Underwater Endoscopic Mucosal Resection Vs Conventional Endoscopic Mucosal Resection for Superficial Nonampullary Duodenal Epithelial Tumors in the Western Setting.

BACKGROUND & AIMS: Conventional endoscopic mucosal resection (C-EMR) is established as the primary treatment modality for superficial nonampullary duodenal epithelial tumors (SNADETs), but recently underwater endoscopic mucosal resection (U-EMR) has emerged as a potential alternative. The majority of previous studies focused on Asian populations and small lesions (≤20 mm). We aimed to compare the efficacy and outcomes of U-EMR vs C-EMR for SNADETs in a Western setting. METHODS: This was a retrospective multinational study from 10 European centers that performed both C-EMR and U-EMR between January 2013 and July 2023. The main outcomes were the technical success, procedure-related adverse events (AEs), and the residual/recurrent adenoma (RRA) rate, evaluated on a per-lesion basis. We assessed the association between the type of endoscopic mucosal resection and the occurrence of AEs or RRAs using mixed-effects logistic regression models (propensity scores). Sensitivity analyses were performed for lesions ≤20 mm or >20 mm. RESULTS: A total of 290 SNADETs submitted to endoscopic resection during the study period met the inclusion criteria and were analyzed (C-EMR: n = 201, 69.3%; U-EMR: n = 89, 30.7%). The overall technical success rate was 95.5% and comparable between groups. In logistic regression models, compared with U-EMR, C-EMR was associated with a significantly higher frequency of overall delayed AEs (odds ratio [OR], 4.95; 95% CI, 2.87-8.53), postprocedural bleeding (OR, 7.92; 95% CI, 3.95-15.89), and RRAs (OR, 3.66; 95% CI, 2.49-5.37). Sensitivity analyses confirmed these results when solely considering either small (≤20 mm) or large (>20 mm) lesions. CONCLUSIONS: Compared with C-EMR, U-EMR was associated with a lower rate of overall AEs and RRAs, regardless of lesion size. Our results confirm the possible role of U-EMR as an effective and safe technique in the management of SNADETs.

Predicting residual neoplasia after a non-curative gastric ESD: validation and modification of the eCura system in the Western setting: the W-eCura score.

OBJECTIVE: To evaluate the risk factors for lymph node metastasis (LNM) after a non-curative (NC) gastric endoscopic submucosal dissection (ESD) and to validate and eventually refine the eCura scoring system in the Western setting. Also, to assess the rate and risk factors for parietal residual disease. DESIGN: Retrospective multicentre multinational study of prospectively collected registries from 19 Western centres. Patients who had been submitted to surgery or had at least one follow-up endoscopy were included. The eCura system was applied to assess its accuracy in the Western setting, and a modified version was created according to the results (W-eCura score). The discriminative capacities of the eCura and W-eCura scores to predict LNM were assessed and compared. RESULTS: A total of 314 NC gastric ESDs were analysed (72% high-risk resection (HRR); 28% local-risk resection). Among HRR patients submitted to surgery, 25% had parietal disease and 15% had LNM in the surgical specimen. The risk of LNM was significantly different across the eCura groups (areas under the receiver operating characteristic curve (AUC-ROC) of 0.900 (95% CI 0.852 to 0.949)). The AUC-ROC of the W-eCura for LNM (0.916, 95% CI 0.870 to 0.961; p=0.012) was significantly higher compared with the original eCura. Positive vertical margin, lymphatic invasion and younger age were associated with a higher risk of parietal residual lesion in the surgical specimen. CONCLUSION: The eCura scoring system may be applied in Western countries to stratify the risk of LNM after a gastric HRR. A new score is proposed that may further decrease the number of unnecessary surgeries.

Risk of residual neoplasia after a noncurative colorectal endoscopic submucosal dissection for malignant lesions: a multinational study.

