Drug-induced linear immunoglobulin A bullous dermatosis mimicking Stevens-Johnson syndrome: a case report.

Linear immunoglobulin A (IgA) bullous dermatosis (LABD) is a rare autoimmune disorder characterized by vesiculobullous mucocutaneous eruptions. LABD also has been reported as a drug-induced reaction. Idiopathic LABD and drug-induced LABD are clinically indistinguishable and can resemble bullous pemphigoid, dermatitis herpetiformis, or bullous erythema multiforme. LABD is diagnosed with direct immunofluorescence (DIF), and idiopathic LABD can be distinguished from drug-induced LABD with a careful medication history. We present the case of a 54-year-old man with drug-induced LABD after ingestion of rimantadine, zanamivir, and azithromycin for presumed influenza. The patient's bullous eruption resolved with discontinuation of the offending medications and treatment with prednisone and pentoxifylline.

Exploring family systems nursing and the community children's nurse's role in caring for children with cystic fibrosis.

Family systems nursing (FSN) theory highlights the potential that exists for the community children's nurse (CCN) to provide active support to families. Knowledge of FSN theory can inform practice in caring for the child and family challenged by cystic fibrosis. FSN theory offers a logical extension to the CCN's role working with families to facilitate and promote the health and well-being of the whole family. Skilled provision of a long-term supportive relationship with families requires appropriate educational preparation and on-going emotional support.

An exploration of best practice in multi-agency working and the experiences of families of children with complex health needs. What works well and what needs to be done to improve practice for the future?

AIMS AND OBJECTIVES: This Appreciative Inquiry study aimed to explore appreciatively examples of best multi-agency working practice with families (mothers, n = 20; fathers, n = 7; children, n = 1) and people working with children with complex needs (n = 41), to determine what works well, why it has worked well and what best practice in the future could be. BACKGROUND: The term 'children with complex health needs' encompasses a diverse group of children and this population is increasing. This diverse group of children often requires high levels of physiological, psychological and social care which brings them and their families into therapeutic contact with a wide range of health, social and education professionals and people from other agencies. DESIGN: The study used appreciative interviews, nominal group workshops and consensus workshops to develop a set of 10 'best practice' guidelines that reflected the views of all participants. Two of these are discussed in detail in this article. All participants were seen as co-researchers whose expert contributions were vital to understanding of what works well and what needs to be done in multi-agency working practice. RESULTS: The study resulted in 'best practice' statements that illuminated 'what works well' in multi-agency working practice that spanned issues including information, decision making, communication, accessibility, collaboration, respect and sharing a common vision. CONCLUSIONS: The guidance that results from this study suggests that parents need the opportunity to share and receive support from other parents who understand the lived reality of caring for a child with complex needs. Parents and people from across various agencies need to work together to ensure that the most appropriate person acts in the role of a long-term coordinator, where the family wants this aspect of support. This study adds a multi-disciplinary and appreciatively oriented focus on what works well in complex care. It contributes to an understanding of the value of an Appreciative Inquiry approach within health-care research. RELEVANCE TO CLINICAL PRACTICE: The guidelines arose from and are grounded in practice and as such they provide clear, workable directions for enhancing practice and for considering what already does work well.

Stabilities of folding of clustered, two-repeat fragments of spectrin reveal a potential hinge in the human erythroid spectrin tetramer.

The large size of spectrin, the flexible protein promoting reversible deformation of red cells, has been an obstacle to elucidating the molecular mechanism of its function. By studying cloned fragments of the repeating unit domain, we have found a correspondence between positions of selected spectrin repeats in a tetramer with their stabilities of folding. Six fragments consisting of two spectrin repeats were selected for study primarily on the basis of the predicted secondary structures of their linker regions. Fragments with a putatively helical linker were more stable to urea- and heat-induced unfolding than those with a putatively nonhelical linker. Two of the less stably folded fragments, human erythroid alpha-spectrin repeats 13 and 14 (HEalpha13,14) and human erythroid beta-spectrin repeats 8 and 9 (HEbeta8,9), are located opposite each other on antiparallel spectrin dimers. At least partial unfolding of these repeats under physiological conditions indicates that they may serve as a hinge. Also less stably folded, the fragment of human erythroid alpha-spectrin repeats 4 and 5 (HEalpha4,5) lies opposite the site of interaction between the partial repeats at the C- and N-terminal ends of beta- and alpha-spectrin, respectively, on the opposing dimer. More stably folded fragments, human erythroid alpha-spectrin repeats 1 and 2 (HEalpha1,2) and human erythroid alpha-spectrin repeats 2 and 3 (HEalpha2,3), lie nearly opposite each other on antiparallel spectrin dimers of a tetramer. These clusterings along the spectrin tetramer of repeats with similar stabilities of folding may have relevance for spectrin function, particularly for its well known flexibility.