Background: The clinical features of pediatric rhabdomyolysis differ from those of the adults with rhabdomyolysis; however, multicenter studies are lacking. This study aimed to investigate the characteristics of pediatric rhabdomyolysis and reveal the risk factors for acute kidney injury (AKI) in such cases. Methods: This retrospective study analyzed the medical records of children and adolescents diagnosed with rhabdomyolysis at 23 hospitals in South Korea between January 2007 and December 2016. Results: Among 880 patients, those aged 3 to 5 years old composed the largest subgroup (19.4%), and all age subgroups were predominantly male. The incidence of AKI was 11.3%. Neurological disorders (53%) and infection (44%) were the most common underlying disorder and cause of rhabdomyolysis, respectively. The median age at diagnosis in the AKI subgroup was older than that in the non-AKI subgroup (12.2 years vs. 8.0 years). There were no significant differences in body mass index, myalgia, dark-colored urine, or the number of causal factors between the two AKI-status subgroups. The multivariate logistic regression model indicated that the following factors were independently associated with AKI: multiorgan failure, presence of an underlying disorder, strong positive urine occult blood, increased aspartate aminotransferase and uric acid levels, and reduced calcium levels. Conclusions Our study revealed characteristic clinical and laboratory features of rhabdomyolysis in a Korean pediatric population and highlighted the risk factors for AKI in these cases. Our findings will contribute to a greater understanding of pediatric rhabdomyolysis and may enable early intervention against rhabdomyolysis-induced AKI.

Purpose: Adenocarcinoma is an extremely rare malignancy in the pediatric population. Research regarding pediatric adenocarcinoma is very rare in Korea. This study aimed to investigate the clinical features of pediatric adenocarcinomas of various primary organ sites in Korea. Materials and Methods: Pediatric patients under 18 years, diagnosed with adenocarcinoma of various sites between January 1995 and December 2016, were included. We retrospectively reviewed patient and tumor characteristics and calculated survival estimates, reported as 5-year survival rate and 95% confidence interval. Results: Of 80 patients (median age, 15 years; range, 10 to 17 years), 37 (46.3%) were men, and 24 (30%) had a family history of cancer or underlying disease relevant to malignancy. The cancer locations were the colon and rectum (n=32), ovaries (n=18), stomach (n=15), lung (n=4), small bowel (n=1), and other sites (n=10). Totally, 54.8% patients (42/77) had stage 3 or 4 disease. The median follow-up period was 2.0 years (range, 0 to 20.4). The 5-year overall survival estimate for all patients, and for those with stomach, colorectal, ovarian, and other cancer sites were 57.9%±11.5%, 58.2%±25.7%, 41.5%±18.2%, 87.5%±16.2%, and 64.0%±34.4%, respectively. The 5-year survival rate differed significantly between categories of adenocarcinomas into gastrointestinal (GI) (44.7%) and non-GI adenocarcinomas (78.8%) (p=0.007). The 5-year survival rate also differed significantly according to carcinoembryonic antigen level (69.3% in < 3 ng/mL, 23.8% in > 3 ng/mL; p < 0.001). Conclusion In pediatric patients, adenocarcinomas arise from various organs and are often diagnosed at advanced stages. Large, prospective studies for their accurate clinical characteristics and prognostic factors are needed.

Parkinson's disease (PD) is an irreversible neurological disorder with related locomotor dysfunction and is haracterized by the selective loss of nigral neurons. PD can be experimentally induced by 6-hydroxydopamine (6-OHDA). It has been reported that reactive oxygen species, which deplete endogenous glutathione (GSH) levels, may play important roles in the dopaminergic cell death characteristic of PD. Fucoidan, a sulfated algal polysaccharide, exhibits anti-inflammatory and anti-oxidant actions. In this study, we investigated whether fucoidan can protect against 6-OHDA-mediated cytotoxicity in SH-SY5Y cells. Cytotoxicity was evaluated by using MTT and LDH assays. Fucoidan alleviated cell damage evoked by 6-OHDA dose-dependently. Fucoidan reduced the number of apoptotic nuclei and the extent of annexin-V-associated apoptosis, as revealed by DAPI staining and flow cytometry. Elevation of lipid peroxidation and caspase-3/7 activities induced by 6-OHDA was attenuated by fucoidan, which also protected against cytotoxicity evoked by buthionine-sulfoximine-mediated GSH depletion. Reduction in the glutathione/glutathione disulfide ratio induced by 6-OHDA was reversed by fucoidan, which also inhibited 6-OHDA-induced disruption of mitochondrial membrane potential. The results indicate that fucoidan may have protective action against 6-OHDA-mediated neurotoxicity by modulating oxidative injury and apoptosis through GSH depletion.
Apoptosis ; Cell Death ; Flow Cytometry ; Glutathione ; Lipid Peroxidation ; Membrane Potential, Mitochondrial ; Nervous System Diseases ; Neurons ; Oxidopamine ; Parkinson Disease ; Reactive Oxygen Species

