No abstract available.
Anemia* ; Chromatids* ; Humans ; Siblings*

No abstract available.
Osteogenesis Imperfecta* ; Osteogenesis*

Menetrier's disease is a rare, characterized by the presence of large rugal folds involving part or all of the stomach. Patients with hypertrophic gastropathy often have distressing abdominal symptoms, weight loss, and edema due to gastric protem loss. A 64 year old male patient was admitted with the chief complaint of epigastric discomfort. Endoscopic, radiologic and histologic examination, revealed characteristic findings of Menetrier's disease. A case of Menetrier's disease is reported with a brief review of literature.
Edema ; Gastritis, Hypertrophic* ; Humans ; Male ; Middle Aged ; Stomach ; Weight Loss

Antemortem diagnosis of inferior vena cava (IVC) and cardiac metastasis of hepatocellular carcinoma (HCC) is difficult but important to decide on treatment strategy. There are only a few cases of cardiac metastasis of HCC which have been diagnosed antemortem by echocardiography. We experienced 3 cases of IVC and Right atrial metastasis and 1 case of Left atrial metastasis of HCC. The tumor was discovered during computed tomography scanning. The patients had exhibited no signs of cardiac involvement. In this case, transesophageal echocardiography was valuable in providing information regarding the exact location of the tumor and its relation to surrounding anatomical structures. Left atrial metastasis of HCC is unusual patterns and probably related to tumor growth from the pulmonary veins following massive metastasis to the lung.
Carcinoma, Hepatocellular* ; Diagnosis ; Echocardiography ; Echocardiography, Transesophageal ; Humans ; Lung ; Neoplasm Metastasis* ; Pulmonary Veins ; Vena Cava, Inferior

PURPOSE: Adequate hemostasis in thyroidectomy is important to reduce postoperative complications including bleeding and hematoma. The object of this study was to evaluate the utility of thyroidectomy using ultrasonically activated shears. METHODS: This was a prospective randomized controlled study. It was conducted on 95 patients who underwent total thyroidectomy between January and March 2009. Patients were divided into two groups according to operation method used: group A (n=49) underwent total thyroidectomy using ultrasonically activated shears, group B (n=46) involved the conventional clamp and tie maneuver. Comparisons included operation time, drain amount, hospitalization, postoperative complications and off-thyroglobulin. RESULTS: The two groups had no significant differences regarding drain amount, hospitalization, postoperative complications and off-thyroglobulin. Operation time was statistically shorter in group A than group B (96.6±22.7 min vs 114.6±24.3 min) (P=.00). CONCLUSION: Thyroidectomy using ultrasonically activated shears reduces operation time significantly, and enables a complete and safe operation without postoperative complications. We recommend the use of ultrasonically activated shears in thyroidectomy.
Hematoma ; Hemorrhage ; Hemostasis ; Hospitalization ; Humans ; Methods ; Postoperative Complications ; Prospective Studies* ; Thyroidectomy*

Polycystic liver is the most common extra-renal manifestation associated with autosomal dominant polycystic kidney disease (ADPKD), comprising up to 80% of all features. Patients with polycystic liver often suffer from abdominal discomfort, dyspepsia, or dyspnea; however, there have been few ways to relieve their symptoms effectively and safely. Therefore, we tried transcatheter arterial embolization (TAE), which has been used in treating hepatocellular carcinoma. We enrolled four patients with ADPKD in Seoul National University Hospital, suffering from enlarged polycystic liver. We embolized the hepatic arteries supplying the dominant hepatic segments replaced by cysts using polyvinyl alcohol particles and micro-coils. The patients were evaluated 12 months after embolization for the change in both liver and cyst volumes. Among four patients, one patient was lost in follow up and 3 patients were included in the analysis. Both liver (33%; 10%) and cyst volume (47.7%; 11.4%) substantially decreased in two patients. Common adverse events were fever, epigastric pain, nausea, and vomiting. We suggest that TAE is effective and safe in treating symptomatic polycystic liver in selected ADPKD patients.
Aged ; Catheterization ; Cysts/*therapy ; Embolization, Therapeutic/instrumentation/*methods ; Female ; Hepatic Artery ; Humans ; Liver/pathology/physiology ; Liver Diseases/pathology/*therapy ; Middle Aged ; Polycystic Kidney, Autosomal Dominant/diagnosis/*therapy ; Polyvinyl Alcohol/therapeutic use ; Tomography, X-Ray Computed

