Infectious endarteritis in aortic coarctation: two spectra of an infrequent disease / Endarterite infecciosa na coarctação da aorta: dois espectros de uma doença pouco frequente

ABSTRACT Objective: To describe two different degrees of clinical commitment and results in the evolution of infectious endarteritis in patients without a previous diagnosis of aortic coarctation. Case description: Two male patients aged 13 and 9 years old were admitted. The first due to a fever for 2 months, which started after dental cleaning, and the second due to high blood pressure, both patients with asthenia and weight loss. In the first case, the transthoracic echocardiogram showed aortic coarctation, and the transesophageal echocardiogram showed the presence of vegetations in the post-coarctation area, without pseudoaneurysms, with blood culture positive for Streptococcus mitis. This patient was treated for six weeks with crystalline penicillin, resolving the infection without complications. The second case was assessed for high blood pressure with a history of fever, and was treated with antibiotics. When performing a transthoracic echocardiogram, aortic coarctation was observed with a saccular image classified as a pseudoaneurysm by angiography and tomography. Blood culture was negative, and the patient developed an episode of hematemesis whose initial etiology could not be determined. Before surgical repair, he had a second episode of copious hematemesis with hypovolemic shock and death. Comments: We need to have a high index of clinical suspicion to establish the diagnosis of aortic coarctation complicated by endarteritis and start the appropriate antibiotic treatment, always maintaining surveillance for the early detection of pseudoaneurysms.

RESUMO Objetivo: Descrever dois diferentes graus de comprometimento clínico e resultados na evolução de endarterite infecciosa em pacientes sem diagnóstico prévio de coarctação da aorta. Descrição do caso: Dois pacientes do sexo masculino com idades entre 13 e nove anos foram internados. O primeiro por febre durante dois meses, iniciada após limpeza dentária. O segundo por hipertensão arterial. Ambos com astenia e perda de peso. No primeiro caso, o ecocardiograma transtorácico mostrou coarctação da aorta e o ecocardiograma transesofágico revelou vegetações na área pós-coarctação, sem pseudoaneurismas. A hemocultura foi positiva para de Streptococcus mitis. Este paciente foi tratado por seis semanas com penicilina cristalina, resolvendo a infecção sem complicações. O segundo caso foi avaliado pela presença de hipertensão arterial, com história de febre tratada com antibióticos. Ao realizar o ecocardiograma transtorácico, observou-se coarctação da aorta com imagem sacular classificada como pseudoaneurisma pela angiografia e tomografia. A hemocultura foi negativa. O paciente desenvolveu um episódio de hematêmese, cuja etiologia inicial não pôde ser determinada. Antes da correção cirúrgica, apresentou um segundo episódio de hematêmese profusa, com choque hipovolêmico e óbito. Comentários: Devemos ter um alto índice de suspeição clínica para poder estabelecer o diagnóstico de coarctação da aorta complicada com endarterite e iniciar o tratamento antibiótico adequado. É preciso manter a vigilância para a detecção precoce de pseudoaneurismas.

Infectious endarteritis in aortic coarctation: two spectra of an infrequent disease.

OBJECTIVE: To describe two different degrees of clinical commitment and results in the evolution of infectious endarteritis in patients without a previous diagnosis of aortic coarctation. CASE DESCRIPTION: Two male patients aged 13 and 9 years old were admitted. The first due to a fever for 2 months, which started after dental cleaning, and the second due to high blood pressure, both patients with asthenia and weight loss. In the first case, the transthoracic echocardiogram showed aortic coarctation, and the transesophageal echocardiogram showed the presence of vegetations in the post-coarctation area, without pseudoaneurysms, with blood culture positive for Streptococcus mitis. This patient was treated for six weeks with crystalline penicillin, resolving the infection without complications. The second case was assessed for high blood pressure with a history of fever, and was treated with antibiotics. When performing a transthoracic echocardiogram, aortic coarctation was observed with a saccular image classified as a pseudoaneurysm by angiography and tomography. Blood culture was negative, and the patient developed an episode of hematemesis whose initial etiology could not be determined. Before surgical repair, he had a second episode of copious hematemesis with hypovolemic shock and death. COMMENTS: We need to have a high index of clinical suspicion to establish the diagnosis of aortic coarctation complicated by endarteritis and start the appropriate antibiotic treatment, always maintaining surveillance for the early detection of pseudoaneurysms.

