Objectives: This study investigated the relationship between clock-drawing test (CDT) performance and neuropsychological functions in patients with chronic schizophrenia. Methods: Thirty-one patients with schizophrenia and 30 healthy controls participated in this study. The CDT was administered in three conditions and analyzed using both quantitative and qualitative scoring systems. Comprehensive neuropsychological tests were administered. Results: The results of the quantitative analysis showed that the schizophrenia group performed significantly worse in all three conditions of the CDT compared with the control group. However, no significant differences were observed between the two groups, when the IQ and educational level were controlled. The qualitative analysis showed that the schizophrenia group exhibited significantly more errors in “graphic difficulty” compared with the control group. In addition, CDT quantitative scores were significantly correlated with visuospatial function, memory, attention and executive functions in patients with schizophrenia. Conversely, each qualitative error type was correlated with specific cognitive domains. Furthermore, “graphic difficulty” and “spatial/ planning deficit” were identified as predictors of depression symptoms in patients with schizophrenia. Conclusion The present study demonstrated that the CDT is useful for assessing cognitive dysfunctions in patients with schizophrenia, while qualitative analyses provide more specific information about cognitive deficits compared with quantitative analyses.

PURPOSE: Isolated noncompaction of the ventricular myocardium(INVM) can present as heart failure or arrhythmias in a child. It is a rare disorder, characterized by prominent trabecular meshwork and deep intertrabecular recesses. We still know little about the diagnosis, symptoms, and clinical outcomes of INVM. METHODS: We included in our study 6 patients who showed ventricular noncompaction on echocardiography. Patients were diagnosed as INVM were excessively prominent trabeculations with deep intertrabecular recesses were found on echocardiography. Patients who had other complex heart lesions such as pulmonary atresia with intact ventricular septum in addition to ventricular noncompaction, were excluded. RESULTS: Age at presentation ranged from 1 day 7 years, with follow up being as long as 6 years. Symptoms at initial presentation were heart murmur, paroxysmal supraventricular tachycardia, cyanosis, feeding intolerance, ventricular tachycardia, and cardiomegaly at fetal screening. Prominent trabeculations and intertrabecular recesses were observed at left ventricular apex in all six patients. All patients were alive at last follow-up. One patient showed WPW syndrome on electrocardiography. Echocardiography revealed decreased systolic function in 4 patients, and decreased systolic and diastolic function in 1 patient. One patient is currently asymptomatic. CONCLUSION: Six patients were diagnosed with INVM with various symptoms at initial presentation. Echocardiography is the most important tool in the diagnosis of INVM due to its morphological characteristics. INVM can rarely be the cause of long term systolic dysfunction, and early detection by echocardiographic screening may be beneficial.
Arrhythmias, Cardiac ; Cardiomegaly ; Child ; Cyanosis ; Diagnosis ; Echocardiography ; Electrocardiography ; Follow-Up Studies ; Heart ; Heart Failure ; Heart Murmurs ; Humans ; Isolated Noncompaction of the Ventricular Myocardium* ; Mass Screening ; Pulmonary Atresia ; Tachycardia, Supraventricular ; Tachycardia, Ventricular ; Trabecular Meshwork ; Ventricular Septum ; Wolff-Parkinson-White Syndrome

Arteriovenous malformations (AVM) are an unusual cause of gastrointestinal (GI) bleeding. But most GI AVM can occur with GI bleeding. When conventional diagnostic studies have failed to reveal the source of GI bleeding, AVM have been one of the most common causes. But the incidence of AVM presented as bleeding of a gastric submucosal tumor is very low. A case of gastric submucosal tumor was experienced and reported as an AVM. This case is herein discussed with review of corresponding literature.
Arteriovenous Malformations* ; Hemorrhage ; Incidence ; Stomach

Leber's hereditary optic neuropathy(LHON) is an optic nerve disease that causes blindness and is associated with maternally inherited mitochondrial DNA(mt DNA) mutations. The most common mitochondrial DNA mutation among LHON patients is a point mutation at the nucleotide 11778 in the subunit 4 of complex I. In one 45-year old male LHON patient with bilateral optic neuropathy, we investigated the presence of a point mutation of mitochondrial DNA and identified a single guanine to adenine transition mutation in the mitochondrial DNA at nucleotide point 11778.
Adenine ; Blindness ; DNA, Mitochondrial* ; Guanine ; Humans ; Male ; Middle Aged ; Optic Atrophy, Hereditary, Leber ; Optic Nerve Diseases ; Point Mutation*

