Juvenile temporal arteritis (JTA) is a localized nodular arteritis confined to the temporal artery without evidence of systemic inflammation, and it occurs mainly in patients younger than 50 years. From the first case report, the pathological features of JTA have been suspected to be the morphological equivalent of Kimura disease (KD), which has been supported further by the concurrent cases of JTA with KD. We present the first case of bilateral JTA accompanying KD, which was confirmed by histological and ultrasound evaluations and supports the hypothesis that JTA is a manifestation of KD. The un-excised JTA lesion was resolved completely after corticosteroid therapy with no recurrence.
Adrenal Cortex Hormones ; Angiolymphoid Hyperplasia with Eosinophilia ; Arteritis ; Giant Cell Arteritis ; Humans ; Inflammation ; Recurrence ; Temporal Arteries ; Ultrasonography

OBJECTIVES: Repetitive and stereotyped behaviors are core symptoms in children with autism spectrum disorders (ASD). The purpose of our study was to investigate the frequency of motor stereotypes in ASD children and their clinical features. METHODS: Among 171 ASD children (age range, 3-15), the ASD group with motor stereotypes was defined according to two items in the Korean version of Autism Diagnostic Interview-Revised (K-ADI-R). We compared the clinical features, behavior problems and severity of other domains in the K-ADI-R and executive functions between the ASD group with motor stereotypes and the ASD group without motor stereotypes. RESULTS: Ninety (52.6%) of 171 ASD children had motor stereotypes. The ASD group with motor stereotypes had a lower intelligence quotient score (62.23 vs. 84.94, p<.001) compared to the ASD group without motor stereotypes. The ASD group with motor stereotypes had more impairments in the social interaction domain [adjusted odds ratio (AOR) 1.11, p=.001] and communication domain (AOR 1.15, p=.008). Thought problems and lethargy were more frequent in the ASD group with motor stereotypes than the ASD group without motor stereotypes (AOR 2.059, p=.034 ; adjusted OR 1.045, p=.046). However, no significant differences in executive function were observed between the ASD group with motor stereotypes and the ASD group without motor stereotypes. CONCLUSION: The ASD group with motor stereotypes showed more impairment in social interaction and communication domains, which are core symptoms of autism. Motor stereotypes may indicate greater severity of ASD.
Autistic Disorder ; Autism Spectrum Disorder* ; Child* ; Executive Function ; Humans ; Intelligence ; Interpersonal Relations ; Lethargy ; Odds Ratio ; Stereotyped Behavior

OBJECTIVES: The purposes of this study were to examine the reliability and validity of the Korean version of Social Communication Questionnaire (K-SCQ) and to determine cut-off scores for diagnosis of autism spectrum disorder (ASD). METHODS: A total of 166 subjects with ASD and their 186 unaffected siblings were recruited through child psychiatry clinics of university hospitals. Board certified child psychiatrists screened all probands suspected to have ASD based on the Diagnostic and Statistical Manual of Mental Disorders, fourth edition. To confirm the diagnoses, the Korean versions of the Autism Diagnostic Observation Schedule and the Autism Diagnostic Interview-Revised (K-ADI-R) were administered to all the subjects. All parents completed the K-SCQ and Social Responsiveness Scale (SRS). The non-ASD siblings were evaluated with the same instruments as the probands with ASD. We performed a factor analysis to examine the structure of K-SCQ. For testing the validity of K-SCQ, we compared the difference in Lifetime and Current scores of probands with ASD and their non-ASD siblings using t-test and analysis of covariance. Correlations between the K-SCQ and other measurements of ASD symptomatology, including K-ADI-R totals and domain scores and SRS, were examined. Receiver operation characteristic curve analysis was performed to extract cutoff scores discriminating affection status. RESULTS: Four factors were extracted through factor analysis of K-SCQ ; 1) social relation and play, 2) stereotyped behavior, 3) social behavior, and 4) abnormal language. Cronbach's internal consistency was .95 in K-SCQ Lifetime, and .93 in K-SCQ Current. There were significant differences in total score of K-SCQ, both in Lifetime and Current between the ASD group and non-ASD siblings group (p<.001). K-SCQ scores were significantly correlated with K-ADI-R subdomain scores and SRS total scores (p<.001). The best-estimate cut-off scores of K-SCQ for diagnosis of ASD were 12 for 48 months and over, and 10 for below 47 months. CONCLUSION: Our findings suggest that the K-SCQ is a reliable and valid instrument for screening autistic symptoms in the Korean population. Lower cut-off scores than the original English version might be considered when using it as a screening instrument of ASD.
Appointments and Schedules ; Autistic Disorder ; Child ; Autism Spectrum Disorder ; Child Psychiatry ; Diagnosis ; Diagnostic and Statistical Manual of Mental Disorders ; Hospitals, University ; Humans ; Mass Screening ; Parents ; Psychiatry ; Reproducibility of Results ; Siblings ; Social Behavior ; Stereotyped Behavior

