Generalized Pustular Psoriasis in Childhood

Generalized pustular psoriasis is a rare form of psoriasis consisting of a generalized eruption of sudden onset with erythema and sterile pustules. In children, generalized pustular psoriasis is even more uncommon and may present as a severe and potentially life-threatening disorder. In this study, we present demographics, clinical aspects, treatment response, and follow-up of seven children with generalized pustular psoriasis. Retrospective study reviewing the records of seven children with generalized pustular psoriasis including age, gender, age of onset, presence of scalp and nail involvement, family history, concomitant diseases, precipitating factors, treatment modalities, and outcome. Age of first symptoms ranged from 1 month to 11 years. All patients received systemic retinoids at one time of the follow-up period. Other treatment modalities included immunosuppressive drugs, biologics, phototherapy, and sulfasalazine. Two patients presented with severe constitutional illness, secondary infection and septic shock, including one fatal outcome. All further cases have remained free of recurrences for a mean period of up to 3 years. In our study, generalized pustular psoriasis presented a wide clinical spectrum in children ranging from mild, asymptomatic outbreaks to more severe, life-threatening episodes. One fatality was observed. Children generally responded well to systemic retinoids. Further studies and long-term follow-up periods are needed to define potential trigger factors, efficacy and safety of different treatment modalities in children with generalized pustular psoriasis.

Factors involved in the T helper type 1 and type 2 cell commitment and osteoclast regulation in inflammatory apical diseases

Periapical chronic lesion formation involves activation of the immune response and alveolar bone resorption around the tooth apex. However, the overall roles of T helper type 1 (Th1), Th2, and T-regulatory cell (Treg) responses and osteoclast regulatory factors in periapical cysts and granulomas have not been fully determined. This study aimed to investigate whether different forms of apical periodontitis, namely cysts and granulomas, show different balances of Th1, Th2 regulators, Treg markers, and factors involved in osteoclast chemotaxis and activation. Gene expression of these factors was assessed using quantitative real-time polymerase chain reaction, in samples obtained from healthy gingiva (n = 8), periapical granulomas (n = 20), and cysts (n = 10). Periapical cysts exhibited a greater expression of GATA-3, while a greater expression of T-bet, Foxp3, and interleukin-10 (IL-10) was seen in granulomas. The expression of interferon-gamma, IL-4, and transforming growth factor-beta was similar in both lesions. Regarding osteoclastic factors, while the expression of SDF-1 alpha/CXCL12 and CCR1 was higher in cysts, the expression of RANKL was significantly higher in granulomas. Both lesions exhibited similar expression of CXCR4, CK beta 8/CCL23, and osteoprotegerin, which were significantly higher than in control. Our results showed a predominance of osteoclast activity in granulomas that was correlated with the Th1 response. The concomitant expression of Treg cell markers suggests a possible suppression of the Th1 response in granulomas. On the other hand, in cysts the Th2 activity is augmented. The mechanisms of periradicular lesion development are still not fully understood but the imbalance of immune and osteoclastic cell activity in cysts and granulomas seems to be critically regulated by Treg cells.

PENCALC: a program for penetrance estimation in autosomal dominant diseases

We present a computer program developed for estimating penetrance rates in autosomal dominant diseases by means of family kinship and phenotype information contained within the pedigrees. The program also determines the exact 95% credibility interval for the penetrance estimate. Both executable (PenCalc for Windows) and web versions (PenCalcWeb) of the software are available. The web version enables further calculations, such as heterozygosity probabilities and assessment of offspring risks for all individuals in the pedigrees. Both programs can be accessed and down-loaded freely at the home-page address http://www.ib.usp.br/~otto/software.htm.

Endothelial dysfunction in cardiovascular and endocrine-metabolic diseases: an update

The endothelium plays a vital role in maintaining circulatory homeostasis by the release of relaxing and contracting factors. Any change in this balance may result in a process known as endothelial dysfunction that leads to impaired control of vascular tone and contributes to the pathogenesis of some cardiovascular and endocrine/metabolic diseases. Reduced endothelium-derived nitric oxide (NO) bioavailability and increased production of thromboxane A2, prostaglandin H2 and superoxide anion in conductance and resistance arteries are commonly associated with endothelial dysfunction in hypertensive, diabetic and obese animals, resulting in reduced endothelium-dependent vasodilatation and in increased vasoconstrictor responses. In addition, recent studies have demonstrated the role of enhanced overactivation ofβ-adrenergic receptors inducing vascular cytokine production and endothelial NO synthase (eNOS) uncoupling that seem to be the mechanisms underlying endothelial dysfunction in hypertension, heart failure and in endocrine-metabolic disorders. However, some adaptive mechanisms can occur in the initial stages of hypertension, such as increased NO production by eNOS. The present review focuses on the role of NO bioavailability, eNOS uncoupling, cyclooxygenase-derived products and pro-inflammatory factors on the endothelial dysfunction that occurs in hypertension, sympathetic hyperactivity, diabetes mellitus, and obesity. These are cardiovascular and endocrine-metabolic diseases of high incidence and mortality around the world, especially in developing countries and endothelial dysfunction contributes to triggering, maintenance and worsening of these pathological situations.

