Friedreich's Ataxia: Cardiac Evaluation of 25 Patients with Clinical Diagnosis and Literature Review

OBJECTIVE - Cardiac evaluation (clinical, electrocardiographic and echocardiographic) of 25 Brazilian patients with clinical diagnosis of Friedreich's ataxia (FA) related to the frequency and the size of GAA repeats (unstable expansion of trinucleotide repeats that results in the disease). METHODS - Clinical and cardiac study including electrocardiogram and echocardiogram of all patients and molecular analysis to detect the frequency and the size of GAA expansion, by polymerase chain reaction analysis. RESULTS - Homozygous GAA expansion was detected in 17 patients (68%) -- all typical cases. In 8 (32%) cases (6 atypical and 2 typical), no GAA expansion was observed, therefore it was not considered Friedreich's ataxia. All patients with GAA expansion (100%) had electrocardiographic abnormalities, and only 25% of the cases without GAA expansion had some abnormality on this exam. However, only 6% of all patients revealed some signals/symptoms suggestive of cardiac involvement. CONCLUSION - A molecular analysis is essential to confirm the diagnosis of Friedreich's ataxia; however, an adequate cardiac evaluation, including an electrocardiogram, was extremely useful to better screening the patients which should perform these molecular analysis.

Cardiac Damage from Chronic Use of Chloroquine: A Case Report and Review of the Literature

Chloroquine has been widely used in rheumatological treatment, but potential severe side effects require careful follow-up. Cardiac damage is not a common consequence, but its clinical relevance has not yet been described. We report the case of a 58-year-old woman with rheumatoid arthritis, in whom chronic chloroquine use resulted in major irreversible cardiac damage. She presented with syncopal episodes due to complete atrioventricular block confirmed by electrophysiological study whose changes were concluded to be irreversible and a permanent pacemaker was indicated. Endomyocardial biopsy was also performed to search for histopathological and ultrastructural cardiac damage. We also reviewed the 22 cases of chloroquine-induced cardiopathy described to date as well as its pathophysiology.

Cardiovascular Research Publications from Latin America between 1999 and 2008. A Bibliometric Study

Background:Cardiovascular research publications seem to be increasing in Latin America overall.Objective:To analyze trends in cardiovascular publications and their citations from countries in Latin America between 1999 and 2008, and to compare them with those from the rest of the countries.Methods:We retrieved references of cardiovascular publications between 1999 and 2008 and their five-year post-publication citations from the Web of Knowledge database. For countries in Latin America, we calculated the total number of publications and their citation indices (total citations divided by number of publications) by year. We analyzed trends on publications and citation indices over time using Poisson regression models. The analysis was repeated for Latin America as a region, and compared with that for the rest of the countries grouped according to economic development.Results:Brazil (n = 6,132) had the highest number of publications in1999-2008, followed by Argentina (n = 1,686), Mexico (n = 1,368) and Chile (n = 874). Most countries showed an increase in publications over time, leaded by Guatemala (36.5% annually [95%CI: 16.7%-59.7%]), Colombia (22.1% [16.3%-28.2%]), Costa Rica (18.1% [8.1%-28.9%]) and Brazil (17.9% [16.9%-19.1%]). However, trends on citation indices varied widely (from -33.8% to 28.4%). From 1999 to 2008, cardiovascular publications of Latin America increased by 12.9% (12.1%-13.5%) annually. However, the citation indices of Latin America increased 1.5% (1.3%-1.7%) annually, a lower increase than those of all other country groups analyzed.Conclusions:Although the number of cardiovascular publications of Latin America increased from 1999 to 2008, trends on citation indices suggest they may have had a relatively low impact on the research field, stressing the importance of considering quality and dissemination on local research policies.