OBJECTIVE: Autophagy plays a vital role in homeostasis by combining organelles and cellular proteins with lysosome under starvation conditions. In addition, autophagy provides tumor cells with a source of energy. Continued autophagy will induce cells death. Here we aim to see if autophagic induction has an effect on conventional chemotherapeutic agents. METHODS: Rapamycin, or mammalian target of rapamycin and paclitaxel, apoptosis-inducing agents were used autophagy in HeLa cervical cancer cells. RESULTS: Growth inhibition of cells was not observed after the application of 0, 10, 20 nM of paclitaxel with or without rapamycin. Using a 5 nM concentration of paclitaxel, rapamycin administration inhibited cell growth significantly compared to no treatment. This implies the synergic antitumor effect of paclitaxel and rapamycin. Paclitaxel itself did not show any autophagic effect on cells but did show cell apoptosis by flow cytometry. Light chain 3, a microtubule-associated protein, which reflect autophagy, was increased with 5 nM of paclitaxel after pretreatment with 10 nM of rapamycin. CONCLUSION: These findings suggest that the autophagic inducer, rapamycin, can potentiate autophagic cell death when added as an apoptosis-inducing chemotherapeutic agent. In conclusion, the control of autophagy may be a future target for chemotherapy.
Apoptosis ; Autophagy ; Cell Death ; Flow Cytometry ; Homeostasis ; Light ; Lysosomes ; Organelles ; Paclitaxel ; Proteins ; Sirolimus ; Starvation ; TOR Serine-Threonine Kinases ; Uterine Cervical Neoplasms

OBJECTIVE: The purpose of this study was to investigate the association between the T102C polymorphism in the serotonin 2A receptor gene and attention-deficit/hyperactivity disorder (ADHD) in Korean patients. METHODS: A total of 189 Korean children with ADHD as well as both parents of the ADHD children and 150 normal children participated in this study. DNA was extracted from blood samples from all of the subjects, and genotyping was conducted. Based on the allele and genotype information obtained, case-control analyses were performed to compare the ADHD and normal children, and Transmission disequilibrium tests (TDTs) were used for family-based association testing (number of trios=113). Finally, according to the significant finding which was showed in the case-control analyses, the results of behavioral characterastics and neuropsychological test were compared between ADHD children with and without the C allele. RESULTS: In the case-control analyses, statistically significant differences were detected in the frequencies of genotypes containing the C allele (chi2=4.73, p=0.030). In the family-based association study, TDTs failed to detect linkage disequilibrium of the T102C polymorphism associated with ADHD children. In the ADHD children, both the mean reaction time and the standard deviation of the reaction time in the auditory continuous performance test were longer in the group with the C allele compared to the group without the C allele. CONCLUSION: The results of this study suggest that there is a significant genetic association between the T102C polymorphism in the serotonin 2A receptor gene and ADHD in Korean children.
Alleles ; Case-Control Studies ; Child ; DNA ; Genotype ; Humans ; Linkage Disequilibrium ; Neuropsychological Tests ; Parents ; Reaction Time ; Receptor, Serotonin, 5-HT2A ; Serotonin

BACKGROUND: The intravenous administration of indigo carmine has been reported to produce transiently increased blood pressure in patients. The goal of this in vitro study was to examine the effect of indigo carmine on phenylephrine-induced contractions in an isolated rat aorta and to determine the associated cellular mechanism with particular focus on the endothelium-derived vasodilators. METHODS: The concentration-response curves for phenylephrine were generated in the presence or absence of indigo carmine. Phenylephrine concentration-response curves were generated for the endothelium-intact rings pretreated independently with a nitric oxide synthase inhibitor, Nomega-nitro-L-arginine methyl ester (L-NAME), a cyclooxygenase inhibitor, indomethacin, and a low-molecular-weight superoxide anion scavenger, tiron, in the presence or absence of indigo carmine. The fluorescence of oxidized dichlorofluorescein was measured in rat aortic vascular smooth muscle cells cultured in the control, indigo carmine alone and tiron plus indigo carmine. RESULTS: Indigo carmine (10(-5) M) increased the phenylephrine-induced maximum contraction in the endothelium-intact rings with or without indomethacin, whereas indigo carmine produced a slight leftward shift in the phenylephrine concentration-response curves in the endothelium-denuded rings and L-NAME-pretreated endothelium-intact rings. In the endothelium-intact rings pretreated with tiron (10(-2) M), indigo carmine did not alter phenylephrine concentration-response curves significantly. Indigo carmine (10(-5) M) increased the fluorescence of oxidized dichlorofluorescein in the vascular smooth muscle cells, whereas tiron abolished the indigo carmine-induced increase in oxidized dichlorofluorescein fluorescence. CONCLUSIONS: Indigo carmine increases the phenylephrine-induced contraction mainly through an endothelium-dependent mechanism involving the inactivation of nitric oxide caused by the increased production of reactive oxygen species.
1,2-Dihydroxybenzene-3,5-Disulfonic Acid Disodium Salt ; Administration, Intravenous ; Animals ; Aorta ; Blood Pressure ; Contracts ; Fluorescence ; Humans ; Indigo Carmine ; Indoles ; Indomethacin ; Muscle, Smooth, Vascular ; Nitric Oxide ; Nitric Oxide Synthase ; Phenylephrine ; Prostaglandin-Endoperoxide Synthases ; Rats ; Reactive Oxygen Species ; Superoxides

