Ultraviolet radiation exposure to the face in patients with xeroderma pigmentosum and healthy controls: applying a novel methodology to define photoprotection behaviour.

BACKGROUND: In xeroderma pigmentosum (XP), the main means of preventing skin and eye cancers is extreme protection against ultraviolet radiation (UVR). Protection is most important for the face. OBJECTIVES: We aimed to assess how well patients with XP adhere to medical advice to protect against UVR by objectively estimating the mean daily dose of UVR to the face. METHODS: We objectively estimated the UVR dose to the face in 36 patients with XP and 25 healthy individuals over 3 weeks in the summer. We used a new methodology which combined UVR dose measurements from a wrist-worn dosimeter with an activity diary record of face photoprotection behaviour for each 15-min period spent outside. A protection factor was associated with each behaviour, and the data were analysed using a negative binomial mixed-effects model. RESULTS: The mean daily UVR dose (weighted for DNA damage capacity) to the face in the patients with XP was 0·13 standard erythemal doses (SEDs) (mean in healthy individuals = 0·51 SED). There was wide variation between patients (range < 0·01-0·48 SED/day). Self-caring adult patients had a very similar UVR dose to the face as cared-for patients (0·13 vs. 0·12 SED/day), despite photoprotecting much more poorly when outside, because the self-caring adults were outside in daylight much less. CONCLUSIONS: Photoprotection behaviour varies widely within the XP group indicating that nonadherence to photoprotection advice is a significant issue. The timing and duration of going outside are as important as photoprotective measures taken when outside, to determine the UVR exposure to the face. This new methodology will be of value in identifying the sources of UVR exposure in other conditions in which facial UVR exposure is a key outcome, particularly in patients with multiple nonmelanoma skin cancers.

Construction and validation of a quality of life questionnaire for neuromuscular disease (INQoL).

BACKGROUND: Because there is no muscle disease specific measure of quality of life (QoL), we wanted to develop and validate an individualized muscle disease specific measure of QoL for adults suitable for both clinical and research use. METHODS: A literature review exploring QoL and its measurement resulted in the development of a theoretical model of QoL. This was used alongside qualitative interviews (n = 41) and a postal survey (n = 252) to design a questionnaire. The psychometric properties, validity (n = 95), reliability (n = 40), and responsiveness (n = 25) of the scale were assessed. RESULTS: The Individualized Neuromuscular Quality of Life questionnaire (INQoL) consists of 45 questions within 10 sections. Four of these focus on the impact of key muscle disease symptoms (weakness, locking [i.e., myotonia], pain, and fatigue), five look at the impact (degree and importance of impact) muscle disease has on particular areas of life, and one section asks about the positive and negative effects of treatment. The questionnaire is structured to allow for variations in the individual characteristics that influence quality of life. Psychometric evaluation established construct validity and test-retest reliability. A preliminary assessment of responsiveness was obtained. CONCLUSIONS: The Individualized Neuromuscular Quality of Life is a validated muscle disease specific measure of quality of life developed from the experiences of patients with muscle disease and can be used for individuals or large samples.

Systematic review of patient and nurse attitudes to depot antipsychotic medication.

BACKGROUND: Depot antipsychotic medication is used widely in the treatment and prophylaxis of severe mental illness. AIMS: To review the literature on patient and nurse satisfaction with, and attitudes towards, depots. METHOD: A systematic search of Medline, Embase, PsycINFO, CINAHL and The Cochrane Library was undertaken, along with citation searches. Studies were selected if satisfaction/attitude data were described in the title or abstract and original data were included. Study quality was rated. RESULTS: The search produced 1374 articles; 22 articles met the inclusion criteria, 18 of which were cross-sectional surveys. Of the 12 studies with relevant data, 10 conveyed a positive opinion of depot medication. Five out of six studies comparing depot with oral medication showed patient preference for depot. CONCLUSIONS: High-quality data examining patient and nurse attitudes regarding depot antipsychotics are sparse. What data there are show a positive attitude to depots from patients. Future randomised controlled trials should include satisfaction as an outcome.

