The polyglandular autoimmune syndrome designates the dysfunction of endocrine and nonendocrine system involving two or more organs on the basis of an autoimmune mechanism. The autoimmune nature of these diseases has been based on the presence of lymphocytic infiltration in the affected gland, organ specific autoantibodies in the serum, cellular immune defects and an association with the HLA DR/DQ genes or immune response genes. This syndrome is usually classified into three classes and their etiology or pathogenesis is still not completely understood. A 28-year-old woman developed vitiligo and insulin dependent diabetes mellitus during the treatment of Graves' disease with antithyroid drug. She had a tendency of spontaneous ketonemia and serum c-peptide levels were low(0.21, 0.16ng/mL: fasting and glucagon stimulated). Thyrotrophin binding inhibitor immunoglobulin and pancreas iIslet cell cytoplasmic antibody were positive. We report here a case of polyglandular autoimmune syndrome, type III manifesting Graves' disease, vitiligo, and insulin dependent diabetes mellitus.
Adult ; Autoantibodies ; Bone Diseases ; C-Peptide ; Cytoplasm ; Diabetes Mellitus ; Fasting ; Female ; Genes, MHC Class II ; Giant Cells ; Glucagon ; Granuloma ; Graves Disease ; Humans ; Immunoglobulins ; Insulin ; Ketosis ; Pancreas ; Pedigree ; Thyrotropin ; Vitiligo

Wernicke encephalopathy is usually accompanied with peripheral neuropathy, known as dry beriberi. In contrast, wet beriberi presenting as cardiovascular symptoms rarely occurs. The acute type of wet beriberi can be fatal, if untreated quickly. It is reported that the cerebellar vermis has a role of the coordination and control of cardiovascular and autonomic reflex activities. We report a 58-year-old man showing acute wet beriberi in Wernicke encephalopathy with cerebellar vermis lesion.
Beriberi* ; Cerebellar Vermis ; Humans ; Middle Aged ; Peripheral Nervous System Diseases ; Reflex ; Wernicke Encephalopathy*

BACKGROUND: High-resolution carotid ultrasonography is considered a fundamental technique for the investigation of the vascular system. However, it is still very unclear whether ultrasonographic studies of carotid arteries are useful for the prediction of cardiovascular events in patients with coronary heart disease. We have tried to assess the usefulness of carotid ultrasonography to predict the severity of coronary artery stenosis in the patients with ischemic heart disease. METHODS: We studied in 80 patients(53 men, 27 women) with acute chest pain, mean ages 63.1 10.8 yr(35 to 84 yrs), who underwent both coronary angiography and carotid ultrasonography with 10 MHz transducer. The patients who had received revascularization procedure were excluded. We classified the patients into two groups, the control group without significant coronary stenosis(23 patients) and the coronary artery disease(CAD) group(57 patients) with significant stenosis(>50%). The intima-media thickness (IMT) was measured in the far wall of CCA at 10 mm proximal to carotid bulb and the abnormal IMT was defined when the measurement was greater than mean IMT+2 SD of control group(