Congenital Heart Disease as a Warning Sign for the Diagnosis of the 22q11.2 Deletion

Background: To alert for the diagnosis of the 22q11.2 deletion syndrome (22q11.2DS) in patients with congenital heart disease (CHD). Objective: To describe the main CHDs, as well as phenotypic, metabolic and immunological findings in a series of 60 patients diagnosed with 22q11.2DS. Methods: The study included 60 patients with 22q11.2DS evaluated between 2007 and 2013 (M:F=1.3, age range 14 days to 20 years and 3 months) at a pediatric reference center for primary immunodeficiencies. The diagnosis was established by detection of the 22q11.2 microdeletion using FISH (n = 18) and/or MLPA (n = 42), in association with clinical and laboratory information. Associated CHDs, progression of phenotypic facial features, hypocalcemia and immunological changes were analyzed. Results: CHDs were detected in 77% of the patients and the most frequent type was tetralogy of Fallot (38.3%). Surgical correction of CHD was performed in 34 patients. Craniofacial dysmorphisms were detected in 41 patients: elongated face (60%) and/or elongated nose (53.3%), narrow palpebral fissure (50%), dysplastic, overfolded ears (48.3%), thin lips (41.6%), elongated fingers (38.3%) and short stature (36.6%). Hypocalcemia was detected in 64.2% and decreased parathyroid hormone (PTH) level in 25.9%. Decrease in total lymphocytes, CD4 and CD8 counts were present in 40%, 53.3% and 33.3%, respectively. Hypogammaglobulinemia was detected in one patient and decreased concentrations of immunoglobulin M (IgM) in two other patients. Conclusion: Suspicion for 22q11.2DS should be raised in all patients with CHD associated with hypocalcemia and/or facial dysmorphisms, considering that many of these changes may evolve with age. The 22q11.2 microdeletion should be confirmed by molecular testing in all patients.

Use of Short-term Circulatory Support as a Bridge in Pediatric Heart Transplantation

Background:Heart transplantation is considered the gold standard therapy for the advanced heart failure, but donor shortage, especially in pediatric patients, is the main limitation for this procedure, so most sick patients die while waiting for the procedure.Objective:To evaluate the use of short-term circulatory support as a bridge to transplantation in end-stage cardiomyopathy.Methods:Retrospective clinical study. Between January 2011 and December 2013, 40 patients with cardiomyopathy were admitted in our Pediatric Intensive Care Unit, with a mean age of 4.5 years. Twenty patients evolved during hospitalization with clinical deterioration and were classified as Intermacs 1 and 2. One patient died within 24 hours and 19 could be stabilized and were listed. They were divided into 2 groups: A, clinical support alone and B, implantation of short-term circulatory support as bridge to transplantation additionally to clinical therapy.Results:We used short-term mechanical circulatory support as a bridge to transplantation in 9. In group A (n=10), eight died waiting and 2 patients (20%) were transplanted, but none was discharged. In group B (n=9), 6 patients (66.7%) were transplanted and three were discharged.The mean support time was 21,8 days (6 to 984h). The mean transplant waiting list time was 33,8 days. Renal failure and sepsis were the main complication and causeof death in group A while neurologic complications were more prevalent en group B.Conclusion:Mechanical circulatory support increases survival on the pediatric heart transplantation waiting list in patients classified as Intermacs 1 and 2.

