A Bilateral Acetabular Physeal Fracture Treated with External Fixation in an Immature Cat.

This study aimed to assess the outcome of a bilateral acetabular physeal fracture treated with external fixation in an immature cat, a surgical technique not usually employed in immature patients. The fixator took 40 days, and it was removed after radiographic bone healing was achieved. No significant complications related to the technique were identified, and the outcome was classified as good based on the functional assessment and pain scales employed. The use of external fixation for stabilising acetabular fractures in immature cats should be considered a viable technical option, especially for minimally invasive stabilisation.

Measurement of the interbronchial angle in acute viral bronchiolitis.

BACKGROUND: Acute viral bronchiolitis (AVB) is the most common lower airway infection in children under 2 years. Attempts to determine disease severity based on clinical and radiological manifestations are a major challenge. Measurements of the anatomy of the trachea and main bronchi are not only limited to pure anthropometry, but are also useful for better care of critically ill patients. The purpose of the study is to verify the association between measurements of the interbronchial angle (ITB) and the severity of respiratory disease. METHODS: A cross-sectional study, which included all patients admitted to the Santo Antônio Children's Hospital, over a period of 1 year, with diagnosis of AVB by respiratory syncytial virus (RSV) was designed. ITB angle was measured and clinical characteristics were analyzed. Quantitative variables were compared and correlation analysis was performed using Pearson's correlation coefficient. A receiving operator characteristic (ROC) curve was performed. P-value <0.05 was statistically significant. RESULTS: A total of 425 patients with AVB due to RSV were included. Most of these patients were male and the median age was 130 days, 91.11% of them required oxygen therapy through a nasal catheter, 3.3% used noninvasive ventilation and 4% used mechanical ventilation. Those who required MV or NIV and intensive care unit support were considered severe. The mean ITB was lower for these patients than for those of lesser severity (p < 0.05). CONCLUSION: The present study demonstrates that there is an association between ITB and AVB severity. The smaller the ITB, the greater the disease severity.

External Fixation for Fracture Stabilization of the Sacrum in 15 Dogs.

This study aimed to evaluate the feasibility, complications, and outcomes of external fixation (EF) for the treatment of sacral fractures in dogs, either as a primary fixation system or as a complementary technique. A total of 15 dogs with sacral fractures were surgically treated using different EF configurations, either as primary or secondary stabilization. The results were evaluated for the extent of fracture reduction, stability during treatment, complications, and bone healing. In most cases, the outcomes were excellent in terms of bone healing, neurological conditions, and pain assessment. The mean bone healing time was 9.45 ± 5.66 weeks. One (6.66%) patient presented a complication due to the technique. In conclusion, the use of EF should be considered for the stabilization of sacral fractures because of its minimal invasiveness, stability, and ease of application.

Retrospective Assessment of Thirty-Two Cases of Pelvic Fractures Stabilized by External Fixation in Dogs and Classification Proposal.

The goals of this study were to evaluate the outcomes of bone healing, patient comfort during the treatment, functional results, and complications in pelvic fractures treated with external fixation, as well as to propose a classification system for the applied external frames. A total of thirty-two canine patients with pelvic fractures of different origins were treated. To provide a better reference for the frames used, an alphanumeric classification system was developed, detailing the frame structure and the number and location of the pins used. In this study, eighty-six fractures were treated in the 32 patients of this work, with an average fixation time of 9.88 ± 4.15 weeks. No major complications were detected in this case cohort, and the outcomes were rated at 9.46 based on a visual assessment scale for the patient's comfort during treatment. Outcomes graded as excellent and good were 96%. The use of external fixation for stabilization of pelvic fractures should be considered as a technical option, especially for minimally invasive stabilization of complex fractures, either as a primary or secondary stabilization.

Report of the Phenotype of a Patient with Roberts Syndrome and a Rare ESCO2 Variant.

Roberts syndrome is a rare autosomal recessive genetic disease. In this report, we report a Brazilian patient with a rare ESCO2 variant. The patient manifested a broad range of clinical findings including the significant, bilateral shortening of the extremities. He deteriorated and passed away at 20 days of age. High-resolution GTG-banded karyotype showed lack of centromeric constriction in some chromosomes, premature centromere separation in others, and repulsion of the heterochromatin regions. Molecular analysis of the ESCO2 gene revealed a deletion of 4 bp involving exon 4 in homozygosity (NM_00107420.2:c.875_878delACAG), which causes loss of ESCO2 function. We describe the clinical presentation caused by a rare ESCO2 variant.

