A5030515052- Inflammatory Bowel Disease
- Gastrointestinal disorders and treatments
- Microscopic Colitis
- Immunodeficiency and Autoimmune Disorders
- Esophageal and GI Pathology
- Gastrointestinal Bleeding Diagnosis and Treatment
- Helicobacter pylori-related gastroenterology studies
- Eosinophilic Esophagitis
- Autoimmune Bullous Skin Diseases
- Hypertrophic osteoarthropathy and related conditions
- Vascular Malformations and Hemangiomas
- Diverticular Disease and Complications
- Immune Response and Inflammation
- PI3K/AKT/mTOR signaling in cancer
- Gastrointestinal motility and disorders
- Genetic factors in colorectal cancer
- Liver Disease and Transplantation
- Tumors and Oncological Cases
- Congenital gastrointestinal and neural anomalies
- Multiple and Secondary Primary Cancers
- Inflammasome and immune disorders
- Cholangiocarcinoma and Gallbladder Cancer Studies
- Plant Virus Research Studies
- Dermatological and Skeletal Disorders
- Intestinal Malrotation and Obstruction Disorders
Miyagi Children's Hospital
2014-2024
Sendai Red Cross Hospital
2005
Chronic enteropathy associated with SLCO2A1 gene (CEAS) is a hereditary disease caused by mutations in the and characterized multiple small intestinal ulcers of nonspecific histology. also causal primary hypertrophic osteoarthropathy (PHO). However, little known about clinical features CEAS or PHO.Sixty-five Japanese patients recruited nationwide survey during 2012-2016 were enrolled this present study. We reviewed information genetically confirmed patients.We identified recessive at 11...
Inborn errors of the NF-κB pathways underlie various clinical phenotypes in humans. Heterozygous germline loss-of-expression and loss-of-function mutations RELA haploinsufficiency, which results TNF-dependent chronic mucocutaneous ulceration autoimmune hematological disorders. We here report six patients from five families with additional autoinflammatory manifestations. These are heterozygous for mutations, all 3' segment gene create a premature stop codon. Truncated RelA proteins expressed...
There are few published registry studies from Asia on pediatric inflammatory bowel disease (IBD). Registry network data enable comparisons among ethnic groups. This study examined the characteristics of IBD in Japanese children and compared them with those European children.This was a cross-sectional multicenter newly diagnosed patients. The Paris classification used to categorize features, results were EUROKIDS data.A total 265 patients initially registered, 22 later excluded for having...
To evaluate the role of colonoscopy in infants and young children clarify distribution colonoscopy-requiring diseases this age group. Cohorts colonoscopies performed at three children's hospitals Japan between April 2011 March 2016 including younger than six years were retrospectively reviewed. In total, 453 276 children. Of these 275 (60.8%) for diagnostic purposes, 177 (39.2%) as follow-up, one case was treatment. The median patient time 2.49 years, there a male-to-female ratio 1.72:1....
ABSTRACT We performed a Japanese nationwide survey of pediatric‐onset chronic nonspecific multiple ulcers the small intestine between January 2000 and July 2013 in 176 institutions pediatric surgery or gastroenterology clarified clinical features associated with genetic abnormalities Solute Carrier Organic Anion Transporter Family, Member 2A1 ( SLCO2A1 ) gene. A total 4 cases (3 girls 1 boy) were diagnosed this series, which had to be differentiated from Crohn disease, Behçet tuberculosis,...
ABSTRACT Background and Aim Peutz–Jeghers syndrome (PJS) juvenile polyposis (JPS) are autosomal dominant diseases associated with high cancer risk. In Japan, knowledge about the prevalence incidence of PJS JPS is lacking despite being crucial for providing appropriate medical support. We aimed to determine these diseases. Methods 2022, a nationwide questionnaire survey was conducted number patients or by sex newly confirmed cases from 2019 2021. The target facilities included...
ABSTRACT Objectives: Causes of early‐onset refractory diarrhea include exudative associated with very inflammatory bowel diseases, osmotic or secretory diarrhea, and protein‐losing enteropathy. Monogenic disorders are included in these yet a comprehensive genetic analysis has not been fully established. Methods: We established targeted gene panels covering all responsible genes for diarrhea. In total, 108 patients from 15 institutions were enrolled this study. collected clinical data...
