Langerhans cell histiocytosis: therapeutic strategy and outcome in a 30‐year nationwide cohort of 1478 patients under 18 years of age

The French national cohort of children with Langerhans cell histiocytosis (LCH) has included 1478 patients since it was established in 1983. LCH therapeutic strategies substantially changed in 1998, so we have divided the cohort into two 15‐year periods. Starting in 1998, therapy duration increased from 6 to 12 months, repeated induction therapy was performed in cases showing a poor response to the first induction with vinblastine and steroids, and refractory disease in a risk organ (RO+) was treated with cladribine and cytarabine. A total of 483 (33%) patients were enrolled before 1998, and 995 (67%) after 1998. Five‐year survival was 96·6% (95% confidence interval: 95·4–97·5%) overall, improving from 92% pre‐1998 to 99% post‐1998 (P < 0·001 adjusted to disease extent). This change was supported by an increase in 5‐year survival from 60% to 92% in the RO+ group. Survival was particularly associated with cladribine and cytarabine among refractory RO+ patients. Disease reactivation was slightly less frequent after 1998, due to better enrolment of single‐system patients, extended therapy duration, and more efficient second‐line therapy. The crude rates of endocrine and neurological sequelae (the most frequent sequelae) appeared to improve over time, but this difference was not observed when the analysis was stratified by disease extent.     

Electronic tracking system and wandering in Alzheimer's disease: A case study

IntroductionWandering is a behavioural disorder, which occurs in Alzheimer's disease or other dementia. People who wander are at risk of physical harm and untimely death. Moreover, wandering behaviour causes a lot of stress to the caregivers. In the last few years, different geolocation devices have been developed in order to minimise risk and manage unsafe wandering. These detection systems rarely meet patients and caregivers’ needs because they are not involved in the devices building process.AimThe aim is to explore the needs and perceptions of wandering persons and their caregivers towards existing tracking devices as well as their acceptability and usability. This paper reports a dyad case.MaterialsThe tracking system tested is presented as a mobile Global Positioning System (GPS) receiver-shaped, including function of telephony and data transfer via GSM/GPRS.MethodDyad patient/caregiver expressed their needs and perceptions towards tracking devices and gave their impressions about the functioning of the tested device at the end of the test.ResultsThe patient focused on the device's shape which he found too voluminous and unaesthetic, and was unable to give an opinion about the device's functioning. The spouse highlighted malfunctions and usage difficulties, which made the device not appropriate to her needs.ConclusionInvolving end-users in the co-design of new technologies is necessary for building tailored devices. Moreover, in this area of dementia care, the person-centred approach is essential to a tailored wandering management.     

Pulmonary opacities in the shape of ribbon streamers. Apropos of a case

The authors report a case of chronic interstitial pneumonia with a very unusual appearance (on plain films and CT); in fact such ribbon-like linear opacities with concentric and pseudo-cavitary disposition have not been previously described. They are comparable but different from the linear opacities described by Carrington in some cases of chronic eosinophilic interstitial pneumonia, which are vertical and peripheral. They are also different from the curvilinear sub-pleural opacities reported in some cases of asbestosis. On the basis of the anatomic findings they discuss the specificity of such radiologic patterns or their sole value as an evolutive state.     

Low high density lipoprotein-2 concentrations in obese male subjects

In this study we have compared the lipoprotein patterns, in particular HDL subfractions, of 34 obese men to those of 34 normoponderal normolipemic men, matched for age and use of tobacco. Obesity was associated with increased VLDL concentrations in only half the subjects. HDL concentrations in all obese subjects were lower than in matched controls. The decrease was most marked in the HDL2 subfraction in which cholesterol and protein contents were decreased by 50%; it was independent of triglyceride levels and not related to the severity of overweight. Moreover, while HDL2 was negatively correlated with BMI (P less than 0.01) when both populations were considered together, the correlation disappeared when calculated separately within each population, suggesting a threshold effect. The low levels of HDL2 might result from discretely altered lipolysis, not sufficient to cause an elevation in fasting triglyceridemia. In this case, HDL2 should prove to be a sensitive index of lipolytic efficiency.