Clinical Journal of Gastroenterology最新文献

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A rare case of a ciliated hepatic foregut cyst with progressive enlargement causing obstructive jaundice. 一个罕见的纤毛肝前肠囊肿进行性扩大引起梗阻性黄疸的病例。
IF 0.8
Clinical Journal of Gastroenterology Pub Date : 2026-10-01 Epub Date: 2026-07-17 DOI: 10.1007/s12328-026-02402-4
Satoshi Watanabe, Takahiro Kubo, Shinsaku Nagamatsu, Takuma Morimoto, Akane Kawasaki, Yuya Matsuo, Shoma Kikukawa, Masakazu Uejima, Kei Moriya
{"title":"A rare case of a ciliated hepatic foregut cyst with progressive enlargement causing obstructive jaundice.","authors":"Satoshi Watanabe, Takahiro Kubo, Shinsaku Nagamatsu, Takuma Morimoto, Akane Kawasaki, Yuya Matsuo, Shoma Kikukawa, Masakazu Uejima, Kei Moriya","doi":"10.1007/s12328-026-02402-4","DOIUrl":"10.1007/s12328-026-02402-4","url":null,"abstract":"<p><p>A 73-year-old woman presented with a 10-day history of anorexia, and laboratory tests revealed obstructive jaundice. Imaging studies showed biliary obstruction caused by a 40‑mm unilocular cystic lesion in hepatic segment 4. The lesion had first been identified 10 years earlier and enlarged over the preceding 5 months. Endoscopic retrograde cholangiopancreatography (ERCP) was performed. Cholangiography revealed a compressive stricture of the bile duct near the hepatic hilum, with complete obstruction of the right intrahepatic bile duct and stenosis of the left intrahepatic bile duct. A biliary stent was placed in the left intrahepatic bile duct. The enlargement of the cystic lesion raised suspicion of malignant involvement. Given the obstruction of the right intrahepatic bile duct, infiltration or adhesion of the cystic lesion to the right intrahepatic bile duct was suspected. An extended right hepatectomy was safely performed, and the lesion was pathologically diagnosed as a ciliated hepatic foregut cyst (CHFC). CHFCs are congenital cystic lesions, that originate from the embryonic foregut. Given the rarity of CHFCs, their natural history and clinical behavior remain poorly understood. CHFCs were traditionally regarded as benign and often managed conservatively. However, recent reports of squamous cell carcinoma arising within CHFCs have raised concerns, and surgical resection is recommended when malignant transformation is suspected. This is the first reported case of a CHFC that remained stable for many years, then rapidly enlarged and caused obstructive jaundice. When CHFCs are identified, clinicians should consider the risks of potential rapid enlargement even without malignant transformation.</p>","PeriodicalId":10364,"journal":{"name":"Clinical Journal of Gastroenterology","volume":" ","pages":"1250-1256"},"PeriodicalIF":0.8,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148469259","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
En bloc resection of intestinal-type adenoma extending to the minor duodenal papilla by underwater endoscopic mucosal resection with a forward-viewing endoscope. 前视内镜下水下粘膜切除术治疗延伸至十二指肠小乳头的肠型腺瘤。
IF 0.8
Clinical Journal of Gastroenterology Pub Date : 2026-10-01 Epub Date: 2026-07-24 DOI: 10.1007/s12328-026-02401-5
Risa Nakamura, Yoshitaka Nawata, Tomoaki Naka, Kimihiro Igarashi, Toru Okuzono, Mareyuki Endo, Tomoki Matsuda
{"title":"En bloc resection of intestinal-type adenoma extending to the minor duodenal papilla by underwater endoscopic mucosal resection with a forward-viewing endoscope.","authors":"Risa Nakamura, Yoshitaka Nawata, Tomoaki Naka, Kimihiro Igarashi, Toru Okuzono, Mareyuki Endo, Tomoki Matsuda","doi":"10.1007/s12328-026-02401-5","DOIUrl":"10.1007/s12328-026-02401-5","url":null,"abstract":"<p><p>We report successful en bloc underwater endoscopic mucosal resection (UEMR) of a lesion involving the minor duodenal papilla. A woman in her 60s presented with a 10-mm depressed lesion in the descending duodenum. White-light imaging and Magnifying endoscopy suggested a tumor involving a minor papilla. Although