Abstract Limited research has investigated the challenges faced by families caring for children with neuronal ceroid lipofuscinosis type 2 (CLN2) disease. Face-to-face, mixed-method, in-depth surveys were conducted with 19 families (23 children) in the UK (n=9) and Germany (n=10) to assess the impact of caring for children with CLN2 disease, using national wellbeing and quality of life (QoL) measures. Primary (n=19) and secondary (n=10) caregivers, adult siblings (n=2), and child siblings (n=2) were included. Caregivers reported reduced health-related QoL compared with age and gender-matched controls (mean utility scores 0.08 and 0.11 lower in Germany and the UK, respectively). Hours of caregiving were significantly higher relative to that provided to a child of normal health, with stress, back pain, and reductions in sleep being recorded. Lower life satisfaction and happiness with partners were also reported, along with significant financial burden. Those caring for children in the late stage of disease were more greatly impacted than those with children in the rapidly progressive stage, or who were bereaved. The results of this study make clear the importance of emotional and practical support for caregivers and siblings coping with CLN2 disease.
Background: Shigella spp is a frequent cause of diarrhea in children. Antimicrobials decrease the duration of diarrhea and pathogen excretion. However, the increasing resistance limits their therapeutic value. Aim: To study Shigella serotype distribution in the Metropolitan Region in Chile, and its relationship with severity of disease, antimicrobial resistance pattern and clonality. Material and methods: During summer 2004-2005, stool samples from children with diarrhea were collected in Cary Blair transpon medium and cultured. Shigella isolates were serotyped using monoclonal and polyclonal commercial antibodies. In vitro activity of ampicillin, amoxicillin/clavulanic acid, chloramphenicol, cotrimoxazol, nalidixic acid, ciprofloxacin, ceftriaxone and azythromycin was determined by minimal inhibitory concentration (MIC). Clonality was studied by pulsed-field gel electrophoresis (PFGE) using Xbal as restriction enzyme. Results: One hundred thirty nine Shigella strains were isolated (77 S sonnei and 62 S flexneri). S sonnei and S flexneri 2a serotypes were responsible for 95% of episodes. Children aged 2-4 years, showed a greater incidence of Shigella infections and 77% of episodes were treated on an ambulatory basis. High resistance levels were observed for ampicillin, cotrimoxazole, amoxicillin-clavulanic acid and chloramphenicol (67%, 60%, 56% and 45%, respectively). We found 11 resistance patterns and 61,2% of strains were multiresistant. There were multiple clones without a strict relationship with resistance patterns. Conclusions: Shigella infections in Metropolitan Region in Chile are associated to a restricted number of serotypes, representing a clonal expansion associated to different antimicrobial resistant patterns