Analysis of SARS-CoV-2 mutations reveals three types of temporal dynamics and one is correlated with international travels

Coronavirus disease 2019 (COVID-19) is a contagious disease caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). This disease has spread globally, causing more than 161.5 million cases and 3.3 million deaths to date. Surveillance and monitoring of new mutations in the virus genome are crucial to our understanding of the adaptation of SARS-CoV-2. Moreover, how the temporal dynamics of these mutations is influenced by control measures and non-pharmaceutical interventions (NPIs) is poorly understood. Using 1 058 020 SARS-CoV-2 from sequenced COVID-19 cases from 98 countries (totaling 714 country-month combinations), we perform a normalization by COVID-19 cases to calculate the relative frequency of SARS-CoV-2 mutations and explore their dynamics over time. We found 115 mutations estimated to be present in more than 3 % of global COVID-19 cases and determined three types of mutation dynamics: High-Frequency, Medium-Frequency, and Low-Frequency. Classification of mutations based on temporal dynamics enable us to examine viral adaptation and evaluate the effects of implemented control measures in virus evolution during the pandemic. We showed that Medium-Frequency mutations are characterized by high prevalence in specific regions and/or in constant competition with other mutations in several regions. Finally, taking N501Y mutation as representative of High-Frequency mutations, we showed that level of control measure stringency negatively correlates with the effective reproduction number of SARS-CoV-2 with High-Frequency or not-High-Frequency and both follows similar trends in different levels of stringency.

Everolimus for severe arrhythmias in tuberous sclerosis complex related cardiac rhabdomyomas.

Intracardiac rhabdomyoma is the most common primary cardiac tumor in children. Most cases are associated with tuberous sclerosis complex (TSC). Most of them are asymptomatic in the neonate and do not require treatment. However, some develop cardiovascular symptoms such as arrhythmias, heart failure, and ventricular inflow/outflow tract obstruction in the neonatal period with early death. Many of these tumors are not candidates for surgical resection and medical management is limited. Treatment with mammalian target of rapamycin (mTOR) inhibitor is currently approved for the management of central nervous tumors and angiomyolipoma in TSC. Two patients with malignant arrhythmias related to nonsurgical multiple rhabdomyomas associated with TSC who were successfully treated with an mTOR inhibitor were described. Everolimus therapy showed significant regression of rhabdomyomas with rapid improvement of arrhythmias and heart failure prior to tumor shrinkage.

Analysis of the Dynamics and Distribution of SARS-CoV-2 Mutations and its Possible Structural and Functional Implications

After eight months of the pandemic declaration, COVID-19 has not been globally controlled. Several efforts to control SARS-CoV-2 dissemination are still running including vaccines and drug treatments. The effectiveness of these procedures depends, in part, that the regions to which these treatments are directed do not vary considerably. Although, it is known that the mutation rate of SARS-CoV-2 is relatively low it is necessary to monitor the adaptation and evolution of the virus in the different stages of the pandemic. Thus, identification, analysis of the dynamics, and possible functional and structural implication of mutations are relevant. Here, we first estimate the number of COVID-19 cases with a virus with a specific mutation and then calculate its global relative frequency (NRFp). Using this approach in a dataset of 100 924 genomes from GISAID, we identified 41 mutations to be present in viruses in an estimated number of 750 000 global COVID-19 cases (0.03 NRFp). We classified these mutations into three groups: high-frequent, low-frequent non-synonymous, and low-frequent synonymous. Analysis of the dynamics of these mutations by month and continent showed that high-frequent mutations appeared early in the pandemic, all are present in all continents and some of them are almost fixed in the global population. On the other hand, low-frequent mutations (non-synonymous and synonymous) appear late in the pandemic and seems to be at least partially continent-specific. This could be due to that high-frequent mutation appeared early when lockdown policies had not yet been applied and low-frequent mutations appeared after lockdown policies. Thus, preventing global dissemination of them. Finally, we present a brief structural and functional review of the analyzed ORFs and the possible implications of the 25 identified non-synonymous mutations.

Worldwide Geographical and Temporal Analysis of SARS-CoV-2 Haplotypes shows Differential Distribution Patterns

Since the identification of SARS-CoV-2, a large number of genomes have been sequenced with unprecedented speed around the world. This marks a unique opportunity to analyze virus spreading and evolution in a worldwide context. Currently, there is not a useful haplotype description to help to track important and globally scattered mutations. Also, differences in the number of sequenced genomes between countries and/or months make it difficult to identify the emergence of haplotypes in regions where few genomes are sequenced but a large number of cases are reported. We propose an approach based on the normalization by COVID-19 cases of relative frequencies of mutations using all the available data to identify major haplotypes. Furthermore, we can use a similar normalization approach to tracking the temporal and geographic distribution of haplotypes in the world. Using 171 461 genomes, we identify five major haplotypes (OTUs) based on nine high-frequency mutations. OTU_3 characterized by mutations R203K and G204R is currently the most frequent haplotype circulating in four of the six continents analyzed. On the other hand, during almost all months analyzed, OTU_5 characterized by the mutation T85I in nsp2 is the most frequent in North America. Recently (since September), OTU_2 has been established as the most frequent in Europe. OTU_1, the ancestor haplotype is near to extinction showed by its low number of isolations since May. Also, we analyzed whether age, gender, or patient status is more related to a specific OTU. We did not find OTUs preference for any age group, gender, or patient status. Finally, we discuss structural and functional hypotheses in the most frequently identified mutations, none of those mutations show a clear effect on the transmissibility or pathogenicity.

A bipartite periplasmic receptor-diguanylate cyclase pair (XAC2383-XAC2382) in the bacterium Xanthomonas citri.

