An Unusual Abscess Associated with Gallbladder Perforation Successfully Treated with Percutaneous Transhepatic Gallbladder Drainage and Endoscopic Ultrasound-guided Abscess Drainage.

Abscesses associated with gallbladder perforation are often confined to the peri-gallbladder region. We herein report a rare case of gallbladder perforation in which the abscess cavity extended into the left upper quadrant. A 79-year-old woman developed gallbladder perforation secondary to acalculous cholecystitis. Computed tomography revealed fluid collection extending from the peri-gallbladder to the dorsal left hepatic lobe in contact with the stomach. We successfully treated percutaneous transhepatic gallbladder drainage and simultaneous endoscopic ultrasound-guided transgastric internal and external abscess drainage. This minimally invasive approach is considered safe and feasible for managing such a rare case.

IgG4-related hepatic inflammatory pseudotumor in a patient with serum IgG4-negative type 1 autoimmune pancreatitis.

IgG4-related disease (IgG4-RD) can cause heterogeneous lesion in various organs. Serum IgG4 levels are useful in monitoring patients with IgG4-RD; however, when it is negative, more careful observation is required. A 58-year-old woman who had been diagnosed with serum IgG4-negative type 1 autoimmune pancreatitis (AIP) 3 years prior visited our hospital for the evaluation of a liver tumor. She had visited a nearby hospital 1 month prior with complaints of a swelling in her right neck, and histological examinations were suggestive of IgG4-related sialadenitis. A positron emission tomography scan showed fluoro-deoxy-glucose accumulation in her right liver lobe; therefore, she was referred to our hospital. Liver tumor biopsy showed inflammatory cell infiltration and storiform fibrosis, without histological findings indicative of a malignancy. Many IgG4-positive cells were detected in immunostaining; thus, an IgG4-related hepatic inflammatory pseudo-tumor was diagnosed. After increasing in steroid dosage, the patient remained recurrence-free with 2 years. To our knowledge, this is the first report of mass-forming IPT for serum IgG4-negative type 1 AIP. Occasionally, IgG4-related IPT may appear in the periphery of the liver, and serum IgG4-negative cases should be more carefully observed because serum IgG4 is not an indicator.

A Case of Successful Endoscopic Extraction of Common Bile Duct Stones After Closing a Duodenal Perforation Using Over-The-Scope Clip.

A 57-year-old man visited our hospital for acute cholangitis due to common bile duct (CBD) stones in March 2021. Biliary stenting was performed without any complications. The cholangitis improved rapidly. He was re-hospitalized to treat the CBD stones in May 2021. Although endoscopic retrograde cholangiopancreatography was performed, endoscopy caused a perforation of the duodenal bulb. We successfully performed endoscopic closure of the duodenal defect using an over-the-scope clip (OTSC®). Considering that mild CBD dilatation of 10 mm can carry an increased risk of stenosis after surgery, we decided to avoid surgery and perform a follow-up endoscopic treatment. He was re-hospitalized in July 2021. The endoscopy revealed OTSC® in the anterior wall of the duodenal bulb and complete healing of the perforation. We carefully advanced the scope to the second portion of the duodenum while avoiding OTSC®, and the ampulla of Vater was identified. We were then able to remove the stones without any complications. OTSC® was effective in closing a duodenal perforation and enabled us to carry out the retreatment safely and successfully.

Intestinal Behçet's disease complicated by myelodysplastic syndrome and secondary pulmonary alveolar proteinosis: a case report.

BACKGROUND: Gastrointestinal lesions, which sometimes develop in Behçet's disease (BD), are referred to as intestinal BD. Although rare, intestinal BD can be accompanied by myelodysplastic syndrome (MDS) with abnormal karyotype trisomy 8, which is refractory to immunosuppressive therapy. Pulmonary alveolar proteinosis is a rare lung complication of BD and MDS. Herein, we present an extremely rare case of intestinal BD presenting with MDS and several chromosomal abnormalities, followed by secondary pulmonary proteinosis. CASE PRESENTATION: A 58-year-old Japanese woman with a 3-year history of genital ulcers and oral aphthae was admitted to our hospital. The patient developed abdominal pain and persistent diarrhea. Colonoscopy revealed multiple, round, punched-out ulcers from the terminal ileum to the descending colon. Intestinal BD was diagnosed and the patient was treated with colchicine, prednisolone, and adalimumab. However, her symptoms were unstable. Bone marrow examination to investigate the persistent macrocytic anemia revealed the presence of trisomy 8, trisomy 9, and X chromosome abnormalities (48, + 8, + 9, X, i(X) (q10) in 12 out of the examined 20 cells). Based on her hypoplastic bone marrow, the patient was diagnosed with low-risk MDS (refractory anemia). At the age of 61, the patient developed pneumonia with fever and diffuse ground-glass opacities on the lung computed tomography (CT). Chest high-resolution CT and histopathology via transbronchial lung biopsy revealed the presence of pulmonary alveolar proteinosis (PAP). These findings combined with the underlying disease led to the diagnosis of secondary PAP. CONCLUSIONS: Secondary pulmonary proteinosis may accompany intestinal BD with MDS and several chromosomal abnormalities. Physicians should pay attention to lung complications, such as PAP, in patients with intestinal BD complicated by MDS. Genetic abnormalities may be associated with the development of such diseases.

(+)-Sesamin-oxidising CYP92B14 shapes specialised lignan metabolism in sesame.

Sesamum spp. (sesame) are known to accumulate a variety of lignans in a lineage-specific manner. In cultivated sesame (Sesamum indicum), (+)-sesamin, (+)-sesamolin and (+)-sesaminol triglucoside are the three major lignans found richly in the seeds. A recent study demonstrated that SiCYP92B14 is a pivotal enzyme that allocates the substrate (+)-sesamin to two products, (+)-sesamolin and (+)-sesaminol, through multiple reaction schemes including oxidative rearrangement of α-oxy-substituted aryl groups (ORA). In contrast, it remains unclear whether (+)-sesamin in wild sesame undergoes oxidation reactions as in S. indicum and how, if at all, the ratio of the co-products is tailored at the molecular level. Here, we functionally characterised SrCYP92B14 as a SiCYP92B14 orthologue from a wild sesame, Sesamum radiatum, in which we revealed accumulation of the (+)-sesaminol derivatives (+)-sesangolin and its novel structural isomer (+)-7´-episesantalin. Intriguingly, SrCYP92B14 predominantly produced (+)-sesaminol either through ORA or direct oxidation on the aromatic ring, while a relatively low but detectable level of (+)-sesamolin was produced. Amino acid substitution analysis suggested that residues in the putative distal helix and the neighbouring heme propionate of CYP92B14 affect the ratios of its co-products. These data collectively show that the bimodal oxidation mechanism of (+)-sesamin might be widespread across Sesamum spp., and that CYP92B14 is likely to be a key enzyme in shaping the ratio of (+)-sesaminol- and (+)-sesamolin-derived lignans from the biochemical and evolutionary perspectives.