GE Portuguese Journal of Gastroenterology最新文献

Introduction: Intraductal papillary mucinous neoplasms (IPMNs) are pancreatic tumours with an associated risk of malignant transformation. Due to the widespread use of imaging techniques, the diagnosis of IPMNs has been rising. The International Evidence-Based Kyoto Guidelines (IKG), the latest update of the International Guidelines, were developed to refine the management of IPMNs. IKG incorporate high-risk stigmata and worrisome features (WF) into the decision-making process. This study evaluates the accuracy of these guidelines in identifying patients requiring surgery.

Methods: A single-centre retrospective cohort study conducted at a University Hospital, including adult patients who underwent pancreatic surgery for IPMNs between 2010 and 2024. Data on demographic characteristics, imaging findings, surgical procedures, and histopathological outcomes were collected. Criteria for surgery, according to the IKG, were retrospectively applied. The diagnostic performance of IKG was assessed through sensitivity, specificity, positive predictive value, negative predictive value, and overall accuracy.

Results: One hundred and six patients (mean age: 67 ± 10 years) were included, and 18.9% had histologically confirmed malignant IPMNs. The sensitivity of IKG in detecting malignancy was 100%, while the specificity was 19.8%. The positive negative predictive values were 22.5% and 100%, respectively. The most predictive factors for malignancy were the presence of lymphadenopathy, abrupt changes in main pancreatic duct calibre, and elevated serum carbohydrate antigen 19-9. An association was observed between the number of WF and the malignancy rate.

Discussion: The IKG demonstrated excellent sensitivity in selecting patients for surgery, ensuring that all malignant cases were identified. However, the low specificity suggests the need for criteria revision to minimize overtreatment. Future guidelines should focus on integrating patient-specific factors, such as age and comorbidities, into decision-making algorithms to optimize management strategies and to consider the weight and number of WF.

Background/aims: To minimize the risk of post-procedure complications, air cholangiography has been proposed as an alternative to contrast cholangiography in patients with hilar biliary obstruction undergoing endoscopic retrograde cholangiography (ERCP). We compared the rate of post-ERCP complication between these two groups.

Methods: We systematically reviewed all studies that compared the safety, success, and complication rate of air versus contrast cholangiography in malignant hilar obstruction patients.

Results: A total of six studies consisting of 450 patients were included in the analysis. The overall rate of post-ERCP complication was less in the air cholangiography group (relative risk [RR]: 0.43, 95% CI: 0.30-0.61, I ² : 1.97%) compared to the contrast cholangiography group. Compared to contrast cholangiography, the pooled risk of post-ERCP cholangitis was significantly lower in the air cholangiography group (RR: 0.25, 95% CI: 0.14-0.44, I ² : 0%). There was no difference in risk of post-ERCP pancreatitis (RR: 0.79, 95% CI: 0.32-1.94, I ² : 0%), bleeding (RR: 1.17, 95% CI: 0.30-4.54), and 30-day mortality (RR: 0.59, 95% CI: 0.23-1.50, I ² : 0%) between the two groups. The pooled odds of clinical (odds ratio [OR]: 1.14, 95% CI: 0.39-3.34, I ² : 0%) and technical success (OR: 2.91, 95% CI: 0.52-16.35, I ² : 0%) were similar between both groups.

Conclusion: In comparison to contrast cholangiography, air cholangiography had a better safety profile and a lower rate of post-ERCP complications.

Introduction: Wilson's disease is a rare autosomal recessive inherited disorder of human copper metabolism. The spectrum of clinical manifestations of liver disease ranges from asymptomatic to cirrhosis and acute liver failure. Unlike the increased frequency of hepatobiliary tumours in other chronic liver diseases, the incidence of these tumours in Wilson's disease is an uncommon event, even in cirrhotic patients.

Case presentation: We describe two clinical cases of cirrhotic patients with Wilson disease diagnosed with cholangiocarcinoma.

Discussion: The occurrence of hepatobiliary malignancies in Wilson's disease is rare, even in cirrhotic patients. The exact reasons for this low prevalence are unclear, with some conflicting data. Copper's role in chromatin stabilization and angiogenesis, along with copper deficiency due to Wilson's disease and its treatment, may reduce tumour growth. The current guidelines do not have specific directives regarding cholangiocarcinoma screening in patients with cirrhosis secondary to Wilson's disease. Despite the lower incidence of malignancies in Wilson's disease, vigilant monitoring is advised.

Background: Gastric peroral endoscopic myotomy has shown promising results for refractory gastroparesis. We share our Indian experience of gastric peroral endoscopic myotomy performed over the last 5 years.

Methods: This is a retrospective study of prospectively maintained data of patients who underwent gastric peroral endoscopic myotomy for refractory gastroparesis at our center. The primary outcome was to assess the clinical success at 3 months measured by ≥50% reduction of gastroparesis cardinal symptom index score from the baseline. The secondary outcomes were to assess the technical success, change in percentage retention at 4 h in gastric emptying scintigraphy at 6 months, change in gastroparesis cardinal symptom index score, Patient Assessment of Upper Gastrointestinal Disorders-Quality of Life score at 1, 6 months and 1 year, respectively, and adverse event rate graded by AGREE classification.

