be present with functional level, but only results from a longitudinal study would be conclusive. Pain was prevalent (19.7%), not treated sufficiently (only 50% receive treatment), and contributes to poor sQoL (p<0.042). Among the other data collected, nutritional level has the greatest influence on general health status (p<0.05). CONCLUSIONS: Systematic spinal fusion cannot be generalized for this population of patients with cerebral palsy and total body involvement. However, health status and quality of life could be improved with better care of nutritional problems and pain.


Assuntos
Paralisia Cerebral/complicações , Escoliose/complicações , Adolescente , Adulto , Criança , Pré-Escolar , Estudos Transversais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estado Nutricional , Dor/etiologia , Qualidade de Vida , Escoliose/cirurgia , Fusão Vertebral
16.
Rev Chir Orthop Reparatrice Appar Mot ; 92(1): 27-32, 2006 Feb.
Artigo em Francês | MEDLINE | ID: mdl-16609614

RESUMO

PURPOSE OF THE STUDY: This study was devoted to better understanding how adults with agenesia of the forearm live their condition. The malformation studied was identical to that of a previous study in children. The present study was designed to provide professionals and parents with a reference system for making therapeutic decisions. MATERIAL AND METHODS: An intensive survey using semi-directive interviews was conducted among adults aged 19 to 37 years, 12 women and 8 men, with unilateral agenesia of the forearm. The majority of the participants had an occupation and an active social and familial life. RESULTS: The survey revealed factors of limitation or progress, expectations and renouncements, marking the life experience of these persons. The prosthesis had a significant impact on their lives. DISCUSSION: Four types of life experience could be identified from the survey data. All four could be understood as a search for equilibrium between internal reality (desired self image) and external reality (image produced by the physical and human environment via the regards of others and obstacles encountered). Several modalities for using a prosthesis, several ways of coping with the absence of a hand, with or without a prosthesis, and several ways of constructing a self-representation were distinguished. Rather than a functional disability, agenesia was found to be perceived as a persistent problem in daily life, generating handicap- or discrimination-producing situations. CONCLUSION: Forearm agenesia creates a situation different from normal and as such makes the question of self-identity and social relations more complex than for others. The comments provided by these young adults who cope with the paradoxical condition of an amputated but functional body demonstrated the central role of the body.


Assuntos
Membros Artificiais , Pessoas com Deficiência/psicologia , Autoimagem , Comportamento Social , Deformidades Congênitas das Extremidades Superiores/psicologia , Atividades Cotidianas , Adulto , Imagem Corporal , Tomada de Decisões , Relações Familiares , Feminino , Antebraço/anormalidades , Inquéritos Epidemiológicos , Humanos , Masculino , Ocupações , Qualidade de Vida , Deformidades Congênitas das Extremidades Superiores/cirurgia
17.
Rev Neurol (Paris) ; 162(4): 485-93, 2006 Apr.
Artigo em Francês | MEDLINE | ID: mdl-16585909

RESUMO

A new scale for motor function measurement has been developed for neuromuscular diseases. After the study of a preliminary and a first version, the validation study included 303 patients, aged 6 to 62 years. Seventy-two patients had Duchenne muscular dystrophy, 32 Becker muscular dystrophy, 30 limb-girdle muscular dystrophy, 39 facio-scapulo-humeral dystrophy, 29 myotonic dystrophy, 21 congenital myopathy, 10 congenital muscular dystrophy, 35 spinal muscular atrophy and 35 hereditary neuropathy. The sensitivity for change was evaluated with 152 patients one year after. The scale comprised 32 items, in three dimensions: standing position and transfers, axial and proximal motor function, distal motor function. High correlations (>0.80) were found between the total score and other scores: Vignos and Brooke grades, Functional Independence Measure, the global severity of disability evaluated with visual analog scales by physicians and physiotherapists. This scale is reliable, does not require any special equipment and is well accepted by patients. It takes an average of 36 min (range 8-75) to complete the scale. Preliminary results of the second evaluation showed good sensitivity to change since last visit, considering rating by patient, investigator or physiotherapist. Also, significant differences in scores are obtained with the greatest deterioration observed in Duchenne patients.


Assuntos
Doenças Neuromusculares/classificação , Índice de Gravidade de Doença , Adolescente , Adulto , Criança , Feminino , Neuropatia Hereditária Motora e Sensorial/fisiopatologia , Humanos , Masculino , Pessoa de Meia-Idade , Atividade Motora , Atrofia Muscular Espinal/fisiopatologia , Distrofias Musculares/classificação , Distrofias Musculares/fisiopatologia , Doenças Neuromusculares/diagnóstico , Doenças Neuromusculares/fisiopatologia , Reprodutibilidade dos Testes , Sensibilidade e Especificidade
18.
Ann Readapt Med Phys ; 48(8): 616-22, 2005 Nov.
Artigo em Francês | MEDLINE | ID: mdl-16023755