Prevalence of Chlamydia trachomatis Infection Diagnosed by Polymerase Chain Reaction in Female Sex Workers in a Northern Mexican City.

PURPOSE: We aimed to determine the association between Chlamydia trachomatis infection and female sex work, and the association between sociodemographic, obstetric, and behavioral characteristics of female sex workers and C. trachomatis infection. METHODS: Through a case-control study design, we studied 201 female sex workers and 201 age-matched women without sex work in Durango City, Mexico. C. trachomatis DNA was detected in cervical swab samples using polymerase chain reaction. RESULTS: C. trachomatis DNA was detected in 32 (15.9%) of the 201 cases and in 6 (3.0%) of the 201 controls (odds ratio [OR] = 6.15; 95% confidence interval [CI]: 2.5-15.0; P < 0.001). The frequency of infection with C. trachomatis in female sex workers did not vary (P > 0.05) regardless of the history of pregnancies, deliveries, cesarean sections, or miscarriages. Regression analysis of the behavioral characteristics showed that infection with C. trachomatis was associated only with consumption of alcohol (OR = 2.39; 95% CI: 1.0-5.71; P = 0.04). Conclusions: We conclude that C. trachomatis infection is associated with female sex work in Durango City, Mexico. This is the first age-matched case-control study on the prevalence of C. trachomatis infection in female sex workers in Mexico using detection of C. trachomatis DNA in cervical samples.

Human Papillomavirus Infection in Female Sex Workers: A Case Control Study.

BACKGROUND: To determine the association of infection with human papillomavirus (HPV) and the occupation of female sex worker; and the correlation of infection with HPV with sociodemographic, clinical and behavioral characteristics of female sex workers. METHODS: We performed a case-control study of 217 female sex workers and 354 women without sex work in Durango City, Mexico. We determined the prevalence of infection with HPV in cervical samples of women using polymerase chain reaction, and HPV genotypes were determined using line probe assay. Bivariate and multivariate analyses were used to assess the association between the characteristics of women and infection. RESULTS: Twelve (5.5%) of the 217 sex workers, and 10 (2.8%) of the 354 control women were positive for HPV DNA (age-adjusted OR = 1.51; 95% CI: 0.62 - 3.68; P = 0.36). Six (50.0%) of the 12 HPV DNA positive sex workers had infections with high-risk genotypes (16, 31, 33, 35, 51, 58). Seven (70%) of the 10 HPV DNA positive control women had infections with high-risk genotypes (16, 18, 56, 58, and 66). The frequency of high risk genotypes in the control women was equal with that found in the female sex workers (P = 0.41). Logistic regression analysis showed that the variable alcohol consumption was associated with HPV infection (OR = 4.0; 95% CI: 1.0 - 16.0; P = 0.04). CONCLUSIONS: No association between HPV infection and female sex work was found in our setting. High risk HPV genotypes were prevalent among the women studied. Results can be used for the design of preventive measures against HPV infection.

Valoración nutricional y hábitos alimenticios en niños de las Comunidades Indígenas Shuar, Morona Santiago - Ecuador / Nutritional assessment and food habits in children of Indigenous Communities Shuar, Morona Santiago - Ecuador / Avaliação nutricional e hábitos alimentares em crianças de Comunidades Indígenas Shuar, Morona Santiago

Resumen Introducción: No existen programas nutricionales significativos, dirigidos al conocimiento del estado nutricional y hábitos alimentarios de las comunidades indígenas Shuar que generen la promoción de programas de salud. La población más vulnerable de la etnia indígena Shuar es la infantil; quienes viven en zonas rurales y urbanas marginales con limitaciones en servicios de salud y educación debido a su asentamiento en zonas selváticas de difícil acceso. Objetivo: Evaluar el estado nutricional y hábitos alimenticios en niños y niñas de 0 a 12 años, mediante el análisis de datos antropométricos y pruebas bioquímicas como línea de base para intervenciones futuras en salud comunitaria. Materiales y métodos: Es un estudio con alcance cuantitativo de tipo no experimental, transversal-descriptivo. Se realizaron historias clínicas nutricionales, recordatorio de 24 horas, frecuencia de consumo de alimentos, así como una confrontación de datos antropométricos y muestra sanguínea para cuantificación de hematocrito capilar. Resultados: El 21,79% del total de niños atendidos en las 3 comunidades indígenas presenta Desnutrición. El 6,41% del total de niños atendidos en las 3 comunidades indígenas presenta bajo peso. El 1,92% del total de niños atendidos en las 3 comunidades indígenas presenta sobrepeso. Conclusión: La desnutrición se debe a múltiples factores, entre los cuales se ha identificado la ingesta de alimentos con escaso valor nutritivo, factores socioeconómico-culturales y a las grandes distancias para obtener alimentos diversificados.

