Key Alford (laughrhythm7)
arker of neurodegeneration in NPC1-disease after onset of clinical symptoms.BACKGROUND Living with a progressive disease as muscular dystrophy (MD) can be challenging for the patient and the entire family from both emotional and practical point of view. We aimed to extend our previously published data about mental health in patients with MDs, also investigating coping profiles of both themselves and their parents. Furthermore, we wanted to verify whether psychological adaptation of patients can be predicted by coping strategies, taking also into account physical impairment, cognitive level and socioeconomic status. METHODS 112 patients with MDs, aged 2-32 were included. Their emotional and behavioural features were assessed through parent- and self-report Achenbach System for Empirically Based Assessment questionnaires and Strength and Difficulties Questionnaires. Development and Well-Being Assessment or Autism Diagnostic Observation Schedule were administered to confirm suspected diagnoses. Coping profile of both parents and patients was assessed through the self-administered New Italian Version of the Coping Orientation to the Problems Experienced questionnaire and its relationship with emotional/behavioural outcome was examined in linear regression analyses. RESULTS High prevalence of intellectual disability and autism spectrum disorders was confirmed in Duchenne MD. Despite the high rate of internalizing symptomatology, we did not report higher rate of psychopathological disorders compared to general population. Parents tend to rely more on positive reinterpretation and less on disengagement coping. Avoidance coping, whether used by parents or patients, and ID, predicted increased emotional/behavioural problems. CONCLUSIONS Psychosocial interventions should address problems of anxiety and depression that people with MDs frequently experience, even through fostering parents' and childrens' engagement coping over disengagement coping.BACKGROUND The prevalence and risk factors of low bone mineral density (BMD) in Asian patients newly diagnosed with inflammatory bowel disease (IBD) have not been fully suggested. AIMS We aimed to examine the prevalence and risk factors of low BMD in young Korean patients newly diagnosed with IBD. METHODS We prospectively enrolled 132 patients aged less than 50 years and newly diagnosed with IBD from six tertiary referral centers in Korea between November 2014 and April 2017. BMD was measured by dual-energy X-ray absorptiometry, and then the Z-score was determined. We defined low BMD as a Z-score ≤ - 1.0. RESULTS Of 68 patients with ulcerative colitis (UC), 22 (32.4%) had low BMD. Also, of 64 patients with Crohn's disease (CD), 24 (37.5%) showed low BMD. Results from multivariate regression analysis identified the risk factors for low BMD as a high level of alkaline phosphatase (ALP) (≥ 140 U/L) (P = 0.010) in UC patients, and being underweight (body mass index ≤ 18.5 kg/m2) (P = 0.017) in CD patients. CONCLUSIONS Our study showed that about one-third of newly diagnosed IBD Asian patients had low BMD. The clinical factors associated with low BMD were a high level of ALP in UC patients, and being underweight, in CD patients. Therefore, measurements of BMD in young patients should be considered at the diagnosis of IBD.The climate change on the impact of grain production potential has significant regional differences. Researchers have studied the grain production potential of various crop combinations or focused on single crop types in a typical area; however, the regional differences of the climate change on the impact of grain production potential were neglected. This paper used the Global Agro-Ecological Zone (GAEZ 3.0) model to focus on the analysis what is the climate change on the impact of grain production potential in different geographic units (Northern Shaanxi Plateau, Guanzhong Basin, Qinba Mountain) in Shaanxi Province of China. The case showed that the precipitation (P