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Biomedical subjects

Jane Davies

Publications and source records attributed to Jane Davies.

13 recordsLinked to original sources

Beta-defensin genomic copy number is not a modifier locus for cystic fibrosis.

Human beta-defensin 2 (DEFB4, also known as DEFB2 or hBD-2) is a salt-sensitive antimicrobial protein that is expressed in lung epithelia. Previous work has shown that it is encoded in a cluster of beta-defensin genes at 8p23.1, which varies in copy number between 2 and 12 in different individuals. We determined the copy number of this locus in 355 patients with cystic fibrosis (CF), and tested for correlation between beta-defensin cluster genomic copy number and lung disease associated with CF. No significant association was found.

Alleles↗

Voriconazole therapy in children with cystic fibrosis.

BACKGROUND: There is increasing evidence for the efficacy of the antifungal voriconazole, particularly in immunosuppression. We describe our experience of using voriconazole in children with CF. METHODS: We performed a retrospective case note review of children with CF treated with voriconazole in a single centre over an 18 month period. RESULTS: A total of 21 children aged 5 to 16 years (median 11.3) received voriconazole for between 1 and 50 (22) weeks. Voriconazole was used as monotherapy in 2 children with recurrent allergic bronchopulmonary aspergillosis (ABPA); significant and sustained improvements in clinical and serological parameters for up to 13 months were observed, without recourse to oral steroids. Voriconazole was used in combination with an immunomodulatory agent in a further 11 children with ABPA, with significant improvement in pulmonary function and serology. 8 children without ABPA but who had recurrent Aspergillus fumigatus isolates and increased symptoms also received voriconazole; this group did not improve with treatment. Adverse effects occurred in 7 children (33%: photosensitivity reaction 3, nausea 2, rise in hepatic enzymes 1, hair loss 1). CONCLUSIONS: Voriconazole may be a useful adjunctive therapy for ABPA in CF. Voriconazole monotherapy appears to be an alternative treatment strategy when oral corticosteroids may not be suitable.

Adolescent↗

Early detection of lung disease in preschool children with cystic fibrosis.

PURPOSE OF REVIEW: Babies with cystic fibrosis are born with essentially normal lungs but rapidly develop inflammation and infection. Evaluation is generally based on history and physical examination until school age, when children become able to perform spirometry. In these 'silent years', however, unrecognized lung damage can occur. Recently, techniques to improve the evaluation of preschool children have been developed, and an assessment of where they fit into clinical and research practice is now needed. This is particularly urgent to select groups of children for new, phenotype-specific therapies, and monitor their effects. RECENT FINDINGS: The techniques in which there have been major recent advances include physiologic methods, particularly indices of gas mixing; imaging techniques, especially high-resolution computed tomographic scanning; and bronchoscopic studies. The clinical roles of these techniques need to be explored, as do comparisons between techniques. SUMMARY: The preschool years need no longer be silent. Some simple techniques such as spirometry can be performed in much younger children than conventionally believed to be practical. Others currently require sophisticated apparatus (lung clearance index) or carry potential risks (radiation, high-resolution computed tomographic scanning). We need good long-term, prospective comparisons of these techniques, with measurement of inflammatory markers. We also need to know which are sufficiently reproducible, and indicative of prognosis, for use as endpoints in clinical trials.

Bronchoscopy↗

The teaching of clinical skills in the context of children's nursing: a UK survey.

The teaching of clinical skills within nursing is currently enjoying a resurgence following the implementation of the 'Fitness for Practice Curriculum'. However, the teaching of clinical skills specifically within children's nursing has received little attention within the research arena. This article presents the data of a national postal survey sent to United Kingdom institutions providing pre-registration child branch studies, to ascertain the current provision of clinical skills teaching. The findings conclude that very few institutions have invested in specific skills laboratories to teach children's nursing students. It was also identified that a wide range of clinical skills are taught to child branch students; however, the resources and realism to clinical practice is limited by the lack of specialist children's skills laboratories or equipment. In conclusion the authors recommend the development of child specific skills laboratories, to augment child branch skills teaching, in order to enhance the realistic simulation of clinical practice.

Benchmarking↗

Cystic fibrosis modifier genes.

Since the recognition that CFTR genotype was not a good predictor of pulmonary disease severity in CF, several candidate modifier genes have been identified. It is unlikely that a single modifier gene will be found, but more probable that several haplotypes in combination may contribute, which in itself presents a major methodological challenge. The aims of such studies are to increase our understanding of disease pathogenesis, to aid prognosis and ultimately to lead to the development of novel treatments.

Bacterial Infections↗

Research applications of bronchoscopy.

The technique of flexible bronchoscopy creates unique research opportunities to investigate the underlying mechanisms of respiratory disease in children, by sampling directly from the airway. Bronchoscopy is, however, invasive and the ethics of performing this type of procedure for research purposes must be considered carefully at the outset. This article discusses the research applications of bronchoscopy in children, focusing on the ethical and safety issues, the types of procedure that can be performed and their application to the investigation of specific diseases. Acceptance of the use of bronchoscopy as a research tool is increasing. Future work should focus on the comparison of invasive and non-invasive techniques, the use of bronchoscopy in longitudinal and interventional studies and the development of new, highly sensitive techniques to allow us to study disease pathogenesis in greater detail. Collaboration between centres should be encouraged to maximise the use of samples obtained.

Bronchoscopy↗

Development and organization of child and adolescent mental health services.

Against the backdrop of involving children and families in their own care and the clear need to protect their interest, this article will consider care within child and adolescent mental health services (CAMHS). Following a brief overview of the incidence and prevalence of mental health problems, the development and organization of CAMHS will be explored, giving consideration to some of the literature that discusses the effectiveness of services. A review of the literature revealed that, while there is some evidence of how children feel about the services they receive, there is not currently a significant amount of literature available. Nevertheless, these views are of value in relation to the development of service provision.

Adolescent↗