<b><i>Introduction:</i></b> Sessile serrated lesions (SSLs) have malignant potential for colorectal cancer in the serrated pathway. Selective endoscopic resection of SSLs would reduce medical costs and procedure-related accidents, but the accurate endoscopic differentiation of SSLs from hyperplastic polyps (HPs) is challenging. To explore the differential diagnostic performance of magnifying colonoscopy in distinguishing SSLs from HPs, we conducted a multicenter prospective validation study in clinical practice. <b><i>Methods:</i></b> Considering the rarity of diminutive SSLs, all lesions ≥6 mm that were detected during colonoscopy and diagnosed as type 1 based on the Japan narrow-band imaging expert team (JNET) classification were included in this study. Twenty expert endoscopists were asked to differentiate between SSLs and HPs with high or low confidence level after conventional and magnifying NBI observation. To examine the validity of selective endoscopic resection of SSLs using magnifying colonoscopy in clinical practice, we calculated the sensitivity of endoscopic diagnosis of SSLs with histopathological findings as comparable reference. <b><i>Results:</i></b> A total of 217 JNET type 1 lesions from 162 patients were analyzed, and 114 lesions were diagnosed with high confidence. The sensitivity of magnifying colonoscopy in detecting SSLs was 79.8% (95% confidence interval [CI]: 74.7–84.4%) overall, and 82.4% (95% CI: 76.1–87.7%) in the high-confidence group. These results showed that the sensitivity of this study was not high enough, even limited in the high-confidence group. <b><i>Conclusions:</i></b> Accurate differential diagnosis of SSLs and HPs using magnifying colonoscopy was challenging even for experts. JNET type 1 lesions ≥6 mm are recommended to be resected because selective endoscopic resection has a disadvantage of leaving approximately 20% of SSLs on site.
Juvenile polyposis syndrome (JPS) is a rare disease characterized by multiple hamartomatous polyps within the gastrointestinal tract. SMAD4 or BMPR1A is known as a causative gene of JPS. Approximately 75% of newly diagnosed cases have an autosomal-dominantly inherited condition, whereas 25% are sporadic without previous history of polyposis in the family pedigree. Some patients with JPS develop gastrointestinal lesions in childhood and require continuous medical care until adulthood. JPS is classified into three categories according to phenotypic features of polyp distributions, including generalized juvenile polyposis, juvenile polyposis coli, and juvenile polyposis of the stomach. Juvenile polyposis of the stomach is caused by germline pathogenic variants of SMAD4 with a high risk leading to gastric cancer. Pathogenic variants of SMAD4 are also associated with hereditary hemorrhagic telangiectasia-JPS complex, inducing regular cardiovascular survey. Despite growing concerns regarding the managing JPS in Japan, there are no practical guidelines. To address this situation, the guideline committee was organized by the Research Group on Rare and Intractable Diseases granted by the Ministry of Health, Labor and Welfare involving specialists from multiple academic societies. The present clinical guidelines explain the principles in the diagnosis and management of JPS with three clinical questions and corresponding recommendations based on a careful review of the evidence and involve incorporating the concept of the Grading of Recommendations, Assessment, Development, and Evaluation system. Herein, we present the clinical practice guidelines of JPS to promote seamless implementation of accurate diagnosis and appropriate management of pediatric, adolescent, and adult patients with JPS.
BackgroundThe standard therapy for acute severe ulcerative colitis (ASUC) is intravenous corticosteroids; however, 30% of UC patients do not recover with corticosteroids alone. Infliximab or cyclosporin is indicated to decrease the rates of colectomy of hospitalized ASUC patients. Although a previous study showed that moderate to severe UC had a rapid response to tofacitinib, few studies have reported the efficacy and safety of tofacitinib for ASUC.We report a case series of successful first-line treatment consisting of high-dose tofacitinib (20 mg/day) administered to ASUC patients with steroid resistance. Case presentationPatients diagnosed with ASUC at our institution between October 2018 and February 2020 were retrospectively evaluated. They were administered high dose of tofacitinib (20 mg) after they had no response to steroid therapy comprising a dose of 1 to 1.5 mg/kg/day. This study included a total of eight patients with ASUC. There were four (50%) men and four (50%) women with a median age of 47.1 years (range, 19–65 years). All patients had a median UC duration of 4 years (range, 0–20 years). Four patients had no previous history of UC.A clinical response was observed in six of the eight patients before they experienced remission. Six patients were able to avoid colectomy. One patient (patient 2) had no response; however, remission was achieved after switching from tofacitinib to infliximab. One patient (patient 6) with no response to tofacitinib underwent total colectomy. When we used tofacitinib during induction and the follow-up phase, only one patient (patient 4) experienced a major adverse event, herpes zoster, which was treated with acyclovir without stopping tofacitinib. ConclusionClinical remission can be achieved with high probability and colectomy can be avoided by administering high-dose tofacitinib to steroid-resistant ASUC patients.
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