Primary cardiac tumors in infancy and childhood are rare, with fibromas being the second most common tumor after rhabdomyomas. Although cardiac fibromas are characteristically benign intramural tumors, they may exhibit exhibit expansile growth resulting in obstruction, valvular dysfunction, as well as other problems so early diagnosis and successful surgical excision are important. We report a case of cardiac fibroma in a 2 month-old male infant. He presented with generalized cyanosis from birth. Echocardiography showed oval round large mass filing the right atrium and ventricle which infiltrated into the lateral wall of the ventricle. Partial excision of the tumor was done after another echocardiogram showed a pericardial effusion and restriction of blood flow to the right ventricle due to the tumor. The resected tumor was ovoid, gray-tan, slightly firm and measuring 5x3x2.5 cm. Histologically, the tumor was composed of spindle-shaped fibroblasts and hyalinized fibrous tissue interdigitating with the surrounding myocardium.
Infant ; Child ; Male ; Female ; Humans

The aim of this study was to evaluate current practice patterns on diagnosis and management of pediatric varicoceles. Questionnaires of approaches to diagnosis and management of pediatric varicoceles were sent electronically to pediatric urologists. Of the 70 questionnaires e-mailed, 37 (53%) responded to the survey. 10 respondents (27%) chose to operate on varicoceles, whereas 9 (24%) chose to observe, and 18 (49%) chose to decide upon treatment depending on the clinical situation. The most important indication for varicocelectomy was a decrease in ipsilateral testicular size (n=29, 78%) followed by testicular or scrotal pain (n=4, 11%) and varicocele grade (n=4, 11%). The optimal age for varicocelectomy was answered as 13.8±2.3 years mean. 32 respondents (86%) have used ultrasonography to aid in the diagnosis of varicoceles, and 26 respondents (70%) have considered repairing varicocele incidentally detected on ultrasonography. In an otherwise asymptomatic patient with varicocele, 17 respondents (46%) considered surgery for grade 3, but 15 respondents (41%) would not repair the varicocele. The most commonly used surgical approach was subinguinal microsurgical (n=19, 51%), followed by inguinal (n=9, 24%) and laparascopic (n=5, 14%) procedures. The most commonly experienced post-operative complication was recurrence (n=22, 59%) followed by persistence (n=13, 35%) and hydrocele (n=10, 27%). 28 respondents (76%) did not have long-term follow-up data including regarding fertility on their varicocele patients. Our survey demonstrates that there is lack of consensus on diagnosis and management of pediatric and adolescent varicoceles among pediatric urologists. A prospective randomized study of pediatric and adolescent varicoceles is needed to assess the outcomes and develop universal management guidelines.
Adolescent ; Consensus ; Diagnosis ; Electronic Mail ; Fertility ; Follow-Up Studies ; Humans ; Infertility ; Korea ; Prospective Studies ; Recurrence ; Surveys and Questionnaires ; Ultrasonography ; Varicocele

To evaluate the diagnostic efficacy of needle aspiration biopsy of seminiferous tubules (NABST) and to represent the redistributed diagnostic results corresponding to testicular volumes and follicle-stimulating hormone (FSH) levels. In this retrospective study, we investigated 65 infertile men with either azoospermia or oligoasthenoteratozoospermia. Following NABST, specimens were stained with hematoxylin and eosin and classified into five histological types. With pre-procedure FSH levels and testicular volumes, we evaluated the probabilities of detecting sperms within biopsy specimens. NABST led to the classification of normal spermatogenesis in 31 cases (47.7%), hypospermatogenesis in 23 cases (35.4%), maturation arrest in 4 cases (6.2%), and Sertoli cell only syndrome in 4 cases (6.2%). The success rate of reaching a histological diagnosis using NABST was 95.4% (62 out of 65 cases). Fourteen patients (21.5%) had a testicular volume <15 cc; of these, 8 patients (57.1%) had normal spermatogenesis, 2 patients (14.3%) had hypospermatogenesis, 2 patients (14.3%) had maturation arrest and 2 patients (14.3%) had Sertoli cell-only syndrome (SCO). Twelve patients (18.5%) had an FSH level ≥10 IU; of these, 6 (50%) had normal spermatogenesis, 2 patients (16.7%) had maturation arrest and 4 patients (33.3%) had SCO. Cases with an FSH level <10 IU were positively associated with a probability of detecting sperm using NABST (p<0.001). NABST is a reliable tool for the histological diagnosis of azoospermic and oligoasthenoteratozoospermic patients. The diagnostic success rate was high and associated with pathological accuracy. NABST is a convenient procedure with few complications.
Asthenozoospermia ; Azoospermia ; Biopsy ; Biopsy, Needle ; Classification ; Diagnosis ; Eosine Yellowish-(YS) ; Follicle Stimulating Hormone ; Hematoxylin ; Humans ; Korea ; Male ; Methods ; Needles ; Oligospermia ; Retrospective Studies ; Seminiferous Tubules ; Sertoli Cell-Only Syndrome ; Spermatogenesis ; Spermatozoa

