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Masaaki Usami,Ichiro Takeuchi,Reiko Kyodo,Yuri Hirano,Kosuke Kashiwagi,Hiroki Fujikawa,Hirotaka Shimizu,Toshinao Kawai,Katsuhiro Arai 대한장연구학회 2022 Intestinal Research Vol.20 No.4
Background/Aims: Very early-onset inflammatory bowel disease (VEO-IBD), defined as IBD diagnosed in patients younger than 6 years, is a challenge for pediatric gastroenterologists. Although there have been reports regarding VEO-IBD in Western countries, those in Asia are still lacking. This study aimed to investigate the clinical features of Japanese VEO-IBD patients.Methods: Patients with VEO-IBD diagnosed between 2006 and 2019 were evaluated retrospectively. The disease phenotypes were classified into ulcerative colitis type (UC-type) and Crohn’s disease type (CD-type), and the clinical features and courses were compared between the phenotypes.Results: Overall, 54 VEO-IBD patients (19 patients with UC-type and 35 patients with CD-type) were evaluated. The median age at onset was 18 months. One patient had severe combined immunodeficiency (SCID), and 9 patients had monogenic IBD. Monogenic IBD was more prevalent in the CD-type patients with perianal disease (CD-type (PD)). The age at onset was significantly lower in the CD-type group (P<0.05). The most common initial symptom was bloody stools (70%), followed by diarrhea (63%), weight loss (24%), fever (20%), and perianal disease (20%). Excluding patients with SCID and monogenic IBD, 23 out of 44 patients (52%) required biologics. The biologics were switched in 11 out of 44 patients (25%), and the majority of these patients (82%) were in the CD-type group. Overall, 9 patients (20%) required intestinal resection or ostomy placement.Conclusions: CD-type tends to occur at an earlier age, and monogenic IBD occurs significantly more frequently in CD-type (PD). Disease severity and treatment should be individualized, owing to the disease heterogeneity.
Mechanism of Ischemic Mitral Regurgitation
Yutaka Otsuji,Masaaki Takeuchi,Ryuzo Sakata,Robert A. Levine,Chuwa Tei 한국심초음파학회 2008 Journal of Cardiovascular Imaging (J Cardiovasc Im Vol.16 No.1
Augmented leaflet tethering due to the outward displacement of the PMs with LV remodeling appears to be a basic mechanism for ischemic MR. Further, annular dilatation and LV dysfunction likely contribute to the development of MR in the presence of augmented tethering. PM dysfunction per se does not usually cause ischemic MR and may occasionally attenuate tethering and MR. The concept of PM dyssynchrony is important and further study to separate effects of PM dyssynchrony from other factors are required. Although surgical annuloplasty is often effective in reversing ischemic MR, the frequency of patients with persistent or recurrent ischemic MR after surgical ring annuloplasty even with advanced down sizing suggests the need for approaches to address tethering. Finally, leaflet tethering in patients with ischemic MR can vary within a single patient and between patients, indicating the multiple and individualized approaches may be required to correct ischemic MR in affected patients. Augmented leaflet tethering due to the outward displacement of the PMs with LV remodeling appears to be a basic mechanism for ischemic MR. Further, annular dilatation and LV dysfunction likely contribute to the development of MR in the presence of augmented tethering. PM dysfunction per se does not usually cause ischemic MR and may occasionally attenuate tethering and MR. The concept of PM dyssynchrony is important and further study to separate effects of PM dyssynchrony from other factors are required. Although surgical annuloplasty is often effective in reversing ischemic MR, the frequency of patients with persistent or recurrent ischemic MR after surgical ring annuloplasty even with advanced down sizing suggests the need for approaches to address tethering. Finally, leaflet tethering in patients with ischemic MR can vary within a single patient and between patients, indicating the multiple and individualized approaches may be required to correct ischemic MR in affected patients.
Ito, Natsuki,Takeuchi, Ichiro,Kyodo, Reiko,Hirano, Yuri,Sato, Takuro,Usami, Masaaki,Shimizu, Hirotaka,Shimizu, Toshiaki,Arai, Katsuhiro The Korean Society of Pediatric Gastroenterology 2021 Pediatric gastroenterology, hepatology & nutrition Vol.24 No.4
Purpose: A change in diagnosis from ulcerative colitis (UC) to Crohn's disease (CD) has been reported in pediatric inflammatory bowel disease; however, only a few clinical characteristics and predictors of this diagnostic change have been reported. We aimed to describe the clinical characteristics of patients with UC who underwent a change in diagnosis to CD and identify variables associated with the change. Methods: The medical records of pediatric patients with UC who were followed up at the National Center for Child Health and Development between 2006 and 2019 were retrospectively reviewed. Clinical data on disease phenotype, laboratory parameters, endoscopic findings, and treatment of patients whose diagnosis changed to CD (cCD) were compared to those of patients whose diagnosis remained UC (rUC). Results: Among the 111 patients initially diagnosed with UC, 11 (9.9%) patients were subsequently diagnosed with CD during follow-up. There was no significant difference between the cCD and rUC groups in terms of sex, age at initial diagnosis, and the extent and severity of disease at initial diagnosis. Albumin and hemoglobin levels were significantly lower in the cCD group than in the rUC group. The proportion of patients who required biologics was significantly higher in the cCD group than in the rUC group (p<0.05). Conclusion: Approximately 10% children initially diagnosed with UC were subsequently diagnosed with CD. Hypoalbuminemia and anemia at initial diagnosis and use of biologics could be predictors of this diagnostic change.
