Clinical Case Summary

​Introduction: With approximately 100 cases annually, leptospirosis is among the rarest zoonotic infectious diseases in Germany. It is caused by Leptospira bacteria, which are transmitted through contact with contaminated water or soil. While most infections are self-limiting, some progress to sepsis and multiorgan failure.
Case report: We report the case of a previously healthy 44-year-old man who presented to the emergency department with fever and diarrhoea. Laboratory tests revealed liver failure with hyperbilirubinemia, coagulopathy, and renal dysfunction. Due to his deteriorating condition and unexplained organ failure, he was admitted to the intensive care unit. His clinical status worsened further, requiring endotracheal intubation due to respiratory insufficiency. CT imaging showed bilateral pneumonia and thickened colonic walls. To improve oxygenation, prone positioning was applied. Microbiological testing detected Leptospira in both blood and urine. Treatment with meropenem led to clinical improvement, and the patient was extubated after seven days. Interestingly post-extubation the patient presented tetraplegia and aphasia, which gradually improved. Brain MRI and lumbal puncture did not show any relevant abnormalities.
Conclusion: Icteric leptospirosis affects approximately 5–10% of symptomatic cases and can progress rapidly, with a mortality rate between 5–15%. In cases of moderate to high clinical suspicion, empiric treatment should be initiated promptly, even before laboratory confirmation. This case illustrates a rare and severe presentation of leptospirosis with multiple organ involvement and transient neurological deficits. Clinicians should consider leptospirosis in the differential diagnosis of acute liver failure and refer severe cases promptly to specialised intensive care centers.

Introduction

Gastric peroral endoscopic myotomy (G-POEM) is an emerging endoscopic technique for the treatment of refractory gastroparesis, particularly benefiting patients with pylorospasm. It is considered a safe procedure with a low risk of adverse events when performed by experienced endoscopists. Our aim was to perform a comprehensive analysis of all adverse events associated with G-POEM.

Aims & Methods

We performed a retrospective analysis of prospectively collected data from all patients who underwent G-POEM for gastroparesis at our centers (Prague, Brno) between November 2017 and April 2025. All procedures were performed by a single endoscopist (JM). The primary outcome was procedure-related adverse events rate and severity occurring during the perioperative and early postoperative period. Adverse events were categorized as follows: deviations from the expected peri and postoperative course were defined as "undesirable events." Undesirable events that met the criteria of the Clavien Dindo surgical classification (C-D) were considered "true adverse events".

Results

Out of 70 procedures, no true adverse or undesirable events occurred in 51 procedures (72.9%). Perioperatively, the following undesirable events occurred in 18 patients: : gastric (4; 5.7%) and duodenal mucosal injuries (2; 2.8%), major bleeding during the procedure treated endoscopically (1; 1.4%), serosal perforation (10; 14.3%), capnoperitoneum requiring puncture (3; 4.3%), and subcutaneous emphysema (1; 1.4%). In the early postoperative period, adverse events occurred in 3 patients: one patient complained of significant abdominal pain necessitating analgetics, subsequently, a small leak from the pyloric region to the subhepatic region was described on CT and gastroscopy confirmed ulceration at the tunnel site. The patient responded well to treatment with intravenous proton pump inhibitors. In two other cases, both involving patients with diabetic gastroparesis, decompensation of diabetes occurred during the hospital stay post-procedure. The following “undesirable” events were considered true adverse events according to the C-D classification: serosal perforation (10; C-D I), leak requiring prolonged hospitalization (1; C-D IIIa) and diabetes decompensation (2; C-D II). These complications occurred in 13 procedures (18.6%).
The mean duration of hospitalization after the procedure was 2 days (range 1-7). Three months after the procedure, 1 patient developed mild dumping syndrome, which was successfully treated conservatively.

Conclusion

G-POEM is a safe procedure. The rate of true adverse events according to Clavien-Dindo classification is low and severe AEs occurs rarely.