BACKGROUND : Endoscopic submucosal dissection (ESD) in colorectal lesions is technically demanding and a significant rate of noncurative procedures is expected. We aimed to assess the rate of residual lesions after a noncurative ESD for colorectal cancer (CRC) and to establish predictive scores to be applied in the clinical setting. METHODS : Retrospective multicenter analysis of consecutive colorectal ESDs. Patients with noncurative ESDs performed for the treatment of CRC lesions submitted to complementary surgery or with at least one follow-up endoscopy were included. RESULTS : From 2255 colorectal ESDs, 381 (17 %) were noncurative, and 135 of these were performed in CRC lesions. A residual lesion was observed in 24 patients (18 %). Surgery was performed in 96 patients and 76 (79 %) had no residual lesion in the colorectal wall or in the lymph nodes. The residual lesion rate for sm1 cancers was 0 %, and for > sm1 cancers was also 0 % if no other risk factors were present. Independent risk factors for lymph node metastasis were poor differentiation and lymphatic permeation (NC-Lymph score). Risk factors for the presence of a residual lesion in the wall were piecemeal resection, poor differentiation, and positive/indeterminate vertical margin (NC-Wall score). CONCLUSIONS : Lymphatic permeation or poor differentiation warrant surgery owing to their high risk of lymph node metastasis, mainly in > sm1 cancers. In the remaining cases, en bloc and R0 resections resulted in a low risk of residual lesions in the wall. Our scores can be a useful tool for the management of patients who undergo noncurative colorectal ESDs.

Juvenile polyps with osseous metaplasia treated by endoscopic mucosal resection.

A 17-year-old male with no previous medical history presented with a 1-year history of rectal bleeding, mucus discharge and occasional rectal prolapse. Colonoscopy revealed several polypoidal growth masses in the distal rectum, formed by multiple sessile polyps with a glistening mucus-covered surface and fleshy, friable appearance, that coalesced forming large conglomerates. Given their complexity and large size, piecemeal endoscopic mucosal resection of the rectal lesions was performed and histopathological examination revealed ulcerated polypoid mucosa with mixed inflammatory cell infiltrate in the lamina propria and dilated cystic mucus-filled glands. Remarkably, bony trabeculae surrounded by osteoblastic cells were also seen. These findings were consistent with juvenile polyps with foci of osseous metaplasia. Osseous metaplasia has been described in a wide variety of tissue types, such as prostate, uterus, breasts, lungs and urinary tract, with respect to both neoplastic and non-neoplastic conditions. However, it is exceedingly rare in colonic polyps and, to the best of our knowledge, only 9 cases have been described in juvenile polyps.

Acute gastrointestinal graft-versus-host disease with cytomegalovirus and Epstein-Barr virus superinfection in a patient with COVID-19.

A 60-year-old female was diagnosed with acute myeloid leukemia. After initial remission with chemotherapy, she relapsed and underwent allogeneic hematopoietic stem cell transplantation (HSCT). Two months later, she presented to emergency department with watery diarrhea, abdominal pain and fever. She also tested positive for SARS-CoV2 on nasopharyngeal swab by polymerase chain reaction (PCR) and both cytomegalovirus (CMV) and Epstein-Barr virus (EBV) were detected in peripheral blood. Flexible sigmoidoscopy showed diffuse edema, erythema and loss of vascular pattern with interspersed segments of mucosal denudation and exudate and bBiopsies revealed epithelial cell apoptosis, diffuse crypt atrophy and dropout, with ulceration and both CMV and EBV were detected in colon mucosa, consistent with acute severe gastrointestinal graft-versus-host disease complicated by CMV and EBV superinfection. Despite starting therapy with methylprednisolone, ganciclovir and rituximab,she presented unfavorable evolution and died after 5 weeks.

Hepatic amyloidosis: a prevalence study and clinical characterization of a rare and severe disease.

BACKGROUND AND AIM: Amyloidosis is a systemic disease characterized by extracellular deposition of amyloid protein, most commonly in the heart and kidney. Hepatic amyloidosis is a rare form of presentation that ranges from mild hepatomegaly and altered liver biochemical tests to acute liver failure. The aims of this study were to evaluate the prevalence of amyloidosis in patients undergoing liver biopsy and describe its main clinical characteristics and prognostic impact. METHODS: A retrospective analysis of all patients with a histological diagnosis of hepatic amyloidosis between January 2010 and December 2019 was performed. MAJOR RESULTS: A total of 7 patients were identified from a total of 1773 liver biopsy procedures (0.4%), with a female predominance (6/7) and median age of diagnosis of 62 years. The most common clinical manifestations included hepatomegaly (4/7), jaundice (2/7) and peripheral edema (2/7), whereas 3/7 patients were asymptomatic. Every patient presented abnormalities in liver biochemical tests, more commonly cholestasis (6/7), but also cytolysis (4/7) or hyperbilirubinemia (2/7). Abnormal imaging findings included hepatomegaly, steatosis or parenchymal heterogeneity. In most patients (5/7), other organs were involved, most commonly with nephrotic syndrome (3/7) and infiltrative cardiomyopathy (3/7). The most common type was AA amyloidosis (3/7) followed by AL amyloidosis (2/7). The 1-year mortality rate was 43% and the median survival was 24 months. CONCLUSIONS: We report a low prevalence (0.4%) of amyloidosis among patients undergoing liver biopsy. Although rare, hepatic amyloidosis is associated with a dismal prognosis and a high index of suspicion is crucial to achieve an early diagnosis. .

