Balance and physical functioning in Spinocerebellar ataxias 3 and 10.

OBJECTIVES: Limitations of functional capacity and balance are common features of the natural history of spinocerebellar ataxias (SCA). However, their onset and progression patterns differ according to subtype. The aim of our study was to compare physical functionality and balance parameters in SCA10 and SCA3 patients, correlating with clinical variables. MATERIALS & METHODS: Cross-sectional study evaluating ninety-five SCA patients (60 with SCA3 and 35 with SCA10) with validated scales for functional independence, balance and the severity of signs and symptoms. RESULTS: The groups were similar in terms of age and gender, and results were adjusted for age at symptom onset. The SCA10 patients had better results for balance and functional independence (p < 0.007). They also had lower scores for disease severity (p < 0.0002) and the subitems gait (p < 0.0005), posture (p < 0.0021) and sitting balance (p < 0.0008). Symptom progression in both groups was similar for patients with a disease duration of up to ten years, but there was a more marked decline in SCA3 patients after this period. CONCLUSIONS: We have shown that disease progression as assessed by balance and physical functioning is slower in SCA10 patients than SCA3 patients, particularly after 10 years of disease. These findings are important as they can help to characterize the disease, assisting in the development of new therapies and rehabilitation programs.

Life-long Maternal Cafeteria Diet Promotes Tissue-Specific Morphological Changes in Male Offspring Adult Rats.

Here, we evaluated whether the exposure of rats to a cafeteria diet pre- and/or post-weaning, alters histological characteristics in the White Adipose Tissue (WAT), Brown Adipose Tissue (BAT), and liver of adult male offspring. Female Wistar rats were divided into Control (CTL; fed on standard rodent chow) and Cafeteria (CAF; fed with the cafeteria diet throughout life, including pregnancy and lactation). After birth, only male offspring (F1) were maintained and received the CTL or CAF diets; originating four experimental groups: CTL-CTLF1; CTL-CAFF1; CAF-CTLF1; CAF-CAFF1. Data of biometrics, metabolic parameters, liver, BAT and WAT histology were assessed and integrated using the Principal Component Analysis (PCA). According to PCA analysis worse metabolic and biometric characteristics in adulthood are associated with the post-weaning CAF diet compared to pre and post weaning CAF diet. Thus, the CTL-CAFF1 group showed obesity, higher deposition of fat in the liver and BAT and high fasting plasma levels of glucose, triglycerides and cholesterol. Interestingly, the association between pre and post-weaning CAF diet attenuated the obesity and improved the plasma levels of glucose and triglycerides compared to CTL-CAFF1 without avoiding the higher lipid accumulation in BAT and in liver, suggesting that the impact of maternal CAF diet is tissue-specific.

Life-long Maternal Cafeteria Diet Promotes Tissue-Specific Morphological Changes in Male Offspring Adult Rats

ABSTRACT Here, we evaluated whether the exposure of rats to a cafeteria diet pre- and/or post-weaning, alters histological characteristics in the White Adipose Tissue (WAT), Brown Adipose Tissue (BAT), and liver of adult male offspring. Female Wistar rats were divided into Control (CTL; fed on standard rodent chow) and Cafeteria (CAF; fed with the cafeteria diet throughout life, including pregnancy and lactation). After birth, only male offspring (F1) were maintained and received the CTL or CAF diets; originating four experimental groups: CTL-CTLF1; CTL-CAFF1; CAF-CTLF1; CAF-CAFF1. Data of biometrics, metabolic parameters, liver, BAT and WAT histology were assessed and integrated using the Principal Component Analysis (PCA). According to PCA analysis worse metabolic and biometric characteristics in adulthood are associated with the post-weaning CAF diet compared to pre and post weaning CAF diet. Thus, the CTL-CAFF1 group showed obesity, higher deposition of fat in the liver and BAT and high fasting plasma levels of glucose, triglycerides and cholesterol. Interestingly, the association between pre and post-weaning CAF diet attenuated the obesity and improved the plasma levels of glucose and triglycerides compared to CTL-CAFF1 without avoiding the higher lipid accumulation in BAT and in liver, suggesting that the impact of maternal CAF diet is tissue-specific.