Abstract Introduction: There are no significant nutritional programs, aimed at understanding the nutritional status and eating habits of the indigenous Shuar communities that generate the promotion of health programs. The most vulnerable population of the Shuar indigenous ethnic group is the infantile population; those who live in marginal rural and urban areas with limitations in health and education services due to their settlement in jungle areas of difficult access. Objective: Evaluate the nutritional status and eating habits of children from 0 to 12 years old, by analyzing anthropometric data and biochemical tests as a baseline for future community health interventions. Material and methods: It is a study with a quantitative scope of a non-experimental, transversal-descriptive nature. Nutritional clinical histories, 24-hour reminder, frequency of food consumption, as well as a comparison of anthropometric data and blood sample for quantification of capillary hematocrit were performed. Results: 21.79% of the total number of children served in the 3 indigenous communities presents Malnutrition. 6.41% of the total number of children served in the 3 indigenous communities is underweight. 1.92% of the total number of children treated in the 3 indigenous communities is overweight. Conclusions: Malnutrition is due to multiple factors, among which has been identified the intake of food with low nutritional value, socioeconomic-cultural factors and the great distances to obtain diversified food.

Resumo Introdução: Não existem programas nutricionais significativos, visando a compreensão do estado nutricional e hábitos alimentares das comunidades indígenas Shuar que geram a promoção de programas de saúde. A população mais vulnerável do grupo étnico indígena Shuar é a população infantil; aqueles que vivem em áreas rurais e urbanas marginais, com limitações nos serviços de saúde e educação, devido ao seu assentamento em áreas de difícil acesso na selva. Objetivo: Avaliar o estado nutricional e hábitos alimentares de crianças de 0 a 12 anos, analisando dados antropométricos e testes bioquímicos como base para futuras intervenções de saúde comunitária. Materiais e métodos: Trata-se de um estudo com escopo quantitativo, de natureza não-experimental, de natureza transversal-descritiva. Foram realizadas histórias clínicas nutricionais, lembrete de 24 horas, frequência de consumo alimentar, comparação de dados antropométricos e amostra de sangue para quantificação do hematócrito capilar. Resultados: 21,79% do total de crianças atendidas nas 3 comunidades indígenas apresenta Desnutrição. 6,41% do total de crianças atendidas nas três comunidades indígenas está abaixo do peso. 1,92% do total de crianças tratadas nas três comunidades indígenas está acima do peso. Conclusões: A desnutrição deve-se a múltiplos fatores, dentre os quais se identificou a ingestão de alimentos de baixo valor nutricional, fatores socioeconômicos-culturais e as grandes distâncias para obtenção de alimentos diversificados.

Glycine Increases Insulin Sensitivity and Glutathione Biosynthesis and Protects against Oxidative Stress in a Model of Sucrose-Induced Insulin Resistance.

Oxidative stress and redox status play a central role in the link between insulin resistance (IR) and lipotoxicity in metabolic syndrome. This mechanistic link may involve alterations in the glutathione redox state. We examined the effect of glycine supplementation to diet on glutathione biosynthesis, oxidative stress, IR, and insulin cell signaling in liver from sucrose-fed (SF) rats characterized by IR and oxidative stress. Our hypothesis is that the correction of glutathione levels by glycine treatment leads to reduced oxidative stress, a mechanism associated with improved insulin signaling and IR. Glycine treatment decreases the levels of oxidative stress markers in liver from SF rats and increases the concentrations of glutathione (GSH) and γ-glutamylcysteine and the amount of γ-glutamylcysteine synthetase (γ-GCS), a key enzyme of GSH biosynthesis in liver from SF rats. In liver from SF rats, glycine also decreases the insulin-induced phosphorylation of insulin receptor substrate-1 (ISR-1) in serine residue and increases the phosphorylation of insulin receptor ß-subunit (IR-ß) in tyrosine residue. Thus, supplementing diets with glycine to correct GSH deficiency and to reduce oxidative stress provides significant metabolic benefits to SF rats by improving insulin sensitivity.