side-viewing endoscopes are conventionally used for the minor papilla, pre-procedural assessment indicated inadequate maneuverability for this specific lesion (ED-580T; Fujifilm Corporation, Tokyo, Japan). Consequently, we strategically used a forward-viewing dual-channel endoscope (GIF-2TQ260M; Olympus Medical Systems, Tokyo, Japan). Utilizing its right-side instrument channel and independent suction capability provided a direct approach and stable field of view, facilitating precise snaring. Histopathology confirmed a 15 × 13-mm intestinal-type adenoma with negative margins. Given the minor papilla was located at the tumor margin and lacked a luminal orifice, the lesion was diagnosed as a superficial non-ampullary duodenal epithelial tumor (SNADET) secondarily extending to the papilla. Forward-viewing endoscopes are viable alternatives for this region depending on lesion orientation. Furthermore, comprehensive pre-procedural assessment, including evaluation for pancreas divisum, may aid in determining treatment strategies, such as the omission of pancreatic stenting. Strategic, case-specific endoscope selection is paramount for safe therapeutic intervention in this complex anatomical region.</p>","PeriodicalId":10364,"journal":{"name":"Clinical Journal of Gastroenterology","volume":" ","pages":"1099-1106"},"PeriodicalIF":0.8,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148577260","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Video-assisted retroperitoneal debridement for infected retroperitoneal fat necrosis following laparoscopic distal pancreatectomy. 视频辅助腹膜后清创治疗腹腔镜远端胰腺切除术后感染腹膜后脂肪坏死。
IF 0.8
Clinical Journal of Gastroenterology Pub Date : 2026-10-01 Epub Date: 2026-08-01 DOI: 10.1007/s12328-026-02414-0
Katsuya Ami, Keiko Kamei, Masaya Nakano, Chihoko Nobori, Yuta Yoshida, Kentaro Tai, Takaaki Murase, Atsushi Takebe, Ippei Matsumoto
{"title":"Video-assisted retroperitoneal debridement for infected retroperitoneal fat necrosis following laparoscopic distal pancreatectomy.","authors":"Katsuya Ami, Keiko Kamei, Masaya Nakano, Chihoko Nobori, Yuta Yoshida, Kentaro Tai, Takaaki Murase, Atsushi Takebe, Ippei Matsumoto","doi":"10.1007/s12328-026-02414-0","DOIUrl":"10.1007/s12328-026-02414-0","url":null,"abstract":"<p><p>Video-assisted retroperitoneal debridement (VARD) is an established and minimally invasive treatment option for infected necrotizing pancreatitis. However, its application in the postoperative setting after pancreatic resection has rarely been described. We report a case of extensive infected retroperitoneal fat necrosis following laparoscopic distal pancreatectomy that was successfully managed using a step-up approach incorporating VARD. An 81-year-old woman underwent laparoscopic distal pancreatectomy for pancreatic ductal adenocarcinoma of the pancreatic tail. On postoperative day (POD) 7, she developed sudden-onset abdominal pain. Computed tomography (CT) demonstrated enlargement of the remnant pancreas with increased peripancreatic fat stranding. Follow-up CT on POD 9 and 12 revealed progressive enlargement of the fluid collection around the pancreatic stump with rapid extension of inflammatory changes into the retroperitoneal space. Conservative management with fasting and antibiotic therapy resulted in temporary improvement in inflammatory markers. However, extensive infected retroperitoneal fat necrosis developed on POD 38. On POD 43, CT-guided percutaneous catheter drainage failed to achieve adequate source control due to highly viscous necrotic debris, necessitating surgical necrosectomy. An additional drainage tube was placed to establish a retroperitoneal access route, after which VARD was performed on POD 55, with removal of 61 g of necrotic fatty tissue. Inflammatory markers rapidly improved, and the patient was discharged in good condition on POD 102. Infected retroperitoneal fat necrosis after laparoscopic distal pancreatectomy was successfully managed using a step-up strategy incorporating VARD. This case highlights VARD as a minimally invasive therapeutic option for postoperative infected retroperitoneal fat necrosis.