Neuronal Ceroid-lipofuscinosis with prominent chorea and without visual manifestations: a case report

A case of neuronal ceroid-lipofuscinosis (NCL) is reported in a 11-year-old girl, whose main symptoms were progressive dementia since the age of 4 years and choreic movements since age 10. Seizures, myoclonus and visual deterioration were absent and optic fundi were normal. A cerebral biopsy disclosed two basic types of stored substance in the cytoplasm of neurons: a) severely balloned nerve cells in cortical layers HI and V contained a non-autofluorescent material, which stained with PAS and Sudan Black B in frozen, but not in paraffin sections; ultrastructurally, these neurons showed abundant corpuscles similar to the membranous cytoplasmic bodies of Tay-Sachs disease and, in smaller amounts, also zebra bodies; b) slightly distended or non-distended neurons in all layers contained lipopigment granules, which were autofluorescent, PAS-positive and sudanophil in both frozen and paraffin sections; their ultrastructure was closely comparable to that of lipofuscin. Similar bodies were found in the swollen segments of axons and in a few astrocytes and endothelial cells. The histochemical and ultrastructural demonstration of large amounts of lipopigments allows a presumptive classification of the case as NCL. However, the presence of involuntary movements, the absence of visual disturbances and the unusual ultrastructural features place the patient into a small heterogeneous group within the NCL. A better classification of such unique instances of the disease must await elucidation of the basic enzymatic defects.

Qualidade de vida e avaliação fisica em intoxicados por mercurio : estudo observacional transversal descritivo

Universidade Estadual de Campinas . Faculdade de Educação Física

Novo protocolo para as ações de saúde bucal coletiva: padronização no armazenamento, distribuição e uso do material de higiene bucal

Este trabalho avaliou o material de higiene bucal usado em escolas para estabelecer um protocolo às ações de higiene bucal coletiva. O estudo foi dividido em duas etapas: 1ª- 20 responsáveis pelos procedimentos coletivos com escolares de Bauru e São José dos Campos - SP responderam a dois questionários sobre o uso de cinco kits de higiene bucal coletiva. A análise estatística foi realizada através do teste Wilcoxon (p < 0,05); 2ª - 178 escolares de 4 a 8 anos de Bauru e Bariri-SP dispensaram na escova uma quantidade de creme dental e dentifrício líquido para a prática da escovação, a qual foi pesada através de uma balança portátil. A análise estatística foi obtida através do coeficiente de correlação de Pearson e a análise de covariância (p< 0,05). O kit 5 obteve graus de satisfação e muita satisfação quando comparado aos kits 1 a 4. A quantidade de creme dental dispensada pelos escolares foi em média 0,41g (Bauru) e 0,48g (Bariri). Não houve diferença estatística entre os escolares de Bauru e Bariri em relação ao dentifrício líquido (média de 0,15g). O dentifrício líquido, através da "técnica da gota", foi considerado prático, dispensando uma pequena quantidade padronizada. O kit 5 demonstrou ser uma boa alternativa ao estabelecimento de um protocolo de ações em saúde bucal coletiva no SUS.

An in vitro comparison of nickel and chromium release from brackets

This study aimed at comparing amounts of nickel (Ni) and chromium (Cr) released from brackets from different manufacturers in simulated oral environments. 280 brackets were equally divided into 7 groups according to manufacturer. 6 groups of brackets were stainless steel, and 1 group of brackets was made of a cobalt-chromium alloy with low Ni content (0.5%). International standard ISO 10271/2001 was applied to provide test methods. Each bracket was immersed in 0.5 ml of synthetic saliva (SS) or artificial plaque fluid (PF) over a period of 28 days at 37ºC. Solutions were replaced every 7 days, and were analyzed by spectrometry. The Kruskal-Wallis test was applied. Amounts of Ni release in SS (µg L-1 per week) varied between groups from "bellow detection limits" to 694, and from 49 to 5,948.5 in PF. The group of brackets made of cobalt-chromium alloy, with the least nickel content, did not release the least amounts of Ni. Amounts of Cr detected in SS and in PF (µg L-1 per week) were from 1 to 10.4 and from 50.5 to 8,225, respectively. It was therefore concluded that brackets from different manufacturers present different corrosion behavior. Further studies are necessary to determine clinical implications of the findings.