High Seroprevalence of Toxoplasma Gondii Infection in Female Sex Workers: A Case-Control Study.

Through an age- and sex-matched case-control study, we sought to determine whether female sex workers have an increased risk of Toxoplasma gondii exposure and to determine the sociodemographic, work, clinical, and behavioral characteristics of these workers associated with T. gondii exposure. Female workers (n = 136) and controls (n = 272) were examined with enzyme-linked immunoassays (EIA) for the presence of anti-Toxoplasma IgG and IgM antibodies. IgM positive sera were additionally tested with enzyme linked-fluorescence immunoassay (ELFA). Anti-T. gondii IgG antibodies were found in 21 (15.44%) of 136 cases and in 10 (3.67%) of 272 controls (OR = 4.05; 95% CI: 1.84-8.89; P = 0.0001). Anti-T. gondii IgG levels higher than 150 IU/ml were found in 13 (9.6%) of 136 cases and in 8 (2.9%) of 272 controls (P = 0.007). Anti-T. gondii IgM antibodies were found in two cases and in six controls by EIA, but all were negative by ELFA. T. gondii seropositivity was associated with being born out of Durango State (OR = 10.47; 95% CI: 2.9-36.8; P < 0.01), injuries during sex work (OR = 6.30; 95% CI: 1.1-33.7; P = 0.03), and soil contact (OR = 4.11; 95% CI: 1.2-14.0; P = 0.02). This is the first report of an association of T. gondii infection and female sex workers.

Malformations detected by abdominal ultrasound in children with congenital heart disease.

BACKGROUND: Extracardiac malformations may be present in patients with congenital heart disease (CHD), bringing greater risk of comorbidity and mortality. OBJECTIVE: Verify frequency and types of abdominal abnormalities detected in children with and without CHD through abdominal ultrasound (AUS), compare the patients in relation to their dysmorphic/cytogenetic findings and perform an estimative of the cost-effectiveness of the screening through AUS. METHODS: We conducted a cross-sectional study with a control cohort. The cases consisted of patients with CHD admitted for the first time in a pediatric intensive care unit; the controls consisted of children without CHD who underwent AUS at the hospital shortly thereafter a case. All patients with CHD underwent AUS, high-resolution karyotype and fluorescence in situ hybridization (FISH) for microdeletion 22q11.2. RESULTS: AUS identified clinically significant abnormalities in 12.2% of the cases and 5.2% of controls (p= 0.009), with a power of significance of 76.6%. Most malformations with clinical significance were renal anomalies (10.4% in cases and 4.9% in controls; p= 0.034). In Brazil, the cost of an AUS examination for the Unified Health System is US$ 21. Since clinically significant abnormalities were observed in one in every 8.2 CHD patients, the cost to identify an affected child was calculated as approximately US$ 176. CONCLUSION: Patients with CHD present a significant frequency of abdominal abnormalities detected by AUS, an inexpensive and noninvasive diagnostic method with good sensitivity. The cost of screening for these defects is considerably lower than the cost to treat the complications of late diagnoses of abdominal malformations such as renal disease.

Malformações detectadas pelo ultrassom abdominal em crianças com cardiopatia congênita / Malformations detected by abdominal ultrasound in children with congenital heart disease

FUNDAMENTO: Malformações extracardíacas podem estar presentes em pacientes com cardiopatia congênita (CC), trazendo maior risco de comorbidade e mortalidade. OBJETIVO: Verificar a frequência e os tipos de anormalidades abdominais detectadas em crianças com e sem CC através do ultrassom abdominal (USA), comparar os pacientes quanto a seus achados dismórficos/citogenéticos e realizar uma estimativa do custo-benefício da triagem pelo USA. MÉTODOS: Foi realizado um estudo transversal com controle. Os casos consistiram de pacientes com CC admitidos pela primeira vez em uma unidade de terapia intensiva pediátrica; os controles consistiram de crianças sem CC submetidas ao USA no hospital logo após cada caso. Todos os pacientes com CC foram submetidos ao USA, ao cariótipo de alta resolução e à hibridização in situ fluorescente (FISH) para microdeleção 22q11.2. RESULTADOS: USA identificou anormalidades clinicamente significativas em 12,2% dos casos e em 5,2% dos controles (p = 0,009), com um poder de significância de 76,6%. A maioria das malformações com significado clínico foi de anomalias renais (10,4% nos casos e 4,9% nos controles, p = 0,034). No Brasil, o custo de um exame de USA pelo Sistema Único de Saúde é de 21 dólares. Uma vez que anormalidades clinicamente significativas foram observadas em um a cada 8,2 pacientes com CC, o custo para identificar uma criança afetada foi de 176 dólares. CONCLUSÃO: Pacientes com CC apresentam uma frequência significativa de anomalias detectadas pelo USA, um método diagnóstico barato e não invasivo, com boa sensibilidade. O custo da triagem para esses defeitos é consideravelmente menor que o custo para tratar as complicações do diagnóstico tardio de malformações abdominais, como a doença renal.

