Fatores associados ao aumento da espessura do septo atrial em lactentes com Síndrome da Hipoplasia do Coração Esquerdo (SHCE): implicações para septostomia percutânea

Introdução: Nos procedimentos usualmente realizados em recém-nascidos acometidos pela Síndrome da Hipoplasia do Coração Esquerdo (SHCE), variações fenotípicas influenciam o sucesso do tratamento, principalmente as características relacionadas ao septo atrial e ao forame oval (FO) no caso de septostomia por cateter. Objetivos: Analisar características macroscópicas do septo atrial de corações com SHCE, a fim de definir e orientar procedimentos terapêuticos nessa estrutura. Métodos: Foram avaliados 18 corações de pacientes falecidos e diagnosticados com SHCE quanto à perviedade e tamanho do FO, abaulamento da lâmina, atresia ou perviedade da válvula mitral e calibre da aorta ascendente e tronco pulmonar. Cortes histológicos do septo atrial foram feitos para medidas da espessura máxima e mínima da lâmina. Resultados: A idade média dos pacientes foi de 34,5 dias (57% do sexo masculino), com espessura do septo atrial médio de 1,90 mm (0,63 - 4,09 mm). O diâmetro médio do tronco e da aorta pulmonar foi de 1,16 cm e 0,22 cm, respectivamente. O FO era patente em 39% dos casos. A valva mitral era atrésica em 21% das amostras. Houve diferença significativa na espessura do septo atrial nos casos com FO patente ou fechada, sendo maior nos casos em que a FO era fechada (p = 0,047). A relação FO / idade apresentou correlação negativa estatisticamente significante com a espessura do septo atrial (r = -0,76 ep <0,05). Conclusões: Os resultados indicam que a perviedade e o tamanho do forame oval têm repercussões na espessura do septo atrial, sugerindo que esse fator pode limitar o sucesso de intervenções terapêuticas, principalmente na septostomia por cateter.Introduction: In the usual procedures performed on newborns affect by Hypoplastic Left Heart Syndrome (HLHS), phenotypic variations influence the success of the treatment, especially the characteristics related to the atrial septum and foramen ovale (FO) in case of catheter septostomy. Objectives: To analyze macroscopics features of the atrial septum of hearts with HLHS in order to define and guide therapeutic procedures on this structure. Methods: 18 hearts of deceased patients diagnosed with HLHS were evaluated for FO patency and size, bulging of its blade, atresia or patency of the mitral valve and caliber of the ascending aorta and pulmonary trunk. Histological sections of the atrial septum were made for measurements of maximum and minimum thickness of the lamina. Results: The mean age of the patients was 34.5 days (57% male), with a mean atrial septal thickness of 1.90 mm (0.63 - 4.09 mm). The mean diameter of the pulmonary trunk and aorta were 1.16 cm and 0.22 cm, respectively. FO was patent in 39% of cases. The mitral valve was atresic in 21% of the specimens. There was a significant difference in the thickness of the atrial septum in cases with patent or closed FO, being greater in cases where the FO was closed (p = 0.047). The ratio FO/age presented a statistically significant negative correlation with atrial septum thickness (r = -0.76 and p <0.05). Conclusions: The results indicate that the patency and size of the oval foramen have repercussions on the thickness of the atrial septum, suggesting that this factor may limit the success of therapeutic interventions, especially catheter septostomy

Factors associated with increased atrial septum thickness in infants with left heart hypoplasia syndrome: implications for percutaneous septostomy

Introduction: Hypoplastic Left Heart Syndrome (HLHS) encompasses a spectrum of cardiac malformations characterized by underdevelopment of the left heart structures and aorta that, despite the low incidence among cases of congenital heart disease have high mortality rates. Phenotypic variations of the anomaly may influence the success of the interventional and surgical procedures, especially the characteristics related to the atrial septum and foramen ovale (FO), in cases where balloon catheter septostomy is necessary to maintain the interatrial shunt.Objectives: To analyze the gross features of the atrial septum of hearts with HLHS in order to define and guide therapeutic procedures on this structure, correlating them with other morphological alterations of the syndrome, such as the anatomy of the heart valves. Methods: We studied 18 hearts of patients diagnosed and deceased with HLHS, all belonging to the anatomic collection of the Laboratory of Pathology of the Heart Institute (InCor) HCFMUSP. The atrial septum was evaluated for FO patency and size, as well as for the presence of bulging of its lamina to one or the other atrial cavity. Other morphological features of the anatomical specimen such as atresia or patency of the mitral valve and caliber of the ascending aorta and pulmonary trunk were annotated. After resection of the atrial septum, histological sections were made for microscopic measurements of the maximum and minimum thickness of the lamina (atrial septum).Results: The mean age of the patients was 34.5 days (57% male), with a mean atrial septal thickness of 1.90 mm (0.63-4.09 mm). The mean diameter of the pulmonary trunk and aorta were 1.16 cm and 0.22 cm, respectively. FO was patent in 39% of cases (mean diameter 4,4 mm, range 4 to 8 mm), with bulging of the lamina to one of the atrial cavities in half of the cases. The mitral valve was atretic in 21% of the specimens. There was a significant difference in the mean thickness of the atrial septum in cases with patent versus closed FO, being thicker in cases where the FO was closed (p = 0.047). The ratio FO/age presented a statistically significant negative correlation with the atrial septum thickness (r = -0.76 and p <0.05).Discussion and Conclusions: The results indicate that the patency and size of the oval foramen may influence the thickness of the atrial septum, suggesting that this factor may limit the success of therapeutic interventions, especially balloon catheter septostomy