Effect of Potato virus X on total phenol and alkaloid contents in Datura stramonium leaves

The present paper reports results of the effect of Potato virus X (PVX) on the contents of total phenols and alkaloids in leaves of Datura stramonium. A significant decrease in the contents of phenols and alkaloids was observed in leaves inoculated with PVX (X-I). However, there was an increase in the percentage of phenols in leaves rubbed with phosphate buffer (C1-I) and in leaves from the nodes immediately above, possibly induced by mechanical injury. Gas chromatography/mass spectroscopy revealed amounts of scopolamine in samples submitted to all treatments, except X-I, in which the amount of this alkaloid was low. High amounts of an unidentified compound (molecular ion m/z 302 and a prominent peak at m/z 129) were noted in extracts from leaves X-I, C1-I and leaves from the nodes immediately above the leaves inoculated with PVX. It is suggested that the synthesis and accumulation of the unidentified compound is a result of stress from mechanical injury and virus inoculation.

Anomalous somatic embryos in Acca sellowiana (O. Berg) Burret (Myrtaceae)

Somatic embryogenesis represents a valuable tool for the studies on the basic aspects of plant embryo development. Today this process is used as a potencial technique for large-scale plant micropropagation although, so far, it has been applied to only a small number of species. However, when somatic embryos are malformed they are considered economically useless. In Acca sellowiana (O. Berg) Burret, an important fruit-producing crop, large amounts of anomalous somatic embryos (76.3%) were found just after 40 days of culture of explants in a 2,4-D containing medium. Among the anomalous forms found in the cotiledonary stage, 12.2% consisted of fused embryos, 40.4% displayed fused cotyledons, 13.0% presented supernumerary cotyledons, and 10.7% showed absence or poorly developed cotyledons, including those without the shoot apical meristem. Histological analyses indicated that the altered embryos were formed either directly from cotyledons, hypocotyl and radicle of the zygotic embryos used as explants, or indirectly from calli formed from these tissue parts. It is suggested that the formation of anomalous somatic embryos, as well as a low frequency of conversion into emblings reflect physiological and/or genetic disturbances triggered by the presence of 2,4-D in the medium. In vitro experimental alternative approaches are discussed in order to lessen the occurrence of malformed somatic embryos.

Reserve carbohydrates and lipids from the seeds of four tropical tree species with different sensitivity to desiccation

Considering the importance of water content for the conservation and storage of seeds, and the involvement of soluble carbohydrates and lipids for embryo development, a comparative study was carried out among the seeds of Inga vera (ingá), Eugenia uniflora (pitanga), both classified as recalcitrant, and Caesalpinia echinata (brazilwood) and Erythrina speciosa (mulungu), considered as orthodox seeds. Low concentrations of cyclitols (0.3-0.5%), raffinose family oligosaccharides (ca. 0.05%) and unsaturated fatty acids (0-19%) were found in the seeds of ingá and pitanga, while larger amounts of cyclitols (2-3%) and raffinose (4.6-13%) were found in brazilwood and mulungu, respectively. These results, in addition to higher proportions of unsaturated fatty acids (53-71%) in orthodox seeds, suggested that sugars and lipids played important role in water movement, protecting the embryo cell membranes against injuries during dehydration.

Avaliação da influência de substâncias fenólicas e carotenoides na anomalia do epicarpo da goiaba, "anelamento"

A anomalia do epicarpo da goiaba, comumente relatada por agricultores e técnicos como o "anelamento juvenil da goiaba", tem causado preocupação devido à desinformação sobre o assunto. O objetivo deste estudo foi analisar quimicamente as concentrações de substâncias fenólicas e carotenoides na região do epicarpo de goiabas afetadas pelo "anelamento", visando a caracterizar essa anomalia previamente relatada. Foram analisadas substâncias fenólicas (taninos, flavonas/flavonóis, antocianinas e fenóis totais) e carotenoides em epicarpos de frutos verdes e maduros de goiabeiras cv. Paluma, com e sem anomalia. O delineamento experimental adotado foi o inteiramente casualizado, sendo estabelecidos seis tratamentos com o epicarpo dos frutos maduro sem anomalia na região inferior (FMSI); frutos maduros sem injuria na região superior (FMSS); frutos verdes sem anomalia na região inferior (FVSI); frutos verdes sem anomalia na região superior (FVSS); frutos verdes com anomalia na região inferior (FVCI); frutos verdes com anomalia na região superior (FVCS). Dentre as substâncias analisadas, os carotenoides, os taninos e os fenóis totais mostram indicativos para a caracterização do anelamento. Tanto substâncias fenólicas quanto carotenoides apresentam propriedades antioxidantes e, dessa forma, poderiam estar relacionadas à defesa antioxidante causada por um fator de estresse ainda desconhecido, que promove o "anelamento" característico apresentado pelas goiabas.