Very Long-Term Prognostic Role of Admission BNP in Non-ST Segment Elevation Acute Coronary Syndrome

Abstract Background: BNP has been extensively evaluated to determine short- and intermediate-term prognosis in patients with acute coronary syndrome, but its role in long-term mortality is not known. Objective: To determine the very long-term prognostic role of B-type natriuretic peptide (BNP) for all-cause mortality in patients with non-ST segment elevation acute coronary syndrome (NSTEACS). Methods: A cohort of 224 consecutive patients with NSTEACS, prospectively seen in the Emergency Department, had BNP measured on arrival to establish prognosis, and underwent a median 9.34-year follow-up for all-cause mortality. Results: Unstable angina was diagnosed in 52.2%, and non-ST segment elevation myocardial infarction, in 47.8%. Median admission BNP was 81.9 pg/mL (IQ range = 22.2; 225) and mortality rate was correlated with increasing BNP quartiles: 14.3; 16.1; 48.2; and 73.2% (p < 0.0001). ROC curve disclosed 100 pg/mL as the best BNP cut-off value for mortality prediction (area under the curve = 0.789, 95% CI= 0.723-0.854), being a strong predictor of late mortality: BNP < 100 = 17.3% vs. BNP ≥ 100 = 65.0%, RR = 3.76 (95% CI = 2.49-5.63, p < 0.001). On logistic regression analysis, age >72 years (OR = 3.79, 95% CI = 1.62-8.86, p = 0.002), BNP ≥ 100 pg/mL (OR = 6.24, 95% CI = 2.95-13.23, p < 0.001) and estimated glomerular filtration rate (OR = 0.98, 95% CI = 0.97-0.99, p = 0.049) were independent late-mortality predictors. Conclusions: BNP measured at hospital admission in patients with NSTEACS is a strong, independent predictor of very long-term all-cause mortality. This study allows raising the hypothesis that BNP should be measured in all patients with NSTEACS at the index event for long-term risk stratification.

Agriculture and short run macroeconomics

Neste trabalho, formula-se um modelo macroeconômico de curto prazo a fim de se derivar as interações entre os setores agrícola e não-agrícola por ocasião da aplicação de políticas de estabilização. As variáveis exógenas são mudanças nas políticas fiscal, monetária e cambial e nos preços internacionais. As variáveis endógenas explicitamente analisadas são renda real para cada setor e preços relativos. Os principais resultados são: (a) os preços relativos tendem a variar quando as variáveis exógenas variam; (b) a produção agrícola e os preços relativos da gricultura tendem a se reduzir face a políticas fiscais e monetárias expansivas mesmo quando a elasticidade-renda de demanda para produtos agrícolas for zero; (c) embora o efeito inflacionario de políticas monetárias e fiscais expansivas seja maior quando a elasticidade de oferta de produtos agrícolas é baixa, os preços nominais da agricultura tendem a crescer no máximo tanto quanto os preços nominais não-agrícolas. Os efeitos de diversas pressuposições a respeito da elasticidades de demanda e de oferta sobre os resultados do modelo são também derivados.

Reproductive characteristics of characid fish species (Teleostei, Characiformes) and their relationship with body size and phylogeny

In this study, I investigated the reproductive biology of fish species from the family Characidae of the order Characiformes. I also investigated the relationship between reproductive biology and body weight and interpreted this relationship in a phylogenetic context. The results of the present study contribute to the understanding of the evolution of the reproductive strategies present in the species of this family. Most larger characid species and other characiforms exhibit a reproductive pattern that is generally characterized by a short seasonal reproductive period that lasts one to three months, between September and April. This is accompanied by total spawning, an extremely high fecundity, and, in many species, a reproductive migration. Many species with lower fecundity exhibit some form of parental care. Although reduction in body size may represent an adaptive advantage, it may also require evolutionary responses to new biological problems that arise. In terms of reproduction, smaller species have a tendency to reduce the number of oocytes that they produce. Many small characids have a reproductive pattern similar to that of larger characiforms. On the other hand they may also exhibit a range of modifications that possibly relate to the decrease in body size and the consequent reduction in fecundity. Examples of changes in the general reproductive pattern include the following: reduction in the size of mature oocytes; increase in fecundity; production of several batches of oocytes; an extended reproductive period or even continuous reproduction that allows individuals to reproduce more than once a year; high growth rates; rapid recruitment of juveniles; presence of more than one reproductive cohort that increases the sexually active population; and multiple independent development of insemination as a reproductive strategy. These changes are possibly associated with adaptive pressures that are related to the reduction in body size. In addition, such reproductive characteristics or novelties may reflect the phylogenetic history of a given species.