Endoscopic Findings and Treatment of Gastric Neoplasms in Familial Adenomatous Polyposis
Sato, Chihiro,Takahashi, Kazuya,Sato, Hiroki,Naruse, Takumi,Nakajima, Nao,Takatsuna, Masafumi,Mizuno, Ken-ichi,Hashimoto, Satoru,Takeuchi, Manabu,Yokoyama, Junji,Kobayashi, Masaaki,Terai, Shuji The Korean Gastric Cancer Association 2022 Journal of gastric cancer Vol.22 No.-
Purpose: Gastric neoplasia is a common manifestation of familial adenomatous polyposis (FAP). This study aimed to elucidate the clinical characteristics, endoscopic features including fundic gland polyposis (FGPsis), and treatment outcomes of gastric neoplasms (GNs) in patients with FAP. Materials and Methods: A total of 35 patients diagnosed with FAP, including nine patients from four pedigrees who underwent esophagogastroduodenoscopy (EGD), were investigated regarding patient characteristics, GN morphology, and treatment outcomes. Results: Twenty-one patients (60.0%) had 38 GNs; 33 (86.8%) and 5 (13.2%) were histologically diagnosed with adenocarcinoma and adenoma, respectively. There were no specific patient characteristics related to GNs.Nodule-type GNs were more prevalent in patients with FGP than without (52.2% vs. 0.0%, P=0.002) in the upper body of the stomach. Conversely, depressed-type GNs were fewer in patients with FGPsis than in those without (13.0% vs. 73.3%, P<0.001). Slightly elevated-type GNs were observed in both groups (34.8% vs. 20.0%, P=0.538). Even within pedigrees, the background gastric mucosa and types of GNs varied. In total, 24 GNs were treated with endoscopic submucosal dissection (ESD) and eight with endoscopic mucosal resection (EMR). EMR was selected for GNs with FGPsis because of the technical difficulty of ESD, resulting in a lower en bloc resection rate (62.5% vs. 100%, P=0.014). Conclusions: Our study indicates the necessity of routine EGD surveillance in patients diagnosed with FAP. Notably, the morphology and location of GNs differed between patients with and without FGPsis. Endoscopic treatment and outcomes require more attention in cases of FGPsis.
Endoscopic Findings and Treatment of Gastric Neoplasms in Familial Adenomatous Polyposis
Sato Chihiro,Takahashi Kazuya,Sato Hiroki,Naruse Takumi,Nakajima Nao,Takatsuna Masafumi,Mizuno Ken-ichi,Hashimoto Satoru,Takeuchi Manabu,Yokoyama Junji,Kobayashi Masaaki,Terai Shuji 대한위암학회 2022 Journal of gastric cancer Vol.22 No.4
Purpose Gastric neoplasia is a common manifestation of familial adenomatous polyposis (FAP). This study aimed to elucidate the clinical characteristics, endoscopic features including fundic gland polyposis (FGPsis), and treatment outcomes of gastric neoplasms (GNs) in patients with FAP. Materials and Methods A total of 35 patients diagnosed with FAP, including nine patients from four pedigrees who underwent esophagogastroduodenoscopy (EGD), were investigated regarding patient characteristics, GN morphology, and treatment outcomes. Results Twenty-one patients (60.0%) had 38 GNs; 33 (86.8%) and 5 (13.2%) were histologically diagnosed with adenocarcinoma and adenoma, respectively. There were no specific patient characteristics related to GNs. Nodule-type GNs were more prevalent in patients with FGP than without (52.2% vs. 0.0%, P=0.002) in the upper body of the stomach. Conversely, depressed-type GNs were fewer in patients with FGPsis than in those without (13.0% vs. 73.3%, P<0.001). Slightly elevated-type GNs were observed in both groups (34.8% vs. 20.0%, P=0.538). Even within pedigrees, the background gastric mucosa and types of GNs varied. In total, 24 GNs were treated with endoscopic submucosal dissection (ESD) and eight with endoscopic mucosal resection (EMR). EMR was selected for GNs with FGPsis because of the technical difficulty of ESD, resulting in a lower en bloc resection rate (62.5% vs. 100%, P=0.014). Conclusions Our study indicates the necessity of routine EGD surveillance in patients diagnosed with FAP. Notably, the morphology and location of GNs differed between patients with and without FGPsis. Endoscopic treatment and outcomes require more attention in cases of FGPsis. Purpose Gastric neoplasia is a common manifestation of familial adenomatous polyposis (FAP). This study aimed to elucidate the clinical characteristics, endoscopic features including fundic gland polyposis (FGPsis), and treatment outcomes of gastric neoplasms (GNs) in patients with FAP. Materials and Methods A total of 35 patients diagnosed with FAP, including nine patients from four pedigrees who underwent esophagogastroduodenoscopy (EGD), were investigated regarding patient characteristics, GN morphology, and treatment outcomes. Results Twenty-one patients (60.0%) had 38 GNs; 33 (86.8%) and 5 (13.2%) were histologically diagnosed with adenocarcinoma and adenoma, respectively. There were no specific patient characteristics related to GNs. Nodule-type GNs were more prevalent in patients with FGP than without (52.2% vs. 0.0%, P=0.002) in the upper body of the stomach. Conversely, depressed-type GNs were fewer in patients with FGPsis than in those without (13.0% vs. 73.3%, P<0.001). Slightly elevated-type GNs were observed in both groups (34.8% vs. 20.0%, P=0.538). Even within pedigrees, the background gastric mucosa and types of GNs varied. In total, 24 GNs were treated with endoscopic submucosal dissection (ESD) and eight with endoscopic mucosal resection (EMR). EMR was selected for GNs with FGPsis because of the technical difficulty of ESD, resulting in a lower en bloc resection rate (62.5% vs. 100%, P=0.014). Conclusions Our study indicates the necessity of routine EGD surveillance in patients diagnosed with FAP. Notably, the morphology and location of GNs differed between patients with and without FGPsis. Endoscopic treatment and outcomes require more attention in cases of FGPsis.