Development of an instrument to evaluate the knowledge that the patient with hepatic cirhosis has about his disease and treatment

Introduction: hepatic cirrhosis (HC) is a disease with high morbidity and mortality in Brazil. Its treatment is complex and requires from lifestyle changes to large surgeries such as liver transplantation. To reach the best treatment results, it is necessary to guarantee a good patient’s adherence to the treatment. Studies indicate that the patient’s self-knowledge about his or her clinical condition is a determining factor in its adherence.Objectives: to assist in the design of an instrument that evaluates knowledge about the disease and treatment of HC. To improve the student knowledge on liver cirrhosis. Development of skills related to physical examination of the patient with HC.Methodology: The development process of the instrument will be divided into 3 stages: construction of the instrument (1st stage), evaluation of content validity and clarity of the instrument (2nd stage) and assessment of the reliability of the instrument (3 rd stage).Results: an instrument to evaluate the cirrhotic patient knowledge about the disease was made, analyzed by specialists and approved in the criteria proposed.Conclusions:1) The development of the instrument followed the steps described in literature.2) The suggestion of the evaluators allowed to restructure the questions making them clearer and more relevant to the proposal of the instrument.3) it was possible to achieve satisfactorily the proposal of the construction of the instrument.Key words: Liver cirrhosis; Self concept; Surveys and questionnaires; Health education

Development of an instrument to evaluate the knowledge that the patient with hepatic cirrhosis has about his disease and treatment

Introdução: cirrose hepática (CH) é uma doença com alta morbidade e mortalidade no Brasil. Seu tratamento é complexo e requer desde mudanças no estilo de vida até se submeter a grandes cirurgias, como o transplante hepático. Para alcançar os melhores resultados é necessário que o paciente tenha uma boa aderência ao tratamento. Estudos indicam que o conhecimento do paciente sobre sua condição clínica é um fator determinante na aderência. Objetivos: criar um instrumento que meça o conhecimento que um paciente com CH tem sobre sua doença e seu tratamento. Methodologia: o processo de desenvolvimento do instrument foi dividido em 3 estágios: construção do instrument (1º estágio), avaliação do conteúdo e claridade do instrument (2º estágio) e avaliação da confiabilidade do instrument (3º estágio). Results: um instrument para avaliar o conhecimento do paciente cirrótico sobre sua doença foi feito, analisado por especialistas e aprovado pelos critérios propostos. Conclusions: 1) O desenvolvimento do instrumento seguiu os passos descritos na literatura. 2) As sugestões dos avaliadores foram seguidas na reestruturação das questões, tornando-as mais claras e relevantes para a proposta do instrumento. 3) Foi possível alcançar de forma satisfatória a proposta de construção do instrumento.Introduction: hepatic cirrhosis (HC) is a disease with high morbidity and mortality in Brazil. Its treatment is complex and requires from lifestyle changes to large surgeries such as liver transplantation. To reach the best treatment results, it is necessary to guarantee a good patient’s adherence to the treatment. Studies indicate that the patient’s self-knowledge about his or her clinical condition is a determining factor in its adherence. Objectives: to assist in the design of an instrument that evaluates knowledge about the disease and treatment of HC. Methodology: The development process of the instrument will be divided into 3 stages: construction of the instrument (1st stage), evaluation of content validity and clarity of the instrument (2nd stage) and assessment of the reliability of the instrument (3 rd stage). Results: an instrument to evaluate the cirrhotic patient knowledge about the disease was made, analyzed by specialists and approved in the criteria proposed. Conclusions: 1) The development of the instrument followed the steps described in literature. 2) The suggestion of the evaluators allowed to restructure the questions making them clearer and more relevant to the proposal of the instrument. 3) it was possible to achieve satisfactorily the proposal of the construction of the instrument

TEP for incarcerated groin hernias: is it feasible for experienced surgeons?

