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Gut ; 71(5): 961-973, 2022 May.
Artigo em Inglês | MEDLINE | ID: mdl-33849943


OBJECTIVE: Recent studies have found aristaless-related homeobox gene (ARX)/pancreatic and duodenal homeobox 1 (PDX1), alpha-thalassemia/mental retardation X-linked (ATRX)/death domain-associated protein (DAXX) and alternative lengthening of telomeres (ALT) to be promising prognostic biomarkers for non-functional pancreatic neuroendocrine tumours (NF-PanNETs). However, they have not been comprehensively evaluated, especially among small NF-PanNETs (≤2.0 cm). Moreover, their status in neuroendocrine tumours (NETs) from other sites remains unknown. DESIGN: An international cohort of 1322 NETs was evaluated by immunolabelling for ARX/PDX1 and ATRX/DAXX, and telomere-specific fluorescence in situ hybridisation for ALT. This cohort included 561 primary NF-PanNETs, 107 NF-PanNET metastases and 654 primary, non-pancreatic non-functional NETs and NET metastases. The results were correlated with numerous clinicopathological features including relapse-free survival (RFS). RESULTS: ATRX/DAXX loss and ALT were associated with several adverse prognostic findings and distant metastasis/recurrence (p<0.001). The 5-year RFS rates for patients with ATRX/DAXX-negative and ALT-positive NF-PanNETs were 40% and 42% as compared with 85% and 86% for wild-type NF-PanNETs (p<0.001 and p<0.001). Shorter 5-year RFS rates for ≤2.0 cm NF-PanNETs patients were also seen with ATRX/DAXX loss (65% vs 92%, p=0.003) and ALT (60% vs 93%, p<0.001). By multivariate analysis, ATRX/DAXX and ALT status were independent prognostic factors for RFS. Conversely, classifying NF-PanNETs by ARX/PDX1 expression did not independently correlate with RFS. Except for 4% of pulmonary carcinoids, ATRX/DAXX loss and ALT were only identified in primary (25% and 29%) and NF-PanNET metastases (62% and 71%). CONCLUSIONS: ATRX/DAXX and ALT should be considered in the prognostic evaluation of NF-PanNETs including ≤2.0 cm tumours, and are highly specific for pancreatic origin among NET metastases of unknown primary.

Deficiência Intelectual , Tumores Neuroendócrinos , Neoplasias Pancreáticas , Talassemia alfa , Proteínas Correpressoras/genética , Genes Homeobox , Proteínas de Homeodomínio , Humanos , Deficiência Intelectual/genética , Chaperonas Moleculares/genética , Recidiva Local de Neoplasia/genética , Tumores Neuroendócrinos/genética , Proteínas Nucleares/genética , Neoplasias Pancreáticas/patologia , Telômero/genética , Telômero/patologia , Fatores de Transcrição/genética , Proteína Nuclear Ligada ao X/genética , Talassemia alfa/genética
Proc (Bayl Univ Med Cent) ; 33(2): 239-240, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-32313472


Splenic infarctions have been reported in cirrhotic patients with comorbidities (e.g., primary biliary cirrhosis), but there is little to no mention of spontaneous splenic infarction in alcoholic hepatitis. This condition is notable, as splenic function has been shown to decrease in alcoholic liver disease, but the exact pathophysiology has been unclear. We describe a relatively young cirrhotic woman who developed splenic infarctions in the setting of alcoholic hepatitis.

Proc (Bayl Univ Med Cent) ; 31(2): 203-204, 2018 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-29706820


We present a case of severe systolic anterior motion developing intraoperatively after aortic valve replacement for aortic valve stenosis.

Proc (Bayl Univ Med Cent) ; 31(4): 404-406, 2018 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-30948967


We performed a retrospective chart review to investigate a potential relation between pulmonary artery (PA) diameter as measured by intraoperative transesophageal echocardiography and PA pressures measured by PA catheter with the aim of determining whether main PA diameter can aid clinicians in the diagnosis of PA hypertension. A total of 82 adult patients undergoing cardiac surgery were included in our study. Main PA diameter showed a moderate correlation with systolic and diastolic pressures, r = 0.576 (95% confidence interval [CI], 0.407-0.703), P < 0.001, and r = 0.504 (95% CI, 0.319-0.648), P < 0.001, respectively. The authors believe that although a moderate correlation exists between main PA diameter and PA pressure, confounding hemodynamic variables prevent main PA diameter from being an accurate and reliable means of diagnosing PA hypertension.