OBJECT: The purpose of this study was to investigate that infection with group A[beta] hemolytic streptococcus may associate the mechanisms that cause or exacerbate some cases of Tourette's disorder and to evaluate the treatment effect of IG therapy, comparing between IG therapy and drug therapy. METHOD: The subjects were divided into three groups composing of the groups with increasing level of ASO titer and the group with normal level of ASO titer, treating with antipsychotics. Children with infection-triggered exacerbation of Tourette's disorder were assigned treatment with IVIG (400mg/kg/daily on 5 consecutive days) or antipsychotic drugs. Symptom severity was rated at baseline, and at 4weeks, at at 8weeks after treatment by use of standard assessment scale of tics. RESULTS: 1) The motor tic score, global severity scores and overall TS impairment rating scores of YGTSS in the group with incresing level of ASO titer were related with ASO titer. 2) Immune therapy was more effective in the group with incresing level of ASO titer than antipsychotic drug therapy. CONCLUSION: These results suggest that increasing level of ASO titer, resulting from group A[beta] hemolytic streptococcal infection has affected worsening the tic symptoms in Touette's disorder and in group with increasing level of ASO titer, IVIG therapy is more effective than drug therapy.
Antipsychotic Agents ; Child ; Drug Therapy ; Humans ; Immunoglobulins, Intravenous ; Streptococcal Infections ; Streptococcus ; Tics* ; Tourette Syndrome*

Background: Nonpharmacological interventions (NPIs) reduce the incidence of respiratory infections. After NPIs imposed during the coronavirus disease 2019 pandemic ceased, respiratory infections gradually increased worldwide. However, few studies have been conducted on severe respiratory infections requiring hospitalization in pediatric patients.This study compares epidemiological changes in severe respiratory infections during pre-NPI, NPI, and post-NPI periods in order to evaluate the effect of that NPI on severe respiratory infections in children. Methods: We retrospectively studied data collected at 13 Korean sentinel sites from January 2018 to October 2022 that were lodged in the national Severe Acute Respiratory Infections (SARIs) surveillance database. Results: A total of 9,631 pediatric patients were admitted with SARIs during the pre-NPI period, 579 during the NPI period, and 1,580 during the post-NPI period. During the NPI period, the number of pediatric patients hospitalized with severe respiratory infections decreased dramatically, thus from 72.1 per 1,000 to 6.6 per 1,000. However, after NPIs ceased, the number increased to 22.8 per 1,000. During the post-NPI period, the positive test rate increased to the level noted before the pandemic. Conclusion Strict NPIs including school and daycare center closures effectively reduced severe respiratory infections requiring hospitalization of children. However, childcare was severely compromised. To prepare for future respiratory infections, there is a need to develop a social consensus on NPIs that are appropriate for children.

Triple A syndrome is a rare genetic disorder caused by mutations in the achalasia-addisonianism-alacrima syndrome (AAAS) gene which encodes a tryptophan aspartic acid (WD) repeat-containing protein named alacrima-achalasia-adrenal insufficiency neurologic disorder (ALADIN). Northern blot analysis shows that the 2.1 kb AAAS mRNA is expressed in various tissues with stronger expression in testis and pancreas. We show that human ALADIN is a protein with an apparent molecular weight of 60 kDa, and expressed in the adrenal gland, pituitary gland and pancreas. Furthermore, biochemical analysis using anti-ALADIN antibody supports the previous finding of the localization of ALADIN in the nuclear membrane. The mutations S544G and S544X show that alteration of S544 residue affects correct targeting of ALADIN to the nuclear membrane.
Adrenal Insufficiency/*genetics ; Antibodies/immunology ; Cloning, Molecular ; DNA, Complementary/genetics ; Esophageal Achalasia/*genetics ; Gene Expression Profiling ; Hela Cells ; Humans ; Lacrimal Apparatus Diseases/*genetics ; Mutagenesis, Site-Directed ; Nerve Tissue Proteins/*analysis/*genetics/immunology ; Nuclear Pore/chemistry ; Nuclear Pore Complex Proteins/*analysis/*genetics/immunology ; RNA, Messenger/analysis/genetics ; Syndrome ; Tissue Distribution