Hereditary hemochromatosis beyond hyperferritinemia: Clinical and laboratory investigation of the patient's profile submitted to phlebotomy in two reference centers in southern Brazil.

Hereditary Hemochromatosis is a disorder characterized by iron deposition in several organs and hyperferritinemia. The most studied variants are linked to the HFE gene. In Brazil, surveys that characterize this population are scarce, with no sampling in the state of Rio Grande do Sul. Our objective is to carry out a data collection focusing on the profile of this population and the influence of the most frequently HFE variants. Two centers were enrolled: Hospital de Clínicas de Porto Alegre and Hospital São Vicente de Paulo. Patients with hyperferritinemia and undergoing phlebotomy were invited. Clinical data were collected, including HFE investigation. Among the descriptive data, the allele frequency of the C282Y variant (0.252) stands out, which differs from the national scenario. Systemic arterial hypertension was the most cited comorbidity. Differences between centers were observed, highlighting higher frequency of H63D cases in HSVP (p<0.01). Genotypes were stratified according to deleterious effect of C282Y variant. Higher transferrin saturation and number of phlebotomies were observed in the C282Y/C282Y cases (p<0.001). Positive family history for hyperferritinemia was more prevalent in compound heterozygotes (p<0.01). The results presented confirm the importance of encouraging such studies and reiterate the need for greater attention to this population.

Ocorrência de linfoma de Burkitt secundário a mieloma múltiplo em paciente tratado com protocolo CyBorD: um relato de caso / Occurrence of Burkitt lymphoma secondary to multiple myeloma in a patient treated with CyBorD protocol: a case report

Introdução: Após tratamento para Mieloma Múltiplo (MM), a transformação em linfoma de burkitt (LB) é incomum, com raros casos relatados.Objetivo: relatar um caso raro de LB secundário a MM, em paciente tratado com protocolo CyBorD. Relato de caso: Nós descrevemos um caso de um homem de 37 anos, negro, senegalês e HIV positivo, que em pouco mais de dois meses após o diagnóstico de MM evoluiu para uma transformação medular que resultou em um linfoma linfoblástico B compatível com LB. Conclusão: Este relato de caso demonstra a possibilidade de transformação maligna de MM para LB, incentivando assim futuras comparações multicêntricas de casos similares num esforço amplo para uma melhor definição desse processo patológico.

Introduction: After treatment for Multiple Myeloma (MM), transformation into burkitt's lymphoma (LB) is uncommon, with rare cases reported. Objective: to report a rare case of LB secondary to MM, in a patient treated with the CyBorD protocol. Case report: We describe a case of a 37-year-old man, black, Senegalese and HIV positive, who in just over 2 months after the diagnosis of MM evolved to a medullary transformation that resulted in a B lymphoblastic lymphoma compatible with LB. Conclusion: This case report demonstrates the possibility of malignant transformation from MM to BL, thus encouraging future multicentric comparisons of similar cases in a broad effort to better define this pathological process.

Primary malignant neoplasms of the cecal appendix: unusual findings in acute appendicitis

Introduction: To evaluate the prevalence of appendix neoplasia correlating with patient profile, histological types and frequency. Methods: Data collection was performed in the Pathology Department of a General Hospital, with the objective of identifying patients diagnosed with malignant cecal appendix tumors by histopathologic study of specimens from acute appendicitis. Results: The prevalence of malignant primary epithelial neoplasia of the appendix was 1%. Fifty percent of the cases were neuroendocrine tumors, 35% were mucinous, and 15% were adenocarcinomas. The mean age at diagnosis was 41.3 (SD, 20.4) years (range 16-81), with a women/men ratio of 3:1. Discussion: Appendiceal neoplasms are rare and should be suspected manly in women over 40 years of age with suggestive symptoms of acute appendicitis. The size, location, extent, margins and presence of mucin are essential findings for the treatment of these patients. (AU)

Right hepatic artery originated from the superior mesenteric artery: What is the standard anatomic position? / Artéria hepática direita originada da artéria mesentérica superior: Qual seu real trajeto anatômico?