PURPOSE: UGT1A1, UGT2B7, and UGT2B15 are well-known pharmacogenes that belong to the uridine diphosphate glucuronyltransferase gene family. For personalized drug treatment, it is important to study differences in the frequency of core markers across various ethnic groups. Accordingly, we screened single nucleotide polymorphisms (SNPs) of these three genes and analyzed differences in their frequency among five ethnic groups, as well as attempted to predict the function of novel SNPs. MATERIALS AND METHODS: We directly sequenced 288 subjects consisting of 96 Korean, 48 Japanese, 48 Han Chinese, 48 African American, and 48 European American subjects. Subsequently, we analyzed genetic variability, linkage disequilibrium (LD) structures and ethnic differences for each gene. We also conducted in silico analysis to predict the function of novel SNPs. RESULTS: A total of 87 SNPs were detected, with seven pharmacogenetic core SNPs and 31 novel SNPs. We observed that the frequencies of UGT1A1 *6 (rs4148323), UGT1A1 *60 (rs4124874), UGT1A1 *93 (rs10929302), UGT2B7 *2 (rs7439366), a part of UGT2B7 *3 (rs12233719), and UGT2B15 *2 (rs1902023) were different between Asian and other ethnic groups. Additional in silico analysis results showed that two novel promoter SNPs of UGT1A1 -690G>A and -689A>C were found to potentially change transcription factor binding sites. Moreover, 673G>A (UGT2B7), 2552T>C, and 23269C>T (both SNPs from UGT2B15) changed amino acid properties, which could cause structural deformation. CONCLUSION: Findings from the present study would be valuable for further studies on pharmacogenetic studies of personalized medicine and drug response.
Asian Continental Ancestry Group/genetics ; European Continental Ancestry Group/genetics ; Female ; Gene Frequency/genetics ; Glucuronosyltransferase/*genetics ; Haplotypes/genetics ; Humans ; Linkage Disequilibrium/genetics ; Male ; Polymorphism, Single Nucleotide/*genetics

BACKGROUND AND OBJECTIVES: Interleukin-21 receptor (IL-21R) gene polymorphism is related with the development of systemic vasculitis. In this study, we investigated the polymorphisms of IL-21R gene in patients with Kawasaki disease (KD). SUBJECTS AND METHODS: We genotyped the promoter region of IL-21R gene (-2500 bp to +1 bp) in 100 patients with KD and 100 healthy controls. All study subjects were Korean. We designed five pairs of primers and performed polymerase chain reaction (PCR) and direct sequencing. We analyzed whole promoter sequences of 200 individuals with comparison to reference sequences of IL-21R gene (NG_012222.1/NC_000016.9). RESULTS: We found five single nucleotide polymorphisms (SNPs) of which minor allele frequency (MAF) >0.01 in the promoter region of IL-21R gene. Those are -1681 G>T (chromosome site 27411802), -379 G>A (27413104), -332 G>C (27413151, rs2214537), -237 A>T (27413246), and -53 G>A (27413430). There is no significant difference in MAF of each SNP between patients with KD and healthy controls except -237 A>T. Twenty five patients with KD had more than 1 SNP in contrast to only seven healthy controls had. The patients with KD have significantly more IL-21R gene polymorphisms than controls (odds ratio: 3.0, 95% confidence interval: 1.6-5.6, p=0.0005). There was no significant correlation between IL-21R gene polymorphisms and the serum level of IL-21. The serum level of total IgE was not significantly correlated with the presence of IL-21R gene polymorphisms. CONCLUSION: Our data suggest that the genetic susceptibility profile for KD may include IL-21R gene.
Gene Frequency ; Genetic Predisposition to Disease ; Humans ; Immunoglobulin E ; Interleukins ; Mucocutaneous Lymph Node Syndrome ; Polymerase Chain Reaction ; Polymorphism, Genetic ; Polymorphism, Single Nucleotide ; Promoter Regions, Genetic ; Receptors, Interleukin-21 ; Systemic Vasculitis

