Search

Background: Few reports of benign recurrent intrahepatic cholestasis (BRIC) have focused on East Asian patients. We describe the clinicopathologic features, genetics, treatment, and outcomes in Japanese BRIC patients. Methods: We recruited patients with BRIC type 1 (BRIC-1) or 2 (BRIC-2) treated at four pediatric centers and one adult center between April 2007 and March 2022. Demographics, clinical course, laboratory results, molecular genetic findings concerning ATP8B1 and ABCB11 genes, histopathology, and treatment response were examined retrospectively. Results: Seven Japanese patients with BRIC were enrolled (four male, three female; four BRIC-1 and three BRIC-2). The median age at onset for BRIC-1 was 12 years; for BRIC-2, it was 1 month. Intermittent cholestatic attacks numbered from one to eight during the 11 years of median follow-up. Six patients received a mainstream education; only one patient attended special education. None developed cirrhosis. Three with BRIC-1 showed compound heterozygosity for a variant ATP8B1 gene, while one was heterozygous; two BRIC-2 patients showed compound heterozygosity in ABCB11 and one was heterozygous. Liver biopsy specimens obtained during cholestatic attacks showed fibrosis varying from none to moderate; inflammation was absent or mild. Rifampicin administered to three patients for cholestatic attacks was effective in all, as was cholestyramine in two of three. Conclusions: To our knowledge, this is the first East Asian multicenter study of BRIC patients. Onset age and number of cholestatic attacks varied. Rifampicin and cholestyramine were effective against attacks. No patient developed cirrhosis; most had normal growth and development. The long-term outcomes were satisfactory. [ABSTRACT FROM AUTHOR]

Background and Aim: Serum leucine‐rich alpha‐2 glycoprotein (LRG) and calprotectin have been studied as disease activity markers in adults with inflammatory bowel disease (IBD). We evaluated them in pediatric IBD patients. Methods: Subjects under 17 years old undergoing care at 11 Japanese pediatric centers were retrospectively assigned to 3 groups representing Crohn's disease (CD), ulcerative colitis (UC), and normal controls (NC) with irritable bowel syndrome or no illness. Serum LRG and calprotectin were measured using commercial enzyme‐linked immunosorbent assay kits. Results: We enrolled 173 subjects, including 74 with CD, 77 with UC, and 22 NC. Serum LRG concentrations in active CD (median, 200 μg/mL) were significantly greater than in remission (81 μg/mL; P < 0.001) or NC (69 μg/mL; P < 0.001). Serum calprotectin concentrations in active CD (2941 ng/mL) also were significantly greater than in remission (962 ng/mL; P < 0.05) or NC (872 ng/mL; P < 0.05). Serum LRG concentrations in active UC (134 μg/mL) were significantly greater than in remission (65 μg/mL; P < 0.01) but not significantly greater than in NC (69 μg/mL); serum calprotectin concentrations in active UC (1058 ng/mL) were not significantly different from those in remission (671 ng/mL) or NC (872 ng/mL). In receiver operating characteristic analyses of LRG, calprotectin, C‐reactive protein, and erythrocyte sedimentation rate for ability to distinguish active IBD from remission, CD and UC showed areas under receiver operating characteristic curves for LRG (0.77 and 0.70, respectively), exceeding those for calprotectin, C‐reactive protein, or erythrocyte sedimentation rate. Conclusions: In pediatric IBD, serum LRG may better reflect disease activity than serum calprotectin, particularly in CD. [ABSTRACT FROM AUTHOR]

Background: The roles and methods of diagnostic colonoscopy in pediatric patients were previously demonstrated. With advances in medical equipment and the increasing need for pediatric endoscopic diagnosis, we compared recent results with those previously reported. Methods: A retrospective analysis was conducted on pediatric patients aged ≤15 years, comparing those who underwent their first diagnostic colonoscopy between 1 January 2007 and 28 February 2015 with those who did so between 1 March 2015 and 28 February 2022 at Kurume University Hospital. Results: A total of 274 patients were included, including 110 in the previous study and 164 in the present study. The main indications were hematochezia in the previous study (63/110, 57.3%) and abdominal pain in the present study (64/164, 39.0%). Ulcerative colitis (74/274, 27.0%) was the most common diagnosis in both studies. The major difference from the previous study was an increase in the number of Crohn's disease and eosinophilic gastrointestinal disorder cases. Bowel preparation with magnesium citrate was significantly increased across all ages in the present study (142/164, 86.6%). Midazolam + pentazocine was used for sedation in most cases (137/164, 83.5%). An ultrathin upper endoscope was mainly used in patients aged ≤6 years, while ultrathin colonoscopes were applied in patients aged 7–12 years. Conclusion: In the present study, appropriate changes were found in the roles and methods of diagnostic colonoscopy in pediatric patients compared to the previous study. The increasing trend of patients presenting with inflammatory bowel disease and eosinophilic gastrointestinal disorder worldwide indicates the importance of colonoscopy in infants and children. [ABSTRACT FROM AUTHOR]

