Late diagnosis of ectodermal dysplasia syndrome.

This case study reports the clinical, skin biopsy and molecular findings in a 56-year-old Filipino man with the autosomal recessive ectodermal dysplasia disorder, Schöpf-Schulz-Passarge syndrome, the precise nature of which was established only after reading of a similar case in this journal. In addition to the late diagnosis, successful clinical management of his acral hyperkeratosis and ulceration has been difficult, with oral retinoids exacerbating the skin fragility.

Injectable vaccines for preventing pneumococcal infection in patients with chronic obstructive pulmonary disease.

BACKGROUND: As chronic obstructive pulmonary disease (COPD) progresses, exacerbations can occur with increasing frequency. One goal of therapy is to prevent these exacerbations, thereby reducing morbidity and associated healthcare costs. Pneumococcal vaccinations are one strategy for reducing the risk of infective exacerbations. OBJECTIVES: To determine the safety and efficacy of pneumococcal vaccination in COPD. The primary outcomes assessed were episodes of pneumonia and acute exacerbations. Secondary outcomes of interest included hospital admissions, adverse events related to treatment, disability, change in lung function, mortality, and cost effectiveness. SEARCH STRATEGY: We searched the Cochrane Airways Group COPD trials register and the databases CENTRAL, MEDLINE and EMBASE using pre-specified terms. The latest searches were performed in March 2010. SELECTION CRITERIA: Randomised controlled trials assessing the effects of injectable pneumococcal vaccine in people with COPD were included. DATA COLLECTION AND ANALYSIS: Two review authors independently extracted data and three review authors independently assessed trial quality. MAIN RESULTS: Seven studies were identified that met the inclusion criteria for this review and were included in the 2010 review update. Two older trials used a 14-valent vaccine and five more recent trials used a 23-valent injectable vaccine.In six studies involving 1372 people, the reduction in likelihood of developing pneumonia with pneumococcal vaccination compared to control did not achieve statistical significance, the odds ratio (OR) was 0.72 (95% confidence interval (CI) 0.51 to 1.01), with moderate heterogeneity present between studies. The reduction in likelihood of acute exacerbations of COPD from two studies involving 216 people was not statistically significant (Peto OR 0.58; 95% CI 0.30 to 1.13).Of the secondary outcomes for which data were available there was no statistically significant effect for reduction in hospital admissions (two studies) or emergency department visits (one study). There was no significant reduction in pooled results from three studies involving 888 people for odds of all-cause mortality for periods up to 48 months post-vaccination (OR 0.94; 95% CI 0.67 to 1.33), or for death from cardiorespiratory causes (OR 1.07; 95% CI 0.69 to 1.66). AUTHORS' CONCLUSIONS: The limited evidence from randomised controlled trials (RCTs) included in this review suggests that, while it is possible that injectable polyvalent pneumococcal vaccines may provide some protection against morbidity in persons with COPD, no significant effect on any of the outcomes was shown. Further large RCTs in this population would be needed to confirm effectiveness of the vaccine suggested by results from longitudinal studies.

Prevalence of risk factors for non-communicable diseases in the Mekong Delta, Vietnam: results from a STEPS survey.

BACKGROUND: Despite the increasing burden of non-communicable diseases (NCD) in Vietnam, information on the prevalence of preventable risk factors for NCD is restricted to the main urban centres of Ha Noi, and Ho Chi Minh City (HCMC). This population-based survey aimed to describe the prevalence of risk factors for NCD in a rural Vietnamese sample. METHODS: This survey was conducted using the WHO "STEPwise approach to surveillance of non-communicable diseases" (STEPS) methodology. Participants (n=1978) were residents of the Mekong Delta region selected by multi-stage sampling. Standardised international protocols were used to measure behavioural risk factors (smoking, alcohol consumption, fruit and vegetable consumption, physical activity), physical characteristics (weight, height, waist and hip circumferences, blood pressure--BP), fasting blood glucose (BG) and total cholesterol (TC). Data were analysed using complex survey analysis methods. RESULTS: In this sample, 8.8% of men and 12.6% of women were overweight (body mass index (BMI)>or=25 kg/m2) and 2.3% of men and 1.5% of women were obese (BMI>or=30 kg/m2). The prevalence of hypertension (systolic BP>or=140 mmHg and/or diastolic BP>or=90 mmHg, or taking medication for hypertension) was 27.3% for men and 16.2% for women. There were 1.0% of men and 1.1% of women with raised BG (defined as capillary whole BG of at least 6.1 mmol/L). CONCLUSION: We provide the first NCD risk factor profile of people living in the Mekong Delta of Vietnam using standardised methodology. Our findings for this predominantly rural sample differ from previous studies conducted in Ha Noi and HCMC, and suggest that it is inappropriate to generalise findings from the big-city surveys to the other 80% of the population.

Optic nerve head drusen and idiopathic intracranial hypertension in a 14-year-old girl.

A 14-year-old girl had a 3-month history of headache and blurred vision. Funduscopy showed bilateral optic disc edema. Findings on brain imaging were normal, and a diagnosis of idiopathic intracranial hypertension was confirmed after lumbar puncture showed an elevated opening pressure of 32 cm H(2)O. Optic nerve head drusen were noted on computed tomography scan and confirmed with B-scan ultrasound. After 2 years, resolution of symptoms coincided with variable compliance to treatment with acetazolamide and concomitant papilledema. In general, optic disc edema poses a clinical conundrum due to the more common occurrence of optic nerve head drusen, potentially resulting in delayed diagnosis and treatment of idiopathic intracranial hypertension.