RESUMO

OBJECTIVES: To study the link between pain, osteopenia and body composition in patients with Duchenne muscular dystrophy and to present a detailed questionnaire to evaluate their pain. MATERIALS AND METHODS: Twenty-two boys with Duchenne muscular dystrophy, mean age 11.4+/-4.0 years, were examined between February and March 2003. They were asked to complete a detailed questionnaire and undergo a global assessment of pain on a visual analog scale and muscular testing. They were also asked about a history of fractures. Their bone mineral content at the lumbar spine and femoral neck levels, as well as their body composition in fat and lean mass, were assessed by dual energy absorptiometry. RESULTS: The mean age for walking incapacity was 8.8+/-1.7 years. The youngest patients, who were still able to walk, had a higher level of pain than patients who depended on wheelchairs. No significant correlation was established between pain and osteopenia. One in 2 patients had spontaneous pain, and mobilization was painful for 21. The score obtained by detailed questioning about pain correlates with the average pain scores on visual analog scales. The bone mineral content was lower, especially in the lower limbs, had decreased before the inability to walk and was correlated with muscular weakness. Fractures were more frequent in mobile patients and usually occurred after a fall. CONCLUSION: Although pain in Duchenne muscular dystrophy has not been extensively studied, it is frequent and significant. Twenty-one patients had moderate to severe pain. The youngest patients had intense pain, especially during mobilisation. To evaluate this pain, we propose to use the mean results of 2 visual analog scales associated with a detailed questionnaire. However, in this study, Duchenne muscular dystrophy, pain and osteopenia were not correlated. Dual X-ray absorptiometry provides interesting information about bone mineral content, fat body mass and lean body mass. The fat body mass was higher than normal in our patients. The bone mineral content and lean body mass were lower than that for normal children, because the dystrophic process advances with age. The fracture prevalence was high, especially in young patients. Falling was the most common mechanism of fracture.


Assuntos
Densidade Óssea/fisiologia , Fraturas Ósseas/etiologia , Distrofia Muscular de Duchenne/complicações , Distrofia Muscular de Duchenne/fisiopatologia , Dor/etiologia , Criança , Humanos , Masculino , Medição da Dor , Inquéritos e Questionários
19.
Neurochirurgie ; 49(2-3 Pt 2): 199-204, 2003 May.
Artigo em Francês | MEDLINE | ID: mdl-12746694

RESUMO

This article presents the four principle steps of assessment of children with cerebral palsy. Weakness of some muscles groups and spasticity of others and the subsequent functional impairment can be identified by observation. Analytical assessments of spasticity with the Ashworth and Tardieu scales were compared in 30 children with cerebral palsy, mean age 8.5 years. The results pointed out the complimentary nature of these two scales: The Gross Motor Function Measure, which is a validated scale, is specific for children with cerebral palsy. This measure provides a useful assessment of outcome and a convenient means of evaluating the time-course. Joint assessment is also essential to identify muscle contracture. Assessment of spasticity in children with cerebral palsy must be conducted by a multidisciplinary team to take into account all the aspects involved.


Assuntos
Paralisia Cerebral/diagnóstico , Espasticidade Muscular/diagnóstico , Criança , Pré-Escolar , Marcha , Humanos , Exame Neurológico , Resultado do Tratamento
20.
Rev Chir Orthop Reparatrice Appar Mot ; 88(4): 337-41, 2002 Jun.
Artigo em Francês | MEDLINE | ID: mdl-12124532

RESUMO

PURPOSE OF THE STUDY: Children with cerebral palsy who cannot walk have an oblique pelvis and scoliosis. There is a certain degree of controversy in the literature on the best way to manage this difficult situation. We present a descriptive analysis of a population of non-ambulatory adults with cerebral palsy in order to formulate hypotheses concerning the factors determining scoliosis. MATERIAL AND METHODS: This descriptive cross-sectional study was conducted in 234 patients aged over 15 years who had cerebral palsy and could not walk. Physical examination and an x-ray of the pelvis and spine in the reclining position were obtained for all patients. The following variables were recorded: luxation and subluxation of the hip, spontaneous deviation attitude, ability or not to turn over in bed, pelvic obliquity, history of bone surgery, defective hip abduction. The statistical analysis accounted for laterality and pelvis obliquity to the scoliosis convexity and the laterality of the hip excentration. RESULTS: Scoliosis was observed in 66.2% of the patients; it was more than 60 degrees in 34.5%. Two basic groups were distinguished: thoracolumbar scoliosis (41.6%) and lumbar scoliosis (41.6%). The prevalence of oblique pelvi was 59.9% with important difference by side: 31.6% right oblique and 68.4% left oblique pelvi. We were unable to find any relationship between the side of the pelvic obliquity and the side of the scoliosis convexity, the side of the hip excentration, or the deviation attitude, but the deviation attitude appeared to be a risk factor for pelvic obliquity, which itself was a risk factor for excentration, which was a risk factor for scoliosis. DISCUSSION: Scoliosis is an important problem in this population. Hip luxation is a direct risk factor for scoliosis, but the deviation attitude and pelvic obliquity are intermediary stages. The prevalence of oblique pelvi was greater on the left than the right. This finding should be confirmed in other series before hypotheses can be formulated concerning this difference.


Assuntos
Paralisia Cerebral/complicações , Luxação do Quadril/diagnóstico por imagem , Luxação do Quadril/etiologia , Ossos Pélvicos/diagnóstico por imagem , Escoliose/diagnóstico por imagem , Escoliose/etiologia , Atividades Cotidianas , Adolescente , Adulto , Fatores Etários , Estudos Transversais , Pessoas com Deficiência , Feminino , Luxação do Quadril/epidemiologia , Luxação do Quadril/fisiopatologia , Humanos , Masculino , Pessoa de Meia-Idade , Ossos Pélvicos/patologia , Prevalência , Radiografia , Amplitude de Movimento Articular , Fatores de Risco , Escoliose/epidemiologia , Escoliose/fisiopatologia , Caminhada
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