The role of endoscopic submucosal dissection in the management of gastric inflammatory fibroid polyps: a single-center experience.

BACKGROUND AND AIM: gastric inflammatory fibroid polyps constitute only 0.1 % of all gastric polyps. They are usually amenable to resection by snare polypectomy. However, on rare occasions, these lesions may require resection by endoscopic submucosal dissection. This study aimed to evaluate the effectiveness and safety of endoscopic submucosal dissection in the management of gastric inflammatory fibroid polyps not amenable to resection with snare polypectomy. METHODS: a retrospective observational study of all consecutive patients who underwent endoscopic submucosal dissection for gastric inflammatory fibroid polyps between January 2011 and December 2020 was performed. RESULTS: there were nine cases of gastric inflammatory fibroid polyps resected by endoscopic submucosal dissection. Most patients were female (7/9) with a mean age of 62.2 years. All gastric inflammatory fibroid polyps were described as solitary antral subepithelial lesions with a mean diameter of 16.7 mm, which appeared well-circumscribed and homogeneous lesions located at muscularis mucosa and submucosa without deeper invasion on endoscopic ultrasound. All lesions were successfully resected by en bloc and complete resection with free margins obtained in 8/9 specimens. Adverse events were reported in 2/9 cases including one intra-procedural bleeding successfully controlled with hemostatic clips and one aspiration pneumonia that evolved favorably. Mean follow-up duration was 33.7 months and no delayed complications or cases of recurrence were reported. CONCLUSIONS: endoscopic submucosal dissection appears safe and effective for the resection of gastric inflammatory fibroid polyps that present as large subepithelial lesions, if performed by experienced endoscopists after adequate characterization by endoscopic ultrasound, with high rates of technical success and low recurrence rates.

Clinical and endoscopic findings in gastrointestinal amyloidosis: a single-center experience.

Gastrointestinal involvement occurs in approximately 4% of cases of systemic amyloidosis. The most common site of amyloid deposition is small bowel, followed by stomach, colorectum and esophagus. Although rare, gastrointestinal amyloidosis may be associated with severe complications including gastrointestinal bleeding or perforation and may be mistaken for malignancy.

Analysis of gunshot residues from nontoxic ammunition: a contribution to health and environmental analysis.

This study's aim was to determine the presence, as well as to evaluate the health and environmental impacts, of chemical elements from firearm shots during shooter practice at outdoor shooting ranges, both in the environment and on the shooters' hands. Two high-precision devices were used for measuring suspended particles that are released during discharge of Taurus PT 100 .40 caliber pistols. The analysis of collected data allowed the identification of specific distribution patterns of samples that were adsorbed. Moreover, samples were collected from the opisthenar area of the hand to investigate both the occurrence and deposition of particles and chemical elements through scanning electron microscopy coupled with energy dispersive spectroscopy (SEM-EDS). The results indicate that copper, lead, and zinc concentration profiles will be able to delimit and reveal more precise details regarding shots made with nontoxic ammunition (NTA). In particular, the residual graphic analysis indicated that the majority of metal deposited in the shooter's hand is zinc. Further, the metals barium and lead also were detected. Due to the exposure to these elements, it could be concluded that engineering controls and administrative management should be sought in order to avoid human overexposure and environmental release of these airborne toxic metals.

Prevalence and Clinical Impact of Abnormal Findings in Routine Upper Digestive Endoscopy Before Bariatric Surgery.