</p>","PeriodicalId":10364,"journal":{"name":"Clinical Journal of Gastroenterology","volume":" ","pages":"1292-1299"},"PeriodicalIF":0.8,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13645863/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148653202","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Rapid intracystic growth of spindle cell-type anaplastic carcinoma derived from intraductal papillary mucinous carcinoma. 导管内乳头状粘液癌衍生的梭形细胞型间变性癌囊内快速生长。
IF 0.8
Clinical Journal of Gastroenterology Pub Date : 2026-09-29 DOI: 10.1007/s12328-026-02457-3
Reiko Yamada, Ayako Okuda, Shoma Sawai, Minako Urata, Yasuaki Shimada, Kenji Nose, Toji Murbayashi, Hayato Nakagawa
{"title":"Rapid intracystic growth of spindle cell-type anaplastic carcinoma derived from intraductal papillary mucinous carcinoma.","authors":"Reiko Yamada, Ayako Okuda, Shoma Sawai, Minako Urata, Yasuaki Shimada, Kenji Nose, Toji Murbayashi, Hayato Nakagawa","doi":"10.1007/s12328-026-02457-3","DOIUrl":"https://doi.org/10.1007/s12328-026-02457-3","url":null,"abstract":"<p><p>Anaplastic carcinoma of the pancreas (ACP) is a rare and aggressive malignancy. We report a case of spindle cell-type ACP arising from intraductal papillary mucinous carcinoma (IPMC) showing rapid intra-cystic growth. A 73 year-old man was incidentally found to have a pancreatic cyst, initially diagnosed as a simple cyst. After 18 months, a solid component appeared within the cyst and rapidly enlarged within one month. Distal pancreatectomy was performed. Histopathology revealed spindle cell-type anaplastic carcinoma arising from invasive IPMC. Despite aggressive histology, curative resection was achieved. The patient remains recurrence-free for 50 months. Rapid changes in pancreatic cysts warrant careful evaluation and timely surgical intervention.</p>","PeriodicalId":10364,"journal":{"name":"Clinical Journal of Gastroenterology","volume":" ","pages":""},"PeriodicalIF":0.8,"publicationDate":"2026-09-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149079982","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Delayed and multisystem immune-related adverse events in MSI-high colon cancer treated with nivolumab plus ipilimumab. 纳武单抗联合伊匹单抗治疗msi高结肠癌的延迟和多系统免疫相关不良事件
IF 0.8
Clinical Journal of Gastroenterology Pub Date : 2026-09-28 DOI: 10.1007/s12328-026-02460-8
Takato Maeda, Masaki Munakata, Hiroshi Numao, Norihiro Hanabata, Koji Shimaya, Shohei Igarashi, Masayoshi Ko, Yukari Fukutoku, Ryoichi Kikuchi, Hirotake Sakuraba
{"title":"Delayed and multisystem immune-related adverse events in MSI-high colon cancer treated with nivolumab plus ipilimumab.","authors":"Takato Maeda, Masaki Munakata, Hiroshi Numao, Norihiro Hanabata, Koji Shimaya, Shohei Igarashi, Masayoshi Ko, Yukari Fukutoku, Ryoichi Kikuchi, Hirotake Sakuraba","doi":"10.1007/s12328-026-02460-8","DOIUrl":"https://doi.org/10.1007/s12328-026-02460-8","url":null,"abstract":"<p><p>Immune checkpoint inhibitors (ICIs) can cause immune-related adverse events (irAEs) involving multiple organs. However, delayed-onset, multisystem irAEs after treatment discontinuation are uncommon. Here, we present a rare case of a man in his 50s with microsatellite instability-high colon cancer who developed multisystem irAEs following treatment with nivolumab plus ipilimumab. The patient developed adrenocorticotropic hormone deficiency after four cycles of combination therapy, which improved with hydrocortisone supplementation. Following eight cycles of nivolumab monotherapy, treatment was discontinued because no cancerous lesions were detected. Six months after the final nivolumab administration, the patient presented with bilateral knee arthritis and elevated hepatobiliary and pancreatic enzyme levels. No evidence of infectious, autoimmune, or IgG4-related disease was found. Liver biopsy revealed portal lymphocytic infiltration with a predominance of CD8-positive cells. The patient was diagnosed with delayed ICI-related arthritis, accompanied by asymptomatic cholangitis and pancreatitis. These irAEs improved rapidly after prednisolone administration. This case demonstrates that irAEs can occur in a delayed and multisystem manner, even after the cessation of ICIs. Thus, clinicians should consider irAEs in the differential diagnosis when new symptoms or unexplained laboratory abnormalities occur in patients with prior ICI exposure.