Clinical perspectives in primary nocturnal enuresis.

Primary nocturnal enuresis (PNE) is prevalent among the pediatric population, but not all professionals are aware of the current research regarding the etiology and treatment of this disorder. This paper presents a broad overview of PNE, including etiology and evaluation, with a specific emphasis on treatment issues. The most current treatments (imipramine, desmopressin acetate arginine vasopressin, enuresis alarms) are discussed, including recent research on their effectiveness. In considering the recent data on long-term efficacy, overall cost, and safety, the treatment of choice appears to be the enuresis alarm for those families who are capable of following protocols. Desmopressin acetate arginine vasopressin is a safe alternative that has the advantage of quick response and ease of administration.

Body dysmorphic disorder: a cognitive behavioural model and pilot randomised controlled trial.

A cognitive behavioural model of body image is presented with specific reference to body dysmorphic disorder (BDD). We make specific hypotheses from the model for testing BDD patients in comparison with: (i) patients with "real" disfigurements who seek cosmetic surgery; (ii) subjects with "real" disfigurements who are emotionally well adjusted; and (iii) healthy controls without any defect. There have been no randomised controlled trials of treatment for BDD and therefore the model has clear implications for the development of cognitive behavioural therapy. This was evaluated in a pilot controlled trial. Nineteen patients were randomly allocated to either cognitive behaviour therapy or a waiting list control group over 12 weeks. There were no significant pre-post differences on any of the measures in the waiting list group. There were significant changes in the treated group on specific measures of BDD and depressed mood. Cognitive behaviour therapy should be further evaluated in a larger controlled trial in comparison with another psychological treatment such as interpersonal therapy and pharmacotherapy.

Body dysmorphic disorder. A survey of fifty cases.

BACKGROUND: Body dysmorphic disorder (BDD) consists of a preoccupation with an 'imagined' defect in appearance which causes significant distress or impairment in functioning. There has been little previous research into BDD. This study replicates a survey from the USA in a UK population and evaluates specific measures of BDD. METHOD: Cross-sectional interview survey of 50 patients who satisfied DSM-IV criteria for BDD as their primary disorder. RESULTS: The average age at onset was late adolescence and a large proportion of patients were either single or divorced. Three-quarters of the sample were female. There was a high degree of comorbidity with the most common additional Axis l diagnosis being either a mood disorder (26%), social phobia (16%) or obsessive-compulsive disorder (6%). Twenty-four per cent had made a suicide attempt in the past. Personality disorders were present in 72% of patients, the most common being paranoid, avoidant and obsessive-compulsive. CONCLUSIONS: BDD patients had a high associated comorbidity and previous suicide attempts. BDD is a chronic handicapping disorder and patients are not being adequately identified or treated by health professionals.

Sucrose as an analgesic agent for infants during immunization injections.

OBJECTIVE: To assess the effectiveness of sucrose as an analgesic agent during routine immunization injections for infants (age range, 2 weeks to 18 months). STUDY DESIGN: Double-blind, randomized control trial. SETTING: Ambulatory care clinic of a large tertiary care center. PARTICIPANTS: A consecutive sample of 285 infants were randomly assigned to one of three treatment groups. INTERVENTION: Subjects received either no intervention or drank 2 mL of sterile water or 2 mL or a 12% sucrose solution 2 minutes before administration of the immunization. MAIN OUTCOME MEASURE: Infants were videotaped during immunization for later interval recording of pain-induced vocalizations. RESULTS: Results were analyzed by using two-way repeated measure analyses of variance. Two-week-old infants who received either the sterile water or sucrose solution cried significantly less than infants who received no intervention (F=5.92,P<.005). For older infants, those who received water or sucrose cried significantly less only if they were administered one injection rather than two injections (F=3.36,P<.05). CONCLUSIONS: We found that when infants drank sucrose or sterile water, significantly fewer pain vocalizations were produced, but only for 2-week-old infants. For older infants, differences were found only when the number of injections was included in the analysis. We expand on previous findings by demonstrating that both the age of the child and the number of painful exposures can attenuate calming effects. In addition, the results suggest that in the absence of nonnutritive sucking, the actual analgesic effects of sucrose may be nonspecific. Further study is needed of the possible analgesic effects of sucrose.

