Recurrence of progressive familial intrahepatic cholestasis (PFIC) type II poses challenges during postoperative liver transplant care. Posttransplant patients with PFIC type II risk developing recurrent cholestasis with normal gamma-glutamyl transferase activity, which mimics the original bile salt export pump (BSEP) protein deficiency and is related to a form of immunoglobulin G antibody (anti-BSEP)-mediated rejection. Bortezomib effectively induces apoptosis of actively antibody-producing plasma cells that may have a role in antibodymediated rejection. In this case, we used bortezomib to treat PFIC type II recurrence after liver transplantation in a child.

Background: This study aimed to evaluate the effect of a prefabricated functional appliance (MyobraceⓇ ) on skeletal, dental, and soft tissue components in children with Class II, division 1 malocclusion. Methods: Thirteen patients with Class II, division 1 malocclusion (9 girls and 4 boys; mean age, 8.2±0.9 years at the start and 9.3±1.0 years at the end of the treatment) were treated with MyobraceⓇ for a mean period of 12.9±4.0 months. Patients were instructed to use the appliance daily for 1 hour and overnight while sleeping. A control group of 10 patients with untreated Class II, division 1 malocclusion (3 girls and 7 boys; mean age, 9.0±1.6 years at the start and 10.4±2.1 years at the end of the observation) was included to eliminate possible growth effects. The mean observation period for this group was 17.7±11.2 months. Lateral cephalograms were taken at the start and end of the treatment, and findings from 41 measurements were analyzed using the V-CephTM program. The mean and standard deviation of cephalometric measurements were analyzed using paired and independent sample t-tests. Results: The treatment group showed significant changes in SNB, ANB, maxillary protrusion, ramus height, proclination of upper anterior teeth, interincisal angle, overjet, and upper lip protrusion compared with the control group. However, only decrease in ANB, maxillary protrusion, overjet, upper lip protrusion, and increase in interincisal angle were significantly higher in the treatment group than in the control group. Conclusion The prefabricated functional appliance induced skeletal, dentoalveolar, and soft tissue changes, resulting in a significant reduction in anteroposterior discrepancy.

BACKGROUND/AIMS: In addition to the globally endorsed Barcelona Clinic Liver Cancer (BCLC) staging system, other algorithms or staging systems have been developed, including the Hong Kong Liver Cancer (HKLC) staging system. This study aimed to validate the HKLC staging system relative to the BCLC staging system for predicting survival for hepatocellular carcinoma (HCC) patients in Korea. METHODS: From 2004 to 2013, 2,571 patients newly diagnosed with HCC were consecutively enrolled at three Korea University medical centers. RESULTS: Both staging systems differentiated survival well (p < 0.001). However, 1-year and 3-year survival were predicted better using the HKLC system than the BCLC system (area under the receiver operating characteristic curve: 0.869 vs 0.856 for 1 year, p=0.002; 0.841 vs 0.827 for 3 years, p=0.010). In hypothetical survival curves, the HKLC system exhibited better median overall survival than the BCLC system (33.1 months vs 19.2 months). In evaluations of prognosis according to either BCLC or HKLC treatment guidelines, risk of death was reduced in the group following only HKLC guidelines compared with the group following only BCLC guidelines (hazard ratio, 0.601; 95% confidence interval, 0.443 to 0.816; p=0.001). CONCLUSIONS: Although both staging systems predicted and discriminated HCC prognoses well, the HKLC system showed more encouraging survival benefits than the BCLC system.
Academic Medical Centers ; Carcinoma, Hepatocellular ; Hong Kong ; Humans ; Korea ; Liver Neoplasms ; Liver ; Neoplasm Staging ; Prognosis ; ROC Curve

