Origem anômala de artéria coronária esquerda do tronco pulmonar: síndrome de Bland-White-Garland

A origem anômala da artéria coronária esquerda a partir do tronco pulmonar, conhecida como síndrome de Bland-White-Garland (BWG), é uma doença rara, que, habitualmente, leva à morte antes do primeiro ano de vida. Os autores relatam o caso de uma criança branca, com 2 anos e 6 meses de idade, portadora da síndrome de BWG, e revisam a apresentação clínica, a fisiopatologia, o diagnóstico e o tratamento desses pacientes.

Polioencefalomalacia experimental em bovinos induzida por toxicose por enxofre

O presente trabalho teve como objetivos avaliar os sinais clínicos, as concentrações do sulfeto de hidrogênio ruminal e as alterações anatomopatológicas associadas à intoxicação experimental por enxofre em bovinos. Foram utilizados dez bezerros mestiços leiteiros, sendo que quatro bovinos ingeriram ração sem sulfato de sódio (G1) e seis consumiram ração com sulfato de sódio (G2). Exames clínicos (temperatura retal, frequência cardíaca e respiratória e motricidade ruminal) e laboratoriais (hemograma, fibrinogênio, proteína plasmática, pH do fluido ruminal, concentração do sulfeto de hidrogênio ruminal, líquido cerebrospinal e histopatológico) foram realizados. A temperatura retal, frequência cardíaca, hemograma, fibrinogênio, proteína plasmática, pH do fluido ruminal e os valores do líquido cerebrospinal estavam dentro dos valores de referência para a espécie. Taquipnéia, hipomotricidade ruminal e elevados valores de sulfeto de hidrogênio ruminal foram observados nos bezerros do grupo G2. Um bezerro do grupo G2 apresentou sinais neurológicos e lesões histopatológicas de PEM. Dois animais de cada grupo foram eutanasiados. Lesões microscópicas foram observadas nos bezerros do G2. Histologicamente as alterações observadas foram necrose neuronal cortical e lesões hemorrágicas nos núcleos basais, tálamo, mesencéfalo, ponte e bulbo. O protocolo experimental constituído por uma dieta rica em carboidrato de alta fermentação, baixa quantidade de fibra efetiva e altos níveis de enxofre (0,52%) ocasionou alterações clinicas e histológicas e elevadas concentrações de sulfeto de hidrogênio ruminal compatíveis com quadro de intoxicação por enxofre.

Insulin secretion, insulin sensitivity, and hepatic insulin extraction in first-degree relatives of type 2 diabetic patients

To identify early metabolic abnormalities in type 2 diabetes mellitus, we measured insulin secretion, sensitivity to insulin, and hepatic insulin extraction in 48 healthy normal glucose-tolerant Brazilians, first-degree relatives of type 2 diabetic patients (FH+). Each individual was matched for sex, age, weight, and body fat distribution with a person without history of type 2 diabetes (FH-). Both groups were submitted to a hyperglycemic clamp procedure (180 mg/dl). Insulin release was evaluated in its two phases. The first was calculated as the sum of plasma insulin at 2.5, 5.0, 7.5, and 10.0 min after the beginning of glucose infusion, and the second as the mean plasma insulin level in the third hour of the clamp procedure. Insulin sensitivity index (ISI) was the mean glucose infusion rate in the third hour of the clamp experiment divided by the mean plasma insulin concentration during the same period of time. Hepatic insulin extraction was determined under fasting conditions and in the third hour of the clamp procedure as the ratio between C-peptide and plasma insulin levels. FH+ individuals did not differ from FH- individuals in terms of the following parameters [median (range)]: a) first-phase insulin secretion, 174 (116-221) vs 207 (108-277) µU/ml, b) second-phase insulin secretion, 64 (41-86) vs 53 (37-83) µU/ml, and c) ISI, 14.8 (9.0-20.8) vs 16.8 (9.0-27.0) mg kg-1 min-1/µU ml-1. Hepatic insulin extraction in FH+ subjects was similar to that of FH- ones at basal conditions (median, 0.27 vs 0.27 ng/µU) and during glucose infusion (0.15 vs 0.15 ng/µU). Normal glucose-tolerant Brazilian FH+ individuals well-matched with FH- ones did not show defects of insulin secretion, insulin sensitivity, or hepatic insulin extraction as tested by hyperglycemic clamp procedures.