Air pollution and hospital admissions for respiratory diseases in the subequatorial Amazon: a time series approach

The objective of the study is to evaluate the effect of the daily variation in concentrations of fine particulate matter (diameter less than 2.5µm - PM2.5) resulting from the burning of biomass on the daily number of hospitalizations of children and elderly people for respiratory diseases, in Alta Floresta and Tangará da Serra in the Brazilian Amazon in 2005. This is an ecological time series study that uses data on daily number of hospitalizations of children and the elderly for respiratory diseases, and estimated concentration of PM2.5. In Alta Floresta, the percentage increases in the relative risk (%RR) of hospitalization for respiratory diseases in children were significant for the whole year and for the dry season with 3-4 day lags. In the dry season these measurements reach 6% (95%CI: 1.4-10.8). The associations were sig-nificant for moving averages of 3-5 days. The %RR for the elderly was significant for the current day of the drought, with a 6.8% increase (95%CI: 0.5-13.5) for each additional 10µg/m3 of PM2.5. No as-sociations were verified for Tangara da Serra. The PM2.5 from the burning of biomass increased hospitalizations for respiratory diseases in children and the elderly.

Comportamentos em saúde entre idosos hipertensos, Brasil, 2006

OBJETIVO: Estimar as prevalências de comportamentos prejudiciais à saúde e de outros fatores de risco cardiovascular entre idosos com hipertensão auto-referida e comparando-as com de não-hipertensos. MÉTODOS: Foram utilizados dados do sistema de Vigilância de Fatores de Risco e Proteção para Doenças Crônicas por Inquérito Telefônico (VIGITEL), referentes aos 9.038 idosos residentes em domicílios com pelo menos uma linha telefônica fixa nas 26 capitais brasileiras e no Distrito Federal em 2006. RESULTADOS: A prevalência de hipertensão auto-referida foi de 55% (IC 95%: 53;57). A maioria dos hipertensos apresentava concomitância de três ou mais fatores de risco (69%; IC 95%: 67;71). Foram observadas altas prevalências de atividades físicas insuficientes no lazer (88%; IC 95%: 86;89) e do consumo de frutas e hortaliças inferior a cinco porções diárias (90%; IC 95%: 88;90) entre hipertensos, seguidas pela adição de sal aos alimentos (60%; IC 95%: 57;63), consumo habitual de carnes gordurosas (23%; IC 95%: 21;25), tabagismo (9%; IC 95%: 7;10) e consumo abusivo de álcool (3%; IC 95%: 2;4). Essas prevalências foram semelhantes às observadas entre não hipertensos (p >0,05), exceto tabagismo. A prevalência do tabagismo foi menor entre hipertensos (razão de prevalência ajustada [RPA] = 0,75; IC 95%: 0,64;0,89) e as prevalências de sobrepeso (RPA= 1,37; IC 95%: 1,25;1,49), dislipidemia (RPA 1,36; IC 95%: 1,26;1,36) e diabetes (RPA= 1,37; IC 95%: 1,27;1,37) foram mais altas. CONCLUSÕES: Os resultados sugerem que, exceto tabagismo, os comportamentos prejudiciais à saúde entre idosos persistem após o diagnóstico da hipertensão arterial.

Gender and schooling inequalities in risk and protective factors for chronic diseases among Brazilian adults, through telephone survey

OBJECTIVES: To assess risk and protective factors for chronic noncommunicable diseases (CNCD) and to identify social inequalities in their distribution among Brazilian adults. METHODS: The data used were collected in 2007 through VIGITEL, an ongoing population-based telephone survey. This surveillance system was implemented in all of the Brazilian State capitals, over 54,000 interviews were analyzed. Age-adjusted prevalence ratios for trends at different schooling levels were calculated using Poisson regression with linear models. RESULTS: These analyses have shown differences in the prevalence of risk and protective factors for CNCD by gender and schooling. Among men, the prevalence ratios of overweight, consumption of meat with visible fat, and dyslipidemia were higher among men with more schooling, while tobacco use, sedentary lifestyle, and high-blood pressure were lower. Among women, tobacco use, overweight, obesity, high-blood pressure and diabetes were lower among men with more schooling, and consumption of meat with visible fat and sedentary lifestyles were higher. As for protective factors, fruit and vegetables intake and physical activity were higher in both men and women with more schooling. CONCLUSION: Gender and schooling influence on risk and protective factors for CNCD, being the values less favorable for men. vigitel is a useful tool for monitoring these factors amongst the Brazilian population.