BACKGROUND: Extracardiac malformations may be present in patients with congenital heart disease (CHD), bringing greater risk of comorbidity and mortality. OBJECTIVE: Verify frequency and types of abdominal abnormalities detected in children with and without CHD through abdominal ultrasound (AUS), compare the patients in relation to their dysmorphic/cytogenetic findings and perform an estimative of the cost-effectiveness of the screening through AUS. METHODS: We conducted a cross-sectional study with a control cohort. The cases consisted of patients with CHD admitted for the first time in a pediatric intensive care unit; the controls consisted of children without CHD who underwent AUS at the hospital shortly thereafter a case. All patients with CHD underwent AUS, high-resolution karyotype and fluorescence in situ hybridization (FISH) for microdeletion 22q11.2. RESULTS: AUS identified clinically significant abnormalities in 12.2% of the cases and 5.2% of controls (p= 0.009), with a power of significance of 76.6%. Most malformations with clinical significance were renal anomalies (10.4% in cases and 4.9% in controls; p= 0.034). In Brazil, the cost of an AUS examination for the Unified Health System is US$ 21. Since clinically significant abnormalities were observed in one in every 8.2 CHD patients, the cost to identify an affected child was calculated as approximately US$ 176. CONCLUSION: Patients with CHD present a significant frequency of abdominal abnormalities detected by AUS, an inexpensive and noninvasive diagnostic method with good sensitivity. The cost of screening for these defects is considerably lower than the cost to treat the complications of late diagnoses of abdominal malformations such as renal disease.

High-sucrose diet increases ROS generation, FFA accumulation, UCP2 level, and proton leak in liver mitochondria.

Obesity, a risk factor for insulin resistance, contributes to the development of type 2 diabetes and cardiovascular diseases. The relationship between increased levels of free fatty acids in the liver mitochondria, mitochondrial function, and ROS generation in rat model of obesity induced by a high-sucrose diet was not sufficiently established. We determined how the bioenergetic functions and ROS generation of the mitochondria respond to a hyperlipidemic environment. Mitochondria from sucrose-fed rats generated H(2)O(2) at a higher rate than the control mitochondria. Adding fatty acid-free bovine serum albumin to mitochondria from sucrose-fed rats significantly reduced the rate of H(2)O(2) generation. In contrast, adding exogenous oleic or linoleic acid exacerbated the rate of H(2)O(2) generation in both sucrose-fed and control mitochondria, and the mitochondria from sucrose-fed rats were more sensitive than the control mitochondria. The increased rate of H(2)O(2) generation in sucrose-fed mitochondria corresponded to decreased levels of reduced GSH and vitamin E and increased levels of Cu/Zn-SOD in the intermembrane space. There was no difference between the levels of lipid peroxidation and protein carbonylation in the two types of mitochondria. In addition to the normal activity of Mn-SOD, GPX and catalase detected an increased activity of complex II, and upregulation of UCP2 was observed in mitochondria from sucrose-fed rats, all of which may accelerate respiration rates and reduce generation of ROS. In turn, these effects may protect the mitochondria of sucrose-fed rats from oxidative stress and preserve their function and integrity. However, in whole liver these adaptive mechanisms of the mitochondria were inefficient at counteracting redox imbalances and inhibiting oxidative stress outside of the mitochondria.

[Comparison of the diagnostic utility from visual inspection with acetic acid and cervical cytology]. / Comparación de la utilidad diagnóstica entre la inspección visual con acido acético y la citología cervical.

