Publications by authors named "Nambu R"

Eosinophilic gastrointestinal disorders (EGIDs) are treated with corticosteroids and food allergen elimination. However, treatment for refractory cases is not standardized. We demonstrate the efficacy of vedolizumab, an anti-α4β7 integrin agent, in 2 children with duodenal ulcers developed by non-eosinophilic esophagitis EGIDs.

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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 and course of refractory UP in a multicenter pediatric cohort.

Methods: Analyzing data obtained between 2013 and 2022 at 10 institutions specializing in pediatric inflammatory bowel disease, we elucidated natural history and factors predicting a need for immunosuppressive UP treatment.

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Introduction: This study evaluated nutrient deficiencies in infants and toddlers with inflammatory bowel disease (IBD) and eosinophilic gastrointestinal disorders (EGIDs), whose primary nutritional source is elemental formulas (EFs).

Methods: The nutrient status of children with IBD and EGID aged 6 months to 6 years was evaluated.

Results: Twenty-one children fed with EFs (EF group) and 25 controls (CL group) were enrolled.

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Article Synopsis
  • * Four patients with STAT3-GOF were treated with Janus kinase inhibitors (JAKi), and their symptoms showed significant improvement after starting treatment.
  • * Laboratory tests indicated that the patients' lymphoid cells had increased STAT3 activation upon stimulation, suggesting that JAKi might be a promising treatment option for managing STAT3-GOF symptoms.
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Background And Aim: Even with increasing numbers of biologic agents available for management of ulcerative colitis (UC), infliximab (IFX) retains an important place in treatment of pediatric patients with this disease. As few reports have addressed outcomes in pediatric UC patients who had to discontinue IFX, we examined clinical course and prognosis after IFX failure in pediatric UC.

Methods: A prospective cohort study of pertinent cases enrolled in the Japanese Pediatric Inflammatory Bowel Disease Registry between 2012 and 2020 was conducted to determine outcomes for pediatric UC patients who received IFX but required its discontinuation during follow-up (IFX failure).

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Objective: To examine whether it is possible to screen for bile acid synthesis disorders (BASDs) including peroxisome biogenesis disorder 1a (PBD1A) and Niemann-Pick type C1 (NPC1) at the time of newborn mass screening by measuring the intermediary metabolites of bile acid (BA) synthesis.

Methods: Patients with 3β-hydroxy-ΔSuchy et al. (2021)-C-steroid dehydrogenase/isomerase (HSD3B7) deficiency (n = 2), 3-oxo-ΔPandak and Kakiyama (n.

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Background: Vedolizumab (VDZ) is a humanized monoclonal antibody that binds to α4β7 integrin expressed in T-lymphocytes and is gut selective. Few studies have evaluated the safety and efficacy of VDZ in pediatric ulcerative colitis (UC) patients, especially from Asia.

Methods: A longitudinal multicenter retrospective study was conducted at 10 Japanese tertiary medical institutions.

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Background: As best practices for treating children with severe-onset ulcerative colitis remain controversial in the era of biologic agents, we prospectively investigated treatments and outcomes in a multicenter cohort.

Methods: Using a Web-based data registry maintained in Japan between October 2012 and March 2020, we compared management and treatment outcomes in an S1 group defined by a Pediatric Ulcerative Colitis Activity Index of 65 or more points at diagnosis with those in an S0 group defined by an index value below 65.

Results: Three hundred one children with ulcerative colitis treated at 21 institutions were included, with follow-up for 3.

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Article Synopsis
  • The study investigated serum levels of leucine-rich alpha-2 glycoprotein (LRG) and calprotectin as markers for disease activity in children with inflammatory bowel disease (IBD), including Crohn's disease (CD) and ulcerative colitis (UC).
  • A total of 173 pediatric patients were analyzed, revealing that LRG levels were significantly higher in active cases of CD compared to remission and normal controls, while calprotectin showed similar trends but was less consistently different in UC.
  • The findings suggest that serum LRG is a potentially more reliable marker of disease activity in pediatric IBD, especially for CD, compared to traditional markers like calprotectin.
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Objectives: To describe the incidence and diagnostic performance of ultrasound for perianal abscess or fistula-in-ano in pediatric patients with perianal inflammation.