BACKGROUND: Hand eczema is one of the most common skin disorders and negatively affects quality of life. However, a large-scale multicenter study investigating the clinical features of patients with hand eczema has not yet been conducted in Korea. OBJECTIVE: To identify the prevalence of various hand diseases, which is defined as all cutaneous disease occurring in hands, and to investigate the clinical features of patients with hand eczema and the awareness about hand eczema in the general population and to compare the prevalence of hand eczema between health care providers and non-health care providers. METHODS: To estimate the prevalence of hand diseases, we analyzed the medical records of patients from 24 medical centers. Patients were assessed by online and offline questionnaires. A 1,000 from general population and 913 hand eczema patients answered the questionnaire, for a total of 1,913 subjects. RESULTS: The most common hand disease was irritant contact dermatitis. In an online survey, the lifetime prevalence of hand eczema was 31.2%. Hand eczema was more likely to occur in females (66.0%) and younger (20~39 years, 53.9%). Health care providers and housewives were the occupations most frequently associated with hand eczema. Winter (33.6%) was the most common season which people experienced aggravation. The 63.0% and 67.0% answered that hand eczema hinders their personal relationship and negatively affects daily living activities, respectively. CONCLUSION: Hand eczema is a very common disease and hinders the quality of life. The appropriate identification of hand eczema is necessary to implement effective and efficient treatment.
Activities of Daily Living ; Dermatitis, Contact ; Eczema* ; Female ; Hand* ; Health Personnel ; Humans ; Korea* ; Medical Records ; Occupations ; Prevalence ; Quality of Life ; Seasons ; Skin ; Surveys and Questionnaires

Although stress-induced cardiomyopathy (SCMP) is a reversible disease and the prognosis is usually excellent, several complications can occur and can result in fatal adverse events. The formation of left ventricular (LV) thrombus is one of these critical complications of SCMP. This report describes a case of SCMP complicated by formation of a LV thrombus that became increasingly mobile as LV contractility recovered, and for which surgical removal was performed. Here, we report a case of SCMP complicated by LV thrombus and review the literature regarding this topic.
Cardiomyopathies* ; Echocardiography ; Humans ; Prognosis ; Takotsubo Cardiomyopathy ; Thrombosis*

L-transposition of the great arteries (L-TGA) is a rare congenital anomaly and could cause complete atrioventricular (AV) block at relatively younger age. We present a case of 43-year-old male who complained of dizziness due to complete AV block. We confirmed L-TGA using transthoracic echocardiography and cardiac computed tomography. Permanent pacemaker was inserted without complications. No invasive treatment including corrective surgery was performed because patient's cardiac function was almost normal and the symptom was completely resolved after pacemaker insertion.
Adult ; Arteries* ; Atrioventricular Block* ; Dizziness ; Echocardiography ; Heart Defects, Congenital ; Humans ; Male

BACKGROUND/AIMS: Prothrombin induced by vitamin K deficiency or antagonist II (PIVKA-II) is a widely used diagnostic marker for hepatocellular carcinoma (HCC). We evaluated the correlation between alcoholic liver disease (ALD) and serum PIVKA-II levels in chronic liver disease (CLD) patients. METHODS: We retrospectively reviewed the medical records of 2,528 CLD patients without HCC. Among these patients, 76 exhibited serum high PIVKA-II levels of >125 mAU/mL (group 1). We categorized 76 control patients matched by age, sex, and the presence of liver cirrhosis from the remaining patients who were negative for serum PIVKA-II (group 2). RESULTS: Group 1 revealed increased antibiotic usage (23.7% vs 2.6%, p<0.001) and incidence of ALD (60.5% vs 14.5%, p<0.001) as well as elevated aspartate aminotransferase (52.5 IU/L vs 30.5 IU/L, p=0.025) and gamma glutamyl transpeptidase (67.5 IU/L vs 36.5 IU/L, p=0.005) levels compared with group 2. Further, group 1 was significantly associated with a worse Child-Pugh class than group 2. In the multivariate analysis, ALD (odds ratio [OR], 7.151; p<0.001) and antibiotic usage (OR, 5.846; p<0.001) were significantly associated with positive PIVKA-II levels. CONCLUSIONS: Our study suggests that ALD and antibiotics usage may be confounding factors when interpreting high serum PIVKA-II levels in patients without HCC. Therefore, serum PIVKA-II levels in patients with ALD or in patients administered antibiotics should be interpreted with caution.
Adult ; Age Distribution ; Aged ; Anti-Bacterial Agents/therapeutic use ; Aspartate Aminotransferases/blood ; Biomarkers/*blood ; Carcinoma, Hepatocellular/blood ; Diagnosis, Differential ; Female ; Humans ; Liver Cirrhosis/blood ; Liver Diseases, Alcoholic/*blood ; Liver Neoplasms/blood ; Male ; Matched-Pair Analysis ; Middle Aged ; Multivariate Analysis ; Protein Precursors/*blood ; Prothrombin/analysis ; Retrospective Studies ; Sex Distribution ; gamma-Glutamyltransferase/blood