The second messenger cyclic diguanylate monophosphate (c-di-GMP) is a central regulator of bacterial lifestyle, controlling several behaviors, including the switch between sessile and motile states. The c-di-GMP levels are controlled by the interplay between diguanylate cyclases (DGCs) and phosphodiesterases, which synthesize and hydrolyze this second messenger, respectively. These enzymes often contain additional domains that regulate activity via binding of small molecules, covalent modification, or protein-protein interactions. A major challenge remains to understand how DGC activity is regulated by these additional domains or interaction partners in specific signaling pathways. Here, we identified a pair of co-transcribed genes (xac2382 and xac2383) in the phytopathogenic, Gram-negative bacterium Xanthomonas citri subsp. citri (Xac), whose mutations resulted in opposing motility phenotypes. We show that the periplasmic cache domain of XAC2382, a membrane-associated DGC, interacts with XAC2383, a periplasmic binding protein, and we provide evidence that this interaction regulates XAC2382 DGC activity. Moreover, we solved the crystal structure of XAC2383 with different ligands, indicating a preference for negatively charged phosphate-containing compounds. We propose that XAC2383 acts as a periplasmic sensor that, upon binding its ligand, inhibits the DGC activity of XAC2382. Of note, we also found that this previously uncharacterized signal transduction system is present in several other bacterial phyla, including Gram-positive bacteria. Phylogenetic analysis of homologs of the XAC2382-XAC2383 pair supports several independent origins that created new combinations of XAC2382 homologs with a conserved periplasmic cache domain with different cytoplasmic output module architectures.

Espesor del tejido adiposo epicárdico en niños y adolescentes con factores de riesgo cardiometabólico / Epicardial adipose tissue thickness in children and adolescents with cardiometabolic risk factors

Objetivo: Determinar la relación del espesor del tejido adiposo epicárdico (TAE) con factores de riesgo cardiometabólico (FRC) en niños y adolescentes. Métodos: Se seleccionaron 77 sujetos de ambos sexos entre 7 y 18 años. Se realizó anamnesis y evaluación de parámetros clínicos, determinación de glucemia, insulina y lípidos y se calculó el HOMA-IR. Se determinó el espesor del TAE mediante ecocardiografía transtorácica. Se formaron 2 grupos, participantes con menos de 2 FRC (cero o un FRC) y participantes con 2 o más FRC. Resultados: El grupo con 2 o más FRC presentó mayores valores de espesor del TAE, insulina y HOMA-IR (p < 0,05). El espesor del TAE mostró una correlación positiva estadísticamente muy significativa con el índice de masa corporal (IMC) (r = 0,561; p = 0,0001), la circunferencia abdominal (r = 0,549; p = 0,0001), la presión arterial sistólica (PAS) (r = 0,256; p = 0,028), la insulina (r = 0,408; p = 0,0001) y el HOMA-IR (r = 0,325; p = 0,005), sin embargo, estas correlaciones fueron no significativas al ajustar para el IMC. El punto de corte para el espesor del TAE como predictor de 2 o más FRC fue de 3,17 mm. El riesgo (odds ratio) de tener 2 o más FRC si presenta un espesor de TAE > 3,17 mm fue de 3,1 (IC: 1,174-8,022, p = 0,02). El IMC fue la variable independiente que más influyó sobre los valores del espesor del TAE y la presencia de 2 o más FRC (AU)

Conclusión: En este grupo de niños y adolescentes se encontró que la relación del TAE con los FRC es dependiente del IMC. Objective: To assess the relationship of epicardial adipose tissue (EAT) thickness with cardiometabolic risk factors (CRFs) in children and adolescents. Methods: Seventy-seven subjects of both sexes aged 7-18 years were selected. Medical history, clinical parameters, and glucose, insulin, and lipid levels were collected. EAT thickness was measured using transthoracic echocardiography. Study subjects were divided into two groups based on whether they had less than two or two or more CRFs. Results: The group with two or more CRFs had higher EAT thickness, insulin, and HOMA-IR values (P < .05). EAT thickness showed a statistically significant positive correlation with body mass index (BMI) (r = 0.561, P = .0001), waist circumference (r = .549, P = .0001), systolic blood pressure (SBP) (r = .256, P = .028), insulin (r = 0.408, P = .0001), and HOMA-IR (r = .325, P = .005). However, these correlations were not significant after adjustment for BMI. The cut-off point for EAT thickness as predictor of two or more CRFs was 3.17 mm. The risk (odds ratio) of having two or more CRFs if EAT thickness was > 3.17 mm was 3.1 (95% CI: 1.174-8.022). BMI was the independent variable that most affected EAT thickness and the presence of two or more CRFs. Conclusion: In this group of children and adolescents, the relationship of EAT thickness with CRFs was found to be dependent on BMI (AU)

Epicardial adipose tissue thickness in children and adolescents with cardiometabolic risk factors.

