Acta gastro-enterologica Belgica最新文献

Hepatitis E is an increasingly recognized cause of acute hepatitis in the Western world and is often complicated by extrahepatic manifestations, particularly neurological ones such as neuralgic amyotrophy (NA). NA is a disease of the brachial plexus, characterized by sudden onset of severe pain and muscle weakness, typically affecting the shoulders and upper limbs and can even extend to the diaphragm. Recent data suggest that its presence is linked to a HEV infection in 10% of the cases. Due to its rarity, there are currently no established treatment guidelines. Here we present a case of a 44-year-old man who developed bilateral NA and phrenic nerve palsy as a consequence of an acute hepatitis E infection.

Neonatal cholestasis is a diagnostic challenge that warrants extensive investigation as there can be serious sequalae such as liver failure, cirrhosis, or other extrahepatic complications. To differentiate the etiology of cholestasis, a distinction can be made between high and low gamma-glutamyltransferase (GGT) cholestasis. Low GGT cholestasis points towards progressive familial intrahepatic cholestasis type 1-2 and 4-6, bile acid synthesis disorders, tight-junction protein type 2 deficiency and some forms of hypopituitarism. Classic galactosemia is generally not included in the differential diagnosis of low GGT cholestasis. Here, we demonstrate low GGT cholestasis in 9 consecutive patients with classic galactosemia at the University Hospitals of Leuven, Belgium. All neonatal cholestasis should be managed with prompt cessation of galactose intake, but in classic galactosemia it can be lifesaving. We now add that low GGT cholestasis increases the likelihood of galactosemia. Conversely, high GGT cholestasis could point to other causes, like biliary atresia, where there may be no need to stop breastfeeding. In galactosemia, we observe a rise in GGT after initiation of a galactose-free diet, which we suggest may be partially explained by the normalization of bile acid transporter glycosylation.

Background: Cholecystitis and pancreatitis are frequent indications for hospitalization in gastroenterology and hepatology, predominantly due to cholelithiasis. Standard management strategies with imaging and cholecystectomy are common practice. This case report highlights the importance of considering atypical causes in patients presenting with symptoms suggestive of cholecystitis and pancreatitis.

Case report: A female patient presented repeatedly to the emergency department with atypical ultrasound findings suggestive of cholecystitis with secondary pancreatitis. Following an episode of severe upper gastrointestinal bleeding, a diagnosis of haemobilia with secondary pancreatitis was established.

Discussion: This case illustrates that haemobilia can present with symptoms resembling calculous cholecystitis and biliary pancreatitis. Diagnosis can be challenging, especially in the absence of recent procedures or known malignancy. Quincke's triad-pain, jaundice, and bleeding-is observed in only a third of cases.

Conclusion: Haemobilia, though rare, is an important differential diagnosis in cases of upper gastrointestinal bleeding and biliary obstruction. A multidisciplinary approach is essential for prompt diagnosis and appropriate treatment.

Background: Hepatic encephalopathy (HE) is a well-known complication of cirrhosis. When HE recurs despite optimal treatment or without a clear precipitant, a portosystemic shunt may be the culprit. We present a case of therapy-refractory HE caused by a spontaneous portosystemic shunt (SPSS).

Clinical case: A 69-year-old male with MASLD-related cirrhosis, with a medical history of biliopancreatic diversion and variceal bleeding, presented with his fifth episode of HE despite maximal pharmacological treatment. Initial lab tests and infectious screen were negative. Abdominal CT scan showed a paraesophageal shunt, supplied by the coronary vein. To address HE, SPSS closure was planned. Since SPSS closure could aggravate PHT and subsequently increase paracentesis frequency or induce variceal bleeding, this was combined with a proximal splenic artery embolization (pSAE). pSAE after SPSS closure resulted in a reduction of hepatic venous pressure gradient (HVPG) from 21mmHg to 15 mmHg. HE symptoms resolved, pharmacological treatment was stopped, and paracentesis became less frequent. Weeks later, the patient was readmitted with abdominal pain, and CT confirmed splenic infarction. Recurrent spontaneous bacterial peritonitis necessitated splenectomy, which was complicated by hemorrhage, septic shock, and death.

