Propranolol in the treatment of infantile haemangiomas: lessons from the European Propranolol In the Treatment of Complicated Haemangiomas (PITCH) Taskforce survey.

BACKGROUND: Oral propranolol is widely prescribed as first-line treatment for infantile haemangiomas (IHs). Anecdotally, prescribing practice differs widely between centres. OBJECTIVES: The Propranolol In the Treatment of Complicated Haemangiomas (PITCH) Taskforce was founded to establish patterns of use of propranolol in IHs. METHODS: Participating centres entered data on all of their patients who had completed treatment with oral propranolol for IHs, using an online data capture tool. RESULTS: The study cohort comprised 1097 children from 39 centres in eight European countries. 76·1% were female and 92·8% had a focal IH, with the remainder showing a segmental, multifocal or indeterminate pattern. The main indications for treatment were periocular location (29·3%), risk of cosmetic disfigurement (21·1%) and ulceration and bleeding (20·6%). In total 69·2% of patients were titrated up to a maintenance regimen, which consisted of 2 mg kg(-1) per day (85·8%) in the majority of cases. 91·4% of patients had an excellent or good response to treatment. Rebound growth occurred in 14·1% upon stopping, of whom 53·9% were restarted and treatment response was recaptured in 91·6% of cases. While there was no significant difference in the treatment response, comparing a daily maintenance dose of < 2 mg kg(-1) vs. 2 mg kg(-1) vs. > 2 mg kg(-1) , the risk of adverse events was significantly higher: odds ratio (OR) 1 vs. adjusted OR 0·70, 95% confidence interval (CI) 0·33-1·50, P = 0·36 vs. OR 2·38, 95% CI 1·04-5·46, P = 0·04, Ptrend < 0·001. CONCLUSIONS: The PITCH survey summarizes the use of oral propranolol across 39 European centres, in a variety of IH phases, and could be used to inform treatment guidelines and the design of an interventional study.

How useful are structured electronic portfolio templates to encourage reflective practice?

BACKGROUND: Some specialties in the U.K. use structured templates in electronic (e) Portfolios to encourage reflective practice. This study looked at completion of an 11-field template by U.K. Paediatric specialty trainees. METHODS: A reflective ePortfolio log from all paediatric specialty trainees in one large U.K. deanery was assessed by two medical educators. The consultant supervisors' opinion of the trainee's standard of reflective practice and outcome of annual review of competence progression was noted. RESULTS: If the 115 trainees, 10 had no reflective logs and 105 had reflective logs ranging in number from 1 to -18 (mean of 5). The structured template was poorly completed by trainees especially sections on what could be done differently and outcomes for the trainee, parents and others. Discrepancy between the evidence of reflection in ePortfolio and trainers assessment of reflective practice was noted. CONCLUSION: An 11-field structured template for reflective practice was not completed well. We suggest four fields as a maximum so as to enable trainees to reflect and note their personal key learning points. There needs to be an emphasis on quality rather than quantity of ePortfolio reflective logs, both in number and length of log aiming for 1-2 well completed reflections per post.

Reliability of multi-station interviews in selection of junior doctors for specialty training.

BACKGROUND: Interviews for junior doctor training posts in the UK are considered high stakes assessments following the introduction of Modernizing Medical Careers. AIMS: To examine the reliability of paediatric recruitment in our Deanery for specialty training (ST) in 2008. METHODS: A total of 225 candidates were interviewed for 88 posts. There were three interview stations (presentation, structured interview and communication) each lasting for 10 min. Two interviewers independently marked candidates at each station on a 5 point Likert scale. Interview scores were analysed to evaluate reliability. RESULTS: Reliability for ST1 interviews was excellent, reasonable for ST2 and ST3 and poor for ST4 (Cronbach's alpha of 0.908, 0.749, 0.759 and 0.648). Generalizability coefficient was good for ST1 interviews (>0.8) but for ST2-ST4 interviews was below acceptable (<0.8). Decision (D) study calculations showed up to 26 stations that were required to achieve minimum reliability. CONCLUSIONS: This three station interview process was not reliable enough for a high stakes assessment. An increased number of stations are required to improve reliability. Further work is required to clarify the optimum number of stations to achieve minimum reliability and feasibility.

Specialist registrars' views on their teaching role.

This study looks at whether UK specialist registrars (SpRs) are involved in teaching and meeting their educational obligations. A questionnaire was distributed to all 133 paediatric SpRs in the region. 92% responded (122/133). All SpRs reported doing some teaching with nearly all teaching junior trainees and students. During daytime hours, 41% of trainees taught for 1 h per week but half this time at night with 21% reporting doing no teaching out of hours. 55% had formal educational training most commonly a "Teach the Teacher" course. Developing SpRs as educators was felt to be best achieved through mandatory training (61%, 74) and themed regional SpR training days (61%, 74). SpRs are making an important contribution to education. Teaching competences should be included in the personal development plan of all trainees and rostered time allocated to SpRs specifically to teach.