BACKGROUND: In Mexico, cervical cancer is the second leading cause of death in women after breast cancer. The human papillomavirus is associated with intraepithelial lesions, detected up to 99.7% of cervical carcinomas. Despite being easy to detect is a condition that many women suffer. OBJECTIVE: To determine the diagnostic utility of the visual inspection with acetic acid of the uterine cervix compared with the cervical cytology. PATIENTS AND METHOD: Study of diagnostic tests. The study was realized in the Centro de Atención Materno Infantil y Planificación Familiar of the Instituto de Investigación Científica, Durango, Mexico, research of the Juárez University of the State of Durango, from August 23, 2005 to November 13, 2006. 1,521 participants were examined who went consecutively to opportune detection of cervical cancer. One doctor practiced the test of acetic acid and cervical cytology to them, and one digital photograph, which was evaluated by three inter-observers triple blind. Those that was positive to anyone of these tests, were remitted to colposcopy and/or biopsy; also to 10% of selected negative population randomly was realized this procedure. Sensitivity, specificity, positive and negative predictive values and exactitude were determined. For the agreement inter-observer index of Kappa was used. RESULTS: Sensitivity, specificity, values predictive positive, negative and exactitude for the visual inspection with acetic acid were 20, 97, 5 and 99%, respectively. For the cervical cytology were of 80, 99, 57 and 99%, respectively. The force of agreement between the interobservant was poor. CONCLUSIONS: In this study cervical cytology was more useful than visual inspection with acetic acid to detect dysplasias or cervical cancer opportunely, due to detect all the positive true cases confirmed by biopsy.

Espectro óculo-aurículo-vertebral em pacientes com defeitos cardíacos congênitos / Oculo-auriculo-vertebral spectrum in patients with congenital heart defects / Espectro óculo-aurículo-vertebral en pacientes con defectos cardíacos congénitos

FUNDAMENTO: Há poucos estudos avaliando a frequência do espectro óculo-aurículo-vertebral (EOAV) nos pacientes com defeitos cardíacos congênitos (DCC). OBJETIVO: Verificar a frequência do EOAV em uma amostra de pacientes com malformações cardíacas graves. MÉTODOS: Avaliamos uma coorte prospectiva de pacientes com DCC, admitidos em uma unidade de terapia intensiva (UTI) cardiopediátrica no Brasil. O diagnóstico de EOAV foi feito com base em dados clínicos, considerando critérios padrões. Os pacientes que preenchiam esses critérios foram submetidos ao cariótipo de alta resolução com bandamento GTG e hibridização fluorescente in situ para a microdeleção 22q11.2. O teste exato de Fisher (P < 0,05) foi usado para as análises estatísticas. RESULTADOS: Durante o período de avaliação, 330 pacientes foram hospitalizados pela primeira vez na UTI, mas trinta deles não participaram deste estudo. Dos 300 pacientes que constituíram a amostragem final, o EOAV foi verificado em três casos (1 por cento). Todos apresentaram estudos citogenéticos normais. CONCLUSÃO: O EOAV parece ser uma condição frequente em pacientes com DCC. Contudo, não podemos excluir a possibilidade de que a frequência de EOAV encontrada em nosso estudo possa ter sido subestimada devido à baixa taxa de detecção pré-natal de DCC e ao acesso limitado dos pacientes ao tratamento médico apropriado em nosso meio. Estudos prospectivos futuros com critérios clínicos bem definidos e pacientes com defeitos leves e graves serão importantes para avaliar o papel do EOAV na população em geral de indivíduos com malformações cardíacas.

BACKGROUND: There have been few studies evaluating the frequency of oculo-auriculo-vertebral spectrum (OAVS) in patients with congenital heart defects (CHDs). OBJECTIVE: To verify the frequency of OAVS in a sample of patients with major heart malformations. METHODS: We evaluated a prospective cohort of patients with CHD admitted in a pediatric cardiac intensive care unit (ICU) in Brazil. The diagnosis of OAVS was made based on the clinical data, considering standard criteria. The patients that met these criteria were submitted to high resolution GTG-Banding karyotype and fluorescence in situ hybridization for 22q11.2 microdeletion. Fisher's exact test (P < 0.05) was used for the statistical analysis. RESULTS: During the period of evaluation, 330 patients were hospitalized for the first time in the ICU, but thirty of them did not participate in the study. Of the 300 patients that constituted the final sample, OAVS was verified in 3 cases (1 percent). All presented normal cytogenetic studies. CONCLUSION: OAVS seems to be a frequent condition among patients with CHDs. However, we cannot exclude the possibility that the frequency of OAVS found in our study might have been underestimated due to the low rate of prenatal detection of CHDs and the limited access of patients to appropriate health care in our region. Future prospective studies with well defined clinical criteria and subjects with mild and major defects will be important to assess the role of OAVS in the general population of subjects with heart malformations.