OBJECTIVE: To assess the relationship of epicardial adipose tissue (EAT) thickness with cardiometabolic risk factors (CRFs) in children and adolescents. METHODS: Seventy-seven subjects of both sexes aged 7-18 years were selected. Medical history, clinical parameters, and glucose, insulin, and lipid levels were collected. EAT thickness was measured using transthoracic echocardiography. Study subjects were divided into two groups based on whether they had less than two or two or more CRFs. RESULTS: The group with two or more CRFs had higher EAT thickness, insulin, and HOMA-IR values (P<.05). EAT thickness showed a statistically significant positive correlation with body mass index (BMI) (r=0.561, P=.0001), waist circumference (r=.549, P=.0001), systolic blood pressure (SBP) (r=.256, P=.028), insulin (r=0.408, P=.0001), and HOMA-IR (r=.325, P=.005). However, these correlations were not significant after adjustment for BMI. The cut-off point for EAT thickness as predictor of two or more CRFs was 3.17mm. The risk (odds ratio) of having two or more CRFs if EAT thickness was >3.17mm was 3.1 (95% CI: 1.174-8.022). BMI was the independent variable that most affected EAT thickness and the presence of two or more CRFs. CONCLUSION: In this group of children and adolescents, the relationship of EAT thickness with CRFs was found to be dependent on BMI.

Espesor de tejido adiposo epicárdico en escolares y adolescentes con obesidad, sobrepeso, y normopeso / Epicardial adipose tissue thickness in children and adolescents with obesity, overweight, and normal weight

Objetivo: Determinar la relación del espesor del tejido adiposo epicárdico (TAE) con el estado nutricional (obesidad, sobrepeso y normopeso) en escolares y adolescentes. Métodos: Estudio observacional, analítico y transversal. Participaron 53 escolares y adolescentes de 6 a 18 años, distribuidos de acuerdo al índice de masa corporal (IMC) en normopeso, sobrepeso y obesidad. Se tomaron medidas antropométricas y de tensión arterial (TA). Se midió el espesor del TAE por ecocardiografía bidimensional. Resultados: Hubo 21 (39,6%) participantes con normopeso, 13 (24,5%) con sobrepeso y 19 (35,9%) con obesidad. El espesor del TAE fue significativamente mayor en el grupo de obesos (3,24±0,46 mm) en comparación con sobrepeso (2,79±0,37 mm; p<0,003) y normopeso (2,20±0,34 mm; p<0,0001), y en el grupo de sobrepeso al compararlo con normopeso (p=0,0001). El espesor del TAE no fue diferente según edad y sexo. Se observó una correlación positiva estadísticamente significativa del espesor del TAE con el IMC (r=0,766; p=0,0001), la circunferencia de cintura (r=0,684; p=0,0001) y la TA sistólica (r=0,376; p=0,005). El análisis de regresión lineal múltiple mostró que el IMC (p=0,0001) fue la variable que más influyó sobre el espesor del TAE. Conclusión: El espesor del TAE aumenta con la adiposidad desde la edad escolar y la adolescencia, y podría ser una herramienta para evaluar riesgo cardiovascular.

Objective: To determine the ratio of the thickness of epicardial adipose tissue (EAT) with nutritional status (obesity, overweight and normal weight) in children and adolescents. Methods: Observational, analytical and cross-sectional study. Fifty-three children and adolescents from 6 to 18 years old were included, distributed according to body mass index (BMI) in obese, overweight and normal weight. Anthropometric measures and blood pressure (BP) were taken. TAE thickness was measured by two-dimensional echocardiography. Results: Twenty-one participants (39.6%) had normal weight, 13 (24.5%) overweight and 19 (35.9%) obesity. The thickness of EAT was significantly higher in the obese group (3.24±0.46 mm) compared to overweight (2.79±0.37 mm, p<0.003) and normal weight (2.20±0.34 mm; p<0.0001), and in the overweight group as compared with normal weight (p=0.0001). TAE thickness was no different by age and gender. A positive statistically significant correlation of thickness of EAT with BMI (r=0.766; p=0.0001), waist circumference (r=0.684; p=0.0001) and systolic BP (r=0.376; p=0.005) was observed. The multiple linear regression analysis showed that BMI (p=0.0001) was the variable that most influenced the thickness of EAT. Conclusion: The thickness of EAT increases with adiposity from school age and adolescence and could be a tool to assess cardiovascular risk.

[Antiadrenergic rescue therapy with amiodarone in children with severe left ventricular dysfunction secondary to scorpion envenomation]. / Terapia de resgate com amiodarona em crianças com grave disfunção ventricular esquerda causada por veneno de escorpião.

