Complement component 3 glomerulonephritis (C3GN) is a rare kidney disease characterized by complement dysregulation that results in prominent complement component 3 (C3) deposition in the kidneys. The clinical course of C3GN varies from mild hematuria to progressive chronic kidney disease. In most patients, C3GN is driven by acquired factors, namely, autoantibodies that target C3 or C5 convertases. Genetic variations in complement-related genes are less frequent. We report the case of a 9-yearold Korean boy who presented with microscopic hematuria and a persistently low C3 level and had biopsy findings of C3GN, with the presence of a C3 gene mutation: a frameshift mutation associated with C3 deficiency. However, the patient did not exhibit any other symptoms of complement deficiency. Direct DNA sequencing of his family members revealed the same genetic mutation in his father and older brother. This case report is significant because there are very few such reports worldwide concerning gene mutations related to C3 deficiency to be discovered in patients with C3GN. Explaining C3GN pathogenesis is challenging; therefore, additional research is required in the future.

C4 glomerulopathy is a recently introduced entity that presents with bright C4d staining and minimal or absent immunoglobulin and C3 staining. We report a case of a 62-year-old man with C4 glomerulonephritis (GN) and uveitis. He presented to the nephrology department with proteinuria and hematuria. The patient also had intermediate uveitis along with proteinuria and hematuria. A kidney biopsy that was performed in light of continuing proteinuria and hematuria showed a focal proliferative, focal sclerotic glomerulopathy pattern on light microscopy, absent staining for immunoglobulin or C3 by immunofluorescence microscopy, with bright staining for C4d on immunohistochemistry, and electron-dense deposits on electron microscopy. Consequently, C4 GN was suggested as the pathologic diagnosis. Although laser microdissection and mass spectrometry for glomerular deposit and pathologic evaluation of the retinal tissue were not performed, this is the first report of C4 GN in Korea and the first case of coexisting C4 GN and uveitis in the English literature.
Biopsy ; Diagnosis ; Glomerulonephritis* ; Hematuria ; Humans ; Immunoglobulins ; Immunohistochemistry ; Kidney ; Korea ; Mass Spectrometry ; Microdissection ; Microscopy ; Microscopy, Electron ; Microscopy, Fluorescence ; Middle Aged ; Nephrology ; Proteinuria ; Retinaldehyde ; Uveitis ; Uveitis, Intermediate*

BACKGROUND: Atrial fibrillation (AF) can be a risk factor for development of significant tricuspid regurgitation (TR). We investigated which clinical and echocardiographic parameters were related to severity of functional TR in patients with lone AF. METHODS: A total of 89 patients with lone AF were enrolled (75 +/- 11 years; 48% male): 13 patients with severe TR, 36 patients with moderate TR, and 40 consecutive patients with less than mild TR. Clinical parameters and echocardiographic measurements including right ventricular (RV) remodeling and function were evaluated. RESULTS: Patients with more severe TR were older and had more frequently persistent AF (each p < 0.001). TR severity was related to right atrial area and tricuspid annular systolic diameter (all p < 0.001). The patients with moderate or severe TR had larger left atrial (LA) volume and increased systolic pulmonary artery pressure (SPAP) than the patients with mild TR (p = 0.04 for LA volume; p < 0.001 for SPAP). RV remodeling represented by enlarged RV area and increased tenting height was more prominent in severe TR than mild or moderate TR (all p < 0.001). Multivariate analysis showed type of AF, LA volume, tricuspid annular diameter and tenting height remained as a significant determinants of severe TR. In addition, tenting height was independently associated with the presence of severe TR (p = 0.04). CONCLUSION: In patients with lone AF, TR was related to type of AF, LA volume, tricuspid annular diameter and RV remodeling. Especially, tricuspid valvular tethering seemed to be independently associated with development of severe functional TR.
Atrial Fibrillation* ; Echocardiography* ; Humans ; Multivariate Analysis ; Pulmonary Artery ; Risk Factors ; Tricuspid Valve Insufficiency*