BACKGROUND/AIMS: To compare the frequency of metabolic syndrome (MetS) and magnitude of insulin resistance, measured by the homeostatic model assessment of insulin resistance (HOMA-IR), between South Korean women with rheumatoid arthritis (RA) and healthy subjects, and to evaluate risk factors for MetS and increased HOMA-IR in patients with RA. METHODS: In a cross-sectional setting, 84 female patients with RA and 109 age-matched healthy female subjects were consecutively recruited at a university-affiliated rheumatology center in South Korea. MetS was defined according to the Third Report of the National Cholesterol Education Program's Adult Treatment Panel (NCEP-ATP III) 2004 criteria. RESULTS: The frequency of MetS did not differ significantly between patients with RA (19%) and healthy subjects (15.6%, p = 0.566), although patients with RA had a higher HOMA-IR compared with healthy subjects (p < 0.001). Patients with RA met the NCEP-ATP III 2004 criteria for high blood pressure more often than healthy subjects (44% vs. 19.3%, p < 0.001), and low high density lipoprotein cholesterol was more prevalent in healthy subjects (33%) than in patients with RA (14.3%, p = 0.004). Although no obvious risk factors for the presence of MetS were identified in patients with RA, higher serum C-reactive protein and disease activity score assessed using the 28-joint count for swelling and tenderness-erythrocyte sedimentation rate significantly contributed to a higher HOMA-IR. CONCLUSIONS: Despite their increased insulin resistance, South Korean women with RA did not have a significantly higher frequency of MetS compared with that in healthy subjects.
Adult ; Aged ; Arthritis, Rheumatoid/diagnosis/*epidemiology ; Biological Markers/blood ; Case-Control Studies ; Chi-Square Distribution ; Cross-Sectional Studies ; Female ; Humans ; Insulin Resistance ; Linear Models ; Logistic Models ; Metabolic Syndrome X/blood/diagnosis/*epidemiology ; Middle Aged ; Multivariate Analysis ; Odds Ratio ; Prevalence ; Republic of Korea/epidemiology ; Risk Assessment ; Risk Factors ; Sex Factors ; Young Adult

OBJECTIVE: A higher prevalence of metabolic syndrome (MetS) has been described in various rheumatic diseases such as rheumatoid arthritis and systemic lupus erythematosus, but no direct studies have investigated the prevalence of MetS in systemic sclerosis (SSc) patients compared with the general population. We compared the frequency of MetS and insulin resistance, as measured by Homeostatic assessment model-insulin resistance (HOMA-IR) between female SSc patients and healthy subjects. METHODS: In a cross-sectional setting, 35 female SSc patients and 84 age and sex-matched healthy subjects were consecutively recruited at a single center of a tertiary hospital between 2009 and 2010. MetS was defined according to the National Cholesterol Education Program's Adult Treatment Panel (NCEP-ATP III) 2004 criteria, using the Asian criteria for central obesity. RESULTS: The frequency of MetS was not significantly higher in SSc patients (20.0%) than in healthy subjects (14.3%, p=0.425), but SSc patients had an increased insulin resistance measured by HOMA-IR (p=0.001). Significantly more patients with SSc had increased triglyceride concentrations (p=0.004) and fewer SSc patients tended to meet the waist circumference criterion (p=0.051) according to the individual MetS features. Additionally, there were no significant differences in age, high-sensitivity C-reactive protein, body mass index, HOMA-IR, disease duration, cumulative glucocorticoids dose, modified Rodnan skin score or the proportion of limited SSc cases according to the absence or presence of MetS in SSc patients. CONCLUSION: Unlike other rheumatic diseases, our preliminary report did not show a significant difference in the prevalence of MetS between female SSc patients and healthy subjects.
Adult ; Arthritis, Rheumatoid ; Asian Continental Ancestry Group ; Body Mass Index ; C-Reactive Protein ; Cholesterol ; Female ; Glucocorticoids ; Humans ; Insulin Resistance ; Lupus Erythematosus, Systemic ; Prevalence ; Rheumatic Diseases ; Scleroderma, Systemic ; Skin ; Tertiary Care Centers ; Waist Circumference

Treatments for patient with ankylosing spondylitis (AS) include non-steroidal anti-inflammatory drugs (NSAIDs), disease-modifying antirheumatic drugs (DMARDs) and anti-tumor necrosis factor-alpha (TNFalpha) agents. However, owing to the well-known nephrotoxicity of NSAIDs and some DMARDs, the use of these drugs is limited in AS patients with renal insufficiency. As the pharmacokinetics and metabolism of anti-TNFalpha agents in patients of end stage renal disease, especially those receiving peritoneal dialysis (PD), have not been investigated well, little is known about treating them with anti-TNFalpha agents. We described the safety and efficacy of etanercept, a soluble fusion protein comprising the TNF receptor 2 in linkage with the Fc portion of immunoglobulin G, in a 40-year-old male AS patient receiving PD.
Adult ; Anti-Inflammatory Agents, Non-Steroidal ; Antirheumatic Agents ; Humans ; Immunoglobulin G ; Kidney Failure, Chronic ; Male ; Metabolism ; Necrosis ; Peritoneal Dialysis* ; Pharmacokinetics ; Receptors, Tumor Necrosis Factor ; Renal Insufficiency ; Spondylitis, Ankylosing* ; Tumor Necrosis Factor-alpha ; Etanercept

Mixed connective tissue disease (MCTD) was first described by Sharp and coworkers in 1972, characterized by symptoms of Raynaud's phenomenon or swollen hands, overlapping clinical features of systemic lupus erythematosus, systemic sclerosis, or polymyositis/dermatomyositis, and the presence of anti-U1 RNP antibody. MCTD is rare in children and constitutes 0.3~0.6% of all rheumatologic patients in pediatric rheumatology database of the United States. Here, we report the first Korean case of a 10-year-old female patient with MCTD, presenting Raynaud's phenomenon in the hands, feet, and tongue.
Child ; Female ; Foot ; Hand ; Humans ; Lupus Erythematosus, Systemic ; Mixed Connective Tissue Disease ; Rheumatology ; Scleroderma, Systemic ; Tongue ; United States