Enhanced differentiation of intraepithelial lymphocytes in the intestine of polymeric immunoglobulin receptor-deficient mice.

To clarify the effect of secretory IgA (sIgA) deficiency on gut homeostasis, we examined intraepithelial lymphocytes (IELs) in the small intestine (SI) of polymeric immunoglobulin receptor-deficient (pIgR(-/-) ) mice. The pIgR(-/-) mice exhibited the accumulation of CD8αβ(+) T-cell receptor (TCR)-αβ(+) IELs (CD8αβ(+) αβ-IELs) after weaning, but no increase of CD8αβ(+) γδ-IELs was detected in pIgR(-/-) TCR-β(-/-) mice compared with pIgR(+/+) TCR-β(-/-) mice. When 5-bromo-2'-deoxyuridine (BrdU) was given for 14 days, the proportion of BrdU-labelled cells in SI-IELs was not different between pIgR(+/+) mice and pIgR(-/-) mice.

Lissencephaly with marked ventricular dilation, agenesis of corpus callosum, and cerebellar hypoplasia caused by TUBA1A mutation.

We described the clinical course and pathological findings in a child with TUBA1A mutation. MRI revealed marked ventricular dilation with thin cortex, poorly differentiated basal ganglia, agenesis of corpus callosum, cerebellar hypoplasia with preserved vermis at 2 months of age. No gain of developmental milestones was observed until she died with respiratory failure at 23 months of age.

A boy with a severe phenotype of succinic semialdehyde dehydrogenase deficiency.

Succinic semialdehyde dehydrogenase (SSADH) deficiency is a rare autosomal recessive disorder affecting γ-aminobutyric acid degradation. We describe here a boy with a severe phenotype of SSADH deficiency. He was referred because of a developmental delay at 4 months of age.

ω-3 fatty acids attenuate mucosal inflammation in premature rat pups.

Background: Necrotizing enterocolitis (NEC) is a devastating intestinal disease of premature infants. Although ω-3 fatty acids are known to have antiinflammatory effects, their effect against NEC remains unclear.

Methods: Mother rats fed a soybean-based, docosahexaenoic acid (DHA)- or eicosapentaenoic acid (EPA)-enriched diet from days 7 to 20 of gestation were examined.

Effects of leukotriene receptor antagonists on peripheral eosinophil counts and serum IgE levels in children with food allergy.

Background: Although the efficacy of leukotriene receptor antagonists (LTRAs) for bronchial asthma is already established, their effect on food allergy remains unclear.

Objective: To investigate the efficacy of LTRAs in children with food allergy.

Methods: This retrospective study examined 65 children with food allergy who were aged between 3 and 36 months (mean 14 ± 9.

Monitoring 6-thioguanine nucleotide concentrations in Japanese children and adolescents with inflammatory bowel disease.

Background And Aim: 6-Mercaptopurine (6-MP) and azathioprine (AZA) are widely used as maintenance therapy in children with inflammatory bowel disease (IBD). However, proper 6-thioguanine nucleotide (6-TGN) concentrations in Japanese children with IBD have not been reported.

Methods: This retrospective review examines 32 ulcerative colitis (UC) patients and 19 Crohn's disease (CD) patients (12.

Increased mucosal expression of GATA-3 and STAT-4 in pediatric ulcerative colitis.

Background: Serum pro-inflammatory cytokine levels are frequently elevated in the acute phase of pediatric inflammatory bowel disease (IBD). Because the role of pro-inflammatory cytokine in the acute phase of pediatric IBD has not been well investigated, the serum levels of pro-inflammatory cytokines and the expression of Th1 and Th2 signaling molecules in mucosa from the acute phase of pediatric IBD were examined.

Methods: Twenty children with ulcerative colitis (UC; mean age, 9.

Overfeeding can cause NEC-like enterocolitis in premature rat pups.

Background: Neonatal necrotizing enterocolitis (NEC) is the most common gastrointestinal emergency in premature infants. The mortality rate associated with NEC is quite high and in most reports ranges from 20 to 30%. Despite extensive studies, the pathogenesis of NEC remains poorly understood.

Acute encephalopathy with biphasic seizures and late reduced diffusion associated with hemophagocytic syndrome.

We reported a girl with HHV-6 infection associated with both acute encephalopathy with biphasic seizures and late reduced diffusion, and hemophagocytic syndrome. She had a prolonged convulsion after a one-day history of febrile illness. Cerebrospinal fluid or brain CT showed no abnormalities on admission and her consciousness was recovered on the next day.