Aim: To identify causes of fever, treatable diseases, and the most helpful investigations in febrile children, who had travelled to the tropics or subtropics in the preceding year. Methods: Prospective observational study of all admissions to children's wards in a district general hospital in Birmingham between January 1997 and July 1999. Children with fever >37.5°C and a history of travel to the tropics or subtropics in the preceding 12 months were included. Data were available on 153/162 children; median age was 5 years (range 0.1-15). A total of 133 (85%) children had visited South Asia; only 18/135 had received malarial prophylaxis. Median time to presentation after travel was four weeks. Children were investigated with full blood count, blood film, and stool culture. Other investigations were performed at the discretion of the admitting paediatrician. Results: Diarrhoeal illness (n = 41) and malaria (n = 22) were the most common diagnoses. A treatable cause for the febrile illness was identified in 70 (46%) children. One or more investigations were positive in 60% of children. Stool culture (17% positive) and blood film (14% positive) were the most helpful investigations. Platelet counts greater than 190 × 10 9 /l had a negative predictive value of 97% for malaria in this population. Conclusions: Children who present with fever and have travelled to the tropics or subtropics in the preceding year, often have a treatable infection. They should have a full blood count, blood film for malarial parasites, stool culture, blood culture, and chest x ray.
Epidermolysis bullosa simplex (EBS) is a blistering skin disease caused in most cases by mis-sense mutations in genes encoding the basal epidermal keratin (K) 5 and K14. The inheritance is usually autosomal dominant and the mutant keratin proteins appear to exert a dominant negative effect on the keratin intermediate filament cytoskeleton in basal keratinocytes. We report a child with a homozygous K14 mutation resulting in the complete absence of K14 protein in the epidermis; remarkably, he only had mild to moderate disease. Electron microscopy of a skin biopsy showed a marked reduction in numbers of keratin intermediate filaments in the basal keratinocytes. Immunofluorescence microscopy using monoclonal antibody LL001 against K14 showed no staining, suggesting a functional knockout of K14. Sequence analysis of genomic DNA revealed a homozygous mutation in codon 31 of K14 that resulted in a premature stop codon further downstream in exon 1. The child's mother, who is unaffected by the disease, is heterozygous for the mutation. The consanguineous father was unaffected and unavailable for testing. The resulting mRNA is predicted to encode a protein of 116 amino acids, of which the first 30 are identical to the normal K14 sequence, and the remaining 86 residues are mis-sense sequence. Four previously reported cases of autosomal recessive EBS with functional knockout of K14 were severely affected by blistering, in contrast to our patient in whom the predicted protein has only the first 30 amino acids of K14 and is therefore the closest to a true knockout of K14 protein yet identified.
BackgroundPolicies for allocating deceased donor kidneys have recently shifted from allocation based on Human Leucocyte Antigen (HLA) tissue matching in the UK and USA. Newer allocation algorithms incorporate waiting time as a primary factor, and in the UK, young adults are also favoured. However, there is little contemporary UK research on the views of stakeholders in the transplant process to inform future allocation policy. This research project aimed to address this issue.MethodsDiscrete Choice Experiment (DCE) questionnaires were used to establish priorities for kidney transplantation among different stakeholder groups in the UK. Questionnaires were targeted at patients, carers, donors / relatives of deceased donors, and healthcare professionals. Attributes considered included: waiting time; donor-recipient HLA match; whether a recipient had dependents; diseases affecting life expectancy; and diseases affecting quality of life.ResultsResponses were obtained from 908 patients (including 98 ethnic minorities); 41 carers; 48 donors / relatives of deceased donors; and 113 healthcare professionals. The patient group demonstrated statistically different preferences for every attribute (i.e. significantly different from zero) so implying that changes in given attributes affected preferences, except when prioritizing those with no rather than moderate diseases affecting quality of life. The attributes valued highly related to waiting time, tissue match, prioritizing those with dependents, and prioritizing those with moderate rather than severe diseases affecting life expectancy. Some preferences differed between healthcare professionals and patients, and ethnic minority and non-ethnic minority patients. Only non-ethnic minority patients and healthcare professionals clearly prioritized those with better tissue matches.ConclusionsOur econometric results are broadly supportive of the 2006 shift in UK transplant policy which emphasized prioritizing the young and long waiters. However, our findings suggest the need for a further review in the light of observed differences in preferences amongst ethnic minorities, and also because those with dependents may be a further priority.
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