Visceral and subcutaneous abdominal adiposity and pulmonary function in 30-year-old adults: a cross-sectional analysis nested in a birth cohort.

BACKGROUND: Several studies have verified body fat distribution in association with pulmonary function (PF), mainly waist circumference, but few have used measures able to distinguish abdominal fat compartments. The present study aims to verify the association of visceral adipose tissue (VAT) and subcutaneous adipose tissue (SAT) with PF measures. METHODS: In 1982, all hospital births occurring in Pelotas, Brazil, were identified and those livebirths have been followed. In 2012-13, the cohort participants were evaluated and VAT and SAT measured using ultrasound; forced expiratory volume in the first second (FEV1) or forced vital capacity (FVC) were patronized in z-scores stratified by sex. The associations were verified using crude and adjusted linear regressions. RESULTS: The present analyses comprised 3438 individuals (1721 women). VAT was inversely associated with spirometric parameters, in both crude and adjusted models. SAT showed inverse associations in the crude analyzes in males and a positive trend after adjustment, except for SAT and FVC in males. To each centimeter of VAT, mean adjusted FEV1 z-scores decreased 0.072 (95% CI -0.107; -0.036) in men and 0.127 (95% CI -0.164; -0.090) in women, and FVC z-scores decreased -0.075 (95% CI -0.111; -0.039) and 0.121 (95% CI -0.158; -0.083), in men and women, respectively. CONCLUSIONS: VAT has a consistent inverse association with FEV1 and FVC in both sexes. On the other hand, SAT showed inconsistent results with PF parameters

Three- dimensional assessment of craniofacial asymmetry in children with transverse maxillary deficiency after rapid maxillary expansion: A prospective study

ObjectiveThe aim of this study was to evaluate craniofacial asymmetry in children with transverse maxillary deficiency, with or without functional unilateral posterior crossbite (UPC), before and after rapid maxillary expansion (RME).Setting and sample populationA sample of 51 children with cone beam computed tomography scans obtained before RME (T1) and a year after RME (T2).Material and methodsThis prospective study consisted of 2 groups: 25 children with functional UPC (6.77 ± 1.5 years) and 26 children without UPC (7.41 ± 1.31 years). Linear and angular measurements were obtained from zygomatic, maxilla, glenoid fossa and mandible, using original and mirrored 3D overlapped models. All right and left side comparisons in both groups and intergroups asymmetries were compared using MANOVA and t test for independent samples, respectively, statistically significant at P < .05.ResultsThe UPC group showed no side differences, but mandibular horizontal rotation at T1, and this asymmetry was improved in T2. The non- UPC group showed at baseline significant lateral asymmetry in orbitale, position of palatine foramen, respectively, in average 2.95 mm and 1.16 mm, and 0.49 mm of average asymmetry in condylar height. The glenoid fossa was symmetric in both groups at T1 and T2.ConclusionsChildren with transverse maxillary deficiency showed slight morphological asymmetry, located in the mandible position in cases of UPC, and in the orbital and maxillary regions in cases without UPC. One year after RME, patients improved their craniofacial asymmetry, with significant changes in the mandible and correction of the mandibular rotation in patients who presented UPC.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/156195/2/ocr12370_am.pdfhttp://deepblue.lib.umich.edu/bitstream/2027.42/156195/1/ocr12370.pd

Early hematopoietic stem cell transplantation in a patient with severe mucopolysaccharidosis II : 7 years follow-up

Mucopolysaccharidosis type II (MPS II - Hunter syndrome) is an X-linked lysosomal storage disorder caused by a deficiency in the enzyme iduronate-2 sulfatase (I2S), leading to the accumulation of the glycosaminoglycans, affecting multiple organs and systems. Enzyme replacement therapy does not cross the blood brain barrier, limiting results in neurological forms of the disease. Another option of treatment for severe MPS, hematopoietic stem cell transplantation (HSCT) has become the treatment of choice for the severe form of MPS type I, since it can preserve neurocognition when performed early in the course of the disease. To date, only few studies have examined the long-term outcomes of HSCT in patients with MPS II. We describe the seven-year follow-up of a prenatally diagnosed MPS II boy with positive family history of severe MPS form, submitted to HSCT with umbilical cord blood cells at 70 days of age. Engraftment after 30 days revealed mixed chimerism with 79% donor cells; after 7 years engraftment remains at 80%. I2S activity 30 days post-transplant was low in plasma and normal in leukocytes and the same pattern is observed to date. At age 7 years growth charts are normal and he is very healthy, although mild signs of dysostosis multiplex are present, as well as hearing loss. The neuropsychological evaluation (Wechsler Intelligence Scale for Children - Fourth Edition - WISC-IV), disclosed an IQ of 47. Despite this low measured IQ, the patient continues to show improvements in cognitive, language and motor skills, being quite functional. We believe that HSCT is a therapeutic option for MPS II patients with the severe phenotype, as it could preserve neurocognition or even halt neurodegeneration, provided strict selection criteria are followed

Socioeconomic determinants of leprosy new case detection in the 100 Million Brazilian Cohort: a population-based linkage study.