OBJECTIVE: Liver arterial irrigation is characterized by a high frequency of variations in its anatomy. The aim of the study was to describe the anatomic position of the right hepatic artery as a brunch of the superior mesenteric artery. METHODS: A total of 5147 intravenous contrast-enhanced computed tomography scans of patients seen at the Radiology Service of the Passo Fundo Clinical Hospital (RS), from October 2016 to December 2017, were selected. 125 patients with anatomic variation of the right hepatic artery were selected. The findings were categorized by the variation of the vascular position, emergence from the superior mesenteric artery and the relationship with other structures. RESULTS: The most frequent position was retropancreatic (88.8%), retroportal (76.8%) and post-choledocian (75.2%), emerging about 2.33 cm from the superior mesenteric artery. CONCLUSION: We have shown that most common variant of the right hepatic artery presents its posterior origin from the pancreatic and hepatic pedicle, and arises close to the origin of the superior mesenteric artery.

OBJETIVOS: A irrigação arterial hepática tem como característica a elevada frequência de variações da anatomia. O objetivo do estudo foi descrever o trajeto anatômico da artéria hepática direita quando originada da artéria mesentérica superior. MÉTODOS: Foram analisadas 5147 tomografias computadorizadas com contraste endovenoso de pacientes atendidos no Serviço de Radiologia do Hospital de Clínicas de Passo Fundo - RS, no período outubro de 2016 a dezembro de 2017. Foram selecionados 125 pacientes portadores de variação anatômica da artéria hepática direita na origem. Os achados foram categorizados pela variação do trajeto vascular, emergência da artéria mesentérica superior e a relação com demais estruturas. RESULTADOS: Obtivemos o trajeto mais frequente desta variação como retropancreático (88,8%), retroportal (76,8%) e pós-coledociano (75,2%), emergindo cerca de 2,33 cm da origem da artéria mesentérica superior. CONCLUSÃO: Demonstramos que na maioria das vezes, a artéria hepática direita variante, apresenta trajeto posterior ao pâncreas e ao pedículo hepático e emerge próxima da origem da artéria mesentérica superior.

Preoperative laparoscopy and peritoneal lavage in gastric adenocarcinoma: can the approach be modified? / Laparoscopia e lavado peritoneal no pré-operatório do adenocarcinoma gástrico: podemos modificar a conduta?

OBJECTIVE: to assess whether laparoscopy with peritoneal lavage is superior to computed tomography for staging gastric adenocarcinoma and whether it can modify the surgical approach. METHODS: we conducted a retrospective study of 46 patients with gastric adenocarcinoma treated by the digestive surgery team of the Passo Fundo Clinics Hospital (RS), from January 2015 to December 2018, and submitted to laparoscopy with preoperative peritoneal lavage. All patients underwent preoperative clinical staging with computed tomography. RESULTS: of the 46 patients analyzed, the majority had tumors located in the cardia (34.8%), poorly differentiated (69.6%), and subtype signet ring cells (65.2%). In 91.3%, the computed tomography scan did not identify peritoneal carcinomatosis or distant metastasis. Among these patients with negative computed tomography for distant disease, 21.8% had positive peritoneal lavage for neoplastic cells and had their therapeutic approaches modified. CONCLUSION: laparoscopy and peritoneal lavage altered the surgical decision in 21.8% of patients, providing a more reliable preoperative staging in gastric adenocarcinoma.

