ABSTRACT The Atlantic Forest is made up of different vegetation types over extensive latitude. Most of its remnants are in mountain ranges, which are responsible for the maintenance of most endemic and threatened species in this domain. Here we present the floristic composition of the Área de Proteção Ambiental Mestre Álvaro (APAMA), a coastal massif at Espírito Santo State with expressive altitudinal amplitude (100-800 a.s.l.) containing remnants never floristically studied. Knowing the composition of this protected area is important because it includes an altitudinal range not yet evaluated in floristic comparisons involving highland and lowland forests between the latitudes 18-21° S. We found 493 species (in 292 genera and 91 families): ten are regional endemic species, nine are new records of occurrence for the state, and 52 are under some degree of threat of extinction. Our results indicate the presence of flora elements' typical of both highland and lowland forests, which makes the APAMA essential in a scenario extremely fragmented. Finally, our data provide an unprecedented floristic list for regional forest restoration projects, as well as an invaluable source of data for biogeographic studies that seek to understand the effect caused by neighboring vegetation types on the composition of lowland forests.

A doença de Gaucher (DG) é um erro inato do metabolismo do grupo das doenças lisossômicas de depósito, sendo a mais freqüente do referido grupo. É de herança autossômica recessiva, portanto com risco de 25% a cada gestação de casal heterozigoto. A doença é resultante da deficiência da beta-glicosidase ácida ou beta-glicocerebrosidase, que leva ao acúmulo de glicolipídios nos macrófagos principalmente em baço, fígado, medula óssea e pulmão. As manifestações clínicas ou fenotípicas da DG vão depender do grau de deficiência da enzima, existindo três tipos: Tipo I, forma não neuropática, afeta crianças e adultos com hepatoesplenomegalia, anemia, trombocitopenia, leucopenia e lesões ósseas; Tipo II, forma neuropática aguda, afeta crianças com 4-5 meses com quadro neurológico grave, hepatoesplenomegalia e comprometimento pulmonar e o Tipo III, forma neuropática crônica, afeta crianças e adolescentes com quadro neurológico menos grave que o Tipo II e ainda pode comprometer fígado, baço e ossos. Um grupo de catorze médicos com experiência no tratamento da DG com reposição enzimática realizaram extensa revisão da literatura, confrontaram com os dados evolutivos dos pacientes brasileiros e chegaram a um consenso quanto aos critérios para iniciar o tratamento, a dose da enzima e freqüência das infusões, do acompanhamento ambulatorial, laboratorial e radiológico. O Grupo Brasileiro de Estudos em Doença de Gaucher e outras Doenças de Depósito Lisossômico (GBDDL) tem o objetivo de estabelecer diretrizes para o diagnostico, tratamento e acompanhamento de pacientes com doença de Gaucher no Brasil. Esta iniciativa pioneira visa uniformizar a conduta no país com relação ao tratamento de DG com reposição enzimática, tratamento de alto custo porém de grande eficácia.

Gaucher disease is one of the inborn errors of metabolism from lysossomal storage diseases and the most frequent of this group. It iss an autosomal recessive disease with reoccurrence risk of 25% in each pregnancy of hetrozygotes parents. The disease results from an b-glucosidase acid or b-glucocerebrosidase deficiency leading to accumulation of glucolipids mainly in the spleen, liver, bone marrow, and lung macrophages. The clinical manifestations of Gaucher disease depend on the enzyme deficiency level. There are three types of the disease, namely, Type I, corresponding to a non-neuropathic form affecting both adults and children with hepatosplenomegaly, anemia, thrombocytopenia, leukopenia, and bone lesions; Type II, corresponding to an acute neuropathic form affecting 4- to 5-month-old babies with a severe neurological condition, hepatosplenomegaly, and lung damage; Type III, corresponding to a chronic neuropathic form affecting both children and teenagers with less severe neurological conditions than Type II, besides being able to cause damage to liver, spleen and bones. A group of fourteen physicians experienced in treating Gaucher disease by enzyme replacement therapy has performed an extensive literature review on the subject and compared data of the evolution of Brazilian patients, having reached a consensus as regards the criteria to start treatment, the enzyme doses and frequency of infusions, as well as medical evaluations and laboratory and radiological monitoring. The purpose of this pioneering initiative is to standardize the conduct within the country, of Gaucher disease treatment using enzyme replacement therapy, which is an expensive treatment but with an expressive efficacy.

Determinaram-se os teores de Mo, Co, Cu, Zn, Mn e Fe solúveis em solução de HC1 6N, a quente, de 33 amostras de materiais usados como corretivos da acidez do solo no Estado de São Paulo, das quais dezesseis eram de calcários sedimentaress, catorze de calcários metamórficos, uma de calcário magmático e duas de escórias de alto forno. Nas amostras de calcários, os teores médios, mínimos e máximos de Mo, Co, Cu, Zn, Mn e Fe, expressos em ppm, foram respectivamente de 0,9 (0,1-5,0), 4,2 (0-46,9), 11,0 (5,4-42,2), 15,2 (7,5-46,0), 810 (30-1760) e 3905 (159-33.110). Nas duas amostras de escórias analisadas, os resultados obtidos foram: Mo 2,5 e 5,3 ppm; Co 2,6 e 11,4 ppm; Cu 2025 e 7800 ppm; Zn 750 e 1250 ppm; Mn 8100 e 38600 ppm e Fe 4522 e 127150 ppm.

Determinations of Mo, Co, Cu, Zn, Mn, and Fe soluble in hot 6N HC1 were carried out on 31 limestones and 2 siderurgy slags employed in the neutralisation of soil acidity in the State of São Paulo. In the limestones, the average, minimum and maximum contents of Mo, Co, Cu, Zn, Mn and Fe, expressed in ppm were: 0.9(0.1-5.0); 4.2(0-46.9); 11.0(5.4-42.2); 15.2(7.5-46.0); 810(30-1760) and 3905(159-33,110). In the two analysed siderurgy slags samples the contents obtained were: Mo 2.5 and 5.3 ppm; Co 2.6 and 11.4 ppm; Cu 2025 and 7800 ppm; Zn 750 and 1250 ppm; Mn 8100 and 38,600 ppm and Fe 4522 and 127,150 ppm.