Influência do cloro, sôbre a composição do caldo da cana de açúcar Co 290, aplicado no solo, na forma de cloreto de sódio

Considering the economic importance of the sugar industry among ourselves, the authors carried out a field experiment (Latin square) with Co 290 sugar cane, on a white sandy soil of Piracicaba, State of São Paulo, Brazil, applying NaCl in increasing rates (from 6.8 to 54.5 grams per plant), in order to study the effects of chlorides, on productivity and on the composition of juice. No toxic or stimulating effect was found, and there was no change in yield, in degree of purity of the juice, in general aspect of plants or in colour of leaves and culms. No difference was observed between potassium sulphate or chloride, as source of potash for sugar cane culture. Data collected and the literature cited suggest: (a) that the use of the variety Co 290 is indicated for soils rich in chlorine, such as the saline soils of the North-east and Atlantic Coast of Brazil; (b) that it is necessary to extend studies in Research Institutes and Agricultural Experiment Stations of the country to verify the behaviour of other varieties of sugar cane in the types of soils mentioned, especially with respect their yielding capacity. The authors are already planning such investigations.

Biologia da Arsenura xanthopus (Walker, 1855) ( Lepidoptera - Adelocephalidae ), praga do «Açoita-Cavalo» (Luehea spp.) e notas sôbre seus inimigos naturais

Biology of Arsenura xanthopus (Walker, 1855) (Lep., Adelocephalidae), a pest of Luehea spp. (Tiliaceae), and notes on its natural enemies. In the beginning of 1950, one of the Authors made some observations about the biology of Arsenura xanthopus (Walker), in Piracicaba, State of S. Paulo, Brazil. From 1951 to 1953, both Authors continued the observations on such an important Adelocephalidae, the caterpillars of which represent a serious pest of Luehea spp. leaves. Actually, in some occasions, the caterpillars can destroy completely the leaves of the trees. The species is efficientely controlled by two natural enemies: an egg parasite (Tetrastichus sp., Hym., Eulophidae) and a fly attacking the last instar caterpillar (Winthemia tricolor (van der Wulp), Dip., Tachinidae). Tetrastichus sp. can destroy 100% of the eggs and the fly, 70 to 100% of the caterpillars. Indeed, facts as such are very interesting because we rarely know of a case of so complete a control of a pest by an insect. A. xanthopus had not yet been mentioned in our literature. Actually neither the systematic bibliography nor the economic one has treated of this species. However, a few other species of Arsenura are already known as living on Luehea spp. According to the Authors' observations, W. tricolor was also unknown by the Brazilian entomological literature. Arsenura xanthopus (Walker, 1855) After giving the sinonimy and a few historical data concerning the species, and its geographical distribution, the Authors discuss its placing in the genus Arsenura Duncan or Rhescyntis Huebner, finishing by considering Arsenura xanthopus as a valid name. The Authors put the species in the family Adelocephalidae, as it has been made by several entomologists. The host plant The species of Tiliaceae plants belonging to the genus Luehea are called "açoita-cavalo" and are well known for the usefulness of their largely utilized wood. The genus comprises exclusively American plants, including about 25 species distributed throughout the Latin America. Luehea divaricata Mart, is the best known species and the most commonly cultivated. Biology of Arsenura xanthopus Our observations show that the species passes by 6 larval stages. Eggs and egg-postures, all the 6 instars of the caterpillars as well as the chrysalid are described. The pupal period is the longest of the cycle, taking from 146 to 256 days. Data on the eclosion and habits of the caterpillars are also presented. A redescription of the adult is also given. Our specimens agreed with BOUVIER's description, except in the dimension between the extremities of the extended wings, which is a little shorter (107 mm according to BOUVlErVs paper against from 80 to 100mm in our individuals). Winthemia tricolor (van der Wulp, 1890) Historical data, geographical distribution and host are first related. W. tricolor had as yet a single known host-; Ar^-senura armida (Cramer). This chapter also contains some observations on the biolcn gy of the fly and on its behaviour when trying to lay eggs on the caterpillars' skin. The female of W. tricolor lays from 1 to 33 eggs on the skin of the last instar caterpillar. The mam region of the body where the eggs are laid are the membranous legs. Eggs are also very numerous oh the ventral surface of the thorax and abdomen. The. preference for such regions is easily cleared up considering the position assumed by the caterpillar when fixed motionless in a branch. In such an occasion, the fly approaches, the victim, puts the ovipositor out and lays the eggs on different parts of the body, mainly on the mentioned regions, which are much more easily reached. The eggs of the fly are firmly attached to the host's skin, being almost impossible to detach them, without having them broken. The minute larvae of the fly enter the body of, the host when it transforms into chrysalid. Chrysalids recentely formed and collected in nature f requentely show a few small larvae walking on its skin and looking for an adequate place to get into the body. A few larvae die by remaining in the skin of the caterpillar which is pushed away to some distance by the active movements of the chrysalid recentely formed. From 1 to 10 larvae completely grown may emerge from the attacked chrysalid about 8 days after their penetrating into the caterpillars' body and soon begin to look for an adequate substratum where they can transform themselves into pupae. In natural conditions, the metamorphosis occurs in the soil. The flies appear within 15 days. Tetrastichus sp. This microhymenoptera is economically the most interesting parasite, being commonly able to destroy the whole pos^ ture of the moth. Indeed, some days after the beginning of the infestation of the trees, it is almost impossible to obtain postures completely free of parasites. The active wasp introduces the ovipositor into the egg of the moth, laying its egg inside, from 80 to 120 seconds after having introduced it. A single adult wasp emerges from each egg. Sarcophaga lambens Wiedemann, 1830 During the observations carried out, the Authors obtained 10 flies from a chysalid that were recognized as belonging to the species above. S. lambens is a widely distributed Sarcophagidae, having a long list of hosts. It is commonly obtained from weak or died invertebrates, having no importance as one of their natural enemies. Sinonimy, list of hosts and distribution are presented in this paper. Control of Arsenura xanthopus A test has been carefully made in the laboratory just to find out the best insecticide for controlling A. xanthopus caterpillars. Four different products were experimented (DDT, Pa-rathion, BHC and Fenatox), the best results having been obtained with DDT at 0,25%. However, the Authors believe in spite of the initial damages of the trees, that the application of an insecticide may be harmful by destroying the natural agents of control. A biological desiquilibrium may in this way take place. The introduction of the parasites studied (Tetrastichus sp. and Winthemia tricolor) seems to be the most desirable measure to fight A. xanthopus.