Estudos dos agrupamentos vegetativos relacionados com as áreas onde foram efetuadas as pesquisas sôbre a febre amarela silvestre no Município de Passos, Estado de Minas Gerais

The work reported here was carried-out on the invitation of Dr. Henry Kumm, Director of the Rockefeller Foundation, and by appointment from Dr. Henrique Aragão, Director of the Instituto Oswaldo Cruz. It was done during the investigation of sylvan yellow fever, in June 1947, with a view to establishing the phyto-ecological conditions of the county of Passos. The pe¬riod was, however, too short for definite conclusions to be reached. Thanks are due to Dr. O. R. Causey, Chief of Research on Yellow Fever for transpor¬tation and other help. THE REGIONAL VEGETATION. Aerial photographs of the county of Passos shoto that it is covered by three great types of vegetation: Rain Forest, Secondary Pasture Land and Scrub.1 Detailed investigation, however, brings out the fact that these correspond to different seres; furthermore, each type presents not only the specific, characteristics of the biological form dominant for the climate, but also are at various stages, which express HABITATS differing from those of the normal sere. The phytogeographic survey of the region shows that most of it is now covered by secondary pasture land (disclimax) in which Melinis minutiflora, v. "fat grass" (fig. 1), predominates. The mosaic of Rain Forest and of small patches of Scrub reveals the effects of human intervention (BARRETO, H. L. de Mello 1); consequently, all the formations have to be regarded as secon¬dary, though some of them probably include relicts of the primitive climax (WARMING, E. 2). On close examination, the Scrub cannot be considered as the climax, because of the following facts: 1. In the zone of Rain-Forest stretches of forest are present in very varied topographic conditions and the reconstitution of the associations show that man has destroyed an ecological unit (fig. 2). 2. In the zone of Scrub the characteristic patches are small. The banks of rivers and brooks, the valleys and ravine and whatever the soil has retained some humidity, is being invaded fry Rain Forest, which seems to be growing under optimum conditions. The Scrub is thus limited to small belts on the calcareous mountains and on sandy soils with alcaline depths (pH abo¬ve 7) which do not retain enough moisture for the Rain Forest that is progres¬sively restricting the area occupied by Scrub. In view of the topographic and present climatic conditions the Rain Forest must consequently be regarded as the regional climax. The presence of ecologically contradictory elements and associations shows that the real problem is that of the fluctuations of the climate of Passos or even of Minas Geraes during the quaternary and recent periods (DAN-SEREAU, P. : 3), a subject on which little is known and which is tied to the evolution of the climate of Brazil (OLIVEIRA, E. : 4) . The transformation of Scrub into Rain Forest has been - observed by the author before, in other parts of Brazil (VELOSO, PL P.: 5) . It seems probable that the Rio Grande has also greatly influenced the change of the regional vegetation, by invading areas of Scrub and dislocating the limit of the Pluvial climate towards the Canastra Range, though there are remnants of Scrub (postclimax) transfor¬med into secondary open country (disclimax, fig. 5) by human devastation and the setting of fire to the land. VEGETATION GROUPS OF THE PLUVIAL TYPE. The map of the region also shows that at the present time the small patches of forest (whether devasted or intact) occupy the least accessible places, such as valleys, peaks and abrupt slopes (fig. 2). Even these are now being destroyed, so that in the near future this forested region will be en¬tirely reduced to poor pasture land unless energetic measures of conservation are undertaken in time. The Special Service for Prophylaxis against Yellow Fever installed two of their four Stations for the Capture of Mosquitos in this area, one of them at Batatal and the other at Cachoeira, which have separate formations each of them composed of several associations. Other vegetation formations were also analysed, from the synecological point of view, so as to ascertain of which degree of succession their associations belong. These phytosociological sur¬veys give an idea of the principal characteristics of each station. BATATAL FORMATION. The abrupt nature of the valley has rendered this location inappropriate for agricultural purposes since colonial times. The relict of the primitive forest climax saved by this circumstance has expanded gradually to zones whose paedologic conditions favour the eatablishment of mesophilous species. The aerial photograph shows two small stretches of forest, one apparently primi¬tive, the other composed of associations belonging to the subclimax of the subsere. CACHOEIRA FORMATION. Aerial photographs show that this station is crossed by a small river, which divides it into two separate parts. The first, which presents ecological conditions similar, though not identical to those of Batatal, is favoured by topography and apparently remains primitive forest. Though the topography of the other, on the whole, favours the establishment of groups belonging to the normal sere of the climax, is has been partly devastated recently and the aspect of the associations has been completely modified. It was is this part that the four posts for the capturing of mosquitos were set up. The first forest is favoured by deposition of organic matter, washed out from the nearby devasted areas by torrential rains, and thus provides, an appropriate HABITAT for the climax species with certain hygrophilous trends of the ecological quasiclimax type. This association seems to have reached a biological equilibrium, as the dominates. Gallesia gorarema and Cariniana legalis (fig. 10), present an optimum vitality with a vigorous habit and a normal evolutionary cycle. The Cariniantum legalis Gallesiosum equilibrium, corresponds however, to a provisory association, because if the moving of soil by torrential rains should cease it would become possible…