Risk factors associated with growth failure in the follow-up of very low birth weight newborns / Fatores de risco associados à falha de crescimento no seguimento de recém-nascidos de muito baixo peso

Abstract Objective: To determine risk factors during neonatal hospital stay and follow-up associated with failure to thrive in the first year of life of very low birth weight newborns. Methods: Study of preterm very low birth weight newborns followed from 2006 to 2013 in a public institutional hospital program. The study included newborns that attended at least one appointment in each of the three periods: Period I, up to 3 months of corrected age (CA); Period II, 4-6 months of CA; and Period III, 7-12 months of CA. The variables were analyzed by logistic regression with XLSTAT 2014 software (Microsoft®, WA, USA). Failure to thrive (Z-score below -2 SD) was classified as a dichotomous dependent variable (0 - failure/1 - success), while the other variables were classified as explanatory variables for the hospitalization periods and for each of the follow-up periods (I, II, and III). Results: Children born adequate for gestational age increased the chance of Z-score for weight at discharge > -2 SD (OR = 10.217; 95% CI: 1.117-93.436). Metabolic bone disease and retinopathy of prematurity in Period I, as well as hospital readmissions in Periods II and III during follow-up increased the chance of Z-score < -2 SD. Conclusion: Failure to thrive is influenced by intrauterine factors and, subsequently, by several morbidities, both in the birth and hospitalization period, as well as in the post-discharge period and thus, such variables should be prioritized in the follow-up.

Resumo Objetivo: Determinar fatores de risco do período de internação neonatal e do seguimento ambulatorial associados à falha de crescimento no primeiro ano de vida de recém-nascidos de muito baixo peso. Métodos: Estudo com crianças nascidas prematuras de muito baixo peso em acompanhamento de 2006 a 2013 em ambulatório de alto risco de um hospital-escola. Incluídas aquelas que fizera pelo menos uma consulta em cada um dos três períodos assim determinados: Período I - até três meses de idade corrigida (IC); Período II - entre quatro e seis meses de IC; e Período III - entre sete e 12 meses de IC. As variáveis foram analisadas por regressão logística com o programa XLStat 2014 (Microsoft®, WA, EUA). A falha de crescimento (escore z abaixo de --2 DP) foi classificada como variável dependente do tipo dicotômica (0 - falha/1 - sucesso) e as demais variáveis foram classificadas como variáveis explicativas para os períodos de internação e para cada um dos períodos de seguimento (I, II e III). Resultados: Nascer adequado para a idade gestacional aumenta a chance de apresentar escore Z do peso na alta hospitalar acima de -2 DP (OR = 10,217; IC95% 1.117-93,436). Doença metabólica óssea e retinopatia da prematuridade durante o Período I e reinternações nos Períodos II e III de seguimento aumentam a chance de escore z abaixo de -2 DP. Conclusão: A falha de crescimento é influenciada por fatores intrauterinos e posteriormente por diversas morbidades, tanto no período da internação como no pós-alta. Tais variáveis estudadas deveriam ter prioridade no seguimento.

Dental panoramic indices and fractal dimension measurements in osteogenesis imperfecta children under pamidronate treatment.

OBJECTIVES: To verify radiomorphometric indices and fractal dimension (FD) in dental panoramic radiographs (DPRs) of children with different types of osteogenesis imperfecta (OI) and also to verify the effect of pamidronate (PAM) treatment in such panoramic analyses. METHODS: In this retrospective study, 197 DPRs of 62 children with OI Types I, III and IV who were in treatment with a comparable dosage of intravenous PAM were selected. The mandibular cortical width (MCW), mandibular cortical index, visual estimation of the cortical width and FD of three standardized trabecular and cortical mandibular regions of interest were obtained from the radiographs. Factorial analysis of variance and Fisher test were used to compare FD and MCW measurements in children with different types of OI for different PAM cycles. RESULTS: Children with all types of OI have thinner and more porous mandibular cortices at the beginning of treatment. There were significant differences between MCW and FD of the cortical bone, regarding different types of OI and number of PAM cycles (p = 0.037 and p = 0.044, respectively). FD measurements of the trabecular bone were not statistically different among OI types nor were PAM cycles (p > 0.05). CONCLUSIONS: Children with OI presented cortical bone alterations after PAM treatment. Both MCW and the FD of the cortical bone were higher in children with OI after PAM treatment. It is argued that cortical bone should be considered for analyzing patients with OI, as well as to monitor the progress of PAM treatment.

Risk factors associated with growth failure in the follow-up of very low birth weight newborns.