Background: The role of preoperative upper digestive endoscopy has been discussed with controversy in bariatric surgery. This study aims to evaluate the prevalence of endoscopic findings in obese patients undergoing bariatric surgery and their clinical impact. Methods: A retrospective observational study of all patients who performed upper endoscopy before bariatric surgery between January 2019 and December 2021 was performed. A total of 612 patients were identified, mostly females (79.2%), with a mean age of 43.9 years. Abnormal endoscopic findings were identified in 474 (77.5%) patients, including erythematous gastropathy (59.2%), reflux esophagitis (13.6%), erosive gastritis (10.6%), and hiatal hernia (8.0%). Importantly, Barrettâ??s esophagus was also identified in four patients and gastric adenocarcinoma in one. All performed gastric biopsies that revealed H. pylori gastritis in 368 (60.1%). Taken together, abnormal endoscopic and histological findings influenced perioperative management of 403 (65.8%) patients, including preoperative medical therapy in 378 (61.8%), endoscopic treatment of mucosal lesions in 47 (7.7%), direct influence in surgical strategy in 27 (7.8%) and contraindication to bariatric surgery in 1 (0.2%). Conclusion: Preoperative upper endoscopy identifies a wide range of abnormal findings in obese patients, which often influence peri-operative management. Therefore, it must be considered in all patients prior to bariatric surgery.

Baseline anaemia increases locally advanced rectal cancer mortality in older patients undergoing preoperative chemoradiation.

PURPOSE: The median diagnosis age of rectal cancer (RC) is 70 years old. The standard of care for locally advanced RC (LARC) is preoperative chemoradiation (CRT) followed by surgery. Anaemia is a frequent condition in older patients but is not a pure consequence of ageing. METHODS: The patients aged 65 years or over, with clinical stage II/III LARC, and treated with preoperative concurrent CRT were retrospectively reviewed. Baseline haemoglobin (Hb) levels were collected. RESULTS: One hundred and seven patients enrolled in this study, but 17 were excluded in relation with treatment disruption. Fifty-seven (63.3%) males and 33 (36.7%) females completed preoperative CRT whose median age at diagnosis was 73. Twenty-five (27.8%) patients presented with anaemia at rectal cancer diagnosis, and median Hb was 13.5 g/dL (IQR = 1.45) and 11.2 g/dL (IQR = 1.35), for non-anaemic and anaemic patients, respectively. For the enrolled older population, only 2 patients reported acute grade 3 toxicity. Baseline anaemia tended to decrease the LARC-free interval and was associated with a significantly higher hazard of all-cause and LARC mortality, approximately 5 times (HR = 5.25; 95% CI 1.48-18.66) and 10 times (HR = 10.09; 95% CI 2.40-42.48), respectively. Patients older than 75 presented a significantly negative impact on overall survival (OS) and LARC-specific survival (HR = 6.20, 95% CI 2.00-19.22; and HR = 7.61, 95% CI 2.08-27.87, respectively). Conversely, no significant impact was found for age-adjusted Charlson comorbidity index on OS, LARC-specific survival and LARC-free interval. CONCLUSIONS: Overall and LARC-specific survival were significantly lower for the baseline anaemic older patients and for those aged 75 years or over.

Factors affecting survival after palliative radiotherapy in patients with lung cancer.

BACKGROUND: Lung cancer is the most common cancer worldwide. It is estimated that 60% of patients with NSCLC at time of diagnosis have advanced disease. The aim of this study was to identify factors that play a major role in the survival of lung cancer patients treated with palliative radiotherapy. MATERIALS AND METHODS: We retrospectively reviewed data of 280 lung cancer patients treated with palliative radiotherapy from January 2013 to December 2017. A multivariate analysis using the proportional hazards model of Cox was conducted. Also, Kaplan Meier curves were used to describe the distribution of survival times of the patients. The level of significance was set at 0.05. RESULTS: The mean age at diagnosis was 65.6 years. About 77.5% of patients were male and 22.5% were female. In our cohort > 95% had stage 4 lung cancer. Most cases were adenocarcinomas (72.5%) and ECOG-PS 0-1 (80.4%). Different sites were submitted to palliative treatment: 120 brain metastases, 96 bone metastases, 53 lung tumour, 8 lymph nodes and 3 lung metastases. Brain as first site of palliative radiotherapy (HR: 1.553, 95% CI: 1.167-2.067, p = 0.003) and ECOG-PS 2-3 compared with ECOG-PS 0-1 (HR: 2.253, 95% CI: 1.546-3.283, p ≤ 0.001) were associated with increased likelihood of lung cancer death. Patients who received biological therapy had 70.7% (p ≤ 0.001) reduction in lung cancer death risk. CONCLUSION: Brain as the first metastatic site treated with radiotherapy and ECOG-PS 2-3 are associated with increased lung cancer death. Biological therapy was associated with decreased death risk.