FUNDAMENTO: Hay pocos estudios evaluando la frecuencia del espectro óculo-aurículo-vertebral (EOAV) en los pacientes con defectos cardíacos congénitos (DCC). OBJETIVO: Verificar la frecuencia del EOAV en una muestra de pacientes con malformaciones cardíacas graves. MÉTODOS: Evaluamos una cohorte prospectiva de pacientes con DCC, admitidos en una unidad de terapia intensiva (UTI) cardiopediátrica en el Brasil. El diagnóstico de EOAV fue hecho con base en datos clínicos, considerando criterios estándares. Los pacientes que llenaban esos criterios fueron sometidos al cariotipo con bandeamiento GTG en alta resolución e Hibridización fluorescente in situ para la microdelección 22q11.2. El Test Exacto de Fisher (P < 0,05) fue usado para los análisis estadísticos. RESULTADOS: Durante el período de evaluación, 330 pacientes fueron hospitalizados por primera vez en la UTI, pero treinta por ciento de ellos no participó de este estudio. De los 300 pacientes que constituyeron el muestreo final, el EOAV fue verificado en tres casos (1 por ciento). Todos presentaron estudios citogenéticos normales. CONCLUSIÓN: El EOAV parece ser una condición frecuente en pacientes con DCC. Con todo, no podemos excluir la posibilidad de que la frecuencia de EOAV encontrada en nuestro estudio pueda haber sido subestimada debido a la baja tasa de detección pre-natal de DCC y al acceso limitado de los pacientes al tratamiento médico apropiado en nuestro medio. Estudios prospectivos futuros con criterios clínicos bien definidos y pacientes con defectos leves y graves serán importantes para evaluar el papel del EOAV en la población en general de individuos con malformaciones cardíacas.

[Oculo-auriculo-vertebral spectrum in patients with congenital heart defects]. / Espectro óculo-aurículo-vertebral em pacientes com defeitos cardíacos congênitos.

BACKGROUND: there have been few studies evaluating the frequency of oculo-auriculo-vertebral spectrum (OAVS) in patients with congenital heart defects (CHDs). OBJECTIVE: to verify the frequency of OAVS in a sample of patients with major heart malformations. METHODS: we evaluated a prospective cohort of patients with CHD admitted in a pediatric cardiac intensive care unit (ICU) in Brazil. The diagnosis of OAVS was made based on the clinical data, considering standard criteria. The patients that met these criteria were submitted to high resolution GTG-Banding karyotype and fluorescence in situ hybridization for 22q11.2 microdeletion. Fisher's exact test (P < 0.05) was used for the statistical analysis. RESULTS: During the period of evaluation, 330 patients were hospitalized for the first time in the ICU, but thirty of them did not participate in the study. Of the 300 patients that constituted the final sample, OAVS was verified in 3 cases (1%). All presented normal cytogenetic studies. CONCLUSION: OAVS seems to be a frequent condition among patients with CHDs. However, we cannot exclude the possibility that the frequency of OAVS found in our study might have been underestimated due to the low rate of prenatal detection of CHDs and the limited access of patients to appropriate health care in our region. Future prospective studies with well defined clinical criteria and subjects with mild and major defects will be important to assess the role of OAVS in the general population of subjects with heart malformations.

Tonsil size as a predictor of cardiac complications in children with sleep-disordered breathing.

OBJECTIVES/HYPOTHESIS: The primary objective was to evaluate the association of palatine (T/P) tonsil size determined by radiography with pulmonary artery pressure (PAP) measured by Doppler echocardiography in children with surgical indication for adenotonsillar hypertrophy. The secondary objective was to evaluate if tonsil size could help to identify children at higher risk of pulmonary artery hypertension (PAH). STUDY DESIGN: Cross-sectional study. METHODS: The study was conducted with a consecutive sample of children with indication of adenotonsillectomy for sleep-disordered breathing. T/P was measured by lateral neck radiography, PAP by echodopplercardiography, and symptoms and quality of life by the Obstructive Sleep Apnea (OSA)-18 questionnaire. T/P was plotted in a receiver operating characteristic (ROC) curve to determine the best cut-off point to identify children with PAH. RESULTS: A total of 45 children participated in the study. The mean age was 72.0 +/- 32.3 months, and six (13%) patients had PAH. Correlation between systolic PAP and T/P was strong (r = 0.624; P < .0001). T/P was significantly higher in patients with PAH (P < .001). OSA-18 score did not significantly correlate with the variables. The cut-off point identified in the ROC was 0.66, which was the minimum T/P where sensitivity was still 100%. Mean systolic pulmonary artery pressure in children with T/P >0.66 was significantly higher than in those with T/P <0.66 (P < .001). CONCLUSIONS: T/P showed a good correlation with PAP in children with adenotonsillar hypertrophy and surgical indication for sleep-disordered breathing. Children with T/P >0.66 can be at greater risk for cardiac complications and should be submitted to complementary studies with echodopplercardiography or given preference for surgery.