BACKGROUND: Children with scorpion envenomation have massive sympathetic activation and variable degrees of left ventricular systolic dysfunction. OBJECTIVE: To evaluate a rescue protocol for children with severe left ventricular dysfunction secondary to scorpion envenomation. METHODS: Four children, after scorpion envenomation, were subjected to a rescue protocol for acute left ventricular dysfunction: Endotracheal intubation and respiratory assistance, electrocardiograms, chest x-Ray, echocardiograms and blood samples for norepinephrine and troponin I serum levels. Samples and echocardiograms were repeated at 12, 24 and 48 hours. Intravenous medications: Dobutamine: 4-6 microg/kg/min. Amiodarone: 3 mg/kg during a 2 hour period. Maintenance: 5 mg/kg/day. Furosemide: 0.5 mg/kg/dose. Diuretics were given when the systemic blood pressure was above percentile fifty. Amiodarone, Dobutamine and Furosemide were administered during the first 48 hours. Beta-adrenergic blockers and angiotensin converting enzyme were given, at 48 hours after admission, once the left ventricular Ejection fraction > 0.35 and the clinical status had improved. RESULTS: On admission, norepinephrine was 1,727.50 +/-794.96 pg/ml, troponin I 24.53 +/- 14.09 ng/ml and left ventricular ejection fraction 0.20 +/- 0.056. At twelve hours, norepinephrine and troponin I serum levels were down to half of the initial values and the ejection fraction increased to 0.32 +/- 0.059. During the next 24 and 48 hours, the ejection fraction rose to 0.46 +/- 0.045, (p<0.01) and norepinephrine and troponin diminished to 526.75 +/- 273.73 (p < 0.02) and 2.20 +/- 2.36 (p<0.02) respectively. CONCLUSION: Amiodarone, by acting as a neuromodulator, is very likely responsible for the early and progressive decrease of serum norepinephrine.

Terapia de resgate com amiodarona em crianças com grave disfunção ventricular esquerda causada por veneno de escorpião / Antiadrenergic rescue therapy with amiodarone in children with severe left ventricular dysfunction secondary to scorpion envenomation / Terapia de rescate con amiodarona en niños con severa disfunción ventricular izquierda ocasionada por veneno de escorpión

Fundamentos: As crianças picadas por escorpião, pressintam ativação maciça do sistema nervoso simpática com vários graus de disfunção sistólica ventricular esquerda. Oobjetivo: Testar um protocolo de resgate em crianças com grave disfunção ventricular esquerda causada por picada de escorpião. Métodos: Quatro crianças após serem picadas por escorpião foram submetidas a: Encubação endotraqueal e suporte respiratório, eletrocardiograma, radiografia de tórax, ecocardiograma e determinação sérica da norepinefrina e troponina I. As análises foram repetidas após 12, 24 e 48 horas. As seguintes medicações intravenosas foram administradas: dobutamina 4-6 μg/kg/min; amiodarona 3 mg/kg durante duas horas, com dose de manutenção de 5 mg/kg/dia; e furosemida 0,5 mg/kg. Amiodarona, dobutamina e furosemida foram administradas durante as primeiras 48 horas. Bloqueadores beta-adrenérgicos e inibidores da enzima conversora da angiotensina foram administrados até 48 após a internação, uma vez que o estado clínico havia melhorado e a fração de ejeção ventricular esquerda encontrava-se acima de 0,35 por cento. Resultados: Na admissão, a dosagem da norepinefrina foi 1.727,50± 794,96 pg/ml, a de troponina I 24,53 ± 14,09 ng/ml e a fração de ejeção do ventrículo esquerdo foi 0,20 ± 0,056. Após 12 horas, os níveis séricos de norepinefrina e de troponina I diminuíram para a metade dos valores iniciais e a fração de ejeção aumentou para 0,32 ± 0,059. Durante as 24 e 48 horas subseqüentes, a fração de ejeção elevou-se para 0,46 ± 0,045 (p<0,01) e a norepinefrina e de troponina I diminuíram para 526,75 ± 273,73 (p< 0,02) e 2,20 ± 2,36 (p<0,02) respectivamente. Conclusão: É bem provável que a amiodarona, ao agir como neuromodulador, seja responsável pela redução rápida e progressiva dos níveis séricos de norepinefrina.

Background: Children with scorpion envenomation have massive sympathetic activation and variable degrees of left ventricular systolic dysfunction. Objective: To evaluate a rescue protocol for children with severe left ventricular dysfunction secondary to scorpion envenomation. Methods: Four children, after scorpion envenomation, were subjected to a rescue protocol for acute left ventricular dysfunction: Endotracheal intubation and respiratory assistance, electrocardiograms, chest x-Ray, echocardiograms and blood samples for norepinephrine and troponin I serum levels. Samples and echocardiograms were repeated at 12, 24 and 48 hours. Intravenous medications: Dobutamine: 4-6 μg/kg/min. Amiodarone: 3 mg/kg during a 2 hour period. Maintenance: 5 mg/kg/day. Furosemide: 0.5 mg/kg/dose. Diuretics were given when the systemic blood pressure was above percentile fifty. Amiodarone, Dobutamine and Furosemide were administered during the first 48 hours. Beta-adrenergic blockers and angiotensin converting enzyme were given, at 48 hours after admission, once the left ventricular Ejection fraction > 0.35 and the clinical status had improved. Results: On admission, norepinephrine was 1,727.50 ±794.96 pg/ml, troponin I 24.53 ± 14.09 ng/ml and left ventricular ejection fraction 0.20 ± 0.056. At twelve hours, norepinephrine and troponin I serum levels were down to half of the initial values and the ejection fraction increased to 0.32 ± 0.059. During the next 24 and 48 hours, the ejection fraction rose to 0.46 ± 0.045, (p<0.01) and norepinephrine and troponin diminished to 526.75 ± 273.73 (p < 0.02) and 2.20 ± 2.36 (p<0.02) respectively. Conclusion: Amiodarone, by acting as a neuromodulator, is very likely responsible for the early and progressive decrease of serum norepinephrine.