Endoscopic treatment of a foreign body-associated colonic perforation.

BACKGROUND: Although the ingestion of foreign bodies is relatively common in the general population, intestinal perforation is rare but usually requires emergent surgery. CASE PRESENTATION: We report a case of an 87-year-old woman that presented with a foreign body-associated colonic diverticular perforation. After multidisciplinary discussion, the foreign body was removed and the perforation was closed using an over-the-scope-clip. Percutaneous drainage of an adjacent intra-abdominal collection was performed. The patient had a favorable clinical evolution and was discharged 15 days after the endoscopic procedure. CONCLUSION: This case highlights the possible growing role of endoscopic treatment of foreign body-associated colonic perforations, especially in high-risk surgical patients.

Prevalence and Risk Factors for Augmented Renal Clearance in a Population of Critically Ill Patients.

BACKGROUND: Critically ill patients show a high, albeit variable, prevalence of augmented renal clearance (ARC). This condition has relevant consequences on the elimination of hydrophilic drugs. Knowledge of risk factors for ARC helps in the early identification of ARC. The aims of this study were evaluation of (1) risk factors for ARC and (2) the prevalence of ARC in critically ill patients over a period of 1 year. METHODS: A retrospective cohort study was performed for all consecutive patients admitted to our intensive care unit (ICU). Augmented renal clearance was defined by a creatinine clearance ≥130 mL/min/1.73 m2. "Patient with ARC" was defined as a patient with a median of creatinine clearance ≥130 mL/min/1.73 m2 over the period of admission. Four variables were tested, Simplified Acute Physiology Score II (SAPS II), male gender, age, and trauma as cause for ICU admission. An analysis (patient based and clearance based) was performed with logistic regression. RESULTS: Of 475 patients, 446 were included in this study, contributing to 454 ICU admissions and 5586 8-hour creatinine clearance (8h-CLCR). Overall, the prevalence of patients with ARC was 24.9% (n = 113). In a subset of patients with normal serum creatinine levels, the prevalence was 43.0% (n = 104). Of the set of all 8h-CLCR measurements, 25.4% (1418) showed ARC. In the patient-based analysis, the adjusted odds ratio was: 2.0 (confidence interval [CI]:1.1-3.7; P < .05), 0.93 (CI: 0.91-0.94; P < .01), 2.7 (CI: 1.4-5.3; P < .01), and 0.98 (CI: 0.96 -1.01; P = .15), respectively, for trauma, age, male sex, and SAPS II. In the clearance-based analysis, the adjusted odds ratio were 1.7 (CI: 1.4-1.9; P < .01), 0.94 (CI: 0.932-0.942; P < .01), and 2.9 (CI: 2.4-3.4; P < .01), respectively, for trauma, age, and male sex. CONCLUSIONS: Trauma, young age, and male sex were independent risk factors for ARC. This condition occurs in a considerable proportion of critical care patients, which was particularly prevalent in patients without evidence of renal dysfunction.

Lewis score: a useful tool for diagnosis and prognosis in Crohn's disease.