Global literature of fiddler crabs, genus Uca (Decapoda, Ocypodidae): trends and future directions

Crabs of the genus Uca Leach, 1814 are characterized by having strong sexual dimorphism and a global distribution. Currently, 97 species have been described and analyzed under several aspects, including population ecology, physiology and ethology. However, there is no general summary of the information from the various literatures. The aim of this study is to perform a scientometric analysis of fiddler crab studies. For this we searched papers available in the Thomson ISI database that contained the words "Uca" OR "fiddler* crab*" between the years 1991 and 2007. For each paper, we researched and recorded the following characteristics: publication year; journal of publication; the first author's nationality; the country where the study was conducted; study type; species studied; and the work area. Our results indicated that there was no increase in the number of articles through the years considered. The Journal of Experimental Marine Biology and Ecology published most of the articles on Uca, indicating the importance of this group as a model for testing ecological hypotheses using experimental approaches. Our results also showed that United States had the highest number of authors and published studies on Uca, following the overall trend in dominance on scientific research. Furthermore, using models with three variables (per capita income, number of species of Uca and extent of coastal countries) we observed that, according to the Akaike Information Criterion, the per capita income was the most important correlate for the number of articles per country (both the author's country and country of study). Additionally, our results show that the species U. pugilator (distributed on the East Coast of the North American continent) was the species most singularly referenced in the papers considered. Moreover, our results indicate that most studies on Uca use a descriptive and local scale. The majority of papers in our literature search reflect studies in population biology, followed by behavioral and physiological characteristics.