BACKGROUND/AIMS: Prothrombin induced by vitamin K deficiency or antagonist II (PIVKA-II) is a widely used diagnostic marker for hepatocellular carcinoma (HCC). We evaluated the correlation between alcoholic liver disease (ALD) and serum PIVKA-II levels in chronic liver disease (CLD) patients. METHODS: We retrospectively reviewed the medical records of 2,528 CLD patients without HCC. Among these patients, 76 exhibited serum high PIVKA-II levels of >125 mAU/mL (group 1). We categorized 76 control patients matched by age, sex, and the presence of liver cirrhosis from the remaining patients who were negative for serum PIVKA-II (group 2). RESULTS: Group 1 revealed increased antibiotic usage (23.7% vs 2.6%, p<0.001) and incidence of ALD (60.5% vs 14.5%, p<0.001) as well as elevated aspartate aminotransferase (52.5 IU/L vs 30.5 IU/L, p=0.025) and gamma glutamyl transpeptidase (67.5 IU/L vs 36.5 IU/L, p=0.005) levels compared with group 2. Further, group 1 was significantly associated with a worse Child-Pugh class than group 2. In the multivariate analysis, ALD (odds ratio [OR], 7.151; p<0.001) and antibiotic usage (OR, 5.846; p<0.001) were significantly associated with positive PIVKA-II levels. CONCLUSIONS: Our study suggests that ALD and antibiotics usage may be confounding factors when interpreting high serum PIVKA-II levels in patients without HCC. Therefore, serum PIVKA-II levels in patients with ALD or in patients administered antibiotics should be interpreted with caution.
Adult ; Age Distribution ; Aged ; Anti-Bacterial Agents/therapeutic use ; Aspartate Aminotransferases/blood ; Biomarkers/*blood ; Carcinoma, Hepatocellular/blood ; Diagnosis, Differential ; Female ; Humans ; Liver Cirrhosis/blood ; Liver Diseases, Alcoholic/*blood ; Liver Neoplasms/blood ; Male ; Matched-Pair Analysis ; Middle Aged ; Multivariate Analysis ; Protein Precursors/*blood ; Prothrombin/analysis ; Retrospective Studies ; Sex Distribution ; gamma-Glutamyltransferase/blood

We report here on a rare case of primary AL hepatic amyloidosis associated with multiple myeloma in a 64-year-old woman. The patient was referred for evaluating her progressive jaundice and right upper quadrant pain. 18F-fluorodeoxyglucose (18F-FDG) positron emission tomography (PET)/computed tomography (CT) showed diffusely and markedly increased 18F-FDG uptake in the liver. Although there have been several case studies showing positive 18F-FDG uptake in pulmonary amyloidosis, to the best of our knowledge, the 18F-FDG PET/CT findings of hepatic amyloidosis or primary hepatic amyloidosis associated with multiple myeloma have not been reported previously.
Amyloidosis/complications/pathology/*radionuclide imaging ; Biopsy, Needle ; Female ; Fluorodeoxyglucose F18/*diagnostic use ; Humans ; Liver/pathology ; Liver Diseases/complications/pathology/*radionuclide imaging ; Middle Aged ; Multiple Myeloma/*complications ; *Positron-Emission Tomography and Computed Tomography ; Radiopharmaceuticals/*diagnostic use

Sarcoidosis is a multi-systemic granulomatous disorder of unknown etiology. The characteristic pathological finding is the presence of non-caseating granulomas. The lungs are primarily affected, however other organs may be involved causing various symptoms and ambiguous laboratory findings can be present. There are a few reported cases of sarcoidosis with elevated tumor markers. We describe a 68-year-old woman presenting with sarcoidosis showing elevated serum carcinoembryonic antigen (CEA). The possibility of cancer arising from serum CEA such as gastrointestinal cancer, breast cancer and lung cancer was excluded. A transbronchial lung biopsy demonstrated a non-caseating granuloma without necrosis. As a result prescribed 30 mg prednisolone daily to the patient and serum CEA was decreased after 1 month of treatment. We report a case of pulmonary sarcoidosis with elevated serum CEA.
Aged ; Biopsy ; Breast Neoplasms ; Carcinoembryonic Antigen ; Female ; Gastrointestinal Neoplasms ; Granuloma ; Humans ; Lung ; Lung Neoplasms ; Necrosis ; Prednisolone ; Sarcoidosis ; Sarcoidosis, Pulmonary ; Biomarkers, Tumor