Introduction

Autoimmune pancreatitis (AiP) is a rare form of chronic pancreatitis. Studies from Japan and Germany suggest that AiP patients may have a higher incidence of malignancies compared to the general population. However, reliable data in this regard are still lacking.

Aims & Methods

We initiated a retrospective, multicenter observational study within the Pancreas 2000 framework to investigate the incidence of malignancy in AiP Patients.In our global multicenter retrospective observational trial, we are currently collecting data from patients with AiP diagnosed after 2005, including type 1, type 2, and not-otherwise-specified (NOS) AIP, regardless of the diagnostic criteria used. Patients with less than 12 months of follow-up after AIP diagnosis and those with immune checkpoint inhibitor-induced pancreatitis are excluded. Our primary outcome is to determine the occurrence of the first invasive cancer in AiP patients. The secondary outcome is to evaluate the characteristics of AiP patients with distinct pancreatic and/or extrapancreatic cancers and the prevalence of all cancer diagnoses within 12 months prior to AiP diagnosis. The standardized incidence ratio of any first invasive cancer after AiP diagnosis will be compared to age-grouped and gender-matched controls in the general population. The expected number of cancers in the general population will be determined using the Cancer Incidence in Five Continents Volume XI (CI5XI) registry.

Results

We will present the current status of recruitment and preliminary data. To date, 16 centers from Europe and America have entered 321 cases. Ethics submissions have already been applied for in an additional 45 centers across four continents (Europe, Asia, America, and Oceania).

Conclusion

To the best of our knowledge, this is the first global observational trial analyzing the risk of developing cancer in patients with AiP.

Clinical Case Summary

A 72-year-old patient with a history of atrial fibrillation on treatment and cirrhosis, presented with subjective fevers and abdominal distention for 2 months. An initial laboratory work-up showed an inflammatory syndrome and a prothrombin level of 40 %. The rest of the work-up was unremarkable. A computed tomography (CT) scan of the abdomen showed dysmorphic liver with signs of portal hypertension and abundant ascites with micronodular infiltration of the greater omentum.
Paracentesis showed ascitic fluid (AF) with total nucleated cells 2400 with 5% neutrophils and 87% lymphocytes and protein levels at 40. Cytology was negative for malignancy.
Initially, the patient was considered for diagnostic laparoscopy to investigate the persistent abdominal symptoms. However, in light of the current epidemiological context, marked by a heightened incidence of tuberculosis in the region, a systematic workup for tuberculosis was prioritized. Direct examination and culture for acid-fast bacilli (AFB) were negative, however Xpert Mtb/Rif was positive. standard chest X-ray showed a normal parenchyma with a small right pleural effusions. Thoracentesis was done and pleural fluid AFB culture was negative.
He was started on isoniazid, rifampin, pyrazinamide and ethambutol and was discharged with a close follow up.

Clinical Case Summary

A 12-year-old girl, previously healthy, was admitted to pediatric ward with jaundice, itchy skin, and
general weakness for about 10 days. Physical examination revealed jaundice, discrete exophthalmos,
and tachycardia. Laboratory tests showed significantly elevated transaminases, total and conjugated
bilirubin and bile acids, with a mild increase in GTP. INR, ammonia and albumin levels remained within the range. In the first stage of diagnosis, infectious factors (primary and secondary hepatotropic viruses), Wilson's disease and alpha-1-antitrypsin deficiency were ruledout. Elevated ANA antibody levels and increased serum IgG titers have been found, but a liver panel was negative. Imaging studies (MRI) showed no changes in the liver and bile ducts. After consultation with the pediatric gastroenterologist thyroid function tests were performed which revealed: undetectable TSH, high fT4 and fT3. In the course of further diagnostics, positive titers of anti-Tg, anti-TSHr, and anti-TPO antibodies and enlarged thyroid in USG were obtained, which led to a diagnosis of Graves' disease. The treatment included methimazole and propranolol, which stabilized the patient's general condition and improved laboratory parameters within a few days. Currently, the patient is continuing methimazole therapy and remains euthyroid, with liver function tests completely normalized.
The clinical case illustrates the coexistence and mutual influence of liver and thyroid diseases. The patient was initially suspected of having AIH induced by autoimmune thyroid disease. The negative liver panel results could indicate a seronegative variant of AIH. The patient was scheduled for a liver biopsy to establish a definitive diagnosis, but the test was postponed due to a marked improvement in liver parameters as a result of the control of thyrotoxicosis. Complete clinical and laboratory improvement after treatment with thionamides, without the use of typical AIH therapy, argues against this diagnosis.