Campomelic syndrome is a very rare skeletal dysplasia with a characteristic pattern of deformity involving the proximal and distal extremities, pelvic and shoulder girdles, thoracic cage and palate. Respiratory compromise often leads to death in early infancy. Etiology has not been determined although evidence suggests genetic heterogeneity. Cytogenetic study revealed high incidence of a 46,XY karyotype in phenotypic females. Recently, we had experienced a case of campomelic dysplasia at amenorrhea 30weeks and termination was done, so we report with a brief review of literature.
Amenorrhea ; Campomelic Dysplasia* ; Congenital Abnormalities ; Cytogenetics ; Extremities ; Female ; Genetic Heterogeneity ; Humans ; Incidence ; Karyotype ; Palate ; Shoulder

Coarctation of aorta(CoA) has been recognized to be cured by corrective operation, so many clinicians have discontinued follow-up early. However, high incidences of cardiovascular morbidity and mortality, of which causes have been known as persistent resting hypertension and exercise-induced hypertension after corrective operation, have been reported during long-term follow-up. And left ventricular mass increase associated with persistent resting hypertension and exercise-induced hypertension has been known as an independent risk factor of cardiovascular disease. So, even for successfully operated CoA patients, increased left ventricular mass as well as resting hypertension and exercise-induced hypertension must be detected early and normalized through persistent observation to adulthood.
Aortic Coarctation ; Cardiovascular Diseases ; Follow-Up Studies ; Heart Ventricles* ; Humans ; Hypertension ; Hypertrophy, Left Ventricular ; Incidence ; Mortality ; Risk Factors

Multiple mycotic pseudoaneurysms that develop after aortic surgery are a rare infectious complication. The clinical course of this disease is severe and associated with a high mortality. Few published cases have described mycotic pseudoaneurysms that are localized mainly in the brain, heart, and aorta. In this case, a 33-year-old woman was admitted with abdominal pain and mild fever after graft surgery for a ruptured thoracic aorta that occurred following a vehicle accident. Thoracoabdominal computed tomography (CT), angiography, and transesophageal echocardiography (TEE) demonstrated multiple pseudoaneurysms and thromboembolic obstructions in the thoracic aorta and its branches. She was treated with anticoagulation, coil embolization, and surgical resection. Microscopically, a resected pseudoaneurysm showed the characteristic features of fungal colonies with thrombi. This patient has been well for 2 months after removing the pseudoaneurysms and treatment with systemic antifungal agents.
Abdominal Pain ; Adult ; Aneurysm, False ; Angiography ; Antifungal Agents ; Aorta ; Aorta, Thoracic ; Brain ; Echocardiography, Transesophageal ; Female ; Fever ; Heart ; Humans ; Thromboembolism ; Transplants

PURPOSE: The peroxisome proliferator-activated receptor gamma (PPARgamma) is a nuclear receptor that regulates expression of mediators of lipid metabolism and the inflammatory response. Thyroid hormone receptor-associated proteins 220 (TRAP220) is an essential component of the TRAP/Mediator complex. The objective of this study was to clarify whether PPARgamma or TRAP220 are significant prognostic markers in resectable colorectal cancer (CRC). MATERIALS AND METHODS: A total of 399 patients who underwent curative resection for CRC were enrolled. We investigated the presence of PPARgamma and TARP220 in CRC tissues and adjacent normal tissues by immunohistochemistry. Correlation between the expression of these factors and clinicopathologic features and survival was investigated. RESULTS: Median age of the patients was 63 years (range, 22 to 87 years), and median follow-up duration 61.1 months (range, 2 to 114 months). PPARgamma and TRAP220 expression showed significant correlation with depth of invasion (p=0.013 and p=0.001, respectively). Expression of TRAP220 also showed association with lymph node metastasis and TNM stage (p=0.001). Compared with patients with TRAP220 negative tumors, patients with TRAP220 positive tumors had longer 5-year disease-free survival (DFS) tendency (p=0.051). Patients who were PPARgamma positive combined with TRAP220 positive had a better 5-year DFS (64.8% vs. 79.3%, p=0.013). In multivariate analysis expression of both PPARgamma and TRAP220 significantly affected DFS (hazard ratio, 0.620; 95% confidence interval, 0.379 to 0.997; p=0.048). CONCLUSION: TRAP220 may be a valuable marker for nodal metastasis and TNM stage. Tumor co-expression of PPARgamma and TRAP220 represents a biomarker for good prognosis in CRC patients.
Colorectal Neoplasms* ; Disease-Free Survival ; Follow-Up Studies ; Humans ; Immunohistochemistry ; Lipid Metabolism ; Lymph Nodes ; Mediator Complex Subunit 1* ; Multivariate Analysis ; Neoplasm Metastasis ; Peroxisomes* ; PPAR gamma* ; Prognosis ; Thyroid Gland