BACKGROUND: Although leprosy is recognised as a disease of poverty, there is little evidence on the specific socioeconomic factors associated with disease risk. To inform targeted strategies for disease elimination, we investigated socioeconomic markers of leprosy risk in Brazil. METHODS: Socioeconomic data from the 100 Million Brazilian Cohort were linked to the Brazilian national disease registry (Sistema de Informação de Agravos de Notificação) for leprosy from Jan 1, 2007, to Dec 31, 2014. Using Poisson regression, we assessed the association of socioeconomic factors with risk of incident leprosy in the full cohort and in children (aged 0-15 years), by leprosy subtype and region of residence. FINDINGS: In an analysis of 23 899 942 individuals including 18 518 patients with leprosy, increased levels of deprivation were associated with an increased risk of leprosy in Brazil. Directions of effect were consistent in children younger than 15 years and across disease subtypes. Individuals residing in regions with the highest poverty in the country (central-west, north, and northeast regions) had a risk of leprosy incidence five-to-eight times greater than did other individuals. Decreased levels of income and education and factors reflecting unfavourable living conditions were associated with an up to two-times increase in leprosy incidence (incidence rate ratio 1·46, 95% CI 1·32-1·62, for lowest vs highest quartile of income per capita; 2·09, 95% CI 1·62-2·72, for lowest vs highest level of education). INTERPRETATION: Within the poorest half of the Brazilian population, the most deprived individuals have the greatest risk of leprosy. Strategies focusing on early detection and treatment in the poorest populations could contribute substantially to global disease control. FUNDING: Medical Research Council, Wellcome Trust, Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (Brazil), the Conselho Nacional das Fundações Estaduais de Amparo à Pesquisa, Economic and Social Research Council, Biotechnology and Biological Sciences Research Council, Conselho Nacional de Desenvolvimento Científico e Tecnológico, and Fundação de Apoio à Pesquisa do Distrito Federal

Socioeconomic risk markers of leprosy in high-burden countries: A systematic review and meta-analysis.

Over 200,000 new cases of leprosy are detected each year, of which approximately 7% are associated with grade-2 disabilities (G2Ds). For achieving leprosy elimination, one of the main challenges will be targeting higher risk groups within endemic communities. Nevertheless, the socioeconomic risk markers of leprosy remain poorly understood. To address this gap we systematically reviewed MEDLINE/PubMed, Embase, LILACS and Web of Science for original articles investigating the social determinants of leprosy in countries with > 1000 cases/year in at least five years between 2006 and 2016. Cohort, case-control, cross-sectional, and ecological studies were eligible for inclusion; qualitative studies, case reports, and reviews were excluded. Out of 1,534 non-duplicate records, 96 full-text articles were reviewed, and 39 met inclusion criteria. 17 were included in random-effects meta-analyses for sex, occupation, food shortage, household contact, crowding, and lack of clean (i.e., treated) water. The majority of studies were conducted in Brazil, India, or Bangladesh while none were undertaken in low-income countries. Descriptive synthesis indicated that increased age, poor sanitary and socioeconomic conditions, lower level of education, and food-insecurity are risk markers for leprosy. Additionally, in pooled estimates, leprosy was associated with being male (RR = 1.33, 95% CI = 1.06-1.67), performing manual labor (RR = 2.15, 95% CI = 0.97-4.74), suffering from food shortage in the past (RR = 1.39, 95% CI = 1.05-1.85), being a household contact of a leprosy patient (RR = 3.40, 95% CI = 2.24-5.18), and living in a crowded household (≥5 per household) (RR = 1.38, 95% CI = 1.14-1.67). Lack of clean water did not appear to be a risk marker of leprosy (RR = 0.94, 95% CI = 0.65-1.35). Additionally, ecological studies provided evidence that lower inequality, better human development, increased healthcare coverage, and cash transfer programs are linked with lower leprosy risks. These findings point to a consistent relationship between leprosy and unfavorable economic circumstances and, thereby, underscore the pressing need of leprosy control policies to target socially vulnerable groups in high-burden countries

Geographic and socioeconomic factors associated with leprosy treatment default: An analysis from the 100 Million Brazilian Cohort.

BACKGROUND: Although leprosy is largely curable with multidrug therapy, incomplete treatment limits therapeutic effectiveness and is an important obstacle to disease control. To inform efforts to improve treatment completion rates, we aimed to identify the geographic and socioeconomic factors associated with leprosy treatment default in Brazil. METHODOLOGY/PRINCIPAL FINDINGS: Using individual participant data collected in the Brazilian national registries for social programs and notifiable diseases and linked as part of the 100 Million Brazilian Cohort, we evaluated the odds of treatment default among 20,063 leprosy cases diagnosed and followed up between 2007 and 2014. We investigated geographic and socioeconomic risk factors using a multivariate hierarchical analysis and carried out additional stratified analyses by leprosy subtype and geographic region. Over the duration of follow-up, 1,011 (5.0%) leprosy cases were observed to default from treatment. Treatment default was markedly increased among leprosy cases residing in the North (OR = 1.57; 95%CI 1.25-1.97) and Northeast (OR = 1.44; 95%CI 1.17-1.78) regions of Brazil. The odds of default were also higher among cases with black ethnicity (OR = 1.29; 95%CI 1.01-1.69), no income (OR = 1.41; 95%CI 1.07-1.86), familial income ≤ 0.25 times Brazilian minimum wage (OR = 1.42; 95%CI 1.13-1.77), informal home lighting/no electricity supply (OR = 1.53; 95%CI 1.28-1.82), and household density of > 1 individual per room (OR = 1.35; 95%CI 1.10-1.66). CONCLUSIONS: The findings of the study indicate that the frequency of leprosy treatment default varies regionally in Brazil and provide new evidence that adverse socioeconomic conditions may represent important barriers to leprosy treatment completion. These findings suggest that interventions to address socioeconomic deprivation, along with continued efforts to improve access to care, have the potential to improve leprosy treatment outcomes and disease control