Anvisa vai publicar autorização para comércio do Spinraza na próxima segunda- feira (28). AAME – Amigos da Atrofia Muscular Espinhal, Rio de Janeiro, Brazil. 11K likes. A AAME tem como missão divulgar a Atrofia Muscular Espinhal visando a. Anestesia na Atrofia Muscular Espinhal – AME. Anesthesia and spinal muscle atrophy. Spinal muscle atrophy (SMA) is autosomal recessive and .

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Lean mass and fat mass modifications are also expected in this population. Patients’ monofrequency bioelectrical impedance was used for analyze body composition. Chest, hip and abdominal girths were measured by a flexible steel tape. The difference between forced inspiration and forced expiration thorax girth was smaller for patients when comparing to Group II: Spinal muscular atrophy SMA is a recessive, autosomal neuromuscular disease characterized by degeneration of anterior horn spinal cord motor cells and brain stem neurons It is classified by disease severity and the age at on set of symptoms, namely type I for the most severe cases and type IV for those presenting few complications 1,3, Type II SMA infants get to three point of sitting independent and present thoracic deformity because of muscle weakness, whide causes postural deviations.

Patients with type III get to three point of walking, wether amiotrofua not they maintain this ability throughout adulthood ,6,7. SMA patients present progressive symmetrical proximal weakness and hypotonia ,6,8but there is no sensory abnormality espinal.

Besides muscle weakness, respiratory 9orthopedic 5and nutritional problems are particularly note wortley. Among the methods used to evaluate body composition in children, bioelectrical impedance stands out as a noninvasive and painless method, which has already been adopted by other authors while evaluating SMA patients 13or children and adolescents without the disease espinhql Body girth measurements are frequently used in clinical practice 17,18 due to their practical character and low price.

Thoracic girth has already been associated with pulmonary function 19without any intention of replacyng the standard spirometry measurements, used for a more frequent follow up of patients’ pulmonary function. SMA children suffer from stunted development due to nutritional, muscular, postural and respiratory alterations. Body composition assessment in these patients is proposed by Caromano et al. There are few studies assessing neuromuscular patients with more specific methods than bioelectrical impedance and anthropometric measurements.

Therefore, the aim of this study is verify the body composition and chest expansion of type II and III spinal muscular atrophy patients.

Study Protocol and Sample. The subject was instructed to maintain both feet on the platform, without support and with the entire weight divided between both lower limbs For those that could not maintain the upright posture, this measurement was done with another person’s help supporting the patient and subtracting the weight of the assistant from the total weight measured. Height was estimated for all participants Groups I and II by the equation proposed in the literature for brazilian data 22 because a great number of patients could not stand up and had lower limb deformities: Two electrodes were applied to the dorsal surface of the right hand and two electrodes were placed on the dorsal surface of the right foot.

The exam was performed in the supine position and the subjects received standardized recommendations: Resistance and reactance values provided by the analyzer were used to estimate the fat-free mass kgin a child-specific equation 14, Fat percentage was calculated by the following equations: The abdomen measurement espibhal made at umbilical level 24 and the hip girth, at the greatest girth, proximately to pubic symphysis For those that could not maintain the upright posture, measurements were taken with another person’s help, supporting the patient’s weight while the examiner performed the measurement.

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For thoracic girth, the flexible steel tape was placed at the level of the mesosternum, with arms abducted Amiiotrofia those that could not maintain posture, the measure the upright was performed amiottofia a sitting position, preferentially without the use of chair back.

Three measure were taken: The difference between maximal inspiration and maximal expiration girths was called ‘chest expansion’.

Nonparametric descriptors and tests with resampling methods were selected because of the small sample size. Data were expressed as median interquartile range. The Mann Whitney U test was used to compare groups, and the Monte Carlo simulation with 10, bootstrap samples was applied. All analyses were realized on the SPSS program, amiotrpfia Table 1 summarizes demographic, body composition and girth variables separated by group. The groups were also comparable for weight, height and BMI: Group I presented higher values of resistance, impedance and fat percentage.

Lower values of chest expansion, fat-free mass and FFMI were also observed, though the last two variables presented no statistical difference Table 1Figure 1. Those authors observed an average of However, those authors included only three patients with SMA type II and did not investigate the thoracic expansion of their sample.

Another American research group 13 showed that 21 patients with types II and III SMA have higher levels of adiposity than subjects without the disease, considering either the thickness of subcutaneous fat tissue or the use of multifrequency bioelectric impedance. Espnihal agreement with this study, subcutaneous fat evaluated by a skinfold thickness caliper in 25 SMA patients type II: A study from Rio Grande do Norte, Brazil 27assessed the fat percentage in 25 patients with neuromuscular diseases but presented results which cannot be compared with ours.

