Open bite is accompanied by decrease in tooth contact and overbite, and causes collapse of occlusal plane, mastication difficulties, speech disorders, changes in appearance, and lower occlusal force than normal. Open bite caused by temporomandibular joint disorder in adults with complete occlusion must be corrected after removal or stabilization of the causative factors. Orthodontic treatment, occlusal adjustment, prosthetic treatment, and surgical treatment can be the option of occlusal correction. This report describes about estimating the cause of occlusion change in two patients who developed an open bite due to mandibular displacement in adults with complete occlusion and different treatment approaches accordingly. In one patient, satisfactory result was obtained in functional and esthetic aspects through occlusal adjustment after stabilization of the temporomandibular joint.

Background: Chronic kidney disease (CKD) has a negative impact on growth and development in children and is a risk factor for neurocognitive impairment; however, there is limited research on the cognitive function of children and adolescents with CKD. This study therefore aimed to investigate the mean intelligence and risk factors for low intelligence in children and adolescents with CKD. Methods: Eighty-one patients with CKD under 18 years old were included in the KoreaN cohort study for Outcomes in patients With Pediatric Chronic Kidney Disease (KNOW-Ped CKD). Participants completed either the Wechsler Intelligence Scale for Children (6–16 years), or Wechsler Adult Intelligence Scale (> 16 years). Results: The mean full-scale intelligence quotient (IQ) was 91 ± 19; 24.7% of participants scored a full-scale IQ below 80. Participants with a short stature (height Z scores < −1.88), failure to thrive (weight Z scores < −1.65), more severe CKD stage (≥ IIIb), longer duration of CKD (≥ 5 years), and those who were Medicare or Medicaid beneficiaries, had significantly lower mean full-scale IQs. Conclusion On linear regression analysis, the association between the full-scale IQ, and longer duration of CKD and growth failure, remained significant after controlling for demographic and clinical variables. It is therefore necessary to investigate cognitive impairment in pediatric patients with CKD who exhibit growth failure or for a longer postmorbid period. It is believed that early interventions, such as kidney transplantation, will have a positive effect on IQ in children with CKD, as the disease negatively affects IQ due to poor glomerular filtration rate over time.

Hyperammonemia is mainly caused by diseases related to liver failure. However, there are also non-hepatic causes of hyperammonemia, such as urinary tract infection (UTI) due to urease-producing organisms. Urease production by these bacteria induces a hydrolysis of urinary urea into ammonia that can cross the urothelial cell membrane and diffuse into blood vessels, leading to hyperammonemia. Delayed diagnosis and treatment of hyperammonemia can lead to lethal encephalopathy that can cause brain damage and life-threatening conditions. In the presence of obstructive uropathy, UTI by urease-producing bacteria can lead to more severe hyperammonemia due to enhanced resorption of ammonia into the systemic circulation. In this report, we present a case of acute severe hyperammonemic encephalopathy leading to brain death due to accumulation of ammonia in blood caused by Morganella morganii UTI in a 10-year-old girl with cloacal anomaly, causing obstructive uropathy even after multiple corrections.

Background: Chronic kidney disease (CKD) has a negative impact on growth and development in children and is a risk factor for neurocognitive impairment; however, there is limited research on the cognitive function of children and adolescents with CKD. This study therefore aimed to investigate the mean intelligence and risk factors for low intelligence in children and adolescents with CKD. Methods: Eighty-one patients with CKD under 18 years old were included in the KoreaN cohort study for Outcomes in patients With Pediatric Chronic Kidney Disease (KNOW-Ped CKD). Participants completed either the Wechsler Intelligence Scale for Children (6–16 years), or Wechsler Adult Intelligence Scale (> 16 years). Results: The mean full-scale intelligence quotient (IQ) was 91 ± 19; 24.7% of participants scored a full-scale IQ below 80. Participants with a short stature (height Z scores < −1.88), failure to thrive (weight Z scores < −1.65), more severe CKD stage (≥ IIIb), longer duration of CKD (≥ 5 years), and those who were Medicare or Medicaid beneficiaries, had significantly lower mean full-scale IQs. Conclusion On linear regression analysis, the association between the full-scale IQ, and longer duration of CKD and growth failure, remained significant after controlling for demographic and clinical variables. It is therefore necessary to investigate cognitive impairment in pediatric patients with CKD who exhibit growth failure or for a longer postmorbid period. It is believed that early interventions, such as kidney transplantation, will have a positive effect on IQ in children with CKD, as the disease negatively affects IQ due to poor glomerular filtration rate over time.

