Purpose: To analyze electrocardiograms (ECGs) of patients with a salt-losing tubulopathy (SLT) and to determine the frequency and risk factors for long QT and arrhythmia. Methods: A total of 203 patients aged <19 years with SLT, specifically Bartter syndrome and Gitelman syndrome, who had a 12-lead ECG were included in this retrospective study. We analyzed the presence of an arrhythmia or prolonged corrected QT (QTc) on ECGs obtained for these patients. Demographic and laboratory data were compared between patients with abnormal and normal ECG findings. Results: Out of the 203 SLT patients, 38 (18.7%) underwent electrocardiography and 10 (40.0%) of 25 patients with inherited SLT had abnormal ECG findings, including prolonged QTc and arrhythmias. The abnormal ECG group had significantly lower serum potassium levels than the normal group (median [interquartile range]: 2.50 mmol/L [2.20–2.83] vs. 2.90 mmol/L [2.70–3.30], p=0.036), whereas other serum chemistry values did not show significant differences. The cutoff level for a significant difference in QTc interval was serum potassium level <2.50 mmol/L. One cardiac event occurred in a 13-year-old boy, who developed paroxysmal supraventricular tachycardia and underwent cardiac ablation. No sudden cardiac deaths occurred in this cohort. Conclusions The incidence of ECG abnormalities in patients with inherited SLT was 40.0%, whereas the ECG screening rate was relatively low (18.7%). Therefore, we recommend ECG screening in patients with inherited SLT, especially in those with serum potassium level <2.50 mmol/L.

PURPOSE: This study validated the musculoskeletal model of the human lower extremity by comparative study between calculated muscle parameters through simulation using modified hill-type model and measured muscle parameters through isokinetic exercise. The relationship between muscle forces and moments participated in motion was quantified from the results of simulation. MATERIALS AND METHODS: For simulation of isokinetic motion, a three-dimensional anatomical knee model was constructed using gait analysis. The EMG-force model was used to determine muscle activation level exciting muscles. The modified Hill-type model was used to calculate individual muscle force and moment in dynamic analysis. This method was validated by comparing analytical data with experimental data. RESULTS: The results showed that there was a significant correlation between calculated torques from simulation and measured torque from isokinetic motion experiments (R=0.97). We also found that muscle forces and moments during knee flexion and extension have nonlinearly proportional or inversely proportional relationship, since lower extremity muscles were simultaneously involved in flexion/extension motion and inner/outer rotation. CONCLUSION: We concluded that the simulation by using musculoskeletal model may be a useful mean to predict and recover musculoskeletal-related diseases, and analyze complicated experiment such as clash condition.
Gait ; Humans ; Knee Joint* ; Knee* ; Lower Extremity ; Muscles ; Torque

CCCTC-binding factor (CTCF) is a transcriptional regulator that binds to a complex DNA motif in various orientations and plays a crucial role in regulating gene expression, chromatin restructuring, and developmental processes. Mutations in the CTCF are associated with neurodevelopmental disorders. Here we report the first Korean case with a de novo heterozygous variant in the CTCF (c.1025G>A; p.Arg342His). She showed global developmental delay, failure to thrive, and dysmorphic face, which are phenotypes consistent with previous reports in the autosomal dominant intellectual developmental disorder 21 (MIM 615502). She also showed clinical features not previously reported, such as antral web and tracheobronchomalacia.Our case follows suit and expands understanding of this rare disorder by reporting common features and, on the other hand, unreported concomitant congenital anomalies.

PURPOSE: Patients with nursing home-acquired pneumonia (NHAP) should be treated as hospital-acquired pneumonia (HAP) according to guidelines published in 2005. However, controversy still exists on whether the high mortality of NHAP results from multidrug resistant pathogens or underlying disease. We aimed to outline differences and factors contributing to mortality between NHAP and community-acquired pneumonia (CAP) patients. MATERIALS AND METHODS: We retrospectively evaluated patients aged 65 years or older with either CAP or NHAP from 2008 to 2014. Patients with healthcare-associated pneumonia other than NHAP or HAP were excluded. RESULTS: Among 317 patients, 212 patients had CAP and 105 had NHAP. Patients with NHAP had higher mortality, more frequently used a ventilator, and had disease of higher severity than CAP. The incidences of aspiration, tube feeding, and poor functional status were higher in NHAP. Twenty three out of 54 NHAP patients and three out of 62 CAP patients had multidrug resistant pathogens (p<0.001). Eleven patients with NHAP died at discharge, compared to 7 patients with CAP (p=0.009). However, there was no association between mortality rate and presence of multidrug-resistant pathogens. The number of involved lobes on chest X-ray [odds ratio (OR)=1.708; 95% confidence interval (CI), 1.120 to 2.605] and use of mechanical ventilation (OR=9.537; 95% CI, 1.635 to 55.632) were significantly associated with in-hospital mortality. CONCLUSION: Patients with NHAP had higher mortality than patients with CAP. The excess mortality among patients with NHAP and CAP was related to disease severity but not to the presence of multidrug resistant pathogens.
Aged ; Aged, 80 and over ; Anti-Bacterial Agents/*therapeutic use ; Community-Acquired Infections/drug therapy/microbiology/mortality ; Cross Infection/drug therapy/*mortality ; *Drug Resistance, Multiple, Bacterial ; Female ; *Hospital Mortality ; Humans ; Male ; *Nursing Homes ; Odds Ratio ; Pneumonia, Bacterial/drug therapy/microbiology/*mortality ; Retrospective Studies

