Quality of life in people with epidermolysis bullosa: a systematic review.

PURPOSE: Individuals with epidermolysis bullosa (EB) present with various clinical manifestations of different severities that affect quality of life (QoL). This systematic review synthesizes the current evidence about the QoL of individuals with EB. METHODS: We included observational studies with people of all age groups, both sexes, and any EB type. Studies with qualitative methodology, chapters of books, meeting proceedings, and abstracts were excluded. RESULTS: In this study, 12 articles comprising 745 individuals were included. More than half of the articles observed lower QoL in individuals with recessive dystrophic EB (RDEB) or junctional EB (JEB). Three articles indicated that EB affected QoL more in women than in men, and one article identified that children with EB suffered more than adults with the disease. Pain was frequently reported. Seven articles identified difficulty in sports, two identified a need for bathing assistance, and three identified eating difficulties. Additionally, participants reported that family relationships and friendships were affected, and they experienced feelings of anxiety and depression. Some of the instruments used evaluated QoL in general dermatologic conditions, and one was specific to EB. CONCLUSION: QoL is more affected in people who have RDEB and JEB. Regarding sex and age, women and children need special care in their monitoring. It is necessary that guidelines on pain management be more disseminated and put into practice. Future studies should use standardized specific instruments to assess the QoL in EB individuals, while considering the particularities of the different age groups.

C-reactive protein in Brazilian adolescents: distribution and association with metabolic syndrome in ERICA survey.

BACKGROUND/OBJECTIVES: C-reactive protein (CRP) is a marker of inflammation that has been shown to be predictive of cardiovascular diseases in adults. To evaluate the distribution of CRP as well as its association with metabolic syndrome and its components. SUBJECTS/METHODS: This is a cross-sectional study on adolescents aged 12-17, participants in the Study of Cardiovascular Risk in Adolescents (ERICA). Anthropometric, biochemical and blood pressure data were collected from 6316 adolescents, selected from a random sample of students in the cities of Brasilia, Fortaleza, João Pessoa, Manaus, Porto Alegre and Rio de Janeiro. Metabolic syndrome was defined by the criteria proposed by International Diabetes Federation for adolescent. Poisson regression model with robust variance, taking into consideration the study's complex sampling design, was used to determine multivariate-adjusted prevalence rate ratios expressing the relationship of metabolic syndrome with CRP. RESULTS: In adolescents with metabolic syndrome, CRP concentrations were five times higher (1.01 mg/l; interquartile range (IQR): 0.54-3.47) compared with those without metabolic syndrome (0.19 mg/l; IQR: 0.10-0.78). In multivariate Poisson regression analysis adjusted by sex, age and skin color, the prevalence of elevated CRP (>3.0 mg/l) was almost three times higher in adolescents with metabolic syndrome than in those without this condition (prevalence ratio (PR): 2.9; 95%CI: 2.0-4.3; P<0.001). Of the metabolic syndrome components, elevated waist circumference, low high-density lipoprotein-cholesterol and high triglycerides were significantly related to CRP in a graded (dose-response) manner. CONCLUSIONS: The association of CRP with metabolic syndrome and its components suggests that inflammation may be useful in assessing cardiovascular risk in adolescents.

Histologic effect of mitomycin C on strabismus surgery in the rabbit.

PURPOSE: To evaluate the efficiency of mitomycin C (MMC) in limiting the postoperative inflammatory response and scarring after strabismus surgery. METHODS: A prospective, two-stage, masked, controlled trial was conducted. In the first stage, the inflammatory response at the extraocular muscle reattachment site was increased after inferior rectus recession in eight rabbits. In the second stage, MMC (0.4 mg/ml) was applied during surgery to the eyes of 22 rabbits with inferior rectus recession. As a control, contralateral eyes were treated with saline solution. Seven weeks later, exenteration was performed, and the sites of muscle reattachment were processed for histologic examinations. The sums of the areas of the granulomas in the extraocular muscle reattachment sites of control and treated eyes were compared. RESULTS: There was no significant inhibitory effect of MMC on the inflammatory response of treated eyes compared with that of control eyes. CONCLUSIONS: The intraoperative use of MMC (0.4 mg/ml) was not effective in controlling the postoperative inflammatory response in rabbit eyes after extraocular muscle surgery. These data do not support the hypothesis that MMC reduces postoperative adhesions after strabismus surgery.

Quantification (grading) of inferior oblique muscle recession for V-pattern strabismus.

PURPOSE: To determine the effectiveness of grading such surgery according to the magnitude of the V pattern and inferior oblique muscle overaction (IOOA). METHODS: We retrospectively reviewed all 53 cases we operated since 1984 for V pattern with IOOA, who had undergone graded inferior oblique recession, recessed according to the anatomical recession table of Apt and Call, ranging from 8mm for V pattern of 12 PD with +1 IOOA to 12mm for 30+ PD with +3 IOOA. RESULTS: A "satisfactory outcome" (defined as = V pattern of 8 PD or less) was observed in 75% of the cases with a preoperative V pattern less than 20 PD, in 70% of those with a preop' V pattern between 20 PD and 29 PD, and in 57% of those with a preop' V pattern greater than 29 PD. There were no overcorrections. All unsatisfactory outcomes were undercorrections. CONCLUSIONS: The principle of grading this surgery is supported and affirmed by these results. However, the results also suggest a need to increase the amount of surgery for all categories and add anterior transposition for larger V patterns.

Characteristics of a pediatric low-vision population.

BACKGROUND: Assessment of the causes of childhood blindness is important to develop preventive and therapeutic strategies that can reduce the incidence of visual impairment. METHODS: A retrospective survey of the clinical records of 395 consecutive children younger than 14 years of age who were attended by the Low Vision Service of State University of Campinas (UNICAMP) was conducted. Data on the age distribution, the causes of low vision, the types of low vision aids prescribed, the follow up of their use, and improvement in visual acuity were obtained. RESULTS: Congenital bilateral toxoplasmic macular scars, optic atrophy, and congenital cataracts were the major causes of low vision in this population. The optical aid most frequently prescribed was a telescope. The improvement of visual acuity with the use of aids was highly significant.