Allogeneic bone marrow transplantation in a 7-year-old girl with congenital erythropoietic porphyria: a treatment dilemma.

Congenital erythropoietic porphyria (CEP, Günther's disease) has a very variable phenotype. In the more severely affected, bone marrow transplantation (BMT) is potentially curative, but is not without risks. We describe a 7-year-old girl with CEP characterized by severe photosensitivity but only mild anaemia, in whom the difficult decision to proceed with allogeneic BMT was made after discussion in a multidisciplinary team. She has shown successful engraftment, accompanied by biochemical and clinical resolution of her metabolic disease. She remains well 3 years later, the oldest patient with CEP receiving BMT to survive beyond 12 months. However, she has experienced significant morbidity including florid cutaneous graft-versus-host disease with postinflammatory hypopigmentation. Her case is important in highlighting the delay in diagnosis not uncommon in this condition and the complex decision-making process involved in proceeding with BMT.

Problem based learning in a junior doctor teaching programme.

BACKGROUND: Problem based learning (PBL) is used increasingly in undergraduate medical education, but there are few postgraduate medical studies. AIM: To compare SHO learning outcomes for a PBL course with a traditional didactic course. METHODS: As part of their protected teaching programme, 14 senior house officers (SHOs) were taught about paediatric dermatology using a traditional didactic course. Six months later, the new SHOs received a PBL course including small group teaching and a study guide. Both the traditional and the PBL group were assessed using multiple choice questions (MCQs), an objective structured clinical examination (OSCE), and pre- and post-course self-assessment sheets. SHOs completed course evaluation sheets. RESULTS: There was no significant difference in learning outcome between the traditional and PBL courses as assessed by the MCQs, OSCE, and self-assessment sheets. The PBL course was well appreciated by SHOs who liked variety in the teaching programme. CONCLUSIONS: The PBL and traditional course had equivalent learning outcomes. PBL adds variety to junior doctor protected teaching programmes and can be a useful tool for doctors working shift patterns.

Improving hospital doctors' working lives: online questionnaire survey of all grades.

BACKGROUND: In 2001, the Department of Health produced the Improving Working Lives (IWL) for Doctors document. This is the first national survey which asks hospital doctors what changes are needed to improve their working lives. METHODS: An online questionnaire was run over a period of six weeks and was open to all doctors of all grades. Doctors were asked to choose their top five factors from a list of 35 diverse choices or to provide alternatives in free text. Demographic data were also collected. RESULTS: 1603 hospital doctors working in the UK completed the online questionnaire. Improved secretarial or managerial support was the first IWL choice for consultants, with different aspects of clinical and non-clinical support representing their top four choices. Junior hospital doctors and staff and associate specialist grades (staff grades, associate specialists, and clinical assistants) identified improved support for education and training as their first choice, while among the female specialist registrars, it was improved support for childcare. Greater opportunities to develop new skills was an important issue for doctors in the surgical specialties and improved access to mentoring was important for all junior doctors, staff and associate specialist grades, and doctors from black and ethnic minority groups. CONCLUSIONS: Hospital doctors in the UK need more support to improve their working lives. The principle needs are better secretarial and managerial support for consultants; education, training, and mentoring for junior doctors and staff and associate specialist grades; and improved opportunities to develop new skills for those in surgical specialties. Support with childcare is an important issue for female specialist registrars. The Department of Health, NHS trusts, deaneries, and Royal Colleges need to endorse policies that promote a training and working environment that will improve working lives for all hospital doctors, ensuring that appropriate and continuing support is available from the time doctors enter the new foundation programmes and proposed run-through grades, to their time spent as consultants in today's NHS.

A keratin 14 'knockout' mutation in recessive epidermolysis bullosa simplex resulting in less severe disease.

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.

Cyclosporine in severe childhood atopic dermatitis: a multicenter study.

BACKGROUND: Severe atopic dermatitis (AD) remains difficult to treat. Cyclosporine is effective in adults but has not previously been investigated in children with AD. OBJECTIVE: The aims were to investigate the efficacy, safety, and tolerability of cyclosporine in severe refractory childhood AD. METHODS: Subjects 2 to 16 years of age were treated for 6 weeks with cyclosporine, 5 mg/kg per day, in an open study. Disease activity was monitored every 2 weeks by means of sign scores, visual analogue scales for symptoms, and quality-of-life questionnaires. Adverse events were monitored. Efficacy and tolerability were assessed with five-point scales. RESULTS: Twenty-seven children were treated. Significant improvements were seen in all measures of disease activity. Twenty-two showed marked improvement or total clearing. Quality of life improved for both the children and their families. Tolerability was considered good or very good in 25 subjects. CONCLUSION: Cyclosporine may offer an effective, safe, and well-tolerated short-term treatment option for children with severe AD.