BACKGROUND: videocapsule endoscopy (VCE) is currently the most sensitive diagnostic tool to detect early small bowel inflammation. A Lewis score (LS) of ≥ 135 as the cutoff value for the presence of significant inflammatory activity in patients undergoing VCE for suspected Crohn's disease (CD) has been suggested as a useful tool for the diagnosis of CD. The aim of this study was to evaluate the diagnostic and prognostic accuracy of the LS in patients with suspected CD undergoing VCE. METHODS: a retrospective single-center study was performed that included patients who underwent VCE for suspected CD between January 2010 and December 2015. Inflammatory activity was assessed with the LS. Patients were grouped according to the criteria of the International Conference on Capsule Endoscopy (ICCE) for the definition of suspected CD; group 1: patients not fulfilling ICCE and group 2: patients with ≥ 2 ICCE criteria. RESULTS: one hundred and ninety-one patients were included, 61% were female and the mean age was 39 ± 14 years. VCE detected significant inflammatory activity (LS ≥ 135) in 81 patients (42%); 24 patients from group 1 (32%) and 57 patients from group 2 (50%) (p = 0.014). During a mean follow-up period of 41 ± 21 months (12-79), a CD diagnosis was determined in 60 patients (31%); 55 patients with LS ≥ 135 (92%) and five patients with LS < 135 (5%) (p < 0.001). The LS showed a good diagnostic accuracy with an AUROC of 0.93 (p < 0.001). During the first year after diagnosis, there was a significant association between a higher LS and the need for immunomodulatory therapy, biological therapy, bowel resection surgery or hospital admission due to a CD flare-up. CONCLUSIONS: the LS (cutoff ≥ 135) is very useful in the diagnosis of CD in patients undergoing VCE. Moreover, higher values of this score was associated with prognostic variables.

How should we select suspected Crohn's disease patients for capsule enteroscopy?

Background: In suspected Crohn's disease (CD), non-diagnostic ileocolonoscopies are often followed by small bowel capsule endoscopy (SBCE). Adequate pre-selection of patients for SBCE is a key to optimize allocation of resources. We aimed to establish a rational approach for the CD diagnostic workflow, based on biochemical profile of patients with suspected CD, targeting an optimization of patients' selection for SBCE. Methods: Multicenter cohort study includes consecutive patients with suspected undergoing SBCE after non-diagnostic ileocolonoscopy. Minimum follow-up period after the capsule enteroscopy was six months. The outcome was confirmation of CD diagnosis. Univariate analysis and logistic regression were performed. Results: In included 220 patients, 62.3% of women were with a mean age of 41 years [26-54]. A confirmed diagnosis of CD was established in 98 patients (44.5%). The initial univariate analysis identified variables above the threshold of marginal statistical association toward CD diagnosis (p < .15). The regression model identified high CRP levels (OR 1.028 p = .128) and low serum Iron (OR 0.990 p = .025) as the independent variables with consistent correlation with CD diagnosis. Those two variables present a suitable discriminative power (AUC = 0.669, p < .001) for the diagnosis of CD. Conclusion: In suspected CD, low serum iron and elevated CRP had a statistically significant association with CD diagnosis, being helpful to identify patients with higher CD probability before SBCE. However, the lack of a proper validation of the model leads us to currently recommend SBCE to all patients with suspected CD and negative ileocolonoscopy, as no specific biochemical profile can be used to confidently exclude small bowel CD.

[The Impact of Patient Transfer After Rupture of an Abdominal Aortic Aneurysm]. / O Impacto da Transferência de Doentes após Rutura de Aneurisma da Aorta Abdominal.

OBJECTIVE: determine whether patient transfer adversely affects the survival of ruptured abdominal aortic aneurysm (rAAA) patients after conventional surgery. METHODS: We performed a retrospective review of all patients undergoing attempted repair of an rAAA at a tertiary center, over January 2008 and December 2014. Patients were divided into those presenting directly to our center and those transferred from another hospital. The main outcome variable was 24-hour or 30-day mortality, with secondary variables including time to surgical treatment, length of intensive care unit stay and total length hospitalization. RESULTS: 78 patients (88% men) underwent attempted open repair of an rAAA during this period, 69% (54 cases) were transferred from another institution. Both groups were similar in terms of demographic characteristics, comorbidities and hemodynamic stability. The overall mortality rate was 51% at 30 days. Transferred patients took twice as long as direct patients to get to the operating room (median 7,9 vs. 3,9 horas, p < 0,05), Although the difference for surgery treatment, there was no difference in 24-hour and 30-day mortality between the transferred group and direct group (26% e 50% vs. 29% e 58%, p < 0,05). Mean intensive care unit stay (median, 12 vs. 4 dias, p = 0,04) and total hospitalization (median 11 vs. 4 dias, p = 0,04) were sustantially superior in the transferred group. CONCLUSIONS: Transfer of patients with RAAA in this series results in a doubling of the time interval between initial patient presentation and arrival in the operating room. This, however, did not result in any disadvantage in the survival rate between the groups. The total length and resources consumption were higher in the transfer group. These results may be attributed to a pre-selection of patients (clinically stable) who are able to tolerate such a delay in surgical treatment, secondary to transfer.