Seasonal variation of soluble carbohydrates and starch in Echinolaena inflexa, a native grass species from the Brazilian savanna, and in the invasive grass Melinis minutiflora

Echinolaena inflexa (Poir.) Chase is an abundant C3 grass species with high biomass production in the Brazilian savanna (cerrado); Melinis minutiflora Beauv. is an African C4 forage grass widespread in cerrado and probably displacing some native herbaceous species. In the present work, we analysed seasonally the content and composition of soluble carbohydrates, the starch amounts and the above-ground biomass (phytomass) of E. inflexa and M. minutiflora plants harvested in two transects at 5 and 130 m from the border in a restrict area of cerrado at the Biological Reserve and Experimental Station of Mogi-Guaçu (SP, Brazil). Results showed that water soluble carbohydrates and starch amounts from the shoots of both species varied according to the time of the year, whilst in the underground organs, variations were observed mainly in relation to the transects. Marked differences in the pattern of the above-ground biomass production between these two grasses relative to their location in the Reserve were also observed, with two peaks of the invasive species (July and January) at the Reserve border. The differences in carbohydrate accumulation, partitioning and composition of individual sugars concerning time of the year and location in the Reserve were more related to the annual growth cycle of both grasses and possibly to specific physiological responses of M. minutiflora to disturbed environments in the Reserve border.

Culex quinquefasciatus vitellogenesis: morphological and biochemical aspects

The vitellogenic process in Culex quinquefasciatus, which is triggered by a blood meal, involves the synthesis, distribution and storage of the nutrients necessary for embryo development. The fat body of an adult female Cx. quinquefasciatus revealed two cell types: large trophocytes and small, eosinophilic, "oenocyte-like" cells, which show no morphological changes throughout the gonotrophic cycle. Trophocytes, which only begin to synthesise vitellogenin (Vg) 12 h post-blood meal (PBM), undergo a series of morphological changes following engorgement. These changes include the expansion of the rough endoplasmic reticulum (RER) and Golgi complex, which are later destroyed by autophagosomes. At 84 h PBM, trophocytes return to their pre-engorgement morphology. The ovarian follicles of non-blood-fed Cx. quinquefasciatus contain a cluster of eight undifferentiated cells surrounded by follicular epithelium. After engorgement, the oocyte membrane facing the perioocytic space increases its absorptive surface by microvilli development; large amounts of Vg and lipids are stored between 24 and 48 h PBM. Along with yolk storage in the oocyte, follicular cells exhibit the development of RER cisternae and electron-dense granules begin to fill the perioocytic space, possibly giving rise to endochorion. Later in the gonotrophic cycle, electron-dense vesicles, which are possible exochorion precursors, fuse at the apical membrane of follicular cells. This fusion is followed by follicular cell degeneration.

Mineralogical characterization of a highly-weathered soil by the Rietveld Method

The mineralogical characterization through mineral quantification of Brazilian soils by X-ray diffraction data using the Rietveld Method is not common. A mineralogical quantification of an Acric Ferralsol from the Ponta Grossa region, state of Paraná, Brazil, was carried out using this Method with X-Ray Diffraction data to verify if this method was suitable for mineral quantification of a highly-weathered soil. The A, AB and B3 horizons were fractioned to separate the different particle sizes: clay, silt, fine sand (by Stokes Law) and coarse sand fractions (by sieving), with the procedure free of chemical treatments. X-ray Fluorescence, Inductively Coupled Plasma Atomic Emission Spectrometry, Infrared Spectroscopy and Mössbauer Spectroscopy were used in order to assist the mineral identification and quantification. The Rietveld Method enabled the quantification of the present minerals. In a general way, the quantitative mineralogical characterization by the Rietveld Method revealed that quartz, gibbsite, rutile, hematite, goethite, kaolinite and halloysite were present in the clay and silt fractions of all horizons. The silt fractions of the deeper horizons were different from the more superficial ones due to the presence of large amounts of quartz. The fine and the coarse sand fractions are constituted mainly by quartz. Therefore, a mineralogical quantification of the finer fraction (clay and silt) by the Rietveld Method was successful.