Growth of herpes simplex type 1 on skin explants of atopic eczema.

In a novel approach to looking at why some children with atopic eczema are susceptible to cutaneous herpes simplex virus (HSV) infections, this study evaluates the hypothesis that HSV replicates more easily on eczematous than normal skin. Growth of HSV on eczematous skin explants was compared with growth on explants from three control groups (psoriasis, Darier's disease and normal skin) over a 2-day period. Growth of HSV was significantly less on normal skin than in atopic eczema, psoriasis and Darier's disease. Virus replicated more quickly, and grew to higher titre within 24h, in eczematous and psoriatic explants than in normal skin. A defect in skin barrier function and host defence factors including local cytokine secretion are discussed as possible mechanisms in causing the increased susceptibility of children with atopic eczema to HSV infection.

Immunological studies of herpes simplex virus infection in children with atopic eczema.

This study examines the role of immune defence mechanisms in herpes simplex virus (HSV) infections in atopic eczema and whether impairment of these mechanisms explains the susceptibility of some children with atopic eczema to cutaneous HSV infections. Ten children with eczema herpeticum and 13 with atopic eczema and recurrent HSV infection affecting multiple skin sites were studied, together with relevant control groups. In all children with atopic eczema, in vitro lymphoproliferation in response to stimulation with concanavalin A (Con A) was significantly decreased and natural killer (NK) cells (CD16 + 56) were reduced compared with non-atopic controls. IL-2 receptors, a marker for lymphocyte activation, were decreased during the acute phase of eczema herpeticum, and for 1 month thereafter. A positive stimulation index (> 3) to HSV antigen, and high HSV IgG antibody titres measured by ELISA, Western blotting and neutralization assay, were seen in children with eczema herpeticum by 6 weeks, and also in children with atopic eczema and recurrent HSV infections. No evidence of an HSV-specific immune defect (either cell-mediated or humoral) was found in atopic eczema. Impairment of cell-mediated immunity in atopic eczema was suggested by the reduced response to Con A. It is likely that reduced numbers of circulating NK cells and a decrease in IL-2 receptors during early eczema herpeticum contribute to the susceptibility of children with atopic eczema to cutaneous HSV infections.

Can admission notes be improved by using preprinted assessment sheets?

Inpatient medical notes often fail to record important details of patient history and findings on clinical examination. To overcome problems with content and legibility of notes we introduced preprinted notes for the admission of children to this hospital. The quality of the information recorded for 100 children whose admissions were clerked with the preprinted notes was compared with that recorded for 100 whose admissions were recorded with the traditional notes. All case notes were selected randomly and retrospectively from traditional notes written from April to October 1993 and from preprinted notes written from October 1993 to April 1994. The quality of information was assessed according to the presence or absence of 25 agreed core clinical details and the number of words per clerking. In admissions recorded with the preprinted notes the mean number of core clinical details present was significantly higher than those recorded with traditional notes (24.0 v 17.6, p < 0.00001). Admissions recorded with the preprinted notes were also significantly shorter (mean 144 words v 184 words, p < 0.0001). The authors conclude that information about children admitted to hospital is both more complete and more succinct when recorded using preprinted admission sheets.

Rapid diagnosis of cutaneous herpes simplex infections using specific monoclonal antibodies.

One hundred children with suspected herpes simplex virus (HSV) infection and 20 controls were studied to compare a rapid immunofluorescence (RIF) test for detection and typing of HSV from smears of lesions with standard viral culture. The RIF test was evaluated for ease of use and speed of diagnosis. RIF and/or culture were positive in 64% of patients. All infections diagnosed by RIF and culture were HSV type 1. In 92% of patients RIF and culture results were in concordance. In 57 cases, RIF and cultures were positive for HSV infection and in 35 cases RIF and cultures were negative for HSV infection. Three patients had inadequate samples for RIF and five children had positive RIF but were culture negative. All controls had negative results both by RIF test and culture. The RIF test demonstrated 100% sensitivity and 95% specificity. The RIF test was type specific, easy to perform and gave diagnosis of HSV infections within an hour of taking the clinical specimen. This study suggests the RIF test is as good, if not more sensitive, in the diagnosis of HSV infections as standard viral culture and has the advantage of speed of diagnosis.