Purpose: In South Korea, investigations into Turner syndrome (TS) prevalence and TS-associated cancer and mortality are lacking.Accurate data were estimated from the National Health Insurance Service (NHIS) and the Rare Diseases Registry (RDR) records. Materials and Methods: Data on patients with TS who were registered in the RDR between 2007 and 2017 were collected. To estimate TS-associated cancer and mortality risk, the data were compared with data of 1:3 age-matched controls. Results: In 2017, 2054 patients with TS were identified from a total population of 26186952 South Korean women; therefore, the prevalence was 7.84 per 100000 persons. TS prevalence across 10-year interval age groups were 11.82, 23.17, 18.37, 10.49, 4.09, and 0.38 for age under 10 years, teenagers, 20s, 30s, 40s, and older than 50, respectively (per 100000 persons). The cancer risk in patients with TS was higher than that of age-matched controls over 5.3 person-years [hazard ratio (HR)=1.82, 95% confidence interval (CI) 1.01–3.27, p=0.045]. Among different types of cancer, thyroid cancer risk in patients with TS was significantly higher than that of age-matched controls (HR=2.78, 95% CI 1.06–7.26, p=0.037). We also observed that TS-associated all-cause mortality risk was higher than that of age-matched controls (HR=3.36, 95% CI 1.59–7.10, p=0.002). Conclusion National prevalence of TS was suggested, and an increased risk of TS-associated thyroid cancer and mortality were observed in this study.

Purpose: In South Korea, investigations into Turner syndrome (TS) prevalence and TS-associated cancer and mortality are lacking.Accurate data were estimated from the National Health Insurance Service (NHIS) and the Rare Diseases Registry (RDR) records. Materials and Methods: Data on patients with TS who were registered in the RDR between 2007 and 2017 were collected. To estimate TS-associated cancer and mortality risk, the data were compared with data of 1:3 age-matched controls. Results: In 2017, 2054 patients with TS were identified from a total population of 26186952 South Korean women; therefore, the prevalence was 7.84 per 100000 persons. TS prevalence across 10-year interval age groups were 11.82, 23.17, 18.37, 10.49, 4.09, and 0.38 for age under 10 years, teenagers, 20s, 30s, 40s, and older than 50, respectively (per 100000 persons). The cancer risk in patients with TS was higher than that of age-matched controls over 5.3 person-years [hazard ratio (HR)=1.82, 95% confidence interval (CI) 1.01–3.27, p=0.045]. Among different types of cancer, thyroid cancer risk in patients with TS was significantly higher than that of age-matched controls (HR=2.78, 95% CI 1.06–7.26, p=0.037). We also observed that TS-associated all-cause mortality risk was higher than that of age-matched controls (HR=3.36, 95% CI 1.59–7.10, p=0.002). Conclusion National prevalence of TS was suggested, and an increased risk of TS-associated thyroid cancer and mortality were observed in this study.

Objective: The purpose of this study is to investigate an association between sex-hormone exposure during fetal period and the development of Alzheimer’s disease (AD). Methods: Twenty four AD patients and 25 normal controls were included from Memory Impairment Clinic of Pusan National University Hospital in Korea. Subjects are measured the ratio of the length of the second to the fourth digit (2D/4D) to estimate fetal sex-hormone exposure. Analysis of covariance was used to investigate an association between fetal sex-hormone exposure and the development of AD after adjusting age, education level, Korean Mini-Mental Status Examination and head circumference. Results: We found that the females with AD had significantly lower 2D/4D ratios than the female controls. In the females withAD, the 2D/4D ratios in the left hand was 0.021 and 0.017 in the right hand compared with the controls. Conclusion Our findings suggest that the development of AD in female may be related with higher testosterone and lower es-trogen exposure during fetal period.

