Background/Aims: Sorafenib is the standard of care in the management of advanced hepatocellular carcinoma (HCC). The purpose of this study was to investigate the characteristics, treatment patterns and outcomes of sorafenib among HCC patients in South Korea. Methods: This population-based retrospective, single-arm, observational study used the Korean National Health Insurance database to identify patients with HCC who received sorafenib between July 1, 2008, and December 31, 2014. A total of 9,923 patients were recruited in this study. Results: Among 9,923 patients, 6,669 patients (68.2%) received loco-regional therapy prior to sorafenib, and 1,565 patients (15.8%) received combination therapy with concomitant sorafenib;2,591 patients (26.1%) received rescue therapy after sorafenib, and transarterial chemoembolization was the most common modality applied in 1,498 patients (15.1%). A total of 3,591 patients underwent rescue therapy after sorafenib, and the median overall survival was 14.5 months compared to 4.6 months in 7,332 patients who received supportive care after sorafenib. The mean duration of sorafenib administration in all patients was 105.7 days; 7,023 patients (70.8%) received an initial dose of 600 to 800 mg. The longest survival was shown in patients who received the recommended dose of 800 mg, subsequently reduced to 400 mg (15.0 months). The second longest survival was demonstrated in patients with a starting dose of 800 mg, followed by a dose reduction to 400–600 mg (9.6 months). Conclusions Real-life data show that the efficacy of sorafenib seems similar to that observed in clinical trials, suggesting that appropriate subsequent therapy after sorafenib might prolong patient survival.

Background and Objectives: Menkes disease (MNK) is a rare X-linked recessive disease, caused by mutations in the copper transporting ATP7A gene that is required for copper homeostasis. MNK patients experience various clinical symptoms including neurological defects that are closely related to the prognosis of MNK patients. Neural stem cells (NSCs) in the hippocampal dentate gyrus (DG) produce new neurons throughout life, and defects in DG neurogenesis are often correlated with cognitive and behavioral problems. However, neurodevelopmental defects in the DG during postnatal period in MNK have not been understood yet. Methods: and Results: Mottled-brindled (Mo Br/y ) mice (MNK mice) and littermate controls were used in this study. In vivo microCT imaging and immunohistochemistry results demonstrate that blood vasculatures in hippocampus are abnormally decreased in MNK mice. Furthermore, postnatal establishment of NSC population and their neurogenesis are severely compromised in the DG of MNK mice. In addition, in vitro analyses using hippocampal neurosphere culture followed by immunocytochemistry and immunoblotting suggest that neurogenesis from MNK NSCs is also significantly compromised, corresponding to defective neurogenic gene expression in MNK derived neurons. Conclusions Our study is the first reports demonstrating that improper expansion of the postnatal NSC population followed by significant reduction of neurogenesis may contribute to neurodevelopmental symptoms in MNK. In conclusion, our results provide new insight into early neurodevelopmental defects in MNK and emphasize the needs for early diagnosis and new therapeutic strategies in the postnatal central nerve system damage of MNK patients.

Objectives: . Studies on the aeroallergen sensitization status of South Koreans based on large-scale data are lacking. Methods: . We analyzed data from 368,156 multiple allergosorbent tests collected by a domestic medical diagnosis company from 3,735 hospitals nationwide from 2018 to 2021. We additionally collected sex, age, and regional data. If the level of an aeroallergen was 0.35 IU/mL or more, the test result for that aeroallergen was defined as positive, and positive cases were defined as those where one aeroallergen was positive. The positive ratio (PR) for aeroallergens was calculated using positive cases. Results: . In total, 347,996 cases were analyzed, excluding cases with missing data. The percentage of positive cases was 56.7%, which was highest in adolescents (74.1%) and lowest in the elderly (47.0%). All four types of mites had high PRs (0.382–0.655), and mold had low PRs (0.023–0.058). Among pollens, the PRs of grasses were generally high (more than 0.14), followed by weeds (approximately 0.10), and the PRs of woods was less than 0.1. For animals, cats and dogs had the highest PRs, at 0.231 and 0.183, respectively. The value for cockroaches was also high, at 0.211. The PRs of indoor aeroallergens, such as mites, molds, and animals, were high in adolescents, and those of pollen and cockroaches were high in the elderly. In Jeju, the PR of Japanese cedars was extremely high (0.222). Conclusion . Koreans were found to be sensitized to a wide variety of aeroallergens. There were significant differences in sensitization patterns according to age and region.