Paracoccidioidomycosis in a Dog: Case Report of Generalized Lymphadenomegaly

Paracoccidioidomycosis (PCM) is a severe systemic mycosis, endemic in Latin America and highly prevalent in Brazil, where it ranks eighth as a mortality cause among infectious and parasitic diseases in humans. The disease in animals has been little explored. It is observed that armadillos can harbor the fungus at high frequencies, although the active disease has not been well documented in this wild mammal. Dogs are susceptible to experimental infection, and the naturally acquired PCM-disease was reported only recently in a dog from Brazil. The present work reports the second case of naturally acquired PCM in a 6-year-old female dog that presented emaciation, lymphadenomegaly, and hepatosplenomegaly. Biochemical and pulmonary radiographic evaluation did not reveal any abnormalities. PCM was diagnosed by clinical findings, culturing, immunohistochemistry, and histopathology of popliteal lymph node. The fungus was recovered from popliteal lymph node, and the molecular analysis showed respective sequencing similarities of 99 and 100% for 803 nucleotides of the Gp43 gene and 592 nucleotides from the ITS-5.8S region of Paracoccidioides brasiliensis. Immunohistochemistry revealed severe lymphadenitis and presented numerous yeasts, which reacted against the gp43 antibody. Histopathology revealed a severe granulomatous lymphadenitis associated with numerous single or multiple budding yeasts. After diagnosis, the dog was successfully treated with itraconazol for 2 years. Veterinarians should be aware of the importance of considering PCM for differential diagnosis, especially in dogs from PCM-endemic areas, whose monophagocytic system involvement is evident.

Systemic response induced by Scorpaena plumieri fish venom initiates acute lung injury in mice

Scorpaena plumieri venomous fish inflicted severe injuries in humans characterized by systemic effects and cardiovascular abnormalities. Although cardiotoxic and hypotensive effects induced in rats by this venom have been studied, little is known about their effect on bronchial epithelial permeability and airway inflammation in mice. The primary goal of this study was to determine whether the intraplantar or intraperitoneal injection of S. plumieri venom results in systemic response, and whether this event initiates acute lung injure. We found that BALB/c mice developed neutrophilic infiltrates, areas of lung hemorrhage and alveolar macrophage activation within 24 h after injection with S. plumieri venom. These histopathological changes were associated with an early increase in BAL fluid protein and early induction of cytokines, chemokines and matrix metaloproteinases, followed by a later increase in BAL fluid neutrophils. These findings provide clear evidence that the injection of S. plumieri venom in footpad or peritoneal cavity of mice results in venom deposition in the airway and initiates a sustained inflammatory response in the lungs. (C) 2007 Elsevier Ltd. All rights reserved.

COMPARATIVE EVALUATION of OCULOMETRIC VARIABLES IN GRAVES' OPHTHALMOPATHY

OBJECTIVES: To estimate oculometric parameters of Graves' ophthalmopathy in comparison to healthy eyes using digital photography and digital image analysis.INTRODUCTION: Graves' ophthalmopathy is the main cause of eye proptosis. Because these protrusions cause clinically perceived distortions in orbital architecture, digital photographs can be used to detect and quantify these changes.METHODS: We carried out a cross-sectional study comprising 12 healthy volunteers and 15 Graves' ophthalmopathy patients with the purpose of evaluating the use of simple, non-invasive digital photography to estimate oculometric parameters of Graves' ophthalmopathy and compare them with the parameters of unaffected eyes. Facial photographs of cases and controls were taken in a standardized manner. Oculometric parameters were compared between the groups and then correlated to proptometer measures.RESULTS: All estimated oculometric variables showed significant differences between the groups, in particular with regard to mediopupilar aperture, lateral height, distance from the iris edge to the lateral boundary of the palpebral fissure, and distance from the higher point of the iris to the lateral limit of the palpebral fissure. The product of medial aperture and horizontal palpebral fissure also revealed greater discrepancy between the groups. Proptometer measures showed significant linear correlation between the distance from the iris edge to the lateral boundary of the palpebral fissure and between the distance from the higher point of the iris to the lateral limit of palpebral fissure (p<0.05).CONCLUSIONS: Comparative analysis of oculometric parameters in Graves' ophthalmopathy suggests that eye proptosis is related to an asymmetric increase in lateral oculometric measures. Standardized digital photographs can be used in clinical practice to objectively estimate oculometric parameters of Graves' ophthalmopathy patients.

Você conhece esta síndrome?