Fundamento: Los niños con picaduras de escorpión sufren activación masiva del sistema nervioso simpático con varios grados de disfunción sistólica ventricular izquierda. Objetivo: Probar un protocolo de rescate en niños con disfunción ventricular severa izquierda ocasionada por picadura de escorpión. Métodos: Cuatro niños tras un escorpión picarlas se sometieron a: incubación endotraqueal y soporte respiratorio, electrocardiograma, radiografía de tórax, ecocardiograma y determinación sérica de la norepinefrina y troponina I. Los análisis se repitieron tras 12, 24 y 48 horas. Las siguientes medicaciones intravenosas se administraron: dobutamina 4-6 mcg/kg/min; amiodarona 3 mg/kg durante dos horas, con dosis de mantenimiento de 5 mg/kg/día; y furosemida 0.5 mg/kg. Amiodarona, dobutamina y furosemida se administraron durante las primeras 48 horas. Bloqueante betaadrenergicos e inhibidores de la enzima convertidora de la angiotensina se administraron hasta 48 tras la internación, una vez que el estado clínico había mejorado y la fracción de eyección ventricular izquierda se hallaba superior a un 0,35 por ciento. Resultados: Al ingreso, la dosificación de la norepinefrina fue 1727,50± 794,96 pg/ml, la de troponina I 24,53 ± 14,09 ng/ml y la fracción de eyección del ventrículo izquierdo fue 0,20 ± 0,056. Tras 12 horas, los niveles séricos de norepinefrina y de troponina I disminuyeron para la mitad de los valores iniciales y la fracción de eyección aumentó para 0,32 ± 0,059. Durante las 24 y 48 horas subsiguientes, la fracción de eyección se elevó para 0,46 ± 0,045 (p<0,01) y la norepinefrina y de troponina I se redujeron para 526,75 ± 273,73 (p< 0,02) y 2,20 ± 2,36 (p<0,02) respectivamente. Conclusión: Es bien probable que la amiodarona, al actuar como neuromodulador, sea responsable de la reducción rápida y progresiva de los niveles séricos de norepinefrina.

Experiência multicêntrica na América do Sul no fechamento perventricular da comunicação interventricular muscular congênita / Perventriculat closure of congenital muscular ventricular septal defect: multicenter experience in South America

Fundamentos: O fechamento perventricular das comunicações interventriculares (CIVs) musculares tornou-se uma altenativa terapêutica atraente em pequenos lactentes com grandes defeitos. Entretanto, a reprodutibilidade desse métodos em vários centros mundiais ainda é uma questão em aberto. Método: Relatamos uma experiência multicêntrica na América do Sul com tal abordagem. No período de julho de 2007 a maio de 2009, nove pacientes não consecutivos (idade e peso médios de 6 meses e 5,5kg, respectivamente) foram submetidos a procedimento no centro cirúgico, sob monitoração da ecocardiográfia transesofágica, utilizando-se dispositivos Amplatzer. Todos os pacientes, com exceção de um, apresentavam insuficiência cardíaca e graus variados de hipertensão pulmonar. Quatro pacientes...

Background: Perventricular closure of muscular ventricular septal defects (VSD) has become an attractive treatment modality for infants. However, its reproducibility worldwide remains to be seen. Methods: We report a multicenter experience in South America. From July, 2007 to May, 2009, nine non-consecutive patients (median age and weight were 6 months and 5.5 kg, respectively) underwent the procedure in the operating room under transesophageal echocardiographic guidance using Amplatzer devices. All patients but one were in congestive heart failure and had pulmonary arterial hypertension. Four patients had coarctation of the aorta and one was submitted to pulmonary artery banding at 6 months of life, which were all repaired at the same session. Eight patients had single defects (six mid-muscular, two apical) measuring 10.3 ± 3.7 mm and one patient had multiple apical defects that required two devices. Results: Ten devices were implanted successfully (median size: 12 mm), and two had to be sutured to the right ventricular wall with a surgical suture. One patient...