OBJECTIVE: To determine risk factors during neonatal hospital stay and follow-up associated with failure to thrive in the first year of life of very low birth weight newborns. METHODS: Study of preterm very low birth weight newborns followed from 2006 to 2013 in a public institutional hospital program. The study included newborns that attended at least one appointment in each of the three periods: Period I, up to 3 months of corrected age (CA); Period II, 4-6 months of CA; and Period III, 7-12 months of CA. The variables were analyzed by logistic regression with XLSTAT 2014 software (Microsoft®, WA, USA). Failure to thrive (Z-score below -2 SD) was classified as a dichotomous dependent variable (0 - failure/1 - success), while the other variables were classified as explanatory variables for the hospitalization periods and for each of the follow-up periods (I, II, and III). RESULTS: Children born adequate for gestational age increased the chance of Z-score for weight at discharge>-2 SD (OR=10.217; 95% CI: 1.117-93.436). Metabolic bone disease and retinopathy of prematurity in Period I, as well as hospital readmissions in Periods II and III during follow-up increased the chance of Z-score<-2 SD. CONCLUSION: Failure to thrive is influenced by intrauterine factors and, subsequently, by several morbidities, both in the birth and hospitalization period, as well as in the post-discharge period and thus, such variables should be prioritized in the follow-up.

Technical evaluation of serological screening tests for anti-Toxoplasma gondii antibodies to prevent unnecessary transfusion risks / Avaliação técnica de testes de triagem sorológica para detecção de anticorpos anti-Toxoplasma gondii como medida preventiva a riscos transfusionais desnecessários

Serological assays to detect anti-Toxoplasma gondii antibodies and specific anamnesis associated with this highly prevalent infection could be necessary in hemotherapy services in order to regulate safer blood supplies thus avoiding unnecessary transfusion risks. Even though, such policies are not yet implemented. In order to evaluate this requirement, our research group designed a questionnaire filled up by 132 volunteer blood donors from Parana Hemotherapy Center (Hemepar) assessing the possible risk factors to toxoplasmic infection. A total of 20 IgG anti-Toxoplasma serological tests were done to check out for positive-reactivity. 60 percent of the selected serum samples reacted positively; from these, 50 percent reported having pets (average of 2 animals per person) and 33 percent of these pets were "semi-free". It suggested the probability of toxoplasmic infection through these animals. Only 2.2 percent of the interviewed individuals were aware of the correct concept of the disease and only 17 percent of them were actually approached by the Blood Center concerning the epidemiological importance of this disease. The current procedures established during blood transfusions are not entirely safe when associated to toxoplasmosis. Today there are no rules that standardize a seroscreening protocol, nor preventive measures for this disease related to hemotherapy services in Brazil, as well as in other services worldwide.

A avaliação da necessidade técnica de implantação de teste sorológico para a detecção de anticorpos anti-Toxoplasma gondii em rotina de serviços de hemoterapia e de uma anamnese com questões ligadas ao ciclo de infecção por Toxoplasma gondii foi feita a partir da aplicação de 132 questionários ao público doador do Centro de Hematologia e Hemoterapia do Paraná (Hemepar) para avaliação dos possíveis fatores de risco ao contágio da toxoplasmose e de 20 testes sorológicos para análise de reatividade positiva a IgG anti-Toxoplasma gondii. Das amostras analisadas sorologicamente, 60 por cento obtiveram reatividade positiva. Destes, 50 por cento possuíam animais domésticos (média de dois animais por pessoa), dos quais 33 por cento dos animais possuia vida semilivre. Após análise das amostras, foi aplicado um estudo de coorte para a formação de um grupo de doadores passíveis de infecção por Toxoplasma gondii e um grupo de não passíveis de infecção, o que não mostrou uma tendência significativa relacionada a fatores de risco. Tal cenário sugere a probabilidade de contágio a partir de animais, porém apenas 2,2 por cento da amostragem conhecia o conceito correto sobre a doença e somente 17 por cento dos doadores foram abordados pelo banco de sangue sobre a importância epidemiológica do parasito. Tais resultados levam a crer que os procedimentos estabelecidos durante as transfusões sangüíneas não se mostram tão seguros, possibilitando o contágio durante uma transfusão. Atualmente, não há nenhuma legislação que estabeleça um protocolo de diagnóstico para esta doença. Este cenário ocorre tanto no Brasil quanto em outros países, não existindo, portanto, a obrigatoriedade da realização dos testes de triagem sorológica para Toxoplasmose por parte dos bancos de sangue.