BACKGROUND: Rhus verniciflua Stokes (RV) has traditionally been used in Korea as an indigenous food (Rhus chicken soup) and as an herbal medicinal plant. While the anticancer, antimicrobial, and anti-inflammatory properties of RV have been actively studied in the medical field, its antioxidant effects in the skin that resist the reactive oxygen species in keratinocytes and fibroblasts is less understood. OBJECTIVE: We designed to evaluate the effects of R. verniciflua Stokes extract (RVE) on the photo-aged skin by an in vitro experiment using human fibroblasts and an in vivo experiment using a photo-aged murine model. METHODS: For the in vitro experiments, human fibroblasts irradiated with ultraviolet (UV) B were treated with RVE or vehicle, and the growth levels and the expression level of type 1 procollagen were compared. For the in vivo experiment, photo-aged mice irradiated with UVB and UVA were administered drinking water with or without RVE, and histological changes and the expression level of type 1 procollagen and matrix metalloprotease (MMP)-13 were compared. RESULTS: In vitro experiments using fibroblasts irradiated with UVB showed that RVE promoted growth and significantly increased the expression of type 1 procollagen as compared to the control group. In the photo-aged mice, RVE increased collagen content in the dermis and promoted the synthesis of type 1 procollagen without any visible decrease in MMP-13 as compared to control group. CONCLUSION: In addition to the previously reported antioxidant effects of RVE, oral intake of RVE effectively inhibited photo-aging in hairless mice by enhancing collagen synthesis.
Aging ; Animals ; Antioxidants ; Chickens ; Collagen ; Dermis ; Drinking Water ; Fibroblasts ; Humans ; In Vitro Techniques ; Keratinocytes ; Korea ; Mice* ; Mice, Hairless ; Plants, Medicinal ; Procollagen ; Reactive Oxygen Species ; Rhus* ; Skin*

BACKGROUND: Generally, it is difficult to differentiate uterine malignant mixed Mullerian Tumor(MMMT) from endometrial carcinom in radiological and clinical aspects. Our purpose is to investigate MR findings that distinguishes MMMT from endometrial carcinoma. MATERIALS AND METHODS: We retrogradely evaluated the magnetic resonance imaging findings of pathologically proven 5 cases of malignant mixed Mullerian Tumor(MMMT) and 13 endometrial carcinomas to know the differential points of these two tumors originating in the endometrial cavity. The size of the mass, presence or absence of myometrial or uterine cervical invasion, growth pattern of the mass, signal intensity and degree and pattern of contrast enhancement were analyzed and compared. RESULTS: The length of the long axis of the MMMT was 1.5-9.0cm(average, 5.7cm) but that of the endometrial carcinoma was 0.5-6.0cm(average, 2.5cm). Invasion of uterine cervix which was found in 3 MMMT cases, dilated the endometrial cavity and the lumen of the uterin cervix and showed the pattern of growing into the external os. Invasion of uterine cervix was found in only one case of endometrial carcinoma. The presence or absence of myometrial invasion, the signal intensity and homogeneity on T1-and T2-weighted images, and the degree and patterns of contrase enhancement showed no significant difference. CONCLUSION: Any specific finding to differentiate MMMT from endometrial carcinoma was not ascertained. However, MMMT can be suspected if the size of the endometrial mass is greater than 5cm and if the mass dilates the enocervical canal and invades the uterine cervix.
Axis, Cervical Vertebra ; Cervix Uteri ; Endometrial Neoplasms* ; Female ; Magnetic Resonance Imaging*