Filmes ultrafinos de ésteres de celulose: preparo, caracterização e imobilização de proteínas

In this study cellulose acetate butyrate (CAB) and carboxymehtylcellulose acetate butyrate (CMCAB) films adsorbed onto silicon wafers were characterized by means of ellipsometry, atomic force microscopy (AFM), sum frequency generation spectroscopy (SFG) and contact angle measurements. The adsorption behavior of lysozyme (LIS) or bovine serum albumin (BSA) onto CAB and CMCAB films was investigated. The amounts of adsorbed LIS or BSA onto CMCAB films were more pronounced than those onto CAB films due to the presence of carboxymethyl group in the CMCAB structure. Besides, the adsorption of BSA molecules on CMCAB films was more favored than that of LIS molecules. Antimicrobial effect of LIS bound to CAB or CMCAB layers was evaluated using Micrococcus luteus as substrate.

Chemical composition and acetylcholinesterase inhibitory activity of essential oils of Myrceugenia myrcioides(Cambess.) O. Berg and Eugenia riedelianaO. Berg, Myrtaceae

The chemical composition of volatile oils from two Myrtaceae species, Myrceugenia myrcioidesand Eugenia riedeliana, both native from the Brazilian Atlantic Rain Forest, was analyzed by GC-MS. Acetylcholinesterase inhibitory activity was colorimetrically evaluated for these oils. For M. myrcioides, monoterpene hydrocarbons represented the major class in the volatile oil, with α-pinene as the most abundant component and a weak inhibitory activity was observed, whilst for E. riedeliana sesquiterpenes were found in higher amounts, being valerianol the major compound, and this oil presented a strong acetylcholinesterase inhibition.

Composição química dos frutos de Campomanesia xanthocarpa Berg-Myrtaceae

Frutos in natura de C. xanthocarpa (Myrtaceae), coletados em diferentes estádios de amadurecimento na Floresta Estadual de Assis, município de Assis - SP, foram avaliados por meio de métodos tradicionais de análise, técnicas cromatográficas e de espectrometria de massas e de emissão ótica quanto à composição nutricional, ao perfil químico do óleo volátil e ao teor de elementos inorgânicos. Os resultados mostraram alto teor de água (81,4%); lipídios (1,9%); carboidratos totais (8,9%); fibra alimentar (6,3%); além de quantidades razoáveis de ácido ascórbico (17,8 mg.100 g-1); e traços de riboflavina (0,09 mg.100 g-1). No óleo volátil (0,2%), pôde-se identificar 62 componentes, correspondendo a 100% dos constituintes do óleo, destacando-se dentre eles os monoterpenos α-pineno (15%), o-cimeno (10,8%), β-pineno (10,5%). Entre os minerais (16), os principais elementos foram o K (2084 mg.kg-1), P (149 mg.kg-1), Mg (135 mg.kg-1) e, como microelementos, o Fe (6,4 mg.kg-1), Cu (93,3 mg.100 g-1) e Pb (1,3 mg.kg-1). O valor energético do fruto (57,3 kcal.100 g-1) deve-se quase que exclusivamente aos teores de carboidratos totais (8,9%).

Nutritional aspects of the prevention and treatment of osteoporosis

Osteoporosis is a global health problem characterized by low bone mass and microarchitectural deterioration of bone tissue with a consequent increase in bone fragility and susceptibility to fracture. Nutrition plays a critical role in reducing the risk of osteoporosis through its effect on all of these fragility factors, especially on the development and maintenance of bone mass. An adequate calcium, vitamin D and protein intake resulted in reduced bone remodeling, better calcium retention, reduced age-related bone loss, and reduced fracture risk. Recent evidence indicates that a healthy dietary pattern including dairy products (mainly fat free), fruit and vegetables and adequate amounts of meat, fish and poultry is positively related to bone health. Furthermore, mineral and vitamin supplementation should be closely monitored by health professionals since it could have adverse effects and be insufficient to ensure optimal protection of bone health.