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Cryptosporidium spp., a significant cause of foodbome infection, have been shown to be resistant to most chemical food disinfectant agents and infective for weeks in irrigation waters and stored fresh vegetal produce. Pulsed UV light (PL) has the potential to inactivate Cryptosporidium spp. on surfaces of raw or minimally processed foods or both. The present study aimed to evaluate the efficacy of PL on viability and in vivo infectivity of Cryptosporidium parvum oocysts present on raspberries, a known source of transmission to humans of oocyst-forming apicomplexan pathogens. The skin of each of 20 raspberries was experimentally inoculated with five 10-pl spots of an oocyst suspension containing 6 X 107 oocysts per ml (Nouzilly isolate). Raspberries were irradiated by PL flashes (4 J/cnr of total fluence). This dose did not affect colorimetric or organoleptic characteristics of fruits. After immunomagnetic separation from raspberries, oocysts were bleached and administered orally to neonatal suckling mice. Seven days after infection, mice were euthanized, and the number of oocysts in the entire small intestine was individually assessed by immunofluorescence flow cytometry. Three of 12 and 12 of 12 inoculated mice that received 10 and 100 oocysts isolated from nonirradiated raspberries, respectively, were found infected. Four of 12 and 2 of 12 inoculated mice that received 103 and 104 oocysts from irradiated raspberries, respectively, were found infected. Oocyst counts were lower in animals inoculated with 103 and 104 oocysts from irradiated raspberries (92 ± 144 and 38 ± 82, respectively) than in animals infected with 100 oocysts from nonirradiated raspberries (35,785 ± 66,221, P = 0.008). PL irradiation achieved oocyst reductions of 2 and 3 log for an inoculum of 103 and 104 oocysts, respectively. The present pilot-scale evaluation suggests that PL is an effective mode of decontamination for raspberries and prompts further applicability studies in industrial contexts. [ABSTRACT FROM AUTHOR]

This work aims to describe the improvements experienced by spinal muscular atrophy (SMA) adult patients with treatments using qualitative methods. While therapeutic treatments of SMA have rapidly developed, difficulties arise to define the specificity of the drugs' effects compared with routine management due to the lack of clinical trials in adults. We performed a secondary analysis of a qualitative study directed by a scientific committee of SMA specialists, carried out in 8 specialized centers and involving 18 adult patients and 31 HCPs interviewed by a sociologist. A thematic analysis of the transcriptions of patients' interviews was performed focused on the perceived effects of treatments. 18 patient interview transcriptions were reviewed. 13 patients report having the experience of a treatment: 11 ongoing, 2 discontinued. According to their SMA type and the severity of their disabilities, they report different kinds of improvements of the treatment described into 3 categories according to the International Classification of Functioning, Disability and Health (ICF): improvement of strength and endurance (body functions and structures), of function (activities) and of fatigue (social participation). Patients with severe disabilities (non-ambulant with severe limitations of distal function of superior limbs, swallowing impairment and/or speech difficulties) report improvement of their speech in volume and endurance as of the strength and endurance of their fingers which improve their manipulation of electric wheelchair, computer and time of social participation. On the other hand, ambulant patients report improvement of their walking endurance and less fatigability in their daily activities. SMA adult patients report improvements that are not measured with existing assessment tools due to their lack of sensitivity. This work highlights the need for more sensitive and reliable assessments and provides insights for their development. [ABSTRACT FROM AUTHOR]

The clinical presentation and prognosis of individuals harbouring four copies of SMN2 gene are not well-known and presymptomatic treatment remains controversial. The aim of this study was to describe the clinical phenotypes associated with 4 SMN2 copies in France. Collected data from all SMA patients with 0SMN1 and 4SMN2 enrolled in the national French Registry (Registre SMA France) were analyzed to better understand epidemiology, clinical presentation and course of the disease. SMN2 copy number was available in 872 patients of 1112 SMA enrolled patients. 140 patients (16.1%) carried four SMN2 copies (0 SMN1 4 SMN2). Median age at onset was 3.5 years (6 months to 20 years) and median follow-up was 32 years. 12 patients (8.6%) never walked independently (SMA type 2). 72% of the cohort was able to stand or walk with support. Independent walking was acquired in 91% (123 SMA3, 5 SMA4) and one third of them lost this ability (median 16 years). Loss of ambulation was significantly earlier in children with onset before 3 years (SMA3a). There was a significant predominance of men in the whole cohort (63%) and in sub-cohorts (SMA2-83%; SMA3-61%; adult population-68%). There was a significant lower risk for women to lose ambulation (p=0.01). 65% of patients used a wheelchair. Scoliosis surgery and ventilation was required in less than 15%. Most SMA patients with 4 S MN2 copies in the French population showed an onset during childhood and a progressive course with absence or loss of ambulation before adulthood. Presymptomatic treatment seems an acceptable option to consider, although identification of individual pejorative markers of early or severe phenotypes would allow more tailored approaches. Our results confirm the phenotypic variability reported in literature, suggesting an overall gender effect in this population. [ABSTRACT FROM AUTHOR]

This study aims to understand how French healthcare professionals (HCPs) deal with the challenge of evaluating spinal muscular atrophy (SMA) adult patients with severe disabilities. SMA assessment is important for guiding care and objectifying the effects of treatments. However, SMA existing assessment tools have limitations especially with adult patients with severe disabilities. We performed a secondary analysis of a qualitative study directed by a scientific committee of SMA specialists, carried out in 8 specialized centers and involving 18 patients and 31 HCPs interviewed by a sociologist. A thematic analysis of the transcriptions of HCPs interviews was performed focused on the evaluation practices for SMA adult patients with severe disabilities (type 1b & 2). 31 HCPs interview transcriptions were reviewed. 26 HCPs report a lack of sufficiently sensitive and accurate evaluations for type 1b & 2 SMA adult patients and 20 HCPs report having to complement them with unofficial evaluations: ad hoc indicators co-decided with patients or in-house non validated instruments. 38 unofficial evaluations were created by HCPs for type 1b or type 2 SMA adult patients to take into account specificities of these patients poorly addressed by existing assessments such as: 1) invasive ventilation 2) swallowing impairment 3) severe limitations of distal function of superior limbs 4) speech difficulties 5) global fatigability. These unofficial evaluations can be classified in 10 domains: 1. respiratory aptitudes 2. swallowing 3. hand and fingers dexterity 4. meal length or structure 5. fatigue 6. finger strength 7. head support 8. pronunciation 9. social participation time 10. ad hoc daily life abilities. Also concerns arise regarding the validity and reliability of such measures. This unprecedented work highlights the needs for sensitive hence reliable and feasible assessment for SMA adult patients with severe disabilities. [ABSTRACT FROM AUTHOR]