Relación entre el índice de masa corporal y las cifras de tensión arterial en adolescentes

Objetivos: Investigar la relación entre el índice de masa corporal (IMC) y las cifras de tensión arterial en adolescentes de la ciudad de Mérida, dada su asociación con el desarrollo de enfermedades crónicas no trasmisibles en el adulto. Métodos: Se estudiaron 385 adolescentes, 62,3% de sexo femenino y 37,7% de masculino, entre 12 y 15 años de edad, con promedio de 15,17±1,71 años. Se les tomaron las medidas antropométricas, para el cálculo del IMC, y la tensión arterial (TA) en posición sentada. Se consideraron normales el IMC y la TA comprendida entre los percentiles 10 y 90 para las curvas venezolanas, de acuerdo a edad y sexo; se consideró sobrepeso y obesidad sobre el pc 90 y Pre-hipertensión (Pre-HTA) e hipertensión arterial (HTA) sobre el pc 90. Resultados: El 75,3% de los adolescentes presentaron un IMC normal, el 11,2% un IMC bajo y el 13,6% sobrepeso y obesidad. El 1,1% presentò TA sistólica sobre el pc 90 y el 6,3% presentó TA diastólica sobre la norma. No se observó asociación del IMC y de la TA con el sexo ni el estrato socio-económico. Se encontró una alta y significativa asociación entre el sobrepeso/obesidad y la Pre-HTA/HTA (p=0,0001). El riesgo de un adolescente con IMC sobre el pc 90 de presentar Pre-HTA o HTA fue 9,76 veces mayor (Odss ratio) que el adolescente con IMC menor al pc 90 (IC 95%: 4,09-23,27; p=0,0001). Conclusión: Se comprobó una asociación estadística entre el IMC y los valores de TA sistólica y diastólica, por lo que adolescentes que tengan IMC altos deben ser seguidos y sometidos a algún tipo de intervención tendiente a disminuir la incidencia de enfermedades crónicas no transmisibles en la edad adulta.

Objective: To investigate the relationship between body mass index (BMI) and the blood pressure (BP) values in adolescents of the city of Mérida, given its association with the development of non-transmissible chronic diseases in adults. Methods: We studied 385 adolescents, 62.3% female and 37.7% male, between 12 and 15 years of age, with average of 15.17 ± 1.71 years. Anthropometric measures and blood pressure (BP) were taken. The body mass index (BMI) was calculated. BMI and BP between 10th and 90th percentile from Venezuelan curves, according to age and sex, were considered normal. Obesity/overweight and pre-hypertension/hypertension (Pre-HTA/HTA) were considered when the BMI and the BP were located above the 90th percentile. Results: A total of 75.3% of the adolescents had a normal BMI, 11.2% a low BMI and 13.6% had overweight and obesity. The systolic BP was above the 90th percentile in 1.1% and the diastolic BP in 6.3% of the adolescents. No association was observed between BMI and BP with the sex and socio-economic stratum. It was found a high and significant association between overweight/obesity and Pre-HTA/HTA (p=0.0001). The risk for Pre-HTA/HTA of an adolescent with a BMI over 90th percentile was 9.76 times higher (Odss ratio) than in adolescents with a BMI less than 90th percentile (95% CI 4.09-23.27; p = 0.0001). Conclusion: It was found a statistical association between BMI and the values of systolic and diastolic BP. Those adolescents with high BMI should be monitored and subject to any intervention aimed at reducing the incidence of non-transmissible chronic diseases in adulthood.

Percutaneous closure of Blalock-Taussig shunts using Gianturco coils.

Permeability of a Blalock-Taussig shunt can increase the risk of endocarditis and ventricular overload. Percutaneous embolization of these shunts gives variable results. We report our experience in 10 patients with percutaneous closure of modified Blalock-Taussig shunts using retrograde arterial embolization with Gianturco coils. The patients' median age was 2.8 years, and their median weight was 12 kg. Most patients had minor stenosis of the distal portion of the anastomosis. In all cases, complete closure of the shunt was achieved without complications using a median of one coil per patient. The technique was feasible, safe, effective, and inexpensive.

Cierre percutáneo de la fístula de Blalock-Taussig con espirales de Gianturco / Percutaneous closure of blalock–taussig shunts using gianturco coils

La permeabilidad de una fístula de Blalock-Taussig incrementa el riesgo de endocarditis y sobrecarga ventricular. Los resultados de la embolización percutánea de estos cortocircuitos son variables. Presentamos la experiencia en el cierre percutáneo de la fístula de Blalock-Taussig modificada, utilizando la embolización arterial retrógrada con espirales de Gianturco, en 10 pacientes. Las medianas de edad y peso fueron 2,8 años y 12 kg, respectivamente. La mayoría presentaba una zona de disminución del diámetro en el extremo distal de la conexión. En todos se consiguió un cierre completo del cortocircuito, con una mediana de 1 dispositivo por paciente, y no se registraron complicaciones. La técnica es factible, segura, efectiva y económica (AU)

Permeability of a Blalock-Taussig shunt can increase the risk of endocarditis and ventricular overload. Percutaneous embolization of these shunts gives variable results. We report our experience in 10 patients with percutaneous closure of modified Blalock-Taussig shunts using retrograde arterial embolization with Gianturco coils. The patients' median age was 2.8 years, and their median weight was 12 kg. Most patients had minor stenosis of the distal portion of the anastomosis. In all cases, complete closure of the shunt was achieved without complications using a median of one coil per patient. The technique was feasible, safe, effective, and inexpensive (AU)

Percutaneous Closure of Blalock-Taussig Shunts Using Gianturco Coils

Permeability of a Blalock-Taussig shunt can increase the risk of endocarditis and ventricular overload. Percutaneous embolization of these shunts gives variable results. We report our experience in 10 patients with percutaneous closure of modified Blalock-Taussig shunts using retrograde arterial embolization with Gianturco coils. The patients’ median age was 2.8 years, and their median weight was 12 kg. Most patients had minor stenosis of the distal portion of the anastomosis. In all cases, complete closure of the shunt was achieved without complications using a median of one coil per patient. The technique was feasible, safe, effective, and inexpensive.