Objective: To verify if endoscopic TEP surgery performed by an experienced surgeon is a feasible procedure to treat incarcerated groin hernia.Methods: This is a retrospective study in which we analyzed data from patients submitted to TEP endoscopic surgery for treatment of incarcerated and non-incarcerated groin hernias. The surgeries were all performed by a single surgeon. We obtained data on gender, age, ASA scores, BMI, hernia site and operating time. The two groups were descriptively analyzed and statistically compared in order to verify how similar the samples were. The operative times were also compared between the groups. The first 65 surgeries were excluded in order to evaluate the hability of an experienced surgeon.Results: 323 surgeries were performed. 32 (9.9%) were cases of incarcerated hernias. In 306 cases (96%), the surgical approach used was TEP, the other 13 cases (4%) were operated by TAPP. All incarcerated hernias were operated by the TEP method. No significant differences on gender, age, hernia site, BMI and ASA score were found between incarcerated and non-incarcerated hernia patients. The operative time was analyzed by site of hernias (left, right and bilateral) and no statistical differences were found between incarcerated and nonincarcerated hernias.Conclusions: 1) There was no significant differences on gender, age, ASA, BMI and site of hernias that could interfere in operative time in both groups. 2) The lack of significant statistical difference on operative time indicate that, for experienced surgeons, the technical difficulty is similar to operate incarcerated and non-incarcerated groin hernias by TEP.Keywords: Herniorraphy; Inguinal hernia; Endoscopy; Learning curve; Operative time

Intussusception reveals MUTYH-related polyposis syndrome and colorectal cancer: a case report

We are reporting a rare case of MUTYH-associated polyposis, a colorectal cancer hereditary syndrome, diagnosticated after an intussusception. Colorectal cancer is an important cause of cancer related mortality that can be manifested by an intussusception, a rare occurrence in adults and almost always related to tumors. Approximately 5% of colorectal cancers can be attributed to syndromes known to cause hereditary colorectal cancer, such as MUTYHassociated polyposis, autosomal genetic syndrome associated with this disease. We present the case of a 44 years old male, that sought medical consultation with a complaint of abdominal discomfort, that after five days changed its characteristics. The patient was sent to the emergency department were a CT-scan revealed intestinal sub-occlusion by ileocolic invagination. Right colectomy was carried out. The anatomic-pathological examination revealed a moderately differentiated mucinous adenocarcinoma and multiples sessile polyps, which led to the suspicion of a genetic syndrome. In the genetics analysis two mutations were observed in the MUTYH gene, and MUTYH-associated polyposis was diagnosticated. This case demonstrates the importance of meticulous analysis of the patient examinations results to identify possible discrete alterations that can lead to improved understanding of disease

Catálogo Taxonômico da Fauna do Brasil: setting the baseline knowledge on the animal diversity in Brazil

The limited temporal completeness and taxonomic accuracy of species lists, made available in a traditional manner in scientific publications, has always represented a problem. These lists are invariably limited to a few taxonomic groups and do not represent up-to-date knowledge of all species and classifications. In this context, the Brazilian megadiverse fauna is no exception, and the Catálogo Taxonômico da Fauna do Brasil (CTFB) (http://fauna.jbrj.gov.br/), made public in 2015, represents a database on biodiversity anchored on a list of valid and expertly recognized scientific names of animals in Brazil. The CTFB is updated in near real time by a team of more than 800 specialists. By January 1, 2024, the CTFB compiled 133,691 nominal species, with 125,138 that were considered valid. Most of the valid species were arthropods (82.3%, with more than 102,000 species) and chordates (7.69%, with over 11,000 species). These taxa were followed by a cluster composed of Mollusca (3,567 species), Platyhelminthes (2,292 species), Annelida (1,833 species), and Nematoda (1,447 species). All remaining groups had less than 1,000 species reported in Brazil, with Cnidaria (831 species), Porifera (628 species), Rotifera (606 species), and Bryozoa (520 species) representing those with more than 500 species. Analysis of the CTFB database can facilitate and direct efforts towards the discovery of new species in Brazil, but it is also fundamental in providing the best available list of valid nominal species to users, including those in science, health, conservation efforts, and any initiative involving animals. The importance of the CTFB is evidenced by the elevated number of citations in the scientific literature in diverse areas of biology, law, anthropology, education, forensic science, and veterinary science, among others