</p>","PeriodicalId":10364,"journal":{"name":"Clinical Journal of Gastroenterology","volume":" ","pages":""},"PeriodicalIF":0.8,"publicationDate":"2026-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149073655","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Response to pembrolizumab after progression on durvalumab in cholangiocarcinoma: a case report of immune checkpoint inhibitor switching. 胆管癌杜伐单抗进展后对派姆单抗的反应:免疫检查点抑制剂切换的病例报告
IF 0.8
Clinical Journal of Gastroenterology Pub Date : 2026-09-26 DOI: 10.1007/s12328-026-02458-2
Akito Furuta, Masatoshi Murakami, Katsuhito Teramatsu, Satoru Kinjo, Kazuhide Matsumoto, Keijiro Ueda, Takeo Yamamoto, Yoshinao Oda, Nao Fujimori, Yoshihiro Ogawa
{"title":"Response to pembrolizumab after progression on durvalumab in cholangiocarcinoma: a case report of immune checkpoint inhibitor switching.","authors":"Akito Furuta, Masatoshi Murakami, Katsuhito Teramatsu, Satoru Kinjo, Kazuhide Matsumoto, Keijiro Ueda, Takeo Yamamoto, Yoshinao Oda, Nao Fujimori, Yoshihiro Ogawa","doi":"10.1007/s12328-026-02458-2","DOIUrl":"https://doi.org/10.1007/s12328-026-02458-2","url":null,"abstract":"<p><p>Currently, combination therapy incorporating immune checkpoint inhibitors (ICIs) is the standard first-line treatment for advanced biliary tract cancer (BTC). However, the relative efficacy of anti-PD-1 and -L1 antibodies remains unclear, and evidence regarding ICI switching after treatment failure is limited. Here, we report a case of advanced distal cholangiocarcinoma in which pembrolizumab demonstrated clinical efficacy after durvalumab failure, with partial response maintained for six months. Pembrolizumab treatment was discontinued because of interstitial pneumonitis requiring corticosteroid therapy. Immunohistochemical analysis demonstrated staining for PD-L1 and -L2 in the tumor cells. Anti-PD-1 antibodies inhibit interactions between PD-L1 and -L2, whereas anti-PD-L1 antibodies do not affect PD-L2. Although the mechanisms underlying the response remain unclear, blockade of the PD-L2-PD-1 axis may have contributed to the clinical response to pembrolizumab after durvalumab failure. This case suggests that switching from anti-PD-L1 to anti-PD-1 therapy may represent an individualized therapeutic option in selected patients with BTC.</p>","PeriodicalId":10364,"journal":{"name":"Clinical Journal of Gastroenterology","volume":" ","pages":""},"PeriodicalIF":0.8,"publicationDate":"2026-09-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149058861","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Reply to the letter to the Editor on "Intraventricular mechanical factors: subtle triggers of takotsubo syndrome following gastric endoscopic interventions". 回复关于“脑室内机械因素:胃内窥镜干预后takotsubo综合征的微妙触发因素”的致编辑的信。
IF 0.8
Clinical Journal of Gastroenterology Pub Date : 2026-09-26 DOI: 10.1007/s12328-026-02454-6
Yoshika Saito, Hiroaki Saito, Masaki Nakashima
{"title":"Reply to the letter to the Editor on \"Intraventricular mechanical factors: subtle triggers of takotsubo syndrome following gastric endoscopic interventions\".","authors":"Yoshika Saito, Hiroaki Saito, Masaki Nakashima","doi":"10.1007/s12328-026-02454-6","DOIUrl":"https://doi.org/10.1007/s12328-026-02454-6","url":null,"abstract":"","PeriodicalId":10364,"journal":{"name":"Clinical Journal of Gastroenterology","volume":" ","pages":""},"PeriodicalIF":0.8,"publicationDate":"2026-09-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149058789","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Recent advances in managing esophageal epiphrenic diverticula: a review of the impact of endoscopy, high-resolution manometry, and POEM. 食管表观憩室治疗的最新进展:内镜检查、高分辨率测压和POEM的影响综述。