BACKGROUND/AIMS: Previous studies have reported a high rate of sustained virologic response (SVR) and a low rate of serious adverse events with the use of daclatasvir (DCV) and asunaprevir (ASV) combination therapy. We evaluated the efficacy and safety of DCV and ASV combination therapy for patients with chronic hepatitis C virus (HCV) genotype 1b infection in real world. METHODS: We enrolled 278 patients (184 treatment-naïve patients) from five hospitals in Daegu and Gyeongsangbuk-do. We evaluated the rates of rapid virologic response (RVR), end-of-treatment response (ETR), and SVR at 12 weeks after completion of treatment (SVR12). Furthermore, we investigated the rate of adverse events and predictive factors of SVR12 failure. RESULTS: The mean age of patients was 59.5 ± 10.6 years, and 140 patients (50.2%) were men. Seventy-seven patients had cirrhosis. Baseline information regarding nonstructural protein 5A (NS5A) sequences was available in 268 patients. Six patients presented with pretreatment NS5A resistance-associated variants. The RVR and the ETR rates were 96.6% (258/267) and 95.2% (223/232), respectively. The overall SVR12 rate was 91.6% (197/215). Adverse events occurred in 17 patients (7.9%). Six patients discontinued treatment because of liver enzyme elevation (n = 4) and severe nausea (n = 2). Among these, four achieved SVR12. Other adverse events observed were fatigue, headache, diarrhea, dizziness, loss of appetite, skin rash, and dyspnea. Univariate analysis did not show significant predictive factors of SVR12 failure. CONCLUSIONS DCV and ASV combination therapy showed high rates of RVR, ETR, and SVR12 in chronic HCV genotype 1b-infected patients in real world and was well tolerated without serious adverse events.

PURPOSE: Therapeutic hypothermia (TH) has become a standard strategy for reducing brain damage in the postresuscitation period. The aim of this study is to investigate the outcomes and current performance of TH with out-of-hospital cardiac arrest (OHCA) survivors through the Korean hypothermia network (KORHN) registry. METHODS: We used the KORHN registry, a web-based, multicenter registry that includes 24 participating hospitals throughout the Republic of Korea. Adult comatose OHCA survivors treated with TH from 2007 to 2012 were included. The primary outcomes were neurologic outcome at hospital discharge and in-hospital mortality. The secondary outcomes were TH performance and adverse events during TH. RESULTS: A total of 930 patients were included; of these, 556 (59.8%) patients survived to discharge and 249 (26.8%) were discharged with good neurologic outcomes. The median time from return of spontaneous circulation (ROSC) to the start of TH was 101 (interquartile range (IQR): 46-200) minutes. The induction, maintenance, and rewarming durations were 150 (IQR: 80-267) minutes, 1440 (IQR: 1290-1440) minutes, and 708 (IQR: 420-900) minutes, respectively. The time from the ROSC to coronary angiography was 1,045 (IQR: 121-12,051) hours. Hyperglycemia (46.3%) was the most frequent adverse event. CONCLUSION: Over one quarter of OHCA survivors (26.8%) were discharged with good neurologic outcome. TH performance was managed appropriately in terms of the factors related to the timing of TH, which were the start time for cooling and the rewarming duration.
Adult ; Brain ; Coma ; Coronary Angiography ; Hospital Mortality ; Humans ; Hyperglycemia ; Hypothermia* ; Hypothermia, Induced ; Korea ; Out-of-Hospital Cardiac Arrest* ; Registries ; Republic of Korea ; Rewarming ; Survivors

The publisher wishes to apologize for incorrectly displaying the author (Seok Beom Gwon) name. We correct his name from Seok Beom Gwon to Seok Beom Kwon.

BACKGROUND AND PURPOSE: Charcot-Marie-Tooth disease (CMT) type 1A (CMT1A) is the demyelinating form of CMT that is significantly associated with PMP22 duplication. Some studies have found that the disease-related disabilities of these patients are correlated with their compound muscle action potentials (CMAPs), while others have suggested that they are related to the nerve conduction velocities. In the present study, we investigated the correlations between the disease-related disabilities and the electrophysiological values in a large cohort of Korean CMT1A patients. METHODS: We analyzed 167 CMT1A patients of Korean origin with PMP22 duplication using clinical and electrophysiological assessments, including the CMT neuropathy score and the functional disability scale. RESULTS: Clinical motor disabilities were significantly correlated with the CMAPs but not the motor nerve conduction velocities (MNCVs). Moreover, the observed sensory impairments matched the corresponding reductions in the sensory nerve action potentials (SNAPs) but not with slowing of the sensory nerve conduction velocities (SNCVs). In addition, CMAPs were strongly correlated with the disease duration but not with the age at onset. The terminal latency index did not differ between CMT1A patients and healthy controls. CONCLUSIONS: In CMT1A patients, disease-related disabilities such as muscle wasting and sensory impairment were strongly correlated with CMAPs and SNAPs but not with the MNCVs or SNCVs. Therefore, we suggest that the clinical disabilities of CMT patients are determined by the extent of axonal dysfunction.
Action Potentials ; Axons ; Charcot-Marie-Tooth Disease ; Cohort Studies ; Humans ; Muscles ; Neural Conduction