Prevention of transfusional Trypanosoma cruzi infection in Latin America

Trypanosoma cruzi is a protozoan infection widely spread in Latin America, from Mexico in the north to Argentina and Chile in the south. The second most important way of acquiring the infection is by blood transfusion. Even if most countries of Latin America have law/decree/norms, that make mandatory the screening of blood donors for infectious diseases, including T. cruzi (El Salvador and Nicaragua do not have laws on the subject), there is usually no enforcement or it is very lax. Analysis of published serologic surveys of T. cruzi antibodies in blood donors done in 1993, indicating the number of donors and screening coverage for T. cruzi in ten countries of Central and South America indicated that the probability of receiving a potentially infected transfusion unit in each country varied from 1,096 per 10,000 transfusions in Bolivia, the highest, to 13.02 or 13.86 per 10,000 transfusions in Honduras and Venezuela respectively, where screening coverage was 100%. On the other hand the probability of transmitting a T. cruzi infected unit was 219/10,000 in Bolivia, 24/10,000 in Colombia, 17/10,000 in El Salvador, and around 2-12/10,000 for the seven other countries. Infectivity risks defined as the likelihood of being infected when receiving an infected transfusion unit were assumed to be 20% for T. cruzi. Based on this, estimates of the absolute number of infections induced by transfusion indicated that they were 832, 236, and 875 in Bolivia, Chile and Colombia respectively. In all the other countries varied from seven in Honduras to 85 in El Salvador. Since 1993, the situation has improved. At that time only Honduras and Venezuela screened 100% of donors, while seven countries, Argentina, Colombia, El Salvador, Honduras, Paraguay, Uruguay and Venezuela, did the same in 1996. In Central America, without information from Guatemala, the screening of donors for T. cruzi prevented the transfusion of 1,481 infected units and the potential infection of 300 individuals in 1996. In the same year, in seven countries of South America, the screening prevented the transfusion of 36,017 infected units and 7, 201 potential cases of transfusional infection.

Chagas infection transmission control: situation of transfusional transmission in Brazil and other countries of Latin America

The transmission of the transfusion-associated Chagas disease is an important mechanism of its dissemination in several Latin American countries. The transmission risk depends on five factors: prevalence of infection in blood donors, degree of serological coverage, sensibility of used tests, safety of obtained results and infection risk. The Southern Cone Iniciative set off by the Pan-American Health Organization, in 1991, is contributing to the implementation of blood law in each endemic country, and to reduce the risk of transfusional transmission of this horrible disease. Despite the clear improvement of Brasilian hemotherapy after 1980 (with the creation of the Blood National Program - Pró-Sangue) and the significant reduction of the chagasic infection among its blood donors; socio-economic, politic and cultural unlevels, prevent it from reaching the necessary universality and security. In order to assure both, the Brazilian Ministry of Health decided to restructure its blood system. In May, 1998, a great program was launched, to reach a specific goal: Blood - 100% with quality safety in all its process until 2003. It was divided in 12 projects, intends to guarantee the quality and self sufficiency in blood and hemoderivates.

The impact of Chagas disease control in Latin America: a review

Discovered in 1909, Chagas disease was progressively shown to be widespread throughout Latin America, affecting millions of rural people with a high impact on morbidity and mortality. With no vaccine or specific treatment available for large-scale public health interventions, the main control strategy relies on prevention of transmission, principally by eliminating the domestic insect vectors and control of transmission by blood transfusion. Vector control activities began in the 1940s, initially by means of housing improvement and then through insecticide spraying following successful field trials in Brazil (Bambui Research Centre), with similar results soon reproduced in São Paulo, Argentina, Venezuela and Chile. But national control programmes only began to be implemented after the 1970s, when technical questions were overcome and the scientific demonstration of the high social impact of Chagas disease was used to encourage political determination in favour of national campaigns (mainly in Brazil). Similarly, large-scale screening of infected blood donors in Latin America only began in the 1980s following the emergence of AIDS. By the end of the last century it became clear that continuous control in contiguous endemic areas could lead to the elimination of the most highly domestic vector populations - especially Triatoma infestans and Rhodnius prolixus - as well as substantial reductions of other widespread species such as T. brasiliensis, T. sordida, and T. dimidiata, leading in turn to interruption of disease transmission to rural people. The social impact of Chagas disease control can now be readily demonstrated by the disappearance of acute cases and of new infections in younger age groups, as well as progressive reductions of mortality and morbidity rates in controlled areas. In economic terms, the cost-benefit relationship between intervention (insecticide spraying, serology in blood banks) and the reduction of Chagas disease (in terms of medical and social care and improved productivity) is highly positive. Effective control of Chagas disease is now seen as an attainable goal that depends primarily on maintaining political will, so that the major constraints involve problems associated with the decentralisation of public health services and the progressive political disinterest in Chagas disease. Counterbalancing this are the political and technical cooperation strategies such as the "Southern Cone Initiative" launched in 1991. This international approach, coordinated by PAHO, has been highly successful, already reaching elimination of Chagas disease transmission in Uruguay, Chile, and large parts of Brazil and Argentina. The Southern Cone Initiative also helped to stimulate control campaigns in other countries of the region (Paraguay, Bolivia, Peru) which have also reached tangible regional successes. This model of international activity has been shown to be feasible and effective, with similar initiatives developed since 1997 in the Andean Region and in Central America. At present, Mexico and the Amazon Region remain as the next major challenges. With consolidation of operational programmes in all endemic countries, the future focus will be on epidemiological surveillance and care of those people already infected. In political terms, the control of Chagas disease in Latin America can be considered, so far, as a victory for international scientific cooperation, but will require continuing political commitment for sustained success.