IF 0.8
Clinical Journal of Gastroenterology Pub Date : 2026-09-24 DOI: 10.1007/s12328-026-02459-1
Daniel L Cohen, Sergei Vosko, Haim Shirin, Anton Bermont, Vered Richter
{"title":"Recent advances in managing esophageal epiphrenic diverticula: a review of the impact of endoscopy, high-resolution manometry, and POEM.","authors":"Daniel L Cohen, Sergei Vosko, Haim Shirin, Anton Bermont, Vered Richter","doi":"10.1007/s12328-026-02459-1","DOIUrl":"https://doi.org/10.1007/s12328-026-02459-1","url":null,"abstract":"<p><p>Epiphrenic diverticula (ED) are pulsion diverticula found in the distal esophagus. They are rare and often associated with esophageal motility disorders. Given technological advances over the past number of years that have impacted the care of patients with ED, it is worthwhile reviewing the recent literature on this entity. As endoscopy is now commonly practiced, there is evidence that ED is mainly being diagnosed endoscopically and that more asymptomatic patients may be diagnosed. The age of patients with ED may also be increasing given increases in life expectancies. Recent studies using high-resolution manometry (HRM), the gold standard for diagnosing motility disorders, have shown that ~ 50-60% of ED patients are diagnosed with a motility disorder according to the Chicago Classification. Studies have also confirmed that the presence of ED is a risk factor for an unsuccessful HRM exam. Finally, with the advent of peroral endoscopic myotomy (POEM), a successful endoscopic treatment is available. While there are several variations to the procedure, such as whether it is performed with septotomy or without, and whether the myotomy is extended through the lower esophageal sphincter and into the stomach or not, POEM has changed the therapeutic algorithm of ED. There is evidence that it has excellent technical and clinical success rates equivalent to that of surgery, but with lower adverse events compared to surgical procedures. In conclusion, recent technological developments have provided a better understanding of ED and offer patients a highly successful, minimally-invasive endoscopic treatment.</p>","PeriodicalId":10364,"journal":{"name":"Clinical Journal of Gastroenterology","volume":" ","pages":""},"PeriodicalIF":0.8,"publicationDate":"2026-09-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149036769","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Staged radiotherapy for symptomatic unresectable giant hepatic hemangiomas with diffuse bilobar involvement. 分期放疗治疗伴有弥漫性双叶累及的症状性不可切除的巨大肝血管瘤。
IF 0.8
Clinical Journal of Gastroenterology Pub Date : 2026-09-24 DOI: 10.1007/s12328-026-02456-4
Kenta Ohmatsu, Sakura Nishiwaki, Mirei Kamori, Yuka Kaizu, Shiho Wada, Miki Tsujii, Sawa Kono, Shunichi Ariizumi, Shuji Sakai, Yaichiro Hashimoto
{"title":"Staged radiotherapy for symptomatic unresectable giant hepatic hemangiomas with diffuse bilobar involvement.","authors":"Kenta Ohmatsu, Sakura Nishiwaki, Mirei Kamori, Yuka Kaizu, Shiho Wada, Miki Tsujii, Sawa Kono, Shunichi Ariizumi, Shuji Sakai, Yaichiro Hashimoto","doi":"10.1007/s12328-026-02456-4","DOIUrl":"https://doi.org/10.1007/s12328-026-02456-4","url":null,"abstract":"<p><p>Although most hepatic hemangiomas require no treatment, giant lesions causing progressive symptoms, consumptive coagulopathy, or compression of adjacent organs may require intervention. Surgery remains the standard treatment but may not be feasible in patients with extensive bilobar disease or excessive operative risk. A 44-year-old woman presented with progressive abdominal fullness caused by multiple giant hepatic hemangiomas involving both hepatic lobes, the largest of