OBJETIVO: avaliar se a laparoscopia com lavado peritoneal é superior à tomografia computadorizada para o estadiamento do adenocarcinoma gástrico e se pode modificar a conduta cirúrgica do paciente. MÉTODOS: estudo retrospectivo de 46 pacientes portadores de adenocarcinoma gástrico tratados pela equipe de cirurgia digestiva do Hospital de Clínicas de Passo Fundo (RS), de janeiro de 2015 a dezembro de 2018, e submetidos à laparoscopia com lavado peritoneal pré-operatório. Todos os pacientes foram submetidos ao estadiamento clínico pré-operatório com tomografia computadorizada. RESULTADOS: dos 46 pacientes analisados, a maioria apresentava tumores localizados na cárdia (34,8%), pouco diferenciados (69,6%) e do subtipo células em anel de sinete (65,2%). Em 91,3% deles a tomografia computadorizada não identificou carcinomatose peritoneal ou metástases à distância. Entre estes pacientes com tomografia computadorizada negativa para doença à distância, 21,8% apresentaram lavado peritoneal positivo para células neoplásicas e tiveram suas condutas terapêuticas modificadas. CONCLUSÃO: a laparoscopia e o lavado peritoneal alteraram a decisão cirúrgica em 21,8% dos pacientes, proporcionando um estadiamento pré-operatório mais fidedigno no adenocarcinoma gástrico.

Prevalence of red blood cell alloimmunization in multiply transfused patients

Introduction: To analyze the prevalence of alloantibodies in multiply transfused patients. Methods: This study was a retrospective, exploratory and descriptive study with a quantitative approach. The study sample comprised 185 patients transfused at a referral service in the city of Passo Fundo, Rio Grande do Sul, from January 2016 to February 2018. Results: Overall, the antibodies identified were as follows: anti-E in 47 patients (18%), anti-D and anti-K in 28 patients each (11%), anti-C in 21 patients (8.1%), and inconclusive antibody results in 23 patients (8.9%). Females were a majority (55.7%), mean age was 48.8 years and mean quantity of blood transfused was 7.2 bags. Cardiovascular disorders were the most common comorbidities, in 39 patients (21.2%), followed by oncological disorders, in 38 patients (18.4%). Conclusion: Alloimmunization is an important and frequent clinical condition that increases the risk of hemolytic reactions and is associated with significant patient morbidity and mortality. (AU)

Artéria hepática direita originada da artéria mesentérica superior: Qual seu real trajeto anatômico? / Right hepatic artery originated from the superior mesenteric artery: What is the standard anatomic position?

RESUMO Objetivos: A irrigação arterial hepática tem como característica a elevada frequência de variações da anatomia. O objetivo do estudo foi descrever o trajeto anatômico da artéria hepática direita quando originada da artéria mesentérica superior. Métodos: Foram analisadas 5147 tomografias computadorizadas com contraste endovenoso de pacientes atendidos no Serviço de Radiologia do Hospital de Clínicas de Passo Fundo - RS, no período outubro de 2016 a dezembro de 2017. Foram selecionados 125 pacientes portadores de variação anatômica da artéria hepática direita na origem. Os achados foram categorizados pela variação do trajeto vascular, emergência da artéria mesentérica superior e a relação com demais estruturas. Resultados: Obtivemos o trajeto mais frequente desta variação como retropancreático (88,8%), retroportal (76,8%) e pós-coledociano (75,2%), emergindo cerca de 2,33 cm da origem da artéria mesentérica superior. Conclusão: Demonstramos que na maioria das vezes, a artéria hepática direita variante, apresenta trajeto posterior ao pâncreas e ao pedículo hepático e emerge próxima da origem da artéria mesentérica superior.

ABSTRACT Objective: Liver arterial irrigation is characterized by a high frequency of variations in its anatomy. The aim of the study was to describe the anatomic position of the right hepatic artery as a brunch of the superior mesenteric artery. Methods: A total of 5147 intravenous contrast-enhanced computed tomography scans of patients seen at the Radiology Service of the Passo Fundo Clinical Hospital (RS), from October 2016 to December 2017, were selected. 125 patients with anatomic variation of the right hepatic artery were selected. The findings were categorized by the variation of the vascular position, emergence from the superior mesenteric artery and the relationship with other structures. Results: The most frequent position was retropancreatic (88.8%), retroportal (76.8%) and post-choledocian (75.2%), emerging about 2.33 cm from the superior mesenteric artery. Conclusion: We have shown that most common variant of the right hepatic artery presents its posterior origin from the pancreatic and hepatic pedicle, and arises close to the origin of the superior mesenteric artery.