Disclosure

No conflict of Interest

Clinical Case Summary

A 77-year-old patient with diabetes insipidus underwent an abdominal computed tomography scan due to upper quadrant abdominal pain. The exam showed concentric thickening of the main and intrahepatic bile ducts. A subsequent magnetic resonance cholangiopancreatography, with diffuse thickening of the main and intrahepatic bile ducts, with diffusion restriction and reduced biliary duct caliber, without lithiasis. Due to the suspiecion of a malignant biliary stricture, the patient was proposed for endoscopic retrograde cholangiopancreatography (ERCP) with cholangioscopy. The cholangiogram showed diffuse rarefaction of the intra and extrahepatic bile ducts, with focal dilatation of the intrahepatic bile ducts. After biliary sphincterotomy, cholangioscopy revealed a narrowed main bile duct extending to the hepatic hilum, with neovascularization, and papillary projections. Multiple biopsies were taken and a plastic stent was placed. Cholangioscopy-guided biopsy revealed granulation tissue and stroma with numerous histiocytes and eosinophils. Immunohistochemistry identified histocytes expressing CD68, S100, and CD1a, consistent with biliary involvement by Langerhans Cell Histiocytosis. The procedure was complicated by acute pancreatitis, requiring Intensive Care Unit admission. The patient’s hydroelectrolytic disorders were caused by pituitary involvement (lymphocytic hypophysitis associated with granulomatous disease). The patient started corticosteroid therapy with clinical and analytical improvement.
Langerhans Cell Histiocytosis is characterized by histiocytes proliferation, with progressive fibrosis due to tissue infiltration. In this case, diabetes insipidus was the first disease manifestation, due to lymphocytic hypophysitis. LCH is a rare cause of secondary sclerosing cholangitis, and early diagnosis through cholangioscopy was essential to initiate appropriate systemic therapy, preventing disease progression with multiorgan involvement.

Clinical Case Summary

Hydatid cyst disease, caused by the Echinococcus parasite, is a zoonotic infection that typically targets the liver, lungs, and nervous system. It commonly occurs in regions such as the Maghreb countries, where it presents a significant health concern. While hydatid cysts in the liver are usually asymptomatic, complications such as cyst rupture, infection, or obstruction can arise, with portal vein involvement being particularly rare. This case report describes a 56-year-old male with a history of surgery for hydatid liver cyst, who presented with portal vein invasion mimicking portal vein thrombosis. Diagnostic imaging, including Color Doppler ultrasound and MRI, revealed a multiloculated cyst in the fourth liver segment with communication to the portal vein, leading to thrombosis and cavernous transformation of the portal vein. Despite the rupture of the hydatid cyst into the portal vein, no anaphylactic shock occurred. Portal vein thrombosis and cavernous transformation due to hydatid cysts represent an exceptional and complex complication of echinococcosis, highlighting the importance of early recognition and appropriate management. Treatment strategies should address both the hydatid liver cyst and associated portal hypertension, with surgical intervention being the cornerstone, including hepatectomies and laparoscopic approaches. This case highlights the necessity of heightened clinical awareness in endemic regions for managing this rare, potentially life-threatening complication.