International migrants could be considered a risk group susceptible to vaccine-preventable diseases. We conducted a measles seroprevalence study among 419 marriage migrant women living in Sinan-gun and Wando-gun, South Jeolla Province, located in the southwestern part of Korea. The overall seroimmunity was 92.8%. The seroimmunity varied considerably according to the country of origin and increased with age. Our current analysis could be valuable in the context of discussions concerning vaccination policies for immigrants in Korea.

PURPOSE: The level of serum prostate-specific antigen (PSA) varies according to both age and race. It is known that the level of PSA increases with age, and that Asians have a lower incidence of prostate cancer and levels of PSA than Caucasians. In this study, the variation in the serum PSA level in samples collected from general populations were used to find an actual standard age-specific PSA reference range for Koreans. MATERIALS AND METHODS: Patients who received serum PSA level check-ups, between November 1998 and July 2005, at 8 domestic hospitals, were selected for the investigation. The PSA levels of 120,439 adult males, aged between 30 and 80 years, were measured, and those lower than 10ng/ml were analyzed. To estimate the increase in the level of serum PSA according to age, a simple linear regression analysis was carried out. RESULTS: The mean PSA level reference ranges according to age were 1.88, 1.92, 2.37, 3.56 and 5.19ng/ml for those in their thirties, forties, fifties, sixties and seventies, respectively. The rates of PSA change were 0.0023, 0.0175 and 0.0499 for those in their forties, fifties and sixties, respectively, indicating the rates of PSA level change increase steady with age, but these increases are greater for those in their fifties and most severe after their sixties. The level of age-specific PSA reference in Korean men was lower than that of men from Western countries. CONCLUSIONS: The age-specific PSA reference levels, as found in pre-existing literature, showed differences between races, with the overall reference levels being low for domestic data. The standard reference level of age-specific PSA for the screening of prostate cancer may be lower in Korean men than those from Western countries.
Adult ; Asian Continental Ancestry Group ; Continental Population Groups ; Humans ; Incidence ; Linear Models ; Male ; Mass Screening ; Prostate-Specific Antigen* ; Prostatic Neoplasms ; Reference Values*

BACKGROUND: Few clinical studies have clarified the prognostic factors that affect clinical outcomes for patients with relapsed or refractory diffuse large B-cell lymphoma (DLBCL) after immunochemotherapy. METHODS: A total of 158 patients with relapsed or refractory DLBCL were enrolled. All patients underwent positron emission tomography/computed tomography (PET/CT) before and after salvage therapy. All enrolled patients previously received the ifosfamide, carboplatin, and etoposide regimen. Clinical outcomes were compared according to several factors (age > or = 65 years, low age-adjusted International Prognostic Index [aa-IPI], maximum standardized uptake value [SUVmax] <6.0 on PET/CT, time to relapse > or =12 months, complete response after salvage therapy). A low aa-IPI, SUVmax <6.0, and time to relapse > or = 12 months were independent prognostic factors for survival. RESULTS: In univariate analysis and multivariate analysis, SUVmax below 6.0 (P<0.001 for progression-free survival (PFS), P<0.001 for overall survival (OS)) and low aa-IPI (P<0.001 for PFS, P<0.001 for OS) were independent prognostic factors associated with favorable outcome. CONCLUSION: The aa-IPI and initial SUVmax were powerful prognostic factors in patients with relapsed or refractory DLBCL.
Carboplatin ; Disease-Free Survival ; Electrons* ; Etoposide ; Humans ; Ifosfamide ; Lymphoma, B-Cell* ; Multivariate Analysis ; Positron-Emission Tomography ; Positron-Emission Tomography and Computed Tomography ; Recurrence ; Salvage Therapy