Background: Pheochromocytoma and paragangliomas (PPGL) are hereditary in approximately 30% to 40% cases. With the advancement of genetic analysis techniques, including next-generation sequencing (NGS), there were attempts to classify PPGL into molecular clusters. With NGS being applied to clinical settings recently, we aimed to review the results of genetic analysis, including NGS, and investigate the association with clinical characteristics in Korean PPGL patients. Methods: We reviewed the medical records of PPGL patients who visited Severance hospital from 2006 to 2019. We documented the clinical phenotype of those who underwent targeted NGS or had known germline mutations of related genes. Results: Among 57 PPGL patients, we found 28 pathogenic germline mutations of susceptibility genes. Before the targeted NGS was implemented, only obvious syndromic feature lead to the Sanger sequencing for the specific genes. Therefore, for the exact prevalence, only patients after the year 2017, when targeted NGS was added, were included (n=43). The positive germline mutations were found in 14 patients; thus, the incidence rate is 32.6%. Patients with germline mutations had a higher likelihood of family history. There were significant differences in the type of PPGLs, percentage of family history, metastasis rate, presence of other tumors, and biochemical profile among three molecular clusters: pseudohypoxic tricarboxylic acid cycle-related, pseudohypoxic von Hippel-Lindau (VHL)/endothelial PAS domain-containing protein 1-related, and kinase-signaling group. Germline mutations were identified in seven PPGL-related genes (SDHB, RET, VHL, NF1, MAX, SDHA, and SDHD). Conclusion We report the expected prevalence of germline mutations in Korean PPGL patients. NGS is a useful and accessible tool for genetic analysis in patients with PPGLs, and further research on molecular classification is needed for precise management.

OBJECTIVES: The purpose of this study was to investigate the role of self-esteem as a moderator of the factors influencing suicidality among middle-schoolers. METHODS: Moderated multiple regression analysis was applied to assess the influence of happiness, depression, and hostility on suicidality and to determine the degree to which self-esteem served as a moderator of those relationships. Data were collected from 268 students at a middle school in Busan, Korea, using a self-administered structured questionnaire. RESULTS: Happiness, depression, and hostility had significant direct effects on suicidality. Self-esteem showed no direct effect, but had a significant moderating effect on the relationship between hostility and suicidal behavior. CONCLUSIONS These results suggest that various interventions, such as counseling programs, should be designed to alleviate hostility and depression and to enhance happiness and self-esteem among early adolescents.

OBJECTIVES: The aim of this study was to evaluate the associations of frailty with perceived neighborhood walkability and environmental pollution among community-dwelling older adults in rural areas. METHODS: The participants were 808 community-dwelling men and women aged 65 years and older in 2 rural towns. Comprehensive information, including demographics, socioeconomic status, grip strength, polypharmacy, perceived neighborhood environment (specifically, walkability and environmental pollution), and frailty, was collected from participants using face-to-face interviews conducted between June and August 2018. Perceived neighborhood walkability was measured using 20 items that were selected and revised from the Neighborhood Environment Walkability Scale, the Neighborhood Walkability Checklist from the National Heart Foundation of Australia, and the Physical Activity Neighborhood Environment Survey. The Kaigo-Yobo Checklist was used to assess participants’ frailty. RESULTS: The overall prevalence of frailty in this community-dwelling population was 35.5%. Sex, age, cohabitation status, educational attainment, employment status, grip strength, and polypharmacy were significantly associated with frailty. In the logistic regression analysis, frailty was associated with low perceived neighborhood walkability (adjusted odds ratio [aOR], 0.881; 95% confidence interval [CI], 0.833 to 0.932; p<0.001) and severe perceived neighborhood environmental pollution (aOR, 1.052; 95% CI, 1.017 to 1.087; p=0.003) after adjusting for sex, age, cohabitation status, educational attainment, employment status, monthly income, grip strength, and polypharmacy. CONCLUSIONS More studies are warranted to establish causal relationships between walkability and environmental pollution and frailty.

OBJECTIVES: The purpose of this study was to investigate the role of self-esteem as a moderator of the factors influencing suicidality among middle-schoolers. METHODS: Moderated multiple regression analysis was applied to assess the influence of happiness, depression, and hostility on suicidality and to determine the degree to which self-esteem served as a moderator of those relationships. Data were collected from 268 students at a middle school in Busan, Korea, using a self-administered structured questionnaire. RESULTS: Happiness, depression, and hostility had significant direct effects on suicidality. Self-esteem showed no direct effect, but had a significant moderating effect on the relationship between hostility and suicidal behavior. CONCLUSIONS: These results suggest that various interventions, such as counseling programs, should be designed to alleviate hostility and depression and to enhance happiness and self-esteem among early adolescents.
Adolescent ; Busan ; Counseling ; Depression ; Happiness ; Hostility ; Humans ; Korea