PURPOSE: The objective of this study was to evaluate the effect of microthreads on removal torque and bone-to-implant contact (BIC). METHODS: Twelve miniature pigs for each experiment, a total of 24 animals, were used. In the removal torque analysis, each animal received 2 types of implants in each tibia, which were treated with sandblasting and acid etching but with or without microthreads at the marginal portion. The animals were sacrificed after 4, 8, or 12 weeks of healing. Each subgroup consisted of 4 animals, and the tibias were extracted and removal torque was measured. In the BIC analysis, each animal received 3 types of implants. Two types of implants were used for the removal torque test and another type of implant served as the control. The BIC experiment was conducted in the mandible of the animals. The P1-M1 teeth were extracted, and after a 4-month healing period, 3 each of the 2 types of implants were placed, with one type on each side of the mandible, for a total of 6 implants per animal. The animals were sacrificed after a 2-, 4-, or 8-week healing period. Each subgroup consisted of 4 animals. The mandibles were extracted, specimens were processed, and BIC was analyzed. RESULTS: No significant difference in removal torque value or BIC was found between implants with and without microthreads. The removal torque value increased between 4 and 8 weeks of healing for both types of implants, but there was no significant difference between 8 and 12 weeks. The percentage of BIC increased between 2 and 4 weeks for all types of implants, but there was no significant difference between 4 and 8 weeks. CONCLUSIONS: The existence of microthreads was not a significant factor in mechanical and histological stability.
Animals ; Biomechanics ; Dental Implants ; Mandible ; Osseointegration ; Swine ; Tibia ; Tooth ; Torque

PURPOSE: We performed a multicenter, prospective study to evaluate the efficacy of imipramine and desmopressin to improve arousability and prevent nocturnal enuresis(NE). MATERIALS AND METHODS: The total of 48 children with NE were given questionnaires that included a scoring system for the assessing arousal from sleep. They were assigned into two groups: group 1(imipramine 25 mg, 3 girls and 16 boys, mean age 7.9 years), group 2(desmopressin 0.2 mg, 9 girls and 9 boys, mean age 7.5 years). The assessment of arousability was repeated 2 weeks and 4 weeks after medication. Eleven children were excluded because of incomplete data. RESULTS: Mean wetting events in group 1 decreased from 8.8 to 5.1 times(2 weeks) and 3.0 times(4 weeks)(p=0.009) versus 10.2 to 5.5 times(2 weeks) and 6.4 times(4 weeks)(p=0.007) for group 2. The mean threshold of arousability in group 1 was 4.9(baseline), 4.4(2 weeks), and 3.7(4 weeks), and, for group 2, 5.1(baseline), 4.8(2 weeks), and 4.8(4 weeks). The two groups were not different(p=0.14, p=0.73). CONCLUSIONS: Imipramine and desmopressin, which are commonly used in treating NE in Korea, influenced wetting events but not arousability.
Arousal ; Child ; Deamino Arginine Vasopressin ; Humans ; Imipramine ; Korea ; Nocturnal Enuresis ; Prospective Studies

