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The relationship between body composition and function in spinal muscular atrophy (SMA) is poorly understood. 53 subjects with SMA were stratified by type and Hammersmith Functional Motor Scale, Expanded score into three cohorts: Low-Functioning Non-Ambulatory (type 2 with Hammersmith score <12, n=19), High-Functioning Non-Ambulatory (type 2 with Hammersmith Score ≥ 12 or non-ambulatory type 3, n=17), and Ambulatory (n=17). Lean and fat mass was estimated using dual-energy x-ray absorptiometry. Anthropometric data was incorporated to measure fat-free (lean mass in kg /stature in m2) and fat (fat mass in kg /stature in m2) mass indices, the latter compared to published age and sex norms. Feeding dysfunction among type 2 subjects was assessed by questionnaire. Fat mass index was increased in the High-Functioning Non-Ambulatory cohort (10.4 ± 4.5) compared with both the ambulatory (7.2 ± 2.1, p = 0.013) and Low-Functioning Non-Ambulatory (7.6 ± 3.1, p = 0.040) cohorts. 12 of 17 subjects (71%) in the High-Functioning Non-Ambulatory cohort had fat mass index >85th percentile for age and gender (connoting “at risk of overweight”) versus 9 of 19 subjects (47%) in the Low-Functioning Non-Ambulatory cohort and 8 of 17 ambulatory subjects (47%). Despite differences in clinical function, a similar proportion of low functioning (7/18, 39%) and high functioning (2/7, 29%) type 2 subjects reported swallowing or feeding dysfunction. Non-ambulatory patients with relatively high clinical function may be at particular risk of excess adiposity, perhaps reflecting access to excess calories despite relative immobility, emphasizing the importance of individualized nutritional management in SMA.

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Objectives-To study the role of exposure, atopy, and smoking in the development of laboratory animal allergy (LAA) in a retrospective cohort study. Methods-Between 1977 and 1993, 225 people received a pre-employment screening when they started a job at a Dutch research institute where they were going to work with laboratory animals. shorter at a higher intensity of exposure, except for those exposed for less than two hours a week. A proportional hazard regression analysis showed that exposure and atopy were significant determinants of LAA. An increased relative risk (RR) was found for non-atopic people exposed to laboratory animal allergens for more than two hours a week. Atopic people had an even higher risk when exposed to laboratory animals for more than two hours a week (RR above 7.3). Sex, smoking, and age were not risk factors. More atopic than non-atopic people were absent from work or transferred because of allergies. Conclusions-This study showed that exposure and atopy are significant predictors of LAA and that the risk of developing LAA remained present for a much longer period (>3 y) than considered before. People who work with laboratory animals are at risk of developing an allergy to the animals they work with. Prevalence rates of 10%-30% have been found, and give an impression of the magnitude of the health risks.' 2 Urinary proteins of laboratory animals are the cause of laboratory animal allergy (LAA).' Mild symptoms of LAA are rhinitis, and skin and eye reactions.' 3'4 Asthma is a more severe form that develops in about 17%-71% of cases of LAA.1 There are some suggestions that most cases develop LAA two to three years from initial exposure to the allergen.' 2 However, the evidence for this is limited and is mainly based on clinical data. No unbiased estimates of time till sensitisation are available from well designed epidemiological studies.Allergen concentrations are known to vary considerably, and depend on stock density, tasks performed, ventilation rates,5 cage design, bedding type, air filtration, and humidity.2 Despite these findings, few studies focused on the relation between exposure intensity and duration and development of LAA. Recent findings from a cross sectional study performed suggest that work related symptoms are related to exposure intensity (expressed either in terms of dust or aeroallergen concentrations) at the time of onset of symptoms of LAA.6 Generally, crude proxies have been used to characterise exposure to allergens. In one cross sectional study the degree of exposure to animals had a positive and significant association with the presence of LAA, but duration of employment was not related to LAA.' Renstrom et at examined the differences for several response variables in an exposed and a matched non-exposed group (36 pairs) which were sampled out of a large group of laboratory animal workers. After two years of follow up no clear differences were found between the two groups in incidence of LAA, specific IgE, and atopy. Work related allergic symptoms were