Background: Cognitive impairment is the second most common clinical manifestation in cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). However, understanding of cognitive impairment in CADASIL has been hampered by lack of consensus on diagnosis of vascular cognitive impairment (VCI). We used vascular impairment of cognition classification consensus study principles (VICCCS-1) and protocols (VICCCS-2) to assess the cognitive impairment in CADASIL. We also evaluated the impact of MRI markers on major and mild VCI in CADASIL. Methods: We prospectively recruited 64 patients who underwent standardized brain MRI and detailed neuropsychological test. MRI analysis included number of lacunes, number of cerebral microbleeds (CMB), normalized volume of white-matter hyperintensities (nWMH), and brain parenchymal fraction (BPF). BPF has been used to measure brain atrophy. The patients were divided into three groups: those with normal cognition (CADASIL-NC, n=14), those with mild VCI (CADASIL-mild VCI, n=38), and those with major VCI (CADASIL-major VCI, n=11). Results: The three groups differed according to age, with the major VCI group being older. The major VCI group had more lacunes, more CMB, more extensive white matter lesions and lower BPF than NC group. There were no significant differences between NC and mild VCI groups in BPF. BPF and age were the independent predictors of major VCI. There was a tendency that women were at higher risk for mild VCI, though it did not reach statistical significance. Women were older than men, but had lower number of lacunes in mild VCI. Conclusions These findings suggest that brain atrophy and age are the main predictors of major VCI in CADASIL.

BACKGROUND: The lesion length of coronary artery stenosis has been regarded as a risk factor for acute complication and long segment stenosis of the coronary artery is associated with a less chance of successful percutaneous transluminal coronary angioplasty(PTCA). Many new interventional techniques auch as excimer laser angioplasty, directional atherectomy, and long-balloon angioplasty catheter have been developed and used for long lesion of coronary artery stenosis. Only a little data is, however, available on long-balloon PTCA. This study was carried out to see the clinical results of PTCA using long-balloon angioplasty catheters. SUBJECTS AND METHODS: Fifty-four coronary arterial stenotic lesions in 49 patients(M:F=32:17, 54.48.9 years)were attempted to dilate with long-balloon PTCA. Clinical diagnoses in 49 patients were acute myocardial infarction in 13, old myocardial infarction in 9, unstable angina in 18, andd stable angina in 9. Lesion length and TIMI(Thrombolysis in Myocardial Infarction) flow of target lesions were measured before PTCA. PTCA was performed in standard fashion using long-balloon angioplasty catheters. Immediate success rate, complications, and retenosis rate were evaluated. RESULTS: The associated risk factors of atherosclerosis were hypertension in 16, hypercholesterolemia in 10, smoking in 27, and diabetes mellitus in 9 patients. The target vessels were 34 left anterior descending arteries, 6 left circumflex arteries, and 14 right coronary arteries. PTCA using long-balloon catheters was performed as an adjunct to suboptimal PTCA using standard balloon catheters in 6 patients. In the remaining 44 patients, long-balloon PTCA was primarily performed due to lesion length greater than 2.0 cm. The overall success rate of long-balloon PTCA was 85.2%, and the procedure-related complications were occurred in 12(22.2%) lesions. Restenosis was revealed in 9(50%) of 18 lesions which were evaluated with follow-up coronary angiography. CONCLUSION: Above results suggest that PTCA using a long-balloon angioplasty catheter is efficacious in dilation long segment stenosis of coronary artery with a low complication rate.
Angina, Stable ; Angina, Unstable ; Angioplasty ; Angioplasty, Balloon, Coronary* ; Arteries ; Atherectomy ; Atherosclerosis ; Catheters ; Constriction, Pathologic ; Coronary Angiography ; Coronary Stenosis ; Coronary Vessels ; Diabetes Mellitus ; Diagnosis ; Follow-Up Studies ; Humans ; Hypercholesterolemia ; Hypertension ; Lasers, Excimer ; Myocardial Infarction ; Risk Factors ; Smoke ; Smoking