Anfíbios Anuros da coleção Adolpho Lutz: III - Hyla claresignata Lutz & B. Lutz, 1939

Hyla claresignata Lutz & Lutz, 1939, is a large species apparently not closely allied to the other known Brazilian hylas. It is characterized by the very small tympanum; the head is short and the snout rounded; the legs are long, the hands and feet unusually large, the latter extensively webbbed. The specific name is derived from the insular, irregular, or roughly triangular, dark spots, with a light halo, found mostly in the dorso-lateral region and on the legs. It belongs to the rain-forest fauna of the Marítime Range. The adult is a bromeliad-dweller and the tadpole rhyacophilous. DESCRIPTION. Vomerine teeth in two separate, oblique, groups, behind the large choanae, parallel to the posterior half of their inner border. Tongue entire, short, very broad and hardly free behind. Snout short, rounded, with distinct canthus rostralis and gradually sloping loreal region. Eye very large and prominent, its horizontal diameter almost equal to the distance between its anterior corner and the tip of the snout. Tympanum very small, less than one third of the diameter of the eye, but distinct, partly covered by a short, heavy ridge. Lateral fingers less than one third webbed; fourth finger slightly longer than the second, just reaching the base of the disk of the third; subarticular tubercles well developed; an angular pollex rudiment, more noticeable in the males. Toes almost completely webbed, the edge of the web inserted at the base of the disk on the third and the fifth; an inner metatarsal tubercle. Skin smooth above, granular beneath, on the throat minutely so. No dermal appendage on the hell. Habit robust, head broader than long, body rather heavy, slightly narrowed in the postaxillary region. Legs long, the tibiotarsal articulation reaching beyond the tip of the snout when adpressed. Type (female): 61 mm. (Fig. 1.) DIAGNOSIS of TADPOLE (by G. Orton). "A large specialized, mountain-stream tadpole, with wide head an elongated, flattened snout, greatly enlarged lips and high tooth formula. Eyes dorsal. Spiracle sinistral, projecting, situated far back on side. Anus dextral. Tooth formula 8/12 to 9/14 in fully grown larvae. Tail with a prominent, vertical dark band across musculature and fins; a second concentration of dark pigment near tip of tail, may or may not form a similar but narrower band. Maximum known total length: 60mm.; head and body length 25mm. (Figs. 6 e 7). For further details see Lutz & Lutz, 1939 and Lutz B. & Orton G. 1946.