Association between dietary fat and skin cancer in an Australian population using case-control and cohort study designs.

BACKGROUND: Human studies of dietary fat as a possible risk factor for cutaneous malignant melanoma (CMM) and non-melanoma skin cancer (NMSC)--principally basal cell carcinoma (BCC) and squamous cell carcinoma (SCC)--have produced inconsistent results. We had the opportunity to examine the association concurrently for all three types of skin cancer in a population-based study in Tasmania, Australia, involving 652 cases of CMM, BCC and SCC and a common set of 471 controls. METHODS: Histopathologically-confirmed cases of CMM, BCC and SCC were ascertained from the Tasmanian Cancer Registry (TCR), and controls were selected at random from the state's electoral roll. We compared subjects categorised by thirds of dietary fat intake score measured by the 'Dobson short fat questionnaire', with logistic regression models that adjusted for age, sex, skin type and usual sun exposure. We then followed all subjects for 56-80 months until 31 August, 2004 for a new NMSC using record linkage with both the TCR and the Births, Deaths, and Marriages registry. Incidence rates were calculated and ratios of rates were estimated using Poisson models. RESULTS: Relative to subjects in the lowest fat intake category, the odds ratios (OR) comparing cases and controls were OR = 0.76 (95% CI: 0.56-1.03) for medium fat intake, and OR = 0.62 (95% CI: 0.45-0.85) for high fat intake, with a significant (p < 0.01) trend of reduced odds ratio with higher category dietary fat intake. Among cases, the incidence rate ratio (IRR) relative to those with lowest fat score was IRR = 0.72 (95% CI: 0.50-1.03) for medium fat intake, and IRR = 0.82 (95% CI: 0.56-1.20) for highest fat intake (linear trend p = 0.30). CONCLUSION: Using the same dietary instrument with two study designs in the same Caucasian population, we found no evidence that high fat intake increases the risk of developing melanoma or non-melanoma skin cancers. Instead, our results suggest a risk reduction for high fat intake.

Oxygen as a therapy for reducing nicotine withdrawal symptoms.

Nicotine addiction leads to withdrawal symptoms in many persons who quit smoking. In addition to craving, the most commonly experienced symptoms are: depression; difficulty sleeping; irritability, frustration, or anger; anxiety; difficulty concentrating; restlessness; decreased heart rate; and increased appetite or weight gain. The severity of withdrawal symptoms are variable, and often lead to relapse. By altering the course of withdrawal symptoms it is possible to improve the chances of the quit attempt and reduce the risk of relapse. There is strong evidence that dopamine is the primary neurotransmitter in the reward pathway in addiction. The use of nicotine replacement therapies and Zyban (sustained release bupropion hydrochloride), both of which are suspected of exploiting dopamine's role in addiction, enhance the likelihood of long-term smoking cessation. Anecdotal reports of oxygen's capacity to reduce both the desire for nicotine and withdrawal symptoms suggest that this may be a therapeutic possibility for those who do not experience success with more traditional cessation approaches. Oxygen may have a favorable effect on nicotine withdrawal, as it appears to alter the balance of central neurotransmitters such as dopamine.

Physical activity and its association with cardiovascular risk factors in Vietnam.

This study examined physical activity in leisure time and at work as estimated by the Global Physical Activity Questionnaire (GPAQ) and the associations between both total and domain-specific physical activity with cardiovascular risk factors in a population-based Vietnamese sample. Participants (n = 1978) were 25- to 64-year-old adults selected by stratified multistage sampling. Leisure activity contributed to <5% of total moderate and vigorous activity and was not associated with cardiovascular risk factors. Total moderate and vigorous activity was associated with body composition (r = -0.16 to -0.22; P < .001), blood glucose (r = -0.07; P < .05), and total cholesterol (r = -0.17; P < .001) for men and with total cholesterol (r = -0.07; P < .05) for women after adjusting for age. Further adjustment for smoking and alcohol intake made negligible changes. These associations were largely driven by work activity, which accounted for 80% of total activity.

The association between smoking and hypertension in a population-based sample of Vietnamese men.

OBJECTIVE: The association between tobacco smoking and blood pressure in epidemiological studies remains unclear despite experimental evidence that smoking elevates blood pressure. This study examined the association between smoking and hypertension in a population-based sample of Vietnamese men. METHODS: The study utilized a population-based sample of men (n = 910) from a survey of risk factors of noncommunicable diseases in Vietnam. Measurements including behavioural risk factors, body composition, and blood pressure were performed according to internationally standardized protocols. Poisson regression was used to obtain prevalence ratios and 95% confidence intervals (CIs). All analyses were performed using complex survey methods. RESULTS: There were significant trends of increasing prevalence of hypertension with increasing years (P = 0.05) and pack-years (P = 0.03) of smoking after adjusting for age, BMI, and alcohol intake. Relative to never-smokers, the risk of hypertension for those who had smoked for 30 years or more and those who had smoked 20 pack-years or more were 1.52 (95% CI 0.95-2.44) and 1.34 (95% CI 0.94-1.91), respectively. Overall, however, current smokers were not at higher risk of hypertension than never-smokers (prevalence ratio = 1.08, 95% CI 0.70-1.68), and ex-smokers were more likely to be hypertensive than either never-smokers (prevalence ratio = 1.81, 95% CI 1.07-3.06) or current smokers (prevalence ratio = 1.67, 95% CI 1.25-2.23), similarly adjusted. CONCLUSION: In this population-based sample, hypertension was associated with smoking in a dose-response manner when characterized as number of years of smoking and lifetime cigarette consumption, but was not associated with current smoking status.