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.

BACKGROUND: Primary hyperoxaluria (PH), a rare inborn error of glyoxylate meta bolism causing overproduction of oxalate, is classified into three genetic subgroups: type 1–3 (PH1–PH3) caused by AGXT, GRHPR , and HOGA1 gene mutations, respectively. We performed a retrospective case series study of Korean pediatric patients with PH.METHODS: In total, 11 unrelated pediatric patients were recruited and their phenotypes and genotypes were analyzed by a retrospective review of their medical records.RESULTS: Mutational analyses revealed biallelic AGXT mutations (PH1) in nine patients and a single heterozygous GRHPR and HOGA1 mutation in one patient each. The c.33dupC was the most common AGXT mutation with an allelic frequency of 44%. The median age of onset was 3 months (range, 2 months-3 years), and eight patients with PH1 presented with end stage renal disease (ESRD). Patients with two truncating mutations showed an earlier age of onset and more frequent retinal involvement than patients with one truncating mutation. Among eight PH1 patients presenting with ESRD, five patients were treated with intensive dialysis followed by liver transplantation (n=5) with/without subsequent kidney transplantation (n=3).CONCLUSION: Most patients presented with severe infantile forms of PH. Patients with two truncating mutations displayed more severe phenotypes than those of patients with one truncating mutation. Sequential liver and kidney transplantation was adopted for PH1 patients presenting with ESRD. A larger nation-wide multicenter study is needed to confirm the genotype-phenotype correlations and outcomes of organ transplantation.
Age of Onset ; Dialysis ; Genetic Association Studies ; Genotype ; Humans ; Hydrogen-Ion Concentration ; Hyperoxaluria, Primary ; Kidney Failure, Chronic ; Kidney Transplantation ; Liver ; Liver Transplantation ; Medical Records ; Organ Transplantation ; Phenotype ; Retinaldehyde ; Retrospective Studies ; Transplants

BACKGROUND: The purpose of this study was to investigate the relationship between metabolic syndrome and depressive symptoms by administering the nine-item depression module from the Patient Health Questionnaire-9 (PHQ-9) to participants from the general population of Korea.METHODS: In total, 8,150 adults participated in the 1st year of the 7th Korea National Health and Nutrition Examination Survey, which was conducted in 2016. Of them, 5,556 participants underwent tests pertaining to the criteria for metabolic syndrome and responded to PHQ-9; 2,594 respondents were excluded. Analysis of covariance was performed to analyze the relationship between the presence of metabolic syndrome and the PHQ-9 score after adjusting for the effects of demographic and hematologic characteristics and underlying diseases.RESULTS: The total PHQ-9 score (mean=2.98) was significantly higher in participants with metabolic syndrome than in those without it (mean=2.59) (p=0.002). Among the individual PHQ-9 items, changes in sleep, thoughts of suicide or self-harm, and depressive mood showed the greatest differences.CONCLUSION: The PHQ-9 scores in Korea were higher in adults with metabolic syndrome, suggesting an association between metabolic syndrome and depressive symptoms.
Adult ; Depression ; Humans ; Korea ; Nutrition Surveys ; Suicide ; Surveys and Questionnaires

OBJECTIVES: The purpose of this study is to inquire into the relationship between the association of suicidal ideation and the plasma C-reactive protein level in the general population.METHODS: This study selected a total of 5,090 subjects who have responded to the survey item on suicidal ideation status, and received the plasma C-reactive protein test, as a research subject using the 2015 data of the National Health and Nutrition Survey. This study conducted a covariance analysis by correcting a potential influence of demographic and hematological factors. Besides, this study intended to define the cut-off value of the optimum plasma C-reactive protein level, which once identified can distinguish between a subject with and without suicidal ideation using the model of the decision tree.RESULTS: The Plasma C-reactive protein level of a subject having suicidal ideation was noted as being significantly higher than the one having no suicidal ideation in the covariance analysis (p=0.046). In addition, the proper cut-off value of the plasma C-reactive protein level between the subjects with and without suicidal ideation was found to be 1.30 mg/L (p=0.003).CONCLUSION: It is noted that the high plasma C-reactive protein level showed a significant correlation with individuals with noted suicidal ideation. In addition, this study has significance in that it presented that the plasma C-reactive protein concentration has the possibility as an accurate and significant marker of suicidal ideation in the general population.
C-Reactive Protein ; Decision Trees ; Humans ; Nutrition Surveys ; Plasma ; Research Subjects ; Suicidal Ideation