Species distribution and antifungal susceptibility profile of Candida spp. bloodstream isolates from Latin American hospitals

From March 1999 to March 2000, we conducted a prospective multicenter study of candidemia involving five tertiary care hospitals from four countries in Latin America. Yeast isolates were identified by classical methods and the antifungal susceptibility profile was determined according to the National Committee for Clinical Laboratory Standards microbroth assay method. During a 12 month-period we were able to collect a total of 103 bloodstream isolates of Candida spp. C. albicans was the most frequently isolated species accounting for 42% of all isolates. Non-albicans Candida species strains accounted for 58% of all episodes of candidemia and were mostly represented by C. tropicalis (24.2%) and C. parapsilosis (21.3%). It is noteworthy that we were able to identify two cases of C. lusitaniae from different institutions. In our casuistic, non-albicans Candida species isolates related to candidemic episodes were susceptible to fluconazole. Continuously surveillance programs are needed in order to identify possible changes in the species distribution and antifungal susceptibility patterns of yeasts that may occurs after increasing the use of azoles in Latin American hospitals.

Prehistoric tuberculosis in America: adding comments to a literature review

Tuberculosis is a prehistoric American human disease. This paper reviews the literature and discusses hypotheses for origins and epidemiological patterns of prehistoric tuberculosis. From the last decades, 24 papers about prehistoric tuberculosis were published and 133 cases were reviewed. In South America most are isolated case studies, contrary to North America where more skeletal series were analyzed. Disease was usually located at the deserts of Chile and Peru, Central Plains in USA, and Lake Ontario in Canada. Skeletal remains represent most of the cases, but 16 mummies have also been described. Thirty individuals had lung disease, 19 of them diagnosed by the ribs. More then 100 individuals had osseous tuberculosis and 26 also had it in other organs. As today, transmission of the infection and establishment of the disease were favored by cultural and life-style changes such as sedentarization, crowding, undernutrition, use of dark and insulated houses, and by the frequency of interpersonal contacts. The papers confirm that despite previous perceptions, tuberculosis seems to have occured in America for millennia. It only had epidemiological expression when special conditions favored its expansion. Occurring as epidemic bursts or low endemic disease, it had differential impact on groups or social segments in America for at least two millennia.

Antimicrobial susceptibility patterns of unusual nonfermentative gram-negative bacilli isolated from Latin America: report from the SENTRY Antimicrobial Surveillance Program (1997-2002)

