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Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) refractory to conventional therapy can lead to marked disability and represents a therapeutic challenge.
Objective:
To report five cases of treatment-refractory disabling CIDP treated with autologous hematopoietic stem cell transplantation (AHSCT).
Methods:
This was a retrospective cohort study from a tertiary care referral center for both neuromuscular disease and AHSCT. Patients with CIDP treated with AHSCT between 2008 and 2020 were included. All patients had major persistent and disabling neuropathic deficits despite combinations of intensive immunosuppressive therapy. The primary outcome measures were: Medical Research Council sum score, Overall Neuropathy Limitations Scale and requirement for ongoing CIDP immunotherapy after transplantation. We also analyzed safety outcomes by documenting all severe AHSCT-related complications.
Results:
Five patients with refractory CIDP underwent AHSCT. Three were classified as manifesting a typical syndrome, two were classified as the multifocal Lewis Sumner variant. The mean age at time of CIDP diagnosis was 33.4 years (range 24–46 years), with a median delay of 46 months (range 21–135 months) between diagnosis and AHSCT. The median follow-up period was 41 months. All five patients were able to wean off CIDP-related immunotherapy. Marked improvements in Medical Research Council scale and overall Neuropathy Limitations Scale were noted in 4/5 patients. One patient with longstanding neurogenic atrophy showed no improvement in disability scales. There were no treatment-related deaths or critical illnesses.
Conclusions:
AHSCT can achieve marked sustained clinical improvement of refractory CIDP and may allow for weaning off long-term complex immunotherapies.
To investigate socio-economic differences in children’s diet, activity and inactivity and changes in these differences over 4 years during which new policies on food in schools were introduced.
Design
Two cross-sectional surveys in which diet was assessed by FFQ and physical activity and inactivity were assessed by interviewer-administered questionnaire. Socio-economic status was assessed by the area-based Scottish Index of Multiple Deprivation.
Setting
Scotland, 2006 and 2010.
Subjects
Children aged 3–17 years (n 1700 in 2006, n 1906 in 2010).
Results
In both surveys there were significant linear associations between socio-economic deprivation and intakes of energy, non-milk extrinsic sugars (NMES) as a percentage of food energy, sugar-sweetened beverages, confectionery, crisps and savoury snacks and leisure-time screen use (all higher among children in more deprived areas), while intakes of fruit, fruit juice and vegetables showed the opposite trend. In 2010 children in more deprived areas engaged in more physical activity out of school than those in more affluent areas, but between 2006 and 2010 there was an overall reduction in physical activity out of school. There were also small but statistically significant overall reductions in intakes of confectionery, crisps and savoury snacks, energy and NMES and saturated fat as a percentage of food energy, but no statistically significant change in socio-economic gradients in diet or activity between the two surveys.
Conclusions
Interventions to improve diet and physical activity in children in Scotland need to be designed so as to be effective in all socio-economic groups.
Ambulance drivers often travel under stressful conditions at high speed while using vehicles with poor high-speed maneuverability. The occupant safety of ambulance vehicles has not yet been addressed by the automotive safety paradigm; particularly for the rear patient compartment. This study had two objectives: (1) to assess by survey the French Emergency Medical Services (EMS) to determine the layout of the vehicle most often used and the EMS personnel's behavior during transport; and (2) to conduct a crash test to analyze the injuries which may affect EMS personnel and patients in the rear patient compartment.
Method
Firstly, a survey was distributed to the 50 largest metropolitan French EMS programs. Secondly, a crash test was performed with a Mobile Intensive Care Unit (MICU) in conditions closest to reality.
Results
Forty-nine of the 50 biggest metropolitan French EMS programs responded to the survey. This represents 108 French MICUs. During the last three years, 12 of 49 EMS programs (24%) identified at least one accident with an MICU, and six of these 12 (50%) suffered at least one death in those accidents. A crash test using a typical French EMS MICU showed that after impact of a collision, the ambulance was moved more than five meters with major consequences for all passengers. A study-approved human cadaver placed in the position of a potential patient was partially thrown from the stretcher with a head impact. The accelerometric reaction of the anthropomorphic manikin head was measured at 48G.
Conclusion
The crash test demonstrated a lack of safety for EMS personnel and patients in the rear compartment. It would be preferable if each piece of medical equipment were provided with a quick release system resistant to three-dimensional 10G forces. The kinetic changes undergone by the “patient” substitute on the stretcher would probably have an effect of causing injury pathology. This study highlights the need for more research and development in this area.
FournierM, ChenaitiaH, MassonC, MicheletP, BehrM, AuffrayJP. Crew and Patient Safety in Ambulances: Results of a Personnel Survey and Experimental Side Impact Crash Test. Prehosp Disaster Med. 2013;28(4):1-6.
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