OBJECTIVE: A significant proportion of children with autism spectrum disorders (ASD) have regression characterized by loss of previously acquired skills. The purpose of this study was to compare demographic, clinical characteristics and autism-related symptomatology of the children who have regression with children who don't have regression. METHODS: The subjects with ASD and their unaffected siblings (SIB) were recruited from the Korean Autism Genetic Study Consortium. Typically developing children (TC) were volunteered from community. The subjects were administered the Korean version of Autism Diagnostic Interview-Revised (K-ADI-R) and the Korean version of Autism Diagnostic Observation Schedule (K-ADOS) to diagnose or exclude ASD. Regression was defined on the basis of K-ADI-R data. The Korean version of Vineland Adaptive Behavior Scale (K-VABS), Aberrant Behavior Checklist (K-ABC) and Social Responsiveness Scale (K-SRS) were obtained from their parents. RESULTS: Regression occurred in 8.33% (n=14) of children with ASD (n=168). Any SIB (n=166) and TC (n=53) did not experience regression. Regression was associated with lower IQ and lower score of K-VABS. There was no difference in autism symptom severity and K-ABC, K-SRS scores, between children with ASD who experienced regression and who did not. CONCLUSION: Regression seems to be a distinctive feature of ASD. Regression is associated with cognitive and more general functions, rather than symptoms specific to autism.
Adaptation, Psychological ; Appointments and Schedules ; Autistic Disorder ; Checklist ; Child ; Autism Spectrum Disorder ; Humans ; Siblings

OBJECTIVE: Communication problems are a prevalent symptom of autism spectrum disorders (ASDs), which have a genetic background. Although several genome-wide studies on ASD have suggested a number of candidate genes, few studies have reported the association or linkage of specific endophenotypes to ASDs. METHODS: Forty-two Korean ASD patients who showed a language delay were enrolled in this study with their parents. We performed a genome-wide scan by using the Affymetrix SNP Array 5.0 platform to identify candidate genes responsible for language delay in ASDs. RESULTS: We detected candidate single-nucleotide polymorphisms (SNPs) in chromosome 11, rs11212733 (p-value=9.76x10(-6)) and rs7125479 (p-value=1.48x10(-4)), as a marker of language delay in ASD using the transmission disequilibrium test and multifactor dimensionality reduction test. CONCLUSION: Although our results suggest that several SNPs are associated with language delay in ASD, rs11212733 we were not able to observe any significant results after correction of multiple comparisons. This may imply that more samples may be required to identify genes associated with language delay in ASD.
Autistic Disorder ; Child ; Autism Spectrum Disorder ; Chromosomes, Human, Pair 11 ; Endophenotypes ; Genome-Wide Association Study ; Humans ; Language Development Disorders ; Multifactor Dimensionality Reduction ; Parents ; Polymorphism, Single Nucleotide

OBJECTIVES: This study was conducted to examine the prevalence and characteristics of savant skills and the relationship between the savant skills and autistic symptoms in Korean ASD children. METHODS: 141 ASD subjects participated in this study and they were divided in to two groups based on the presence or lack of savant skills. The domain scores and total scores of the K-ADI-R, K-ASDS and SRS were used for evaluating the ASD symptoms between the groups. RESULTS: Memory (n=47) was the most prevalent savant skill in the savant ASD group (n=60). The savant ASD group had a statistically higher mean age and IQ score than did the nonsavant ASD group. Despite their high IQ profile, the savant ASD group showed a higher restricted, repetitive and stereotype behavior score on the K-ADI-R and higher language and cognitive scores on the K-ASDS than did the nonsavant ASD group. CONCLUSIONS: These results suggest savant syndrome in ASD might be related to the severity of some subdomain of autistic symptoms even though their IQ scores were higher than nonsavant ASD patients.
Autistic Disorder ; Child ; Autism Spectrum Disorder ; Humans ; Memory ; Prevalence

BACKGROUND: Dermatological diseases can occur with atopic dermatitis. OBJECTIVE: The purpose of this study was to analyze diseases associated with atopic dermatitis in Koreans. METHODS: From November, 2007, to May, 2008, 948 patients with atopic dermatitis who visited the department of dermatology at 19 hospitals were evaluated for associated diseases. RESULTS: Of 948 patients, 53.8% (510) had symptoms associated with other dermatological diseases. In order of frequency, diseases associated with atopic dermatitis included acne, hand/foot eczema, seborrheic dermatitis, urticaria, warts, and recurrent herpes simplex. The number of associated diseases did not change significantly with the severity of atopic dermatitis. However, the incidence of hand/foot eczema and eczema herpeticum correlated significantly with the severity of atopic dermatitis. CONCLUSION: There is a distinct pattern of diseases associated with atopic dermatitis in Koreans.
Acne Vulgaris ; Dermatitis, Atopic ; Dermatitis, Seborrheic ; Dermatology ; Eczema ; Herpes Simplex ; Humans ; Incidence ; Kaposi Varicelliform Eruption ; Methylmethacrylates ; Polystyrenes ; Urticaria ; Warts