Results: Between 2019 and 2024, 16 patients underwent gastric peroral endoscopic myotomy in our unit. The median (IQR) age was 40 (30.5, 48.75), and 10 were females (62.5%). The most common etiology was idiopathic (11/16, 68.8%). The clinical success at 3 months was 72.4% (8/11), which was sustained at 6 months and 1 year of follow-up to 71.7% (5/7). The technical success was 100%. There was a significant reduction in gastroparesis cardinal symptom index score, Patient Assessment of Upper Gastrointestinal Disorders-Quality of Life score at 1, 3, 6 months and 1 year. Similarly, there was a significant reduction in the percentage retention at 4 h in gastric emptying scintigraphy at 6 months. One patient needed intraoperative needle aspiration of the capnoperitoneum while another had hematemesis after discharge, which was managed conservatively.

Conclusion: Our first Indian experience of gastric peroral endoscopic myotomy for refractory gastroparesis shows similar outcomes compared to global literature with gastric peroral endoscopic myotomy emerging as a preferred modality in these subsets of patients.

Background: Pancreatic cystic neoplasms (PCNs) are lesions with malignant potential that should be addressed differently according to clinical/morphological criteria, to ensure appropriate cancer prevention and while avoiding overtreatment.

Summary: Surgical resection remains the gold standard for high-risk PCNs (at least one high-risk stigmata, multiple "worrisome" features), whereas low-risk PCNs should be monitored through imaging to mitigate the non-negligible risk of postoperative complications. Endoscopic ultrasound (EUS)-guided therapies, such as injection or radiofrequency, may offer a minimally invasive and effective therapeutic option particularly for patients unfit for surgery, although their impact on prognostic (i.e., mortality) remains uncertain.

Key messages: This review summarizes the latest recommendations on surgical management and post-resection follow-up of PCNs and the potential role of EUS ablation in the treatment algorithm.

Introduction: Hepatocellular injury is characterized by elevated levels of aspartate aminotransferase (AST) and alanine aminotransferase (ALT). In contrast, alkaline phosphatase levels are usually elevated in cholestatic diseases. Due to the multiples causes, it is crucial to define the etiology for proper management. The most common diagnoses are viral hepatitis, autoimmune hepatitis, Wilson's disease, drug-induced liver injury, acute alcoholic hepatitis, and ischemic hepatitis. Extrahepatic causes such as endocrine and cardiovascular conditions should also be considered.

Case presentation: A 46-year-old male presented with a 2-month history of elevated transaminase levels that progressed to jaundice and dark urine (choluria) in the preceding 30 days. During the investigation, it was discovered that the hepatocellular injury was caused by hypoperfusion secondary to a retroperitoneal catecholamine-secreting sympathetic paraganglioma, in which the patient achieved a favorable outcome with complete preservation of liver function.

Conclusions: This case highlights the importance of considering rare causes of hepatocellular injury.

Introduction: Ulcerative colitis (UC) and Crohn's disease (CD) are inflammatory bowel diseases (IBDs) characterised by chronic inflammation of the digestive tract. Patients with IBD often present a suboptimal disease control due to the limited efficacy of current treatments and inadequate disease management. A suboptimal disease control is often associated with poor quality of life (QoL) and a higher economic burden. The objective of this study was to assess the proportion of suboptimal disease control in daily clinical practice, the associated impact on QoL, and the economic burden in patients with UC and CD in Portugal.

Methods: This was a non-interventional, multicentre study, wherein clinician-reported outcomes, patient-reported outcomes, and retrospective data were collected from medical chart review from May 17, 2022, to October 17, 2022, in five hospital centres in Portugal. The primary outcomes of this study were to estimate: (1) the proportion of CD and UC patients with suboptimal disease control, according to the STRIDE-II based recommendations, and (2) the associated impact on QoL of optimal and suboptimal controlled CD or UC.

Results: Of 130 patients included (67 with CD and 63 with UC), 56.7% of CD and 31.7% of UC patients were reported to have suboptimal disease control according to STRIDE-II based recommendations. Notably, 82.1% and 54.0% of the patients with CD and UC were currently on targeted immunomodulator (TIM). Patients with CD and UC and suboptimal disease control showed an impaired QoL compared to those with optimal control. Mean annual healthcare costs were substantially higher among patients with CD with optimal control and patients with UC with suboptimal control.

Conclusion: Despite the high rate of treatment with TIM, a considerable proportion of patients with IBD had a suboptimal disease control, indicating a substantial disease burden. Ensuring effective disease management of these patients is crucial as a suboptimal disease control may negatively affect the patient's long-term clinical outcomes and QoL.

Introduction: Basal layer type squamous cell carcinoma (BLSCC) is a rare subtype of esophageal carcinoma imposing challenges in the diagnosis and endoscopic treatment with prognostic implications. We report a case of BLSCC successfully treated by complete circumferential endoscopic submucosal dissection.

Case presentation: A 73-year-old man with a history of a stable non-Hodgkin's lymphocytic lymphoma presented with dysphagia. After a demanding diagnostic workup, an almost circumferential and extensive tenuous flat lesion of the distal esophagus was found. In a multidisciplinary team discussion, a circumferential endoscopic dissection approach was decided, which proved to be curative. The final histopathological diagnosis was BLSCC, a challenging diagnosis of a rare form of squamous cell neoplasia. There were no adverse events, including esophageal stenosis, or recurrence during a 12-month follow-up.

Conclusion: Endoscopic submucosal dissection is an effective and safe therapeutic option for this rare type of esophageal squamous cell carcinoma, even with extensive esophageal involvement.