Short-and long-term effects of proallatotoxin (Ethoxyprecocene II) on Rhodnius prolixus females

Oogenesis and oviposition can be inhibited in female of Rhodnius prolixus by means of short-term experiment (first reproductive cycle) of a single dose of ethoxyprecocene II given by ingestion. The inhibition is dose-dependent as measured by oocyte growth, egg maturation and egg deposition. In a long-term experiment (second and third reproductive cycles) egg production and oogenesis can be partially or totally re-established by subsequent blood meals without ethoxyprecocene II. These findings suggest that in female R. prolixus, damage caused to corpus allatum by ethoxyprecocene II, in certain cases, is not irreversible.

Dinosoma clupeola sp. n. (Hemiuridae) and Pseudoacanthostomum floridensis Nahhas & Short, 1965 (Acanthostomidae), digenetic trematodes in Brazilian Marine fishes

Dinosoma clupeola sp. n. is described from Harengula clupeola, and resembles D. hawaiiense Yamaguti, 1970, from which it differs in the entire, elongate-saccular seminal vesicle, tegument weakly plicated, smaller size of body and internal organs, and slightly larger and narrower eggs. Pseudoacanthostomum floridensis Nahhas & Short, 1965 is referred to Netuma barba, which represents a new host record.

Short and long-term effects of azadirachtin A on development and egg production of Rhodnius prolixus

Azadirachtin A was given through a blood meal to 4th-instar larvae and to adult females of Rhodnius prolixus. Development (ecdysis) and egg production were inhibited in a dose-dependent manner. Long-term experiments with subsequent four feedings on azadirachtin-free blood were performed with 4th-instar larvae and with adult females. Only in the low-dose azadirachtin larval groups (0.01 and 0.1 microng/ml of blood), development was partially restored; after a single 1.0 microng/ml treatment about 50% of the treated larvae were still alive 120 days later without any adult emergence. Similarly fed females had a dose-dependent lower survival and egg deposition rate. The results are discussed in relation to the mode of azadirachtin A action.

Influence of short time exposure to an insect growth regulator, hexaflumuron, on mortality and adult emergence of vector mosquitoes

Hexaflumuron, an insect growth regulator (IGR), was found to greatly affect the development of immatures and emergence of adults of three species of vector mosquitoes, Culex quinquefasciatus, Aedes aegypti and Anopheles stephensi, when larvae were subjected to short time exposure of < or = 1h. This IGR could completely prevent adult emergence even at a minimum exposure time of 10 min at 0.001, 0.01 and 0.1 mg/l. On treatment, larval and pupal mortality as well as varying degrees of morphogenetic abnormalities were induced in immatures and adults of the three species. Four weeks of control achieved in a slow moving sullage canal breeding Culex quinquefasciatus indicates that this IGR can be of use in such breeding habitats.

Bacillus thuringiensis: fermentation process and risk assessment: a short review

Several factors make the local production of Bacillus thuringiensis (Bt) highly appropriate for pest control in developing nations. Bt can be cheaply produced on a wide variety of low cost, organic substrates. Local production results in considerable savings in hard currency which otherwise would be spent on importation of chemical and biological insecticides. The use of Bt in Brazil has been limited in comparison with chemical insecticides. Although Bt is imported, some Brazilian researchers have been working on its development and production. Fermentation processes (submerged and semi-solid) were applied, using by-products from agro-industries. As the semi-solid fermentation process demonstrated to be interesting for Bt endotoxins production, it could be adopted for small scale local production. Although promising results had been achieved, national products have not been registered due to the absence of a specific legislation for biological products. Effective actions are being developed in order to solve this gap. Regardless of the biocontrol agents being considered atoxic and harmless to the environment, information related to direct and indirect effects of microbials are still insufficient in many cases. The risk analysis of the use of microbial control agents is of upmost importance nowadays, and is also discussed.