A feasibility study of cell phone and landline phone interviews for monitoring of risk and protection factors for chronic diseases in Brazil

The study objective was to evaluate the feasibility of interviews by cell phone as a complement to interviews by landline to estimate risk and protection factors for chronic non-communicable diseases. Adult cell phone users were evaluated by random digit dialing. Questions asked were: age, sex, education, race, marital status, ownership of landline and cell phones, health condition, weight and height, medical diagnosis of hypertension and diabetes, physical activity, diet, binge drinking and smoking. The estimates were calculated using post-stratification weights. The cell phone interview system showed a reduced capacity to reach elderly and low educated populations. The estimates of the risk and protection factors for chronic non-communicable diseases in cell phone interviews were equal to the estimates obtained by landline phone. Eligibility, success and refusal rates using the cell phone system were lower than those of the landline system, but loss and cost were much higher, suggesting it is unsatisfactory as a complementary method in such a context.

Prevalence and social distribution of risk factors for chronic noncommunicable diseases in Brazil

OBJETIVOS: Evaluar los factores de riesgo de enfermedades crónicas no transmisibles (ECNT) e identificar las desigualdades sociales relacionadas con su distribución en la población adulta brasileña.MÉTODOS: Se estudiaron los factores de riesgo de ECNT (entre ellos el consumo de tabaco, el sobrepeso y la obesidad, el bajo consumo de frutas y vegetales [BCFV], la insuficiente actividad física en el tiempo de ocio [IAFTO], el estilo de vida sedentario y el consumo excesivo de alcohol) en una muestra probabilística de 54369 adultos de 26 capitales estatales de Brasil y el Distrito Federal en 2006. Se utilizó el Sistema de Vigilancia de los Factores Protectores y de Riesgo para Enfermedades Crónicas No Transmisibles por Entrevistas Telefónicas (VIGITEL), un sistema de encuestas telefónicas asistido por computadora, y se calcularon las prevalencias ajustadas por la edad para las tendencias en cuanto al nivel educacional mediante la regresión de Poisson con modelos lineales. RESULTADOS: Los hombres informaron mayor consumo de tabaco, sobrepeso, BCFV, estilo de vida sedentario y consumo excesivo de alcohol que las mujeres, pero menos IAFTO. En los hombres, la educación se asoció con un mayor sobrepeso y un estilo de vida sedentario, pero con un menor consumo de tabaco, BCFV e IAFTO. En las mujeres, la educación se asoció con un menor consumo de tabaco, sobrepeso, obesidad, BCFV e IAFTO, pero aumentó el estilo de vida sedentario CONCLUSIONES: En Brasil, la prevalencia de factores de riesgo para ECNT (excepto IAFTO) es mayor en los hombres que en las mujeres. En ambos sexos, el nivel de educación influye en la prevalencia de los factores de riesgo para ECNT

Determination of banana fruit susceptibility to post-harvest diseases: wound anthracnose, quiescent anthracnose and crown rot

Introduction. This protocol aims at ( a) evaluating the resistance to post-harvest diseases within different genotypes of bananas, and ( b) comparing different origins of bananas ( geographic origin, physiological stage, etc.) for their susceptibility to post-harvest diseases. The principle, key advantages, starting plant material, time required and expected results are presented. Materials and methods. Materials required and details of the twelve steps of the protocol ( fruit sampling and inoculum preparation, wound anthracnose resistance study, quiescent anthracnose resistance study and crown-rot resistance study) are described. Results. Typical symptoms of the different diseases are obtained after artificial inoculation.

Measurement of fungicide efficacy on post-harvest diseases: wound anthracnose, quiescent anthracnose, crown rot

Introduction. This protocol aims at evaluating (a) the efficacy of new fungicides for the control of post-harvest diseases, (b) the efficacy of various application methods for the chemical control of post-harvest diseases, and (c) the quality of the fungicide solution during the same packing day where this solution is recycled. The principle, key advantages, starting plant material, time required and expected results are presented. Materials and methods. Materials required and details of the eighteen steps of the protocol (fruit sampling and inoculum preparation, wound anthracnose study, quiescent anthracnose study, and crown-rot study) are described. Results. Comparison between untreated control bananas and bananas treated with fungicide allows the calculation of the fungicide treatment efficacy.