Nevus blanco esponjoso familiar / Family nevus spongiosus albus

El nevus blanco esponjoso (NBE) es una rara condición autosómica dominante, caracterizada por placas blancas bilaterales en la mucosa, de aspecto esponjoso, blandas a la palpación y que pueden escamarse. Los tratamientos son paliativos; y el uso de antibióticos, en especial la tetraciclina, ha demostrando buenos resultados en su control. Este trabajo presenta tres casos clínicos de una familia afectada por NBE, donde se discuten los posibles diagnósticos diferenciales y conductas terapéuticas indicadas. Un paciente masculino de 52 años de edad acudió a la clínica aquejado de lesiones blancas bilaterales. El paciente notó las lesiones 30 años antes, sin lograr un diagnóstico final de las mismas. Después de la anamnesis y del examen clínico fue realizada una biopsia incisional. La reunión de los datos clínicos e histopatológicos llevó al diagnóstico de NBE. Se le solicitó al paciente que indagase entre sus familiares con respecto a lesiones semejantes. Se detectó que el hijo de 19 años y la hija de 25 eran portadores de placas blancas en la mucosa yugal. Como no había afectación estética, se optó por no intervenir en las lesiones. El nevus blanco esponjoso es una lesión genética que debe ser diferenciada de otras patologías localizadas y sistémicas importantes, que tienen repercusiones serias para el individuo. Como no hay un tratamiento curativo para el NBE, el papel del cirujano dentista es diagnosticar esta lesión, aclarar al paciente sobre la naturaleza benigna y autolimitante del NBE y si fuera necesario desde el punto de vista estético, aplicar diferentes modalidades terapéuticas(AU)

The aim of present paper is to introduce three clinical cases from a family affected from nevus spongiosus albus (NSA) and also to discuss the possible differential diagnoses as well as the therapeutical behaviors to be adopted. Clinical case: A man aged 52 seen in our clinic due to bilateral white lesions noted 30 years ago without achieve a final diagnosis of lesions. After anamnesis and physical examination an incision biopsy was taken. The clinical and histopathological data collection allows making the NSA diagnosis. Thus, it was necessary to inquire again into the patient's relatives regarding the existence of similar lesions proving the presence of white plaques in oral mucosa in a son aged 19 and a daughter aged 25. The nevus spongiosus albus is an uncommon genetic lesion that must to be differentiated from other significant localized and systemic pathologies with serious repercussions for the subjects. Since there is not a curative treatment for the NSA, the role of the surgeon-dentist is to diagnose that lesion, to explain clearly to patient on the benign and self-limiting origin of this entity and if it is necessary from the aesthetic point of view, to apply the different therapeutical modalities to control the plaques(AU)

Displasia fibrosa da mandíbula / Fibrous dysplasia of mandible

A displasia fibrosa da mandíbula é uma patologia óssea benigna que representa cerca de 2% de todos os tumores ósseos. Relatamos o caso de uma paciente de 11 anos de idade, com queixa de dor em região mandibular esquerda há 1 semana, com história de abaulamento da mesma região há 3 meses. Após realização de exames de imagem, foi diagnosticado displasia fibrosa da mandíbula. O objetivo deste relato é revisar as principais características clínicas, radiológicas e possíveis tratamentos para o caso, dando a devida importância para o seguimento ambulatorial pós-tratamento

Fibrous dysplasia is a benign bone disease which represents about 2% of all bone tumors. Here we report the case of an 11-year-old who complained of pain at the left mandible area for a week, with history of arching in this area for 3 months. Imaging examinations led to the diagnosis of fibrous dysplasia of the mandible. The aim of this work is to review the main clinical and radiological characteristics and possible treatments for the case, giving due importance to ambulatory follow-up care