Enhancing refusal skills: identifying contexts that place adolescents at risk for unwanted sexual activity.

Refusal skills training is one approach to reducing school-age pregnancy and sexually transmitted disease. The generalization of these skills is dependent, in part, on the relevance to participants of the training scenarios. We identified and cross-validated relevant contexts for inclusion in scenarios for refusal skills training. Fifty-six sexually active female adolescents identified 59 contexts associated with unwanted sexual activity. Fifty-one additional subjects rated these on 9-point Likert scales according to how common and difficult each was for them. Unsupervised activity where alcohol is present or feeling an obligation to engage in sexual activity emerged as high risk contexts. The most common and difficult contexts provide points of departure for the development of relevant training scenarios for refusal skills training, thereby contributing to generalization of these skills and reducing the risk of unwanted sexual activity within this population.

Pharmacists' poison prevention advice to the elderly.

Up to 36% of childhood ingestions take place in grandparents' homes. We surveyed the 1544 registered pharmacists in the state of Nebraska by a mailed anonymous questionnaire concerning their poison prevention practices with the elderly. Of the 26% respondents, 75% always or usually gave older adults a choice of child-resistant containers but estimated that 65% chose non-child-resistant containers. Fifty-two percent reported that they asked older adults about children who could possibly ingest medications, and 59% reported that they had poison prevention material available. However, over 50% of pharmacists reported that they neither actively counseled nor handed out poison prevention material to the elderly, and only 9% reported that they specifically advise older adults about poison prevention. If all pharmacists targeted poison control education to the elderly, childhood poisoning by drugs could be reduced by one-third.

Nebraska family practitioners' infant feeding recommendations.

The authors conducted an anonymous survey of 220 Nebraska family and general practitioners' attitudes and practice recommendations for infant feeding. Most practitioners' attitudes reflect published American Academy of Pediatrics (AAP) guidelines regarding using commercial formula for bottle-feeding babies rather than evaporated milk-based formulations. Ninety-two per cent agreed with recommendations relating to the need for fluoride supplementation when fluoride was unavailable in the water supply and 93% agreed that whole cow's milk was inappropriate in the infant's first year. Another 68% felt similarly about evaporated milk formulas. However, 32% of board certified and 53% of nonboard certified physicians continue to believe that early solid foods will reduce night waking. In 80% of the cases, practice recommendations disagreed with AAP guidelines by prescribing vitamin supplements for bottle-feeding babies receiving proprietary infant formulas. Additionally, two-thirds of the practitioners recommended unnecessary water complements and 62% suggested formula supplementation for breastfeeding babies. Solid foods and whole cow's milk for both breastfeeding and bottle-feeding babies often were recommended earlier than the second half of the first year.

Assessment of urinary protein excretion in the adolescent: effect of body position and exercise.

We examined the effects of body position and exercise on the random urinary protein/creatinine ratio (Up/Ucr) in healthy adolescents, to provide reference data to be used in a simplified evaluation of proteinuria. Random urine samples were obtained during recumbency and after both ambulation and exercise in 116 subjects. The Up/Ucr was significantly (P less than 0.0001) higher with increasing levels of activity, and was widely variable, especially during the upright and postexercise periods. No sex-related differences in Up/Ucr were noted, except during recumbency, when values were significantly (P less than 0.001) higher in females. The urinary dipstick was found to be less sensitive as a tool to define abnormal degrees of recumbent proteinuria. We conclude that body position and exercise have significant effects on protein excretion, and suggest that the Up/Ucr in recumbent and upright urine samples will be useful in the evaluation of proteinuria.