The antimicrobial susceptibility of 176 unusual non-fermentative gram-negative bacilli (NF-GNB) collected from Latin America region through the SENTRY Program between 1997 and 2002 was evaluated by broth microdilution according to the National Committee for Clinical Laboratory Standards (NCCLS) recommendations. Nearly 74% of the NF-BGN belonged to the following genera/species: Burkholderia spp. (83), Achromobacter spp. (25), Ralstonia pickettii (16), Alcaligenes spp. (12), and Cryseobacterium spp. (12). Generally, trimethoprim/sulfamethoxazole (MIC50, < 0.5 µg/ml) was the most potent drug followed by levofloxacin (MIC50, 0.5 µg/ml), and gatifloxacin (MIC50, 1 µg/ml). The highest susceptibility rates were observed for levofloxacin (78.3%), gatifloxacin (75.6%), and meropenem (72.6%). Ceftazidime (MIC50, 4 µg/ml; 83.1% susceptible) was the most active beta-lactam against B. cepacia. Against Achromobacter spp. isolates, meropenem (MIC50, 0.25 µg/ml; 88% susceptible) was more active than imipenem (MIC50, 2 µg/ml). Cefepime (MIC50, 2 µg/ml; 81.3% susceptible), and imipenem (MIC50, 2 µg/ml; 81.3% susceptible) were more active than ceftazidime (MIC50, >16 µg/ml; 18.8% susceptible) and meropenem (MIC50, 8 µg/ml; 50% susceptible) against Ralstonia pickettii. Since selection of the most appropriate antimicrobial agents for testing and reporting has not been established by the NCCLS for many of NF-GNB species, results from large multicenter studies may help to guide the best empiric therapy.

Increased resistance to first-line agents among bacterial pathogens isolated from urinary tract infections in Latin America: time for local guidelines?

Emerging resistance phenotypes and antimicrobial resistance rates among pathogens recovered from community-acquired urinary tract infections (CA-UTI) is an increasing problem in specific regions, limiting therapeutic options. As part of the SENTRY Antimicrobial Surveillance Program, a total of 611 isolates were collected in 2003 from patients with CA-UTI presenting at Latin American medical centers. Each strain was tested in a central laboratory using Clinical Laboratory Standard Institute (CLSI) broth microdilution methods with appropriate controls. Escherichia coli was the leading pathogen (66%), followed by Klebsiella spp. (7%), Proteus mirabilis (6.4%), Enterococcus spp. (5.6%), and Pseudomonas aeruginosa (4.6%). Surprisingly high resistance rates were recorded for E. coli against first-line orally administered agents for CA-UTI, such as ampicillin (53.6%), TMP/SMX (40.4%), ciprofloxacin (21.6%), and gatifloxacin (17.1%). Decreased susceptibility rates to TMP/SMX and ciprofloxacin were also documented for Klebsiella spp. (79.1 and 81.4%, respectively), and P. mirabilis (71.8 and 84.6%, respectively). For Enterococcus spp., susceptibility rates to ampicillin, chloramphenicol, ciprofloxacin, and vancomycin were 88.2, 85.3, 55.9, and 97.1%, respectively. High-level resistance to gentamicin was detected in 24% of Enterococcus spp. Bacteria isolated from patients with CA-UTI in Latin America showed limited susceptibility to orally administered antimicrobials, especially for TMP/SMX and fluoroquinolones. Our results highlight the need for developing specific CA-UTI guidelines in geographic regions where elevated resistance to new and old compounds may influence prescribing decisions.

The second internal transcribed spacer of nuclear ribosomal DNA as a tool for Latin American anopheline taxonomy: a critical review

Among the molecular markers commonly used for mosquito taxonomy, the internal transcribed spacer 2 (ITS2) of the ribosomal DNA is useful for distinguishing among closely-related species. Here we review 178 GenBank accession numbers matching ITS2 sequences of Latin American anophelines. Among those, we found 105 unique sequences corresponding to 35 species. Overall the ITS2 sequences distinguish anopheline species, however, information on intraspecific and geographic variations is scarce. Intraspecific variations ranged from 0.2% to 19% and our analysis indicates that misidentification and/or sequencing errors could be responsible for some of the high values of divergence. Research in Latin American malaria vector taxonomy profited from molecular data provided by single or few field capture mosquitoes. However we propose that caution should be taken and minimum requirements considered in the design of additional studies. Future studies in this field should consider that: (1) voucher specimens, assigned to the DNA sequences, need to be deposited in collections, (2) intraspecific variations should be thoroughly evaluated, (3) ITS2 and other molecular markers, considered as a group, will provide more reliable information, (4) biological data about vector populations are missing and should be prioritized, (5) the molecular markers are most powerful when coupled with traditional taxonomic tools.