Objectives. To determine and compare the prevalence of disturbed sleep in JIA and JDM and the relationship of sleep disturbance to pain, function, disease activity and medications.Methods. One hundred fifty-five patients (115 JIA, 40 JDM) were randomly sampled and were mailed questionnaires. Sleep disturbance was assessed by the sleep self-report (SSR) and the children's sleep habits questionnaire (CSHQ). Fatigue, pain and function were assessed by the paediatric quality of life inventory (PedsQL) and disease activity by visual analogue scales (VASs). Joint counts were self-reported.Results. Eighty-one per cent responded, of whom 44% reported disturbed sleep (CSHQ > 41); there were no differences between disease groups. Poor reported sleep (SSR) was highly correlated with PedsQL fatigue (r = 0.56, P < 0.0001). Fatigue was highly negatively correlated with quality of life (r = -0.77, P < 0.0001). The worst pain intensity in the last week was correlated to sleep disturbance (r = 0.32, P = 0.0005). Fatigue was associated with prednisone and DMARD use.Conclusions. Sleep disturbance and fatigue are prevalent among children with different rheumatic diseases. Sleep disturbance and fatigue are strongly associated with increased pain and decreased quality of life. Strategies aimed at improving sleep and reducing fatigue should be studied as possible ways of improving quality of life for children with rheumatic illness.

Background: Although biphasic defibrillation waveforms appear to be superior to monophasic waveforms in terminating VF, their relative benefit, in out-of-hospital resuscitation are incompletely understood. Prior comparisons of defibrillation waveform efficacy in out-of-hospital cardiac arrest (OHCA) are confined to patients presenting in a shockable rhythm and resuscitated by first responder (basic life support). This effectiveness study compared monophasic and biphasic defibrillation waveform for conversion of ventricular arrhythmias in all OHCA treated with advance life support (ALS).Methods and results: This prospective randomized controlled trial compared the rectilinear biphasic (RLB) waveform with the monophasic damped sine (MDS) waveform, using step-up energy levels. The study enrolled OHCA patients requiring at least one shock delivered by ALS providers, regardless of initial presenting rhythm. Shock success was defined as conversion at 5 s to organized rhythm after one to three escalating shocks, We report efficacy results for the cohort of patients treated by ALS paramedics who presented with an initially shockable rhythm who had not received a shock from a first responder (MDS: n = 83; RLB: n = 86). Shock success within the first three ascending energy shocks for RLB (120, 150, 200 J) was superior to MDS (200, 300, 360 J) for patients initially presenting in a shockable rhythm (52% versus 34%, p = 0.01). First shock conversion was 23% and 12%, for RLB and MDS, respectively (p = 0.07). There were no significant differences in return of spontaneous circulation (47% versus 47%), survival to 24 h (31% versus 27%), and survival to discharge (9% versus 7%). Mean 24 h survival rates of bystander witnessed events showed differences between waveforms in the early circulatory phase at 4-10 min post event (mean (S.D.) RLB 0.45 (0.07) versus MDS 0.31 (0.06), p = 0.0002) and demonstrated decline as time to first shock increased to 20 min.Conclusion: Shock success to an organized rhythm comparing step-up protocol for energy settings demonstrated the RLB waveform was superior to MDS in ALS treatment of OHCA. Survival rates for both waveforms are consistent with current theories on the circulatory and metabolic phases of out-of-hospital cardiac arrest. (c) 2005 Elsevier Ireland Ltd. All rights reserved.

OBJECTIVE:Childhood dermatomyositis (DM) is often a chronic disease, lasting many years. It has traditionally been treated with long-term corticosteroid therapy; side effects are often seen. For more than a decade, methotrexate (MTX) has been safely used for the treatment of juvenile arthritis. Here, we report use of MTX as first-line therapy for DM, along with aggressively tapered corticosteroids, in an attempt to reduce treatment-related side effects.METHODS:We studied an inception cohort of 31 children with DM who were rigorously followed up in our myositis clinic, and compared them with a control group of 22 patients with incident cases of juvenile DM who received treatment just before we instituted a policy of first-line therapy with MTX. The mean starting dosage of MTX in the study group was 15 mg/m(2)/week.RESULTS:Both groups had similar improvement in strength and physical function; however, the median time during which patients in the study group received corticosteroids was 10 months, compared with 27 months for controls (P < 0.0001). As a result, the cumulative prednisone dose in the study group was approximately half that in the control group (7,574 mg versus 15,152 mg; P = 0.0006). The study group had greater height velocity during the first year of treatment and a smaller increase in the body mass index over the first 2 years. In the control group, the relative risk of cataracts developing was 1.95 (95% confidence interval 1.05-4.17). Side effects of MTX were rarely observed.CONCLUSION:Use of MTX in conjunction with an aggressively tapered course of prednisone may be as effective as traditional long-term corticosteroid therapy for children with DM, while decreasing the cumulative dose of corticosteroids.