Fechamento percutâneo da Comunicação Interventricular muscular congênita / Percutaneous closure of congenital muscular ventricular septal defects

tratamento percutaneo da comunicação interventricular (CIV)congênita vem sendo realizado com bons resultados nos últimos 10 anos. Relatamos nossa experiência com a oclusão percutânea da CIV muscular congênita com próteses Amplatzer avaliando exequibilidade, segurança e eficâcia do método: Método: No período de setembro de 2002 a dezembro de 2007, foram realizados 9 procedimentos em múltiplos centros em 8 pacientes não-concecutivos (mediana de idade de 6 anos e de peso de 26 kg), sob anestesia geral e monitoração pela ecocardiografia transesofágica. Todas as CIVs eram únicas (7 na região trabecular média e 1 na porção anterior) e tinham diâmetro médio de 6,0 mais ou menos 2,1 mm. Houve embolização imediata de um dispositivo com resgate percutâneo. O procedimento foi repetido com sucesso após um ano. Todos os casos restantes foram realizados com sucesso, som complicações maiores. O índice de oclusão foi de 100 por cento no seguimento. Conclusão: Nessa pequena série de pacientes, a oclusão percutânea da CIV muscular congênita única com próteses Amplatzer foi um procedimento de fácil execução, seguro e altamente efizaz.

Background: Percutaneous treatment of muscular ventricular septal defects (VSD) has been performed with good results in the last 10 years. We report our experience with the percutaneous closure of congenital muscular VSDs with Amplatzer devices assessing the feasibility, safety and efficacy of the procedure. Methods: From 9/2002 to 12/ 2007, 9 procedures were performed in multiple centers in 8 non-consecutive patients (median age: 6 years; median weight: 26 kg) under general anesthesia and transesophageal echocardiographic monitoring. All VSDs were single (7 in the trabecular region and 1 anterior) and had a mean diameter of 6.0 ± 2.1 mm. There was one device embolization with immediate percutaneous device retrieval. This procedure was successfully repeated after year. The other cases were completed successfully without major complications. The rate of complete closure was 100% at follow-up. Conclusion: In this small series of patients, percutaneous closure of congenital single muscular VSD was relatively easy to perform, safe and highly effective.

Síndrome de kawasaki: presentación de un caso poco común / Kawasaki syndrome: presentation of an uncommon case

La enfermedad de Kawasaki es un proceso vasculítico multisistémico cuya etiología es poco conocida. La presentación clínica es florida y su evolución está condicionada al inicio temprano de la terapia específica con gammaglobulinas, de allí la importancia de un diagnóstico precoz. Habitualmente estos casos cursan con dilatación del lecho arterial coronario siendo excepcional la aparición de alteraciones a nivel de arterias de mayor calibre, menos común aneurismas a nivel cerebral, que confiere un peor pronóstico para estos pacientes. Presentamos el caso de lactante de 3 meses de edad con aneurisma de ambas arterias coronarias, arteria subclavia izquierda, cerebral media derecha y ambas Iliacas, y obstrucción de las mismas.

Kawasaki disease, a multisystem vasculitis of unknown etiology can present in various ways. It is crucial to make an early diagnosis, and consequently give gamma globulin in order to abort its insidious evolution and not infrequently fatal outcome. Aneurysms and obstruction of the coronary arteries are the most characteristic presentations. Other large vessels may be involved, with the cerebral circulation being affected least often but having the worst prognosis. We present a clinical case of a 3 month old breast fed infant with aneurysms and obstructions in both coronary arteries, the left subclavian, right middle cerebral, and both iliac arteries.

[Right patent ductus arteriosus with an ipsilateral aortic arch: percutaneous closure with amplatzer devices]. / Conducto arterioso persistente a la derecha con arco aórtico ipsolateral: cierre percutáneo con dispositivos Amplatzer.

The association of a right aortic arch with an ipsilateral patent ductus arteriosus is rare, especially when there are no other intracardiac anomalies. We report three female patients aged 26, 35 and 9 years with this combination in whom previous attempts at surgical closure by thoracotomy and sternotomy were unsuccessful and who subsequently underwent successful percutaneous closure of the defects using Amplatzer devices. In two patients, although angiography demonstrated the presence of type-A patent ductus arteriosus, it was not possible to determine the minimum diameter accurately and it was necessary to measure it using a sizing balloon. An Amplatzer duct occluder was used in two patients and an Amplatzer muscular ventricular septal defect occluder, in the other. In all patients, full closure was confirmed in the catheterization laboratory and the patients were discharged on the same day with no complications. Percutaneous closure of a right patent ductus arteriosus associated with a right aortic arch is feasible, safe and effective.