To identify the Helicobacter pylori antigens operating during early infection in sera from infected infants using proteomics and immunoblot analysis. Two-dimensional (2D) large and small gel electrophoresis was performed using H. pylori strain 51. We performed 2D immunoglobulin G (IgG), immunoglobulin A (IgA), and immunoglobulin M (IgM) antibody immunoblotting using small gels on sera collected at the Gyeongsang National University Hospital from 4–11-month-old infants confirmed with H. pylori infection by pre-embedding immunoelectron microscopy. Immunoblot spots appearing to represent early infection markers in infant sera were compared to those of the large 2D gel for H. pylori strain 51. Corresponding spots were analyzed by matrix-assisted laser desorption/ionization time of flight-mass spectrometry (MALDI-TOF-MS). The peptide fingerprints obtained were searched in the National Center for Biotechnology Information (NCBI) database. Eight infant patients were confirmed with H. pylori infection based on urease tests, histopathologic examinations, and pre-embedding immunoelectron microscopy. One infant showed a 2D IgM immunoblot pattern that seemed to represent early infection. Immunoblot spots were compared with those from whole-cell extracts of H. pylori strain 51 and 18 spots were excised, digested in gel, and analyzed by MALDI-TOF-MS. Of the 10 peptide fingerprints obtained, the H. pylori proteins flagellin A (FlaA), urease β subunit (UreB), pyruvate ferredoxin oxidoreductase (POR), and translation elongation factor Ts (EF-Ts) were identified and appeared to be active during the early infection periods. These results might aid identification of serological markers for the serodiagnosis of early H. pylori infection in infants.
Biotechnology ; Electrophoresis ; Flagellin ; Gels ; Helicobacter pylori* ; Helicobacter* ; Humans ; Immunoblotting ; Immunoglobulin A ; Immunoglobulin G ; Immunoglobulin M ; Infant* ; Microscopy, Immunoelectron ; Peptide Elongation Factors ; Peptide Mapping ; Proteomics ; Pyruvate Synthase ; Serologic Tests ; Spectrum Analysis ; Urease

Cystinuria is an inherited disorder characterized by defective renal reabsorption of cystine and dibasic amino acids leading to nephrolithiasis. This study was conducted to analyze the genotypes and phenotypes of pediatric patients with cystinuria. Eight children from Seoul National University Hospital and Asan Medical Center presenting with cystinuria from January 2003 to June 2016 were retrospectively analyzed. Mutational studies were performed by direct sequencing. Two of the 8 were male and 6 were female. The median ages at onset and diagnosis were 1.5 (range, 0.3–13.6) and 2.6 (range, 0.7–16.7) years, respectively. The median followed up was 7.7 (range, 3.4–14.0) years. Mutational analyses were performed in 7 patients and revealed biallelic SLC3A1 mutations (AA genotype) in 4 patients, a single heterozygous SLC3A1 mutation (A- genotype) in 1 patient, biallelic SLC7A9 mutations (BB genotype) in 1 patient, and a single heterozygous SLC7A9 mutation (B- genotype) in 1 patient. Two of the mutations were novel. No genotype-phenotype correlations were observed, except for earlier onset age in patients with non-AA genotypes than in patients with the AA genotype. All patients suffered from recurrent attacks of symptomatic nephrolithiasis, which lead to urologic interventions. At the last follow-up, 3 patients had a mild-to-moderate degree of renal dysfunction. This is the first study of genotypic and phenotypic analyses of patients with cystinuria in Korea.
Age of Onset ; Amino Acids, Diamino ; Child ; Chungcheongnam-do ; Cystine ; Cystinuria* ; Diagnosis ; Female ; Follow-Up Studies ; Genetic Association Studies ; Genotype* ; Humans ; Korea ; Male ; Nephrolithiasis ; Phenotype* ; Renal Reabsorption ; Retrospective Studies ; Seoul