Objective: To provide information and recommendations for assisting women and their I physicians in making the decisions necessary to establish or exclude the presence of cancer when a lump is felt in the breast. Evidence: Guidelines are based on a systematic review of published evidence and expert opinion. References were identified through a computerized citation search using MEDLINE (from 1966) and CANCERLIT (from 1985) to January 1996. Nonsystematic review of breast cancer literature continued to January 1997. Benefits: Exclusion or confirmation of the presence of cancer with the minimum of intervention and delay. Recommendations: Investigation of women with a breast lump or suspicious change in breast texture starts with a history, physical examination and usually mammography. The clinical history should establish how long the lump has been noted, whether any change has been observed and whether there is a history of biopsy or breast cancer. Risk factors for breast cancer should be noted, but their presence or absence should not influence the decision to investigate a lump further. The physical examination of the breast should aim to identify those features that distinguish malignant from benign lumps. Mammography can often clarify the nature of the lump and detect clinically occult lesions in either breast. Fine-needle aspiration can establish whether the lump is solid or cystic. When a tumour is solid, cells can be obtained for cytologic examination. Ultrasonography is an alternative method to fine-needle aspiration for distinguishing a cyst from a solid tumour. Whenever reasonable doubt remains as to whether a lump is benign or malignant, a biopsy should be carried out. When surgical biopsy is used, the aim is to remove the whole lump in one piece along with a surrounding cuff of normal tissue. Core biopsy, either clinically or image-guided, can usually establish or exclude malignancy, thus reducing the need for surgical biopsy. Thermography and light scanning are not recommended diagnostic procedures. The value of magnetic resonance imaging is still under investigation. It is nota routine diagnostic procedure at this time. The choice of procedure should take into account the experience of the diagnostician and availability of the technology in question. The work-up should be completed expeditiously and the patient kept fully informed throughout. Even when malignancy is not found, it may be prudent, in some cases, to arrange followup surveillance. Validation: Guidelines were reviewed and revised by the Writing Committee, expert primary reviewers, secondary reviewers selected from all regions of Canada and by the Steering Committee. The final document reflects a consensus of all these contributors.

Subjects greater than or equal to 18 yr of age with serum alpha(1)-antitrypsin (alpha(1)-AT) levels less than or equal to 11 mu M or a ZZ genotype were followed for 3.5 to 7 yr with spirometry measurements every 6 to 12 mo as part of a National Heart, Lung, and Blood Institute Registry of Patients with Severe Deficiency of Alpha-1-Antitrypsin. Among all 1,129 enrollees, 5-yr mortality was 19% (95% CI: 16 to 21%). In multivariate analyses of 1,048 subjects who had been contacted greater than or equal to 6 mo after enrolling, age and baseline FEV1% predicted were significant predictors of mortality. Results also showed that those subjects receiving augmentation therapy had decreased mortality (risk ratio [RR] = 0.64, 95% CI: 0.43 to 0.94, p = 0.02) as compared with those not receiving therapy. Among 927 subjects with two or more FEV1 measurements greater than or equal to 1 yr apart, the mean FEV1 decline was 54 ml/yr, with more rapid decline in males, those aged 30 to 44 yr, current smokers, those with FEV1 35 to 79% predicted, and those who ever had a bronchodilator response. Among all subjects, FEV1 decline was not different between augmentation-therapy groups (p = 0.40). However, among subjects with a mean FEV1 35 to 49% predicted, FEV1 decline was significantly slower for subjects receiving than for those not receiving augmentation therapy (mean difference = 27 ml/yr, 95% CI: 3 to 51 ml/yr; p = 0.03). Because this was not a randomized trial, we cannot exclude the possibility that these differences may have been due to other factors for which we could not control.