Background: Biliary atresia (BA) is among the commonest indications for liver transplantation (LT) in children. We examined whether serum matrix metalloproteinase‐7 (MMP‐7) is useful for diagnosis of BA in Japanese infants, and whether serum MMP‐7 concentrations before and after Kasai portoenterostomy (KP) predicted LT within a year. Methods: Subjects under 6 months old at eight pediatric centers in Japan were enrolled retrospectively, including patients with cholestasis and normal controls (NC) without liver disease. Patients with cholestasis were divided into groups representing BA versus cholestasis from other causes (non‐BA). Serum samples were collected from patients with BA at diagnosis and 1 and 4 weeks after KP, as well as from non‐BA and NC. Results: Serum MMP‐7 concentrations were significantly higher in BA at diagnosis (median, 89.1 ng/ml) than in non‐BA (11.0; p < 0.001) or NC (10.3; p < 0.001). Receiver operating characteristic (ROC) analysis of MMP‐7 for BA versus non‐BA yielded an area under the ROC curve of 0.99 (95% confidence interval, 0.96–1.00). An optimal cut‐off value of 18.6 ng/ml for serum MMP‐7 in diagnosing BA demonstrated sensitivity and specificity of 100% and 90%, respectively. Serum MMP‐7 before and 1 week and 4 weeks after KP did not differ significantly between BA requiring only KP and BA requiring LT after KP. Conclusion: Serum MMP‐7 is a useful marker for diagnosis of BA in Japanese infants, but it could not predict LT within a year. [ABSTRACT FROM AUTHOR]

The rheological properties of agarose solutions were examined under the effect of entanglement coupling between agarose chains. Agarose solutions were prepared by using an ionic liquid 1-butyl-3-methylimidazolium chloride as a solvent. The concentration of agarose was varied from 1.1 × 101-2.1 × 102 kg m−3. The master curves of the angular frequency (ω) dependence of the storage modulus ( G′) and the loss modulus ( G″) showed a rubbery region in the middle ω region and a flow region at low ω region, respectively. The molecular weight between entanglements ( M e) for agarose was calculated from the plateau modulus. Moreover, M e for agarose melt was determined to be 2.3 × 103 from the concentration dependence curve of M e. By using well-known empirical relations in polymer rheology, information on molecular characteristics of sample agarose was derived. © 2011 Wiley Periodicals, Inc. J Appl Polym Sci, 2012 [ABSTRACT FROM AUTHOR]

Concentrated solutions of cellulose and amylose were prepared with an ionic liquid 1-butyl-3-methylimidazolium chloride (BmimCl), which was chosen as a good solvent for these polysaccharides. Dynamic viscoelasticity of the concentrated solutions was examined to obtain the molecular weight between entanglements, Me. The value of Me in the molten state ( Me,melt), a material constant that reflecting the entanglement properties, was determined for cellulose and amylose by extrapolating Me to the 'melt.' A marked difference in Me,melt was found: 3.2 × 103 for cellulose and 2.5 × 104 for amylose. The value of Me,melt for cellulose, which is composed of β-(1,4) bonding of D-glucose units, is very close to those for polysaccharides with a random-coil conformation such as agarose and gellan in BmimCl. The much larger Me,melt for amylose can be attributed to the helical nature of the amylose chain, α-(1,4)-linked D-glucose units. The effect of concentration on the zero-shear viscosity for the solutions of cellulose and amylose was also examined. © 2011 Wiley Periodicals, Inc. J Polym Sci Part B: Polym Phys, 2011 [ABSTRACT FROM AUTHOR]

The article presents the case study of 7-year-old Japanese girl had recurrent febrile attacks including abdominal pain lasting 2–3 days and elevated serum C-reactive protein. Topics include Interleukin-1 inhibitors including canakinumab have been effective in some patients with colchicine-resistant Familial Mediterranean fever (FMF) being a systemic autoinflammatory disorder characterized by recurring episodes of fever and serosal, and child with crFMF, permitting withdrawal of colchicine.

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. In this retrospective observational study, we describe results in 7 children with IgAV who were treated with dapsone for abdominal pain resistant to CS. Dapsone rapidly relieved abdominal pain in all 7 patients, who then were tapered off CS without relapse. Side effects of mild methemoglobinemia and hemolysis appeared to be manageable with planned monitoring and dose adjustment; a single patient who discontinued dapsone had fatigue and hypoxia associated with methemoglobinemia. No side effects were life-threatening. Dapsone may be considered as a therapeutic option for gastrointestinal symptoms refractory to CS in children with IgAV., (Copyright © 2022 by the American Academy of Pediatrics.)