BACKGROUND: Some patients with type 2 diabetes mellitus (T2DM) do not develop diabetic kidney disease (DKD) despite the presence of advanced diabetic retinopathy (DR). We aimed to investigate the presence of DKD and its risk factors in patients with T2DM and advanced DR. METHODS: We conducted a cross-sectional study in 317 patients with T2DM and advanced DR. The phenotypes of DKD were divided into three groups according to the urine albumin/creatinine ratio (uACR, mg/g) and estimated glomerular filtration rate (eGFR, mL/min/1.73 m²): no DKD (uACR <30 and eGFR ≥60), non-severe DKD (uACR ≥30 or eGFR <60), and severe DKD (uACR ≥30 and eGFR <60). Mean systolic and diastolic blood pressure, mean glycosylated hemoglobin (HbA1c) level, and HbA1c variability (standard deviation [SD] of serial HbA1c values or HbA1c-SD) were calculated for the preceding 2 years. RESULTS: The prevalence of no DKD, non-severe DKD, and severe DKD was 37.2% (n=118), 37.0% (n=117), and 25.8% (n=82), respectively. HbA1c-SD and the triglyceride/high density lipoprotein cholesterol (TG/HDL-C) ratio correlated positively with uACR and negatively with eGFR. Multiple linear regression analyses showed that the HbA1c-SD and TG/HDL-C ratio were significantly related with eGFR. Multiple logistic regression analyses after adjusting for several risk factors showed that HbA1c-SD and the TG/HDL-C ratio were significant risk factors for severe DKD. CONCLUSION: The prevalence of DKD was about 60% in patients with T2DM and advanced DR. HbA1c variability and TG/HDL-C ratio may affect the development and progression of DKD in these patients.
Blood Pressure ; Cholesterol ; Cholesterol, HDL ; Cross-Sectional Studies ; Diabetes Mellitus, Type 2* ; Diabetic Nephropathies* ; Diabetic Retinopathy* ; Glomerular Filtration Rate ; Hemoglobin A, Glycosylated ; Humans ; Linear Models ; Lipoproteins ; Logistic Models ; Phenotype ; Prevalence ; Risk Factors* ; Triglycerides

POEMS syndrome is multisystemic disorder characterized polyneuropathy, organomegaly, endocrinopathies, monoclonal gammapathy. A 38 year-old man visited our hospital because of progressive general weakness, hyperpigmentation. We diagnosed him as idiopathic primary adrenal insufficiency. The symptoms of general weakness were improved after steroid replacement therapy. But after 6 months, he revisited ER with chief complaints of abdominal distension and edema in lower extremities. He had also polyneuropathy, hepatosplenomegaly, IgA light chain monoclonal gammopathy, hyperpigmentation and thickening of the skin. Bone marrow biopsy showed that the plasma cells, mainly mature forms, are about 40% of all nucleated elements and on tissue section. We think this is the first case of POEMS syndrome presenting as primary adrenocortical insufficiency in Korea, so we report the case with reviews of other literatures. And we recommend performing a rapid ACTH stimulation test in all POEMS syndrome in order to detect adrenal insufficiency.
Addison Disease* ; Adrenal Insufficiency ; Adrenocorticotropic Hormone ; Adult ; Biopsy ; Bone Marrow ; Edema ; Humans ; Hyperpigmentation ; Immunoglobulin A ; Korea ; Lower Extremity ; Multiple Myeloma* ; Paraproteinemias ; Plasma Cells* ; Plasma* ; POEMS Syndrome* ; Polyneuropathies ; Skin