PURPOSE: We aimed to discover clinical and angiographic predictors of microvascular dysfunction using the index of microcirculatory resistance (IMR) in patients with ST-segment elevation myocardial infarction (STEMI). MATERIALS AND METHODS: We enrolled 113 patients with STEMI (age, 56+/-11 years; 95 men) who underwent primary percutaneous coronary intervention (PCI). The IMR was measured with a pressure sensor/thermistor-tipped guidewire after primary PCI. The patients were divided into three groups based on IMR values: Low IMR [<18 U (12.9+/-2.6 U), n=38], Mid IMR [18-31 U (23.9+/-4.0 U), n=38], and High IMR [>31 U (48.1+/-17.1 U), n=37]. RESULTS: The age of the Low IMR group was significantly lower than that of the Mid and High IMR groups. The door-to-balloon time was <90 minutes in all patients, and it was not significantly different between groups. Meanwhile, the symptom-onset-to-balloon time was significantly longer in the High IMR group, compared to the Mid and Low IMR groups (p<0.001). In the high IMR group, the culprit lesion was found in a proximal location significantly more often than in a non-proximal location (p=0.008). In multivariate regression analysis, age and symptom-onset-to-balloon time were independent determinants of a high IMR (p=0.013 and p=0.003, respectively). CONCLUSION: Our data suggest that age and symptom-onset-to-balloon time might be the major predictors of microvascular dysfunction in STEMI patients with a door-to-balloon time of <90 minutes.
Aged ; Angiography/*methods ; Female ; Humans ; Male ; Microcirculation ; Middle Aged ; Myocardial Infarction/physiopathology/*surgery ; Operative Time ; *Percutaneous Coronary Intervention ; Regression Analysis

Fibrous dysplasia, a rare bony disease, is characterized by substitution of normal bone with immature tissue embedded in a fibrous stroma. The localization of fibrous dysplasia only at the middle turbinate is an extremely rare event. The causes of fibrous dysplasia are still unknown. Recently, attention has been focused on a defect in the adenylate cyclase signal transduction system found in the pathological tissues. Nasal endoscopy shows turbinate enlargement that can be mistaken for a concha bullosa. We report a case of fibrous dysplasia confined in the bilateral middle turbinates.
Adenylyl Cyclases ; Endoscopy ; Signal Transduction ; Turbinates*

Malignant pericardial mesothelioma is a rare and progressive cardiac tumor. There is no established standard treatment and the prognosis is poor. Most patients were retrospectively diagnosed from surgery or autopsy due to absence of specific clinical manifestation. Most patients with pericardial mesothelioma have demonstrated constrictive physiology on echocardiography or cardiac catheterization. Therefore, pericardial mesothelioma was often misdiagnosed as other causes of constrictive pericarditis. We report a case of primary pericardial mesothelioma misdiagnosed as pericardial metastasis of unknown origin.
Autopsy ; Cardiac Catheterization ; Cardiac Catheters ; Echocardiography ; Heart Neoplasms ; Humans ; Mesothelioma ; Neoplasm Metastasis ; Pericarditis, Constrictive ; Pericardium ; Prognosis ; Retrospective Studies

BACKGROUND AND OBJECTIVES: Recent studies have implicated inflammation as playing an important role in the occurrence, persistence and recurrence of atrial fibrillation and that C-reactive protein is a useful marker of the inflammation. The purpose of this study is to evaluate the association between serum CRP levels and the risk of atrial fibrillation. SUBJECTS AND METHODS: This study was performed on 9,487 subjects (5,263 men and 4,224 women; mean age: 58.8+/-6.6 years) who underwent medical check-ups at the Health Promotion Center in Kanbuk Samsung Hospital. 9,438 normal control subjects and 49 atrial fibrillation patients were included in the study. The CRP was measured using a highly sensitive Behring Nephelometer II. RESULTS: When comparing the two groups, there were significant differences in age, gender and the presence of hypertension and cerebrovascular accident, and these are the previously known risk factors for atrial fibrillation. After adjustment was made for the clinical significant variables of atrial fibrillation, multiple regression analysis revealed that the hsCRP levels were not associated with the risk for atrial fibrillation (p=0.52). CONCLUSION: The inflammatory markers (CRP, WBC count) were not predictive of a higher risk for atrial fibrillation in the Korean population.
Adult* ; Atrial Fibrillation* ; C-Reactive Protein* ; Female ; Health Promotion ; Humans ; Hypertension ; Inflammation ; Male ; Recurrence ; Risk Factors ; Stroke