High levels of T lymphocyte activation in Leishmania-HIV-1 co-infected individuals despite low HIV viral load

Background: Concomitant infections may influence HIV progression by causing chronic activation leading to decline in T-cell function. In the Americas, visceral (AVL) and tegumentary leishmaniasis (ATL) have emerged as important opportunistic infections in HIV-AIDS patients and both of those diseases have been implicated as potentially important co-factors in disease progression. We investigated whether leishmaniasis increases lymphocyte activation in HIV-1 co-infected patients. This might contribute to impaired cellular immune function. Methods: To address this issue we analyzed CD4(+) T absolute counts and the proportion of CD8(+) T cells expressing CD38 in Leishmania/HIV co-infected patients that recovered after anti-leishmanial therapy. Results: We found that, despite clinical remission of leishmaniasis, AVL co-infected patients presented a more severe immunossupression as suggested by CD4(+) T cell counts under 200 cells/mm(3), differing from ATL/HIV-AIDS cases that tends to show higher lymphocytes levels (over 350 cells/mm(3)). Furthermore, five out of nine, AVL/HIV-AIDS presented low CD4(+) T cell counts in spite of low or undetectable viral load. Expression of CD38 on CD8(+) T lymphocytes was significantly higher in AVL or ATL/HIV-AIDS cases compared to HIV/AIDS patients without leishmaniasis or healthy subjects. Conclusions: Leishmania infection can increase the degree of immune system activation in individuals concomitantly infected with HIV. In addition, AVL/HIV-AIDS patients can present low CD4(+) T cell counts and higher proportion of activated T lymphocytes even when HIV viral load is suppressed under HAART. This fact can cause a misinterpretation of these laboratorial markers in co-infected patients.

Prevalence of non-communicable diseases in Brazilian children: follow-up at school age of two Brazilian birth cohorts of the 1990's

Background: Few cohort studies have been conducted in low and middle-income countries to investigate non-communicable diseases among school-aged children. This article aims to describe the methodology of two birth cohorts, started in 1994 in Ribeirao Preto (RP), a more developed city, and in 1997/98 in Sao Luis (SL), a less developed town. Methods: Prevalences of some non-communicable diseases during the first follow-up of these cohorts were estimated and compared. Data on singleton live births were obtained at birth (2858 in RP and 2443 in SL). The follow-up at school age was conducted in RP in 2004/05, when the children were 9-11 years old and in SL in 2005/06, when the children were 7-9 years old. Follow-up rates were 68.7% in RP (790 included) and 72.7% in SL (673 participants). The groups of low (<2500 g) and high (>= 4250 g) birthweight were oversampled and estimates were corrected by weighting. Results: In the more developed city there was a higher percentage of non-nutritive sucking habits (69.1% vs 47.9%), lifetime bottle use (89.6% vs 68.3%), higher prevalence of primary headache in the last 15 days (27.9% vs 13.0%), higher positive skin tests for allergens (44.3% vs 25.3%) and higher prevalence of overweight (18.2% vs 3.6%), obesity (9.5% vs 1.8%) and hypertension (10.9% vs 4.6%). In the less developed city there was a larger percentage of children with below average cognitive function (28.9% vs 12.2%), mental health problems (47.4% vs 38.4%), depression (21.6% vs 6.0%) and underweight (5.8% vs 3.6%). There was no difference in the prevalence of bruxism, recurrent abdominal pain, asthma and bronchial hyperresponsiveness between cities. Conclusions: Some non-communicable diseases were highly prevalent, especially in the more developed city. Some high rates suggest that the burden of non-communicable diseases will be high in the future, especially mental health problems.

Effect of physical exercise on risk factors for chronic diseases in obeses women

This study aimed to evaluate the effects of physical exercise on body weight reduction. For 12 weeks, 22 obese women (BMI>30 kg/m(2)) were submitted to a physical exercise program. At the beginning and at the final of the program there were evaluated: BMI, waist (WC) and hip circumferences (HC), and waist-hip ratio (WHR); body composition by DEXA; hemoglobin and erythroctye, total cholesterol, HDL and LDL, triacylglycerol and blood glucose; aerobic power. At the final of the program, aerobic power, hemoglobin and erythrocyte values were significantly increased, confirming the physical training effects. Related to anthropometric values, only the visceral fat (WC, HC and WHR) were reduced. The exercise shows to be an important supporting in the body weight loss program, not exactly promoting body weight loss, but lowering risk factors to develop chronic diseases.