Bony deformities and fragility fractures in end-stage renal disease (ESRD) patients on long-term hemodialysis can be caused by either osteoporosis or chronic kidney disease-mineral and bone disorder (CKD-MBD). Correct identification of the underlying mechanism is critical since the treatment methods differ, and one treatment approach could negatively affect the other. Cervical kyphosis, severe enough to require immediate surgical treatment, can be caused by uncontrolled CKD-MBD, albeit in limited cases. This report presents the case of a 61-year-old female with an 11-year history of hemodialysis treatment and severe cervical kyphosis with myelopathy, which required 2-stage spinal surgeries. Our report calls for a careful diagnostic approach in ESRD patients with skeletal disorders, the points to consider before calcium replacement, and early detection of fragility fractures in them. Moreover, early mobilization and weight-bearing after the surgical procedure may lead to better neurological and functional improvements.

Background: This study aimed to determine the parameters for worsening oxygenation in non-severe coronavirus disease 2019 (COVID-19) pneumonia. Methods: This retrospective cohort study included cases of confirmed COVID-19 pneumonia in a public hospital in South Korea. The worsening oxygenation group was defined as that with SpO2 ≤94% or received oxygen or mechanical ventilation (MV) throughout the clinical course versus the non-worsening oxygenation group that did not experience any respiratory event. Parameters were compared, and the extent of viral pneumonia from an initial chest computed tomography (CT) was calculated using artificial intelligence (AI) and measured visually by a radiologist. Results: We included 136 patients, with 32 (23.5%) patients in the worsening oxygenation group; of whom, two needed MV and one died. Initial vital signs and duration of symptoms showed no difference between the two groups; however, univariate logistic regression analysis revealed that a variety of parameters on admission were associated with an increased risk of a desaturation event. A subset of patients was studied to eliminate potential bias, that ferritin ≥280 μg/L (p=0.029), lactate dehydrogenase ≥240 U/L (p=0.029), pneumonia volume (p=0.021), and extent (p=0.030) by AI, and visual severity scores (p=0.042) were the predictive parameters for worsening oxygenation in a sex-, age-, and comorbid illness-matched case-control study using propensity score (n=52). Conclusion Our study suggests that initial CT evaluated by AI or visual severity scoring as well as serum markers of inflammation on admission are significantly associated with worsening oxygenation in this COVID-19 pneumonia cohort.

PURPOSE: Discovery of models predicting the exact prognosis of epithelial ovarian cancer (EOC) is necessary as the first step of implementation of individualized treatment. This study aimed to develop nomograms predicting treatment response and prognosis in EOC. MATERIALS AND METHODS: We comprehensively reviewed medical records of 866 patients diagnosed with and treated for EOC at two tertiary institutional hospitals between 2007 and 2016. Patients’ clinico-pathologic characteristics, details of primary treatment, intra-operative surgical findings, and survival outcomes were collected. To construct predictive nomograms for platinum sensitivity, 3-year progression-free survival (PFS), and 5-year overall survival (OS), we performed stepwise variable selection by measuring the area under the receiver operating characteristic curve (AUC) with leave-one-out cross-validation. For model validation, 10-fold cross-validation was applied. RESULTS: The median length of observation was 42.4 months (interquartile range, 25.7 to 69.9 months), during which 441 patients (50.9%) experienced disease recurrence. The median value of PFS was 32.6 months and 3-year PFS rate was 47.8% while 5-year OS rate was 68.4%. The AUCs of the newly developed nomograms predicting platinum sensitivity, 3-year PFS, and 5-year OS were 0.758, 0.841, and 0.805, respectively. We also developed predictive nomograms confined to the patients who underwent primary debulking surgery. The AUCs for platinum sensitivity, 3-year PFS, and 5-year OS were 0.713, 0.839, and 0.803, respectively. CONCLUSION: We successfully developed nomograms predicting treatment response and prognosis of patients with EOC. These nomograms are expected to be useful in clinical practice and designing clinical trials.
Area Under Curve ; Disease-Free Survival ; Humans ; Medical Records ; Nomograms ; Ovarian Neoplasms ; Platinum ; Prognosis ; Recurrence ; ROC Curve