A síndrome de Rothmund-Thomson é distúrbio autossômico recessivo de expressividade variável associado a mutações do gene RecQL4. Caracteriza-se por poiquilodermia, alopecia, defeitos de crescimento e desenvolvimento, catarata juvenil, alterações dentárias e esqueléticas e predisposição ao câncer cutâneo e ao osteossarcoma. Relata-se caso de paciente de 29 anos de idade com lesões cutâneas desde a infância, catarata bilateral antes dos 20 anos e carcinoma espinocelular aos 26 anos de idade.

Dysphonia and laryngeal sequelae in paracoccidioidomycosis patients: a morphological and phoniatric study

The larynx is the third most commonly involved organ in paracoccidioidomycosis (PCM). While a few studies have evaluated laryngeal sequelae, there have not been any investigations of voice abnormalities in PCM patients. To evaluate persistent dysphonia and laryngeal lesions, we studied 15 normal subjects and 30 post-treatment PCM patients, i.e., 15 with only pulmonary and 15 with both laryngeal and pulmonary involvement. Perceptual and acoustic voice analysis were performed with all patients, while endoscopic studies were also conducted with the 15 laryngeal patients. Voice analysis showed instability by perceptual analysis (P < 0.01) in both groups, but more severe dysphonia was noted in the laryngeal group (P < 0.01). The dysponia, seen in 66.7% of these patients (dysphonia index < 7.0), was characterized by roughness and breathness. The Dr. Speech (Tiger Electronics) analysis program did not accept five voices from the laryngeal group due to the severe dysphonia. Jitter was elevated in five laryngeal lesion patients. Endoscopy showed that 80% of patients with laryngeal lesion had two or more laryngeal structures involved. Vocal fold alterations were seen in all laryngeal lesion patients, which included involvement of the arythenoids, epiglottis, and vestibular folds. This first functional study of laryngeal sequelae in PCM revealed frequent and severe dysphonia that may have important social consequences for patients.

Microbiota intestinal de indivíduos que sofreram acidente ocupacional com materiais biológicos e que realizaram profilaxia anti-retroviral

Avaliar a microbiota intestinal de indivíduos que sofreram acidente ocupacional com materiais biológicos e receberam anti-retrovirais foi o objetivo deste estudo. O grupo de estudo constou de 23 indivíduos com idade entre 18-45 anos, sendo 13 doadores de sangue e 10 que sofreram acidente ocupacional. Foram avaliados a microbiota intestinal, antropometria e exames laboratoriais pré, pós e 30 dias após o término da medicação. Zidovudina mais lamivudina foi utilizada em 70% dos indivíduos associado ao nelfinavir, 20% ao efavirenz e 10% ao ritonavir. As alterações nutricionais e dietéticas-laboratoriais e de microbiota intestinal foram analisadas em três momentos. M1: até dois dias do início da profilaxia; M2: no último dia da profilaxia e M3: 30 dias após o término da profilaxia. Náuseas, vômitos e diarréia estiveram presentes em 50% no segundo momento do estudo. Sobrepeso em 70%, desnutrição e eutrofia em 10%, dos indivíduos, não se modificaram durante o estudo. Transaminases, triglicérides, LDL-colesterol se elevaram no segundo momento e normalizaram 30 dias após término da medicação. Houve redução significativa dos Lactobacillus, Bifidobacterium e Bacteróides nos três momentos. Uso de anti-retrovirais provocou impacto significativo na microbiota intestinal dos indivíduos, sem recuperação em 30 dias.

O significado do trabalho em equipe na reabilitação de pessoas com malformação craniofacial congênita

Este estudo objetivou compreender o significado do trabalho em equipe dos profissionais de reabilitação em anomalias craniofaciais. Realizou-se análise fenomenológica por contemplar a compreensão e interpretação do sentido considerando o sujeito. Entrevistou-se 12 profissionais de diferentes áreas, norteadas pela questão: O que significa para você trabalhar em equipe na reabilitação de anomalias craniofaciais? Resgatando-se os temas: Capacitação para o trabalho, Dificuldade para trabalho em equipe, Relação com paciente e família, Condições de trabalho e A inserção do profissional na equipe. A análise visou refletir o fenômeno engendrando convergências e divergências destacando a explicitação das diferenças e aprendizado contínuo.