Methods: We included 45 patients with perianal inflammation who underwent ultrasonography. To demonstrate the diagnostic performance of ultrasound for fistula-in-ano, a definite diagnosis of perianal abscess, and fistula-in-ano was determined as that proven through magnetic resonance imaging (MRI) or computed tomography (CT).

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Article Synopsis
  • Immunosuppressive therapies for inflammatory bowel disease (IBD) can impact how well vaccines work and their safety, making vaccination decisions crucial before starting treatment.
  • A consensus was created to help guide both physicians and patients in Japan about vaccinations for those with IBD, focusing on vaccine-preventable diseases, while excluding COVID-19 and cervical cancer.
  • The consensus addresses 19 key clinical questions across four topics, providing essential information for managing vaccination in pediatric and adult IBD patients, promoting informed decision-making in everyday medical practice.
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Capsule endoscopy (CE) and balloon-assisted enteroscopy (BAE) have become indispensable techniques for the diagnosis and management of small bowel disease in both adult and pediatric cases. However, relevant differences exist in the indications between these cohorts, with body weight and age having particular relevance in decisions for the latter. Both CE and BAE are designed for adult physique and they were not widely used among children.

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Article Synopsis
  • STXBP2 is a gene linked to intracellular trafficking and is associated with familial hemophagocytic lymphohistiocytosis (HLH) and inflammatory bowel disease (IBD), but its exact impact is still not fully understood.
  • A novel mutation in STXBP2 was found in a boy suffering from severe diarrhea and HLH, who underwent a series of treatments including stem cell transplantation.
  • Despite treatments alleviating some symptoms, the patient continued to experience mild colitis and persistent diarrhea, indicating that STXBP2-related IBD may involve both excessive inflammation and issues with the gut's protective barrier.
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Purpose: Toxins produced by infection (CDI) can cause enteritis and diarrhea. Although the number of pediatric CDI cases is increasing, the clinical management of pediatric CDI, including patient characteristics and prognosis, remains unclear. This study aimed to elucidate the background and clinical course of patients with CDI and evaluate the reliability of diagnostic tests in a tertiary pediatric hospital in Japan.

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Immunoglobulin A vasculitis (IgAV) is a systemic small-vessel vasculitis. Although corticosteroids (CS) are the primary treatment for gastrointestinal manifestations associated with IgAV, some patients develop refractory or recurrent symptoms such as vomiting and abdominal pain despite CS treatment. Dapsone, a synthetic sulfone antimicrobial, has been used to treat cutaneous purpura in IgAV, but few authors have reported its use for refractory gastrointestinal symptoms.

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Purpose: The incidence of lithium button battery ingestion has been increasing recently, which results in severe complications. We aimed to demonstrate the association between the corrosion of lithium button batteries in the esophagus on radiographs and their complications.

Methods: The nine pediatric patients included in this study were classified into two groups based on the presence of severe complications.

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Melena, or tarry black stool, is not a rare symptom encountered in pediatric clinical practice, and the bleeding source varies from the upper gastrointestinal tract to the small intestine. Endoscopy is effective in identifying bleeding, but it does not always identify the source of bleeding. Endoscopic examination in children is commonly challenging, and there are no detailed reports about the causes of melena in children.

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Very early-onset inflammatory bowel disease (VEO-IBD) is defined as IBD onset before 6 years of age and some cases are caused by unique monogenic disorders that require specific treatments such as stem cell transplantation. We identified three children with VEO-IBD of whom two had monogenic disorders. In cases 1 and 2, ultrasound revealed isolated colonic distribution and the loss of wall stratification.

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