OBJECTIVE: To evaluate the feasibility of robot-assisted laparoscopic myomectomy in multiple myomas over 10. METHODS: A retrospective study was conducted for 662 patients who underwent robot-assisted laparoscopic myomectomy and open myomectomy by a single operator in a tertiary university hospital. RESULTS: A total of 30 women underwent removal of 10 or more uterine myomas by robotics and 13 patients were selected for this study. The average number of myomas removed was 13.7 (range 10–20). The maximum diameter of the myomas was 6.8 cm (range 5.0–10.0 cm). The sum of the diameters of each myoma was 34.7 cm (range 20.0–54.5 cm) and the mass of resected myomas for each case was 229.1 g (range 106.8–437.9 g). In no case was the robotic procedure converted into conventional laparoscopy or laparotomy, and all patients recovered without any major complications. In comparison with 13 cases of open myomectomy during the same period, robotic surgery took longer time than open surgery (360.5 vs. 183.8 minutes; P=0.001) but had shorter postoperative hospital days after surgery (mean 2.5 vs. 3.5 days; P=0.003). CONCLUSION: Robot-assisted laparoscopic myomectomy could be an alternative to laparotomic myomectomy for numerous myomas over 10 in number.
Female ; Humans ; Laparoscopy ; Laparotomy ; Leiomyoma ; Minimally Invasive Surgical Procedures ; Myoma ; Retrospective Studies ; Robotic Surgical Procedures ; Robotics ; Uterine Myomectomy

We report a case of successful heart transplantation after 67 days of support with venoarterial extracorporeal membrane oxygenation (ECMO) in a patient who underwent surgery for type A aortic dissection and myocardial infarction complicated by irreversible myocardial damage and a deep sternal wound infection. During ECMO support, left heart vent and distal limb perfusion were performed. Mediastinitis was treated with mediastinal washout and irrigation. Multiple complications from peripheral ECMO were successfully managed.
Cardiomyopathies ; Extracorporeal Membrane Oxygenation* ; Extremities ; Heart Transplantation* ; Heart* ; Humans ; Mediastinitis* ; Myocardial Infarction ; Perfusion ; Shock ; Transplantation ; Wound Infection

PURPOSE: Behcet's disease (BD) is a chronic inflammatory disease characterized by orogenital ulcers, skin and ocular lesions, in addition to articular, vascular, and neurologic symptoms. Carpal tunnel syndrome (CTS), can also occur in BD patients secondary to inflammation in the connective tissues, vessels, and tendons, as well as nerve involvement in BD itself. However, reports of patients who have CTS in BD are rare. The aim of this study was to evaluate the clinical characteristics of CTS in BD patients. MATERIALS AND METHODS: Retrospective analysis of the medical records of 1750 BD patients, and 14 (0.8%) BD patients who were diagnosed with CTS was performed at the BD Specialty Clinic of Severance Hospital. Patient demographics, disease activity/severity for both diseases, and the clinical characteristics of CTS in BD were recorded and analyzed. RESULTS: All 14 BD patients with CTS were women. Twelve patients (85.7%) were diagnosed with active BD. The CTS was mild in 8 patients (57.2%), moderate in 3 patients (21.4%), and severe in 3 patients (21.4%). Ten patients (71.4%) had BD prior to the diagnosis of CTS, and these 10 patients all had active BD. CONCLUSION: CTS can occur as a result of the inflammation associated with BD and can also be the presenting symptom of nerve involvement in BD. CTS can also develop as the initial symptom of BD. Therefore, a higher degree of suspicion should be maintained for CTS in patients with BD and vice versa; however, the exact relationship is uncertain.
Adult ; Age Distribution ; Aged ; Behcet Syndrome/complications/diagnosis/*epidemiology ; Carpal Tunnel Syndrome/diagnosis/*epidemiology ; Female ; Humans ; Inflammation ; Male ; Middle Aged ; Retrospective Studies ; Sex Distribution

We report a case of successful heart transplantation after 67 days of support with venoarterial extracorporeal membrane oxygenation (ECMO) in a patient who underwent surgery for type A aortic dissection and myocardial infarction complicated by irreversible myocardial damage and a deep sternal wound infection. During ECMO support, left heart vent and distal limb perfusion were performed. Mediastinitis was treated with mediastinal washout and irrigation. Multiple complications from peripheral ECMO were successfully managed.
Cardiomyopathies ; Extracorporeal Membrane Oxygenation ; Extremities ; Heart Transplantation ; Heart ; Humans ; Mediastinitis ; Myocardial Infarction ; Perfusion ; Shock ; Transplantation ; Wound Infection