Streptococcal toxic shock syndrome (STSS) is an acute, progressive illness that manifests with fever, hypotension, and accelerated multi-organ failure. It is usually caused by Group A Streptococcus (Streptococcus pyogenes). STSS due to non-group A streptococci is rare, but its incidence has recently increased. We report here on two cases of STSS caused by Group B Streptococcus (Streptococcus agalactiae) and Group G Streptococcus (Streptococcus dysagalactiae).
Fever ; Hypotension ; Incidence ; Shock, Septic ; Streptococcus ; Streptococcus agalactiae

PURPOSE: To evaluate the clinical results between interlocking intramedullary nail with fibular fixation and nail only for treating distal tibiofibular diaphyseal fractures. MATERIALS AND METHODS: From March 2003 to September 2006, 19 distal tibiofibular fractures were antegrade nailed after anatomical reduction and fixation of fibular fractures, and another 37 fractures fixed with nails only. Average age of patients was 48.6 years. These two groups were compared by VAS (visual analogue scale)&ankle ROM according to degree of comminution and fracture configuration. The statistical analysis was evaluated by t-test. RESULTS: There was no statistical difference between fibular fixation group and non-fixation group in VAS score according to fracture comminution and configuration (p>0.05). However, compared according to fracture configuration, mean ankle eversion of fibular fixation group in oblique fractures was 18.3 degrees, and that of non-fixation group was 12.5 degrees (p<0.05). In addition, mean ankle plantar flexion, dorsiflexion, inversion and total ankle ROM of fibular fixation group in spiral fractures was 40.0, 20.0, 30.0 and 108.3 degrees of each and that of non-fixation group was 38.3, 18.5, 27.0 and 101.7 degrees (p<0.05). CONCLUSIONS: In oblique and spiral fractures of distal tibiofibular diaphysis, interlocking intramedullary nail with fibular fixation had the advantage in postoperative ankle ROM. So, it can be a worthy method for the treatment of distal tibiofibular diaphyseal fractures.
Animals ; Ankle ; Diaphyses ; Fracture Fixation, Intramedullary ; Humans ; Imidazoles ; Nails ; Nitro Compounds

BACKGROUND AND OBJECTIVES It is being increasing recognized that the morbidities of migraine and balance disorders are interrelated. In fact, migrainous vertigo (MV) is one of frequent causes of recurrent vertigo in patients presenting to specialized dizziness clinics. Nevertheless, not many studies have reported clinical manifestations and treatment. Therefore, the aim of study was designed to assess clinical features and treatment patterns by a nationwide multicenter study. MATERIALS AND METHODS Patients between 9 and 74 years of age who visited 17 Korean tertiary referral centers and 1 clinic from February to March 2009 were investigated using two forms of questionnaires. RESULTS Overall, 318 patients with MV were enrolled. MV was responsible for ~8.45% of visits to the specialized dizziness clinics. One hundred seventy-five of these patients had definite MV and were included in assessing the clinical features. Vertigo characteristics of patients with definite MV were various. Vertigo was regularly as sociated with headache in 87% of the patients. The duration of vertigo ranged from seconds to days. For the treatment patterns, an acute and prophylactic therapies were carried in most clinics. There were no differences in either acute or prophylactic therapies between department of neurology and otorhinolaryngology. CONCLUSIONS The results of this study suggest that MV the clinical features of MV also varies in Korea. In addition, most clinics provide similar patterns of practice in treatment for MV. The syndrome of MV deserves further research activity as it is relatively common and clinically relevant.
Dizziness ; Headache ; Humans ; Korea ; Migraine Disorders ; Neurology ; Prospective Studies ; Tertiary Care Centers ; Vertigo