which measured approximately 30 cm. Five years earlier, central bisectionectomy had been complicated by massive intraoperative bleeding, and the residual lesions subsequently enlarged. Because further surgery was considered high risk, staged three-dimensional conformal radiotherapy was performed to avoid simultaneous irradiation of the entire liver. The left-lobe lesion received 30 Gy in 15 fractions, followed approximately 2 years later by the same regimen for the enlarging right-lobe lesion. Both treatments resulted in marked tumor regression and symptomatic improvement. Fibrin degradation products and D-dimer levels decreased, fibrinogen levels normalized, and thrombocytopenia partially improved in association with the radiographic response. No grade 2 or higher treatment-related adverse events occurred. These findings suggest that staged bilateral radiotherapy may represent a feasible strategy for selected patients with symptomatic, unresectable giant hepatic hemangiomas and diffuse bilobar involvement for whom surgery is not feasible.</p>","PeriodicalId":10364,"journal":{"name":"Clinical Journal of Gastroenterology","volume":" ","pages":""},"PeriodicalIF":0.8,"publicationDate":"2026-09-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149036818","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Anti-TNF-refractory inflammatory bowel disease associated with a novel XIAP frameshift variant successfully treated with ustekinumab. ustekinumab成功治疗与新型XIAP移码变异相关的抗tnf难治性炎症性肠病。
IF 0.8
Clinical Journal of Gastroenterology Pub Date : 2026-09-22 DOI: 10.1007/s12328-026-02394-1
Natsuki Ito, Takahiro Kudo, Hidetaka Eguchi, Keisuke Jimbo, Yasushi Okazaki, Toshiaki Shimizu, Hiromichi Shoji
{"title":"Anti-TNF-refractory inflammatory bowel disease associated with a novel XIAP frameshift variant successfully treated with ustekinumab.","authors":"Natsuki Ito, Takahiro Kudo, Hidetaka Eguchi, Keisuke Jimbo, Yasushi Okazaki, Toshiaki Shimizu, Hiromichi Shoji","doi":"10.1007/s12328-026-02394-1","DOIUrl":"https://doi.org/10.1007/s12328-026-02394-1","url":null,"abstract":"<p><p>X-linked inhibitor of apoptosis protein (XIAP) deficiency is a monogenic immunodeficiency disorder with a broad clinical spectrum that includes inflammatory bowel disease (IBD). The inflammatory mechanisms underlying XIAP-associated IBD remain incompletely understood, and resistance to anti-tumor necrosis factor (TNF) therapy represents a significant therapeutic challenge. We report a case of a pediatric patient with XIAP-associated IBD caused by a novel pathogenic frameshift variant in the XIAP gene (c.498dupA, p.Leu167Ilefs*30). The clinical course was characterized by refractoriness to anti-TNF therapy, suggesting a non-TNF-dominant inflammatory phenotype. Although hematopoietic stem cell transplantation represents the only curative option, interleukin-12/23 blockade using ustekinumab (UST) was considered following an inadequate response to standard medical therapies. Subsequent treatment with UST was associated with sustained clinical and endoscopic improvement, supporting the involvement of an IL-12/23-related inflammatory axis in this patient. This case illustrates a distinct phenotype of XIAP-associated IBD, characterized by resistance to TNF inhibition and susceptibility to IL-12/23 blockade using UST, associated with a novel pathogenic XIAP variant. Rather than demonstrating treatment efficacy alone, these findings underscore the value of integrating genetic diagnosis with treatment response to gain insight into disease-driving inflammatory pathways and to guide individualized management strategies in monogenic IBD.</p>","PeriodicalId":10364,"journal":{"name":"Clinical Journal of Gastroenterology","volume":" ","pages":""},"PeriodicalIF":0.8,"publicationDate":"2026-09-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149024275","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
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