Recently the incidence of coronary artery diseases in young patients has been increased in Korea, which may be related with the changes in dietary habits, stress and smoking. This report presents an 18-year-old male patient who suffered from acute non-ST elevation myocardial infarction. He had a history of 1.5 pack-years smoking, but no other risk factors. Depression of ST segments more than 3 mm in V1-6, I and aVL was documented on a twelve-lead electrocardiogram. Two-Dimensional echocardiography revealed hypokinetic anteroseptal wall motion, and myocardial SPECT demonstrated severe, reversible perfusion defects in the anteroseptal wall. A diagnostic coronary angiogram and an intravascular ultrasound revealed total occlusion in the proximal left anterior descending coronary artery due to heavy atheromatous plaque. The lesion was treated successfully by a coronary balloon and stent. He has no major adverse cardiovascular event on nine-month clinical follow-up after discharge.
Adolescent ; Coronary Artery Disease ; Coronary Vessels ; Depression ; Echocardiography ; Electrocardiography ; Follow-Up Studies ; Food Habits ; Humans ; Incidence ; Korea ; Male* ; Myocardial Infarction* ; Perfusion ; Risk Factors ; Smoke ; Smoking ; Stents ; Tomography, Emission-Computed, Single-Photon ; Ultrasonography

A 25-year-old woman presented with effort-induced chest pain. Physical examination revealed different blood pressures, 180/100 mmHg in right arm and 100/60 mmHg in left arm. Resting electrocardiogram was normal, but down-slope depression of ST segment more than 3 mm in V3-6, II, III, aVF developed at the stage 1 of treadmill exercise test. Stress Thallium-201 scan showed severe ischemia in the anteroseptal and lateral wall of left ventricle. Diagnostic coronary angiogram showed critical stenosis in the ostium of left main coronary artery. The left subclavian artery was occluded totally with well-developed collateral circulation. The patient underwent ostioplasty of left coronary ostium using pericardial patch, and her symptom improved after surgery. Follow-up coronary angiogram one year after surgery showed patent coronary artery ostium with good flow and myocardial perfusion improved on follow-up Thallium-201 SPECT. She has no major cardiac events during 7-year clinical follow-up.
Adult ; Arm ; Chest Pain ; Collateral Circulation ; Constriction, Pathologic ; Coronary Vessels* ; Depression ; Electrocardiography ; Exercise Test ; Female ; Follow-Up Studies* ; Heart Ventricles ; Humans ; Ischemia ; Perfusion ; Physical Examination ; Subclavian Artery ; Takayasu Arteritis* ; Tomography, Emission-Computed, Single-Photon

OBJECTIVE: The synovium in rheumatoid arthritis(RA) is characterized by an increase in the thickness of lining layer and infiltration of cells into the sublining area. Histomorphologic studies of RA have indicated that initial destruction is more closely related to the presence of transformed appearing proliferating synovial cells than to the presence of subsynovial or periarticular inflammation. Based on the fact that synovial lining cells have some properties of transformed appearing cells, we examined the expressions of Fos, Jun and Myc oncoproteins in the synovial tissue from patients with rheumatoid arthritis and osteoarthritis. METHODS: Synovial tissues from 15 patients with RA and 15 with osteoarthritis(OA) were studied by the immunohistochemical staining technique. Nine of 15 RA specimen were from arthroscopic synovectomy and the other 6 were from total knee replacement arthroplasty. RESULT: In all specimen studied, Myc and Fos were expressed in the synovial lining cells and Myc, Fos and Jun were expressed in the sublining cells, including lymphocytes, other inflammatory cells and blood vessels. Lymphocytes in the diffuse infiltrates showed increased expression of three oncoproteins compared to lymphocytes in the nodular aggregates. When oncoprotein expressions in RA were compared to OA, Fos and Myc expressions in the synovial lining cell layer were significantly higher in RA than in OA and Jun, Fos and Myc expressions in inflammatory cells were significantly higher in RA than in OA. The expressions of Fos and Myc were significantly correlated with the degree of synovial hypercellularity. In RA, the expressions of all three oncoproteins were increased in synovectomy group than joint replacement group. CONCLUSION: We observe that there are increased expressions of Myc, Fos and Jun in RA synovium than OA. These changes are more prominent in synovectomy group than joint replacement group, which suggest the differential expression of oncoproteins according to disease progression.
Arthritis, Rheumatoid ; Arthroplasty ; Arthroplasty, Replacement, Knee ; Blood Vessels ; Disease Progression ; Humans ; Inflammation ; Joints ; Lymphocytes ; Oncogene Proteins ; Oncogenes ; Osteoarthritis ; Synovial Membrane*