BACKGROUND: Measurement of peak expiratory flow rate (PEFR) in a follow-up examination for a chronic airway disease is useful because it has the advantages of being a simple measurement and can be repeated during examination. The aim of this study was to examine the annual decrease of PEFR in asthma and chronic obstructive pulmonary disease (COPD) patients and to confirm the factors which influence this decrease. METHODS: From May, 2003 to September, 2010, the annual decrease of PEFR was obtained from asthma and COPD patients attending an outpatient pulmonary clinic. PEFR was measured using a Mini-Wright peak flow meter (Clement Clarke International Ltd. UK), and we conducted an analysis of factors that influence the change of PEFR and its average values. RESULTS: The results showed an annual decrease of 1.70+/-12.86 L/min the asthmatic patients and an annual decrease of 10.3+/-7.32 L/min in the COPD patients. Age and FEV1 were the predictive factors influencing change in asthma, and FEV1 and smoking were the predictive factors influencing change in COPD. CONCLUSION: We confirmed the annual decreasing PEFR in patients with chronic airway disease and identified factors that work in conjunction with FEV1 to influence the change.
Asthma ; Follow-Up Studies ; Forced Expiratory Volume ; Humans ; Outpatients ; Peak Expiratory Flow Rate ; Pulmonary Disease, Chronic Obstructive ; Smoke ; Smoking

Thrombocytopenia-associated multiple organ failure (TAMOF) has a high mortality rate when not treated, and early detection of TAMOF is very important diagnostically and therapeutically. We describe herein our experience of early detection of TAMOF, using an automated hematology analyzer. From 498,390 inpatients, we selected 12 patients suspected of having peripheral schistocytosis, based on the results of red blood cell (RBC) parameters and a volume/hemoglobin concentration (V/HC) cytogram. We promptly evaluated whether the individual patients had clinical manifestations and laboratory findings were consistent with TAMOF. Plasma exchanges were then performed for each patient. All 12 patients had TAMOF. The mean values of RBC parameters were significantly higher in all of the patients than with the reference range, however, 3 patients had % RBC fragments within the reference range. The mean value of ADAMTS-13 activity was slightly lower in patients compared with the reference range. Of the 12 patients, remission was obtained in 9 patients (75%) within 4 to 5 weeks using plasma exchanges. Three patients died. An increased percentage of microcytic hyperchromic cells with anisocytosis and anisochromia indicated the presence of schistocytes, making it an excellent screening marker for TAMOF. Identification of TAMOF with RBC parameters and a V/HC cytogram is a facile and rapid method along with an automated hematology analyzer already in use for routine complete blood cell counting test.
Adult ; Aged ; Aged, 80 and over ; Erythrocyte Indices ; Erythrocytes, Abnormal/pathology ; Female ; Hematologic Tests ; Hemoglobins/metabolism ; Humans ; Male ; Middle Aged ; Multiple Organ Failure/*blood/*diagnosis/etiology ; Thrombocytopenia/*blood/complications/*diagnosis

BACKGROUND/AIMS: Although only a few sporadic cases of Q fever have been reported in Korea, a total of 13 cases have been seen in our area. We performed this study to evaluate the clinical characteristics of these cases of acute Q fever. METHODS: Demographic features, clinical manifestations, laboratory and radiologic findings, and therapeutic outcomes of all cases were evaluated. Q fever was diagnosed using an indirect micro-immunofluorescence assay (MIFA) and polymerase chain reaction (PCR). RESULTS: A total of 13 patients with acute Q fever seen from January 2006 to August 2008 at three teaching hospitals in the Daegu Metropolitan City area were enrolled. The mean age was 49 years old (range, 24~76), and the male to female ratio was 11:2. Six (46.2%) cases had a history of animal contact. Fever (100%) was the most common manifestation, followed by myalgia (84.6%), headache (61.5%), anorexia (61.5%), and chills (61.5%). All cases were diagnosed with high titers of anti-phase II antibody (IgM> or = 1:50, IgG> or =1:200) and positive nested PCR for the 27-kDa OMP com-1 gene of Coxiella burnettii in the blood. In three cases, liver biopsies revealed the presence of compact fibrin-ring granulomas. No characteristics of pneumonia were diagnosed on chest X-rays. The predominant presentation was acute febrile illness with hepatitis, including three cases (27.3%) of severe cholestatic hepatitis. The most frequently used antimicrobial agent was doxycycline (84.6%), followed by azithromycin (7.7%). CONCLUSIONS: Acute Q fever may be added to the list of differential diagnosis of patients with acute febrile illness and hepatitis in the Daegu Metropolitan City area.
Animals ; Anorexia ; Azithromycin ; Biopsy ; Chills ; Coxiella ; Diagnosis, Differential ; Doxycycline ; Female ; Fever ; Granuloma ; Headache ; Hepatitis ; Hospitals, Teaching ; Humans ; Korea ; Liver ; Male ; Pneumonia ; Polymerase Chain Reaction ; Q Fever ; Thorax