Vulvar carcinoma in young patients and its relationship with genital warts

We report the occurrence of aggressive vulvar carcinoma associated with condyloma acuminata in three patients: under 33 years old. Discussion of the role of the human papilloma virus (HPV) in the development of vulvar cancer is also presented. Three patients with condyloma associated with aggressive vulvar squamous cell carcinoma, in situ (1 case) and invasive (2 cases), documented by biopsy and/or vulvectomy are presented. In situ hybridization (ISH) was used to characterize the subtypes of HPV. One patient with erythematous systemic lupus developed in situ carcinoma after 5 years. The other two cases also developed aggressive multicentric, invasive squamous cell carcinoma after 10 years of diagnosis of condyloma. In all cases HPV cytological abnormalities were seen throughout the pathological examination. HPV 16 and 18 were present in cells of invasive squamous cell carcinoma in cases 2 and 3. HPV 6 and 11 were detected only in the condyloma area in case 2. HPV 30 was seen only in the condyloma area in case 3. This report emphasizes the need for biopsies of all unusually persistent or treatment-resistant condylomas, particularly in young and/or immunoisuppressed patients.

Sintomas depressivos e ansiosos em mães de recém-nascidos com e sem malformações

OBJETIVO: comparar as condições emocionais de mães cujos filhos nascem com malformações visíveis (Grupo M) com as das mães de crianças eutróficas (Grupo E) logo após o nascimento. MÉTODO: foram avaliados os sintomas de ansiedade e depressão de 22 mães de cada grupo por meio do Inventário de Depressão de Beck (BDI) e do Inventário de Ansiedade Traço-Estado (IDATE). Foram excluídas as mães portadoras de deficiência sensorial incapacitante, HIV, distúrbios psiquiátricos e síndromes genéticas. Os dados foram complementados com consultas a prontuários médicos da criança e da mãe. Para análise comparativa entre as medianas dos grupos foi utilizado o teste não-paramétrico U de Mann-Whitney; para amostras independentes e para os escores indicativos de sintomas clínicos, o teste exato de Fisher e o teste do χ2. RESULTADOS: houve diferenças significativas nas medianas dos escores das três subescalas (ansiedade-traço, ansiedade-estado e disforia/depressão) entre os dois grupos de mães. Houve uma porcentagem significativamente maior de mães do Grupo M com escores indicativos de sinais clínicos para depressão ou ansiedade no pós-parto imediato e, para ambos os quadros, quando comparadas com mães do Grupo E. Os resultados podem ser decorrentes de traços de personalidade materna, visto que os índices de ansiedade-traço eram significativamente maiores nas mães de crianças malformadas, mas especialmente pelo estado da criança, seu encaminhamento para a UTI e sua condição de vida futura. CONCLUSÕES: a porcentagem de mães de recém-nascidos com malformações visíveis que apresentou escores indicativos de sinais clínicos para ansiedade, depressão e ambos sugerem a necessidade de suporte, individual ou em grupo.

Motile sperm organelle morphology examination is stricter than Tygerberg criteria

The present study aimed to evaluate the correlation between the motile sperm organelle morphology examination (MSOME) and a well-known sperm morphology classification (Tygerberg criteria). For MSOME, spermatozoa were analysed at x8400 magnification by inverted microscope equipped with Nomarski differential interference contrast optics, Uplan Apo x 100 oil/1.35 objective lens and variable zoom lens. By Tygerberg criteria, the semen underwent morphological evaluation as described in the literature. Regression analysis demonstrated significant positive correlation between percentage of normal sperm forms by Tygerberg criteria and by MSOME (r = 0.83, P < 0.0001). However, the incidence of normal spermatozoa by Tygerberg criteria (9.4%) was significantly higher (P < 0.0001) than under MSOME (3.3%). Despite the highly positive correlation, MSOME is a much stricter criterion of sperm morphology classification, since it identifies vacuoles and chromatin abnormalities that are not evaluated with the same precision by the analysis of Tygerberg criteria. MSOME should be included among the routine criteria for semen analysis. In addition, MSOME should be used for selection of spermatozoa for intracytoplasmic sperm injection based on the already published literature, as this is a good selection tool.

Effect of physiological overload on pregnancy in women with mitral regurgitation

Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)

Correlation between semen analysis by motile sperm organelle morphology examination and sperm DNA damage

Regression analysis of 538 semen samples demonstrated that percentages of normal nuclear sperm and all spermatozoa with abnormalities of nuclear form at high magnification had significant negative correlation with percentages of DNA fragmentation. on the other hand, there was a positive correlation between percentages of spermatozoa with nuclear vacuoles and those with DNA fragmentation. (Fertil Steril (R) 2010;94:1937-40. (C) 2010 by American Society for Reproductive Medicine.)