BACKGROUND AND OBJECTIVES: Regular exercise protects us against numerous chronic diseases, and reduces the risk of cardiovascular disease. Also, C-reactive protein, a marker of inflammation, is known to be associated with the risk and prognosis of cardiovascular disease. The present study was designed to assess the effects of regular exercise on the level of high sensitive C-reactive protein (hsCRP). SUBJECTS AND METHODS: The serum level of hsCRP and the anthropometric indices of adiposity, metabolic variables, blood pressure (BP) and several cardiovascular risk factors were measured in 18445 subjects. A prospective study of 42 subjects with high baseline levels of hsCRP was conducted. After 3 months of regular aerobic exercise their hsCRP levels were rechecked and the association between regular exercise and hsCRP analyzed using a cross sectional and prospective study. RESULTS: Age, smoking, hypertension, diabetes and body mass index were found to be independent determinants of an elevated hsCRP level, but exercise was not found to be associated with a decreased hsCRP level in the cross-sectional study. In the prospective study, the mean serum hsCRP value was significantly reduced after 3 months in the exercise group (3.02+/-1.06 vs. 2.05+/-1.23 p=0.015), but not in the controls (3.03+/-1.09 vs. 2.57+/-1.11 p=0.104). However, the interval changes in hsCRP levels were not different in the exercise compared to the control group (p=0.660). CONCLUSION: These results suggest that exercise is not an independent determinant of the CRP level. Three months of regular aerobic exercise show a tendency to be associated with a reduction in the hsCRP level, but this putative effect of exercise on the hsCRP level was not significant in healthy subjects. Further larger sample, prospective cohort studies will be required to establish the effect of exercise on the hsCRP level.
Adiposity ; Blood Pressure ; Body Mass Index ; C-Reactive Protein* ; Cardiovascular Diseases ; Chronic Disease ; Cohort Studies ; Cross-Sectional Studies ; Exercise ; Hypertension ; Inflammation ; Prognosis ; Prospective Studies ; Risk Factors ; Smoke ; Smoking

BACKGROUND AND OBJECTIVES: There is a seasonal variation in the incidence of diabetes mellitus and cardiovascular disease. Insulin resistance is important in the development of both non-insulin dependent diabetes mellitus (Type II DM) and cardiovascular disease. Hyperinsulinemia, induced by an insulin resistant state, causes the development of a chronic inflammatory response in vascular structures, which is one of the main causes of cardiovascular disease. There have been several studies on seasonal variation in insulin resistance. Therefore, seasonal variation in insulin resistance were investigated to discover if any relationship existed. SUBJECTS AND METHODS: Korean people examined at a health care center were included. The subjects were divided into four groups, according to the season, and a homeostasis model assessment index (HOMA) calculated, as an indicator of insulin resistance, and compared between the groups. RESULTS: Using the homeostasis model assessment, the highest insulin resistance was observed during winter. Also, the body mass index and waist-hip ratio were highest during winter. CONCLUSION: Compensating for other metabolic factors (age, gender, body mass index, waist-hip ratio and season), insulin resistance was still found to be higher during winter. Seasonal variation in insulin resistance can be associated with the incidence of DM or cardiovascular disease. Therefore, a prospective study will be needed.
Body Mass Index ; Cardiovascular Diseases ; Delivery of Health Care ; Diabetes Mellitus ; Homeostasis ; Hyperinsulinism ; Incidence ; Insulin Resistance* ; Insulin* ; Seasons* ; Waist-Hip Ratio

BACKGROUND AND OBJECTIVES: It is well known that, regardless of whether a person has cardiovascular diseases, the reduction of heart rate after exercise reflects the impairment of the autonomic nervous system. It is also a predictive factor of death rate and it correlates to insulin resistance. Therefore, we assessed these correlations in normoglycemic subjects. SUBJECTS AND METHODS: Exercise stress testing was performed according to the Bruce protocol. Anthropometric indices of adiposity, metabolic variables, blood pressure (BP) and several cardiovascular risk factors were measured. The HOMA index was used as the insulin resistance, and the impairment of the autonomic nervous system was assessed by measuring the reduction of heart rate for 2 minutes after the cessation of exercise. RESULTS: The reduction of heart rate during 2 minutes after the cessation of exercise statistically correlated with the HOMA index, gender, age, body mass index, the waist circumference, heart rate during rest, the maximum heart rate, serum total cholesterol concentration, serum high density lipoprotein cholesterol concentration and serum low density lipoprotein cholesterol concentration, (p<0.05). However, on multiple regression analysis, the HOMA index, gender, heart rate during rest, and the maximum heart rate significantly correlated to the reduction of heart rate during 2 minutes after the cessation of exercise. CONCLUSION: In individuals with normal serum glucose levels, even after adjustment was made for other factors, the reduction of heart rate after the cessation of exercise correlated to insulin resistance. Hence, in individuals with normal serum glucose levels, efforts to improve insulin resistance have to be made, and prospective study on this subject is required.
Adiposity ; Autonomic Nervous System ; Blood Glucose ; Blood Pressure ; Body Mass Index ; Cardiovascular Diseases ; Cholesterol ; Cholesterol, HDL ; Cholesterol, LDL ; Exercise Test ; Heart Rate* ; Heart* ; Humans ; Insulin Resistance* ; Insulin* ; Mortality ; Risk Factors ; Waist Circumference