Tracheal compression by vascular anomalies in adults is uncommon and most related reports are of children. A 79-year-old woman without any respiratory history underwent a lumbar spine surgery under general anesthesia. She suddenly developed airway obstruction after a position change from supine to prone. A fiberoptic bronchoscopy showed the obstruction of endotracheal tube. The obstruction was relieved after we changed the depth of endotracheal tube and supported the patient's neck with a cotton roll. The surgery ended without any other event and the patient recovered safely. A computed tomography revealed the rightward tracheal deviation and tortuous innominate artery contact with trachea. The patient didn't manifest any respiratory related symptoms during postoperative period, and she was discharged without any treatment.
Adult ; Aged ; Airway Obstruction ; Anesthesia, General ; Brachiocephalic Trunk ; Bronchoscopy ; Child ; Female ; Humans ; Neck ; Postoperative Period ; Prone Position ; Spine ; Trachea

A 48-year-old healthy woman was admitted in our hospital for elective hemorrhoidectomy. She developed sudden headache and chest pain, and showed sinus bradycardia, arrhythmia and hypotension forty minutes after spinal anesthesia with 0.5% hyperbaric bupivacaine. An EKG showed ST depression and an transthoracic echocardiogram performed in PACU demonstrated mild LV dysfunction with hypokinesia of LV inferolateral wall. An coronary angiography on postoperative day 1 revealed normal coronary vessel and akinesia of LV inferior wall. Levels of CK-MB and Troponin I were mildly elevated. With medical therapy, the patient's symptoms improved and recovered without any complication.
Anesthesia, Spinal ; Arrhythmias, Cardiac ; Bradycardia ; Bupivacaine ; Cardiomyopathies ; Chest Pain ; Coronary Angiography ; Coronary Vessels ; Depression ; Electrocardiography ; Female ; Glycosaminoglycans ; Headache ; Hemorrhoidectomy ; Humans ; Hypokinesia ; Hypotension ; Middle Aged ; Troponin I

BACKGROUND: The manifestation of Membranoproliperative glomerulonephritis (MPGN) is variable from asymptomatic hematuria, nephrotic syndrome to advanced chronic kidney disease. The etiology, pathogenesis, and treatment of MPGN are unclear. The incidence of idiopathic MPGN is also rare in Korea, and the clinical course was rarely studied. So we investigated clinical course of idiopathic MPGN type 1. METHODS: From March 1990 to November 2004, renal biopsy was done in about 1500 patients in Dong-A universty hospital. Among them, 16 patients were diagnosed as idiopathic MPGN. Fourteen patients were observed over 6 months. We analyzed clinical data of these patients retrospectively. RESULTS: Male to female ratio of total 16 patients is 2.2:1. Mean age of patients was 37.6 years (14-76 years) at the time of diagnosis. Thirteen cases (81%) had nephrotic range proteinuria, 8 cases (50%) had hypertention, 12 cases (75%) had edema, and 4 cases (25%) had decreased renal fuction (serum creatinine>1.2 mg/dL) at the time of diagnosis. Average observation time was 55.7+/-37.4 (6-122)months. Six cases ended up with progressive renal failure. Three cases achieved complete remission. Renal survival time for 50% of cases was 92 months. In our study, edema and decreased renal fucntion at the time of diagnosis were related with later development of progressive renal failure (p<0.05). Of the total 14 cases, 4 cases ware treated conservatively, 6 cases were treated with anti-platelet agents, and 4 cases were treated with anti-platelet agents and prednisone. In conservative treatment group, 1 case ended up with progressive renal failure and 1 case achieved complete remission. In anti-platelet agents treatment goup, 4 cases ended up progressive renal failure. In combined anti-platelet agents and prednisone treatment group, 1 case ended up progressive renal failure and 2 cases achieved complete remission. However, the effect of each treatment is inconclusive because the number of the patients are too small. CONCLUSIONS: We suspect that deceased renal function and edema at the time of diagnosis may be risk factor predicting progressive renal failure in patients with idiopathic MPGN type 1.
Biopsy ; Diagnosis ; Edema ; Female ; Glomerulonephritis ; Glomerulonephritis, Membranoproliferative* ; Hematuria ; Humans ; Incidence ; Korea ; Male ; Nephrotic Syndrome ; Prednisone ; Proteinuria ; Renal Insufficiency ; Renal Insufficiency, Chronic ; Retrospective Studies ; Risk Factors