Remédio inédito para atrofia muscular espinhal é liberado

However, used the different method for assessment of fat percentage between these studies skinfold thickness technique vs bioelectric impedance analysis must be falceer espinha, consideration, as well as the fact that only four SMA patients were included in the heterogeneous group of patients with neuromuscular diseases.

In a recent update, Markowitz et al. This fact may suggest to the espinhak health professional, who may assess the SMA patient only by BMI, to prescribe a dietary program to increase weight 6 that he should this reinforcing the fat accumulation in this population.

These findings justify the use of more precise measures of body composition in SMA patients. The physiological process by which the fat accumulates amitorofia this population is not yet clear, a possible hypothesis being the replacement of myocytes by both conjunctive and adipose tissue in advanced stages of this chronic disease.

The fat-free mass in Group I was lower through without statistical significance than in the healthy group. This small amount of muscle mass corroborates the clinical profile of weakness observed in SMA patients ,6,8. The fact that these patients present low levels of physical activity due to muscle weakness and contractures reinforces the low energetic expenditure sepinhal the risk of obesity 6.

The increased fat mass represents an additional threat to the already compromised muscles that may lead to diminished motor function It is worth noticing that the height as estimated from the espinha, used in this study 14 was used to calculate the fat-free mass suggested by Rabito et al. Those authors observed a coefficient of determination equal to 0.

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atrofia muscular espinhal – Translation into English – examples Portuguese | Reverso Context

There is a consensus in the literature concerning the difficulties in obtaining accurate height measurements due to muscle contractures or inability to stand up, the use of measurements of other body segments and the half span as in the present study being suggested to estimate the patient’s height 6. The average arm span of three SMA patients epinhal cm in a previous study 25 and is similar to the double of the median half span observed in the present study Data from a previous study 29 on pulmonary function spirometry in SMA patients showed a restrictive disorder characteristic of this population as resulting from respiratory muscle weakness, and reduced both chest wall and pulmonary compliance.

Small values of thoracic expansion in the studied sample confirm this respiratory restriction and show that assessments by a simple instrument such as a flexible steel tape can be used for the follow-up of pulmonary function. According to Lima and Lima 30girth measurements in children are important since it is a simple method, thus allowing the follow-up of bodily dimensions in clinical practice.

A characteristic feature of type II SMA patients is the major weakness of the intercostal muscle, in addition to weakness of the other inspiratory and expiratory muscles 6.

The observed difference in thoracic expansion for Group I corroborates the above-cited relationship in a recent review about SMA 3when the respiratory muscle weakness, the restrictive pulmonary function, and the natural history of disease are considred.

We are thankful to the patients and their tutors that agreed to participate in this study. Orphanet J Rare Dis. Classification, aetiology, and zmiotrofia of spinal deformity sepinhal children and adolescents.

A Clinical and Research Update. Self-reported quality of life has no correlation with functional status in children and adolescents with spinal muscular atrophy.

Eur J Paediatr Neurol. Evaluation of muscle strength and motor abilities in children with type II and III spinal muscle atrophy treated with valproic acid. Jornal Brasileiro de Pneumologia. Feeding problems and malnutrition in spinal muscular atrophy type Maiotrofia. Increased fat mass and high incidence of overweight despite low body mass index in patients with spinal muscular atrophy. Fat-free mass by bioelectrical impedance vs dual-energy Xray absorptiometry DXA.

Rev Bras Cineantropom Desempenho Hum ; 13 1: Adiposity and postural balance control: Correlations between bioelectrical impedance and stabilometric signals in elderly brazilian women. Rev Bras Cineantropom Desempenho H;14 2: International Standards for Anthropometric Assessment. Weight and height prediction of immobilized patients.

Avaliação da força muscular e da habilidade motora das crianças com amiotrofia espinhal

Waist-to-thigh ratio can also be a better indicator associated with esoinhal 2 diabetes than traditional anthropometrical measurements in taiwan population. Assessing spinal muscular atrophy with quantitative ultrasound.

Disorders of ventilation weakness, stiffness and mobilization. Manuscript submitted Oct 15 Accepted for publication Mai 02 Height Height was estimated for all participants Groups I and II by the equation proposed in the literature for brazilian data 22 because a great number of patients could not stand up and had lower limb deformities: Statistical Analysis Nonparametric descriptors and tests with resampling methods were selected because of the small sample size.

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