Objetivo: Determinar se a transferência inter-hospitalar afecta adversamente a sobrevida de doentes com rutura de aneurisma da aorta abdominal (rAAA), submetidos a cirurgia convencional. Métodos: Efetuamos uma análise retrospetiva dos rAAA tratados sequencialmente por cirurgia convencional, num centro de referenciação terciária, no período compreendido entre janeiro 2008 e dezembro de 2014. Os casos foram divididos entre os que se apresentram diretamente neste centro e os que foram alvo de transferência. Analisou-se a mortalidade às 24 horas, a mortalidade aos 30 dias e, como outcomes secundários, incluíram-se o tempo de chegada ao bloco operatório, duração de internamento na unidade de cuidados intensivos (UCI) e o tempo de internamento total. Resultados: Neste período, um total de 78 casos (88% do género masculino) foram submetidos a cirurgia convencional por rAAA, 69% (54 casos) transferidos de outra instituição. Ambos os grupos mostraram semelhanças nas características demográficas, bem como no perfil de comorbilidades e estadio de estabilidade hemodinâmica. A mortalidade global foi de 51% aos 30 dias. O tempo de chegada ao bloco operatório do grupo transferido foi 2 vezes superior (mediana 7,9 vs. 3,9 horas, p < 0,05). Apesar da diferença para início da prestração de cuidados cirúrgicos específicos, a mortalidade às 24 horas e aos 30 dias não diferiu entre entre o grupo transferido e o direto (26% e 50% vs. 29% e 58%, p < 0,05) respectivamente. O tempo de internamento na UCI (mediana, 12 vs. 4 dias, p = 0,04) e a duração total de internamento (mediana 11 vs. 4 dias, p = 0,04) foram substancialmente maiores para o grupo transferido. Conclusões: O resultado da transferência inter-hospitalar, nesta série de doentes, resultou numa duplicação do tempo de admissão no bloco operatório Contudo, não se verificou qualquer desvantagem em relação à sobrevida entre os grupos. A duração da hospitalização e consumo de recursos foi maior no grupo transferido. Estes resultados podem dever-se à possível seleção de doentes transferidos (clinicamente estáveis) que conseguem tolerar o atraso na instituição do tratamento cirúrgico, decorrente da transferência.

Phenotypic heterogeneity of hereditary diffuse gastric cancer: report of a family with early-onset disease.

BACKGROUND AND AIMS: The time course for the development of clinically significant hereditary diffuse gastric cancer (HDGC) is unpredictable. Little is known about the progression from preclinical, indolent lesions to widely invasive, aggressive phenotypes. Gastroendoscopy often fails to detect early lesions, and risk-reducing/prophylactic total gastrectomy (PTG) is the only curative approach. We present an HDGC family with early-onset disease in which clinical and histologic findings provided insight into the understanding of different HDGC phenotypes. METHODS: The proband was diagnosed at age 18 years with widely invasive, metastatic DGC. CDH1 genetic testing identified a pathogenic, germline CDH1 variant (c.1901C>T, p.Ala634Val). Thirty family members were tested, and 15 CDH1 carriers were identified. RESULTS: Six family members had PTG, with negative preoperative workup. The proband's 14-year-old sister is the youngest patient, reported to date, to have PTG after negative preoperative biopsy sampling. Intramucosal HDGC foci were detected in all PTG specimens (1-33). In contrast to the "indolent" phenotype of these foci, the aggressive DGC from the proband showed pleomorphic cells, absent E-cadherin expression, increased proliferation (Ki-67 index), and activation of oncogenic events (p53, pSrc and pStat3 overexpression). All family members had Helicobacter pylori gastritis. Cag-A-positive strains were detected in all specimens, except in the proband's sister. CONCLUSIONS: HDGC is a heterogeneous disease regarding clinical behavior, endoscopic findings, histopathologic features, and immunophenotypic/molecular profile. The presence of bizarre, pleomorphic cells in endoscopic biopsy specimens is suggestive of advanced disease and should prompt clinical intervention. The involvement of a full multidisciplinary team is essential for the management of these patients.