This multicenter, cross-sectional study aimed to elucidate the efficacy and safety of AdvanCE capsule endoscope delivery device for children in Japan.The present analyzed 183 cases small bowel (SBCE) insertion performed using 154 patients aged less than 18 years at participating institutions between 2013 2017. Statistical analyses were conducted identify factors contributing completion rate entire intestine examination, transit time, adverse events technical issues.The commonest reason was...
The benefit of balloon-assisted enteroscopy (BAE) had been recently documented in pediatric patients, but previous reports are based on single institution experiences. We evaluated the feasibility BAE 8 tertiary referral hospitals throughout Japan.This was a prospective, multi-institutional study. Patients younger than 18 years were enrolled between April 2014 and March 2017 to undergo double-balloon or single-balloon enteroscopy. Data collected prospectively using standardized...
Abstract Prostaglandin E-major urinary metabolite (PGE-MUM) is a biomarker reflecting ulcerative colitis (UC) activity. This prospective observational study aimed to evaluate the usefulness of PGE-MUM via rapid chemiluminescent enzyme immunoassay in detecting endoscopic remission (ER) and histologic (HR) pediatric UC (6–16 years) comparison with fecal calprotectin (FCP). ER HR were defined as Mayo score (MES) 0 Matts’ histological grades (Matts) 1 or 2, respectively. A total 104 39...
S状結腸憩室症による結腸膀胱瘻および結腸穿孔をきたした1例を経験したので報告する。症例は73歳の男性で, 既往歴に肝硬変症を有し, 頻尿と糞尿を主訴として来院した。大腸内視鏡検査や注腸造影検査ではS状結腸に多数の憩室を認め, さらに注腸造影検査時に施行したCT検査では膀胱内に空気と造影剤を認めた。以上から, 結腸膀胱瘻を伴うS状結腸憩室症と診断した。手術を考慮中に, S状結腸憩室穿孔による汎発性腹膜炎が生じたため, 緊急手術を施行した。開腹すると, S状結腸憩室穿孔および結腸膀胱瘻を認めた。全身状態が不良なため, 憩室穿孔部および結腸膀胱瘻部を含むS状結腸を切除し, 人工肛門を造設した。また結腸膀胱瘻が存在した膀胱壁は縫合閉鎖した。術後2カ月現在, 経口摂取可能で膀胱炎は治癒している。
Abstract Background Although ulcerative proctitis (UP) in children is considered relatively mild, some patients have proximal disease extension and require immunosuppressive treatment. We investigated clinical characteristics course of refractory UP a multicenter pediatric cohort. Methods Analyzing data obtained between 2013 2022 at 10 institutions specializing inflammatory bowel disease, we elucidated natural history factors predicting need for compared given immunosuppressants and/or...
Abstract Prostaglandin E-major urinary metabolite (PGE-MUM) is a biomarker reflecting ulcerative colitis (UC) activity. This prospective observational study aimed to evaluate the usefulness of PGE-MUM via rapid chemiluminescent enzyme immunoassay in detecting endoscopic remission (ER) and histologic (HR) pediatric UC (6–16 years) comparison with fecal calprotectin (FCP). ER HR were defined as Mayo score (MES) 0 Matts’ histological grades (Matts) 1 or 2, respectively. A total 104 39...
Abstract Background and Aim Even with increasing numbers of biologic agents available for management ulcerative colitis (UC), infliximab (IFX) retains an important place in treatment pediatric patients this disease. As few reports have addressed outcomes UC who had to discontinue IFX, we examined clinical course prognosis after IFX failure UC. Methods A prospective cohort study pertinent cases enrolled the Japanese Pediatric Inflammatory Bowel Disease Registry between 2012 2020 was conducted...
months after the start.Pre-emptive treatment with an antiemetic to reduce adverse events was underused.
Abstract Background Biologics are widely used for paediatric Crohn's disease (CD) and highly effective; however, primary inefficacy the emergence of secondary during long-term use remain challenges. Although it is desirable biologics in patients to be safe usable long-term, there few detailed reports on comparison persistence rates patients. Methods This was a multicentre prospective cohort study using web-based registry system Japan that performed between October 2012 March 2020. New...