Effect of Roux-en-Y gastric bypass in the control of erosive esophagitis

Introduction: Gastroesophageal reflux disease (GERD) is a multifactorial disease associated with environmental and genetic factors. Obesity is among the risk factors for its development, which also correlates with an increase in severity of clinical presentation and a higher incidence of complications associated with reflux. Methods: This historical cohort study included a sample of 249 patients who had undergone bariatric surgery using the Roux-en-Y gastric bypass technique at the Hospital São Vicente de Paulo, Passo Fundo, southern Brazil, from January 2014 to December 2015. Results: Of 249 patients, 77.9% (190 patients) were female and the mean age was 38 years. The occurrence of reflux esophagitis was 81.1% (196 patients) in the preoperative period and 31.3% (75 patients) in the postoperative period. With regard to bariatric treatment response to control moderate and severe esophagitis (grades B, C and D), there was a reduction in prevalence from 62 (25%) to 12 (5%) patients (p<0.05). Conclusions: Bariatric surgery using the Roux-en-Y gastric bypass technique is effective in the control of reflux esophagitis. Regression is observed mainly in cases of moderate and severe esophagitis. (AU)

Laparoscopia e lavado peritoneal no pré-operatório do adenocarcinoma gástrico: podemos modificar a conduta? / Preoperative laparoscopy and peritoneal lavage in gastric adenocarcinoma: can the approach be modified?

RESUMO Objetivo: avaliar se a laparoscopia com lavado peritoneal é superior à tomografia computadorizada para o estadiamento do adenocarcinoma gástrico e se pode modificar a conduta cirúrgica do paciente. Métodos: estudo retrospectivo de 46 pacientes portadores de adenocarcinoma gástrico tratados pela equipe de cirurgia digestiva do Hospital de Clínicas de Passo Fundo (RS), de janeiro de 2015 a dezembro de 2018, e submetidos à laparoscopia com lavado peritoneal pré-operatório. Todos os pacientes foram submetidos ao estadiamento clínico pré-operatório com tomografia computadorizada. Resultados: dos 46 pacientes analisados, a maioria apresentava tumores localizados na cárdia (34,8%), pouco diferenciados (69,6%) e do subtipo células em anel de sinete (65,2%). Em 91,3% deles a tomografia computadorizada não identificou carcinomatose peritoneal ou metástases à distância. Entre estes pacientes com tomografia computadorizada negativa para doença à distância, 21,8% apresentaram lavado peritoneal positivo para células neoplásicas e tiveram suas condutas terapêuticas modificadas. Conclusão: a laparoscopia e o lavado peritoneal alteraram a decisão cirúrgica em 21,8% dos pacientes, proporcionando um estadiamento pré-operatório mais fidedigno no adenocarcinoma gástrico.

ABSTRACT Objective: to assess whether laparoscopy with peritoneal lavage is superior to computed tomography for staging gastric adenocarcinoma and whether it can modify the surgical approach. Methods: we conducted a retrospective study of 46 patients with gastric adenocarcinoma treated by the digestive surgery team of the Passo Fundo Clinics Hospital (RS), from January 2015 to December 2018, and submitted to laparoscopy with preoperative peritoneal lavage. All patients underwent preoperative clinical staging with computed tomography. Results: of the 46 patients analyzed, the majority had tumors located in the cardia (34.8%), poorly differentiated (69.6%), and subtype signet ring cells (65.2%). In 91.3%, the computed tomography scan did not identify peritoneal carcinomatosis or distant metastasis. Among these patients with negative computed tomography for distant disease, 21.8% had positive peritoneal lavage for neoplastic cells and had their therapeutic approaches modified. Conclusion: laparoscopy and peritoneal lavage altered the surgical decision in 21.8% of patients, providing a more reliable preoperative staging in gastric adenocarcinoma.