Conclusion: This case highlights the potential of combining SPSS closure and pSAE, two procedures that have not been combined before to our knowledge, to effectively reduce HE and HVPG, enabling cessation of anti-encephalopathic therapy and lowering paracentesis frequency. However, altered vascular anatomy can lead to complications such as splenic infarction. Although splenic infarction occurred as a complication, we believe this novel combined procedure warrants further investigation.

Neuroendocrine neoplasms (NEN) are rare tumors originating from neuroendocrine cells, commonly found in the gastrointestinal tract and the pulmonary tract. Metastatic welldifferentiated neuroendocrine tumors (NET) grade 3 present unique challenges, as they are positioned between the more indolent NET grade 1-2 and the aggressive neuroendocrine carcinomas (NEC). Due to the scarcity of data regarding the optimal systemic treatment for metastatic NET grade 3 and aggressive NET grade 2 subtypes, guidelines remain inconclusive. This retrospective study analyzed data from the NETwerk database, encompassing patients treated with the capecitabinetemozolomide (CAPTEM) regimen between June 2016 and January 2024. The cohort included patients with NET grades 1-3 and NEC. The study focused on assessing the efficacy and safety of CAPTEM. In total, data from 36 patients was analyzed. The median progression-free survival (mPFS) was 13 months, and median overall survival (mOS) was 17 months. Overall response rate (ORR) was 25.8%, and the disease control rate (DCR) was 67.7%. NET grade 2 patients had the highest mPFS, while NET grade 3 exhibited the most favorable mOS. Subgroup analysis showed that panNEN had superior mPFS and mOS compared to other primary tumor sites, with significant differences in mOS based on NEN type. Safety analysis in 20 patients indicated good tolerance and safety . CAPTEM is an efficient and safe regimen for metastatic NEN, with promising outcomes in NET grade 2-3 patients. The promising findings pave the way for further exploration into various aspects of CAPTEM, to better define its position in the therapeutic landscape of NEN.

Background: Chronic diarrhea is one of the more common reasons for referral to a gastro-enterologist. Chronic diarrhea can have a broad range of causes, making it a disease entity with a very extensive differential diagnosis. In a proportion of patients, however, a cause for the chronic diarrhea cannot be found and these patients are said to have chronic idiopathic diarrhea (CID).

Methods: A Delphi model was used to establish a diagnostic strategy for patients presenting with chronic diarrhea that maximizes the chance for a positive diagnosis while minimizing the number and invasiveness of the investigations. In addition, the participating experts sought consensus on the different treatment options that can be used in these patients.

Results: While a general consensus was reached on the required diagnostic tests for CID, marked differences were observed on the treatment preferences and strategies for these patients among the different experts. The main reason for this is the lack of solid scientific evidence with the different treatment options in this setting (i.e., most data have been generated in patients with IBS-D).

Conclusion: the Delphi-like process that was used for this initiative proved to be a useful vehicle to fuel discussions on the management of CID among experts with different backgrounds and to sketch the current clinical practice.

Background: Lichen planus is a mucocutaneous chronic inflammatory disorder that can also affect the esophagus in a minority of patients. Esophageal lichen planus (ELP) is likely an underdiagnosed and underreported disease entity, although it can lead to significant morbidity with esophageal stenosis or malignant transformation.

Methods: A non-systematic search of Pubmed was performed using search terms 'esophageal lichen planus' and variants thereof combined with terms related to prevalence, diagnosis, and management.

Results: The cutaneous and oral forms of lichen planus are relatively common, but certain mucosal forms such as genital lichen planus are rare and esophageal planus (also a mucosal form) is very rare. ELP occurs predominantly in middle-aged women. Current guidelines regarding lichen planus do not mention a screening strategy for ELP. Diagnosis of ELP is based on endoscopic and histopathological criteria. The foremost complication of ELP is formation of esophageal strictures. Malignant transformation of ELP to squamous cell carcinoma, although rare, has also been reported. We summarize the current evidence regarding optimal management of ELP.

Conclusion: Even if esophageal lichen planus is a rare cause of esophageal strictures, it is important to raise awareness of this condition among gastroenterologists and endoscopists. The cornerstone of ELP treatment is an anti-inflammatory agent in conjunction with endoscopic dilatation of strictures. Further research is needed to devise an evidence-based treatment algorithm that describes the optimal anti-inflammatory drug in different stages/severities of ELP.