PURPOSE: Nocternal enuresis is a common disorder. Tricyclic antidepressant and desmopressin have been accepted pharmacological treatment for this disorder. We conducted a cooperative study to investigate the efficacy and adverse reactions of imipramine, desmopressin and combination treatment in children with primary monosymptomatic nocturnal enuresis(PMNE). METHODS: Data from a large multicenter study were analysed. In the period of 8 months in 2002, the study comprised of 168 children(78 boys and 90 girls, 5 to 15 years old) with PMNE for imipramine, desmopressin or combination treatment. Before treatment a history, physical examination and laboratory tests were performed and the children were observed for 2 weeks. Response rate, adverse reactions and enuresis episodes after stopping drug administration were evaluated after 12-weeks of imipramine, desmopressin or combination of both. RESULTS: After 4 weeks, the frequency of bed wetting in all treated patients decreased during treatment significantly. Even though a 30-50% reduction in the number of wet nights were 68.6%, 74.4% and 86.1% during 12 weeks treatment by imipramine, desmopressin and both of them respectively, there was no significant difference between them. The most common adverse reaction was decreased appetite from imipramine administration. But no serious drug-related adverse events were reported. CONCLUSION: Efficacy of the combination therapy of imipramine and desmopressin in PMNE appears not to be better than either drug alone. It is necessary to pay attention on account of adverse reactions during imipramine treatment even though imipramine and desmopressin were generally well tolerated.
Appetite ; Child ; Deamino Arginine Vasopressin* ; Enuresis ; Female ; Humans ; Imipramine* ; Nocturnal Enuresis* ; Physical Examination

Hydrops fetalis is diagnosed when abnormal fluid collections are manifest in two or more fetal compartmnets including abdominal ascite, pleural effusion, percardial effusion, skin edema, polyhydroamniosis and placental edema. Although fetal hydrops was historically most commonly associated with Rh blood group isoimmunization, the availability of Rh immunoglobulin has increased the proportion of fetuses affected due to nonimmune etiologies. Neuroblastoma is a malignant tumor which originates in the autonomous nervous system. Congenital neuroblastoma is the most common solid malignant tumor of the neonatal period, incidence ranges 1:10,000 of all live births, retroperitoneal space being the most frequent localization. We have experienced a case of nonimmune hydrops fetalis with neuroblastoma at 32 weeks of gestation in 39 year old woman and reported that with brief review of related literatures.
Adult ; Edema ; Female ; Fetus ; Humans ; Hydrops Fetalis* ; Immunoglobulins ; Incidence ; Live Birth ; Nervous System ; Neuroblastoma* ; Pleural Effusion ; Pregnancy ; Retroperitoneal Space ; Skin

OBJECTIVE: Degradation of the extracellular matrix and the basement membrane is believed to be associated with tumor invasion and metastasis. To evaluate the roles of Matrix metalloproteinase-2 (MMP-2) in cervical cancer and its utility as a potential diagnostic tumor biomarker, we investigated the expression of MMP-2 in cervical cancer tissue and sera. METHODS: Through the zymography of MMP-2 in cervical cancer tissues from 35 patients, we investigated the extent and distribution of MMP-2. Also, up-regulation of its gene expression was examined by reverse transcription-polymerase chain reaction (RT-PCR) analysis. Then, we attempted to investigate by ELISA serologic responses in the sera obtained from 35 cervical cancer patients, and examined its utility as diagnostic and prognostic tumor marker in cervical cancer by comparing it with conventional tumor markers SCCA and CEA. RESULTS: In zymography, the active form of MMP-2 was detected in 66kDa. In cervical cancer the active form of MMP-2 is more strongly reacted with the substrates than those in normal tissues. Also, the mRNA of MMP-2 was more up-regulated in cervical cancer tissue than in normal one. MMP-2 was detected in the sera of cervical cancer patients, and its amount differed according to clinical situations. While it was not more effective than previous tumor markers SCCA and CEA in diagnosis, it was more useful in monitoring of the cancer. The effect was especially remarkable when it was combined with any other conventional tumor markers. CONCLUSIONS: The results suggest that the expressions of MMP-2 correlate with cervical cancer invasion and metastasis. Also, it may be useful in the monitoring of the cervical cancer lesion.
Basement Membrane ; Diagnosis ; Enzyme-Linked Immunosorbent Assay ; Extracellular Matrix ; Gene Expression ; Humans ; Matrix Metalloproteinase 2* ; Neoplasm Metastasis ; RNA, Messenger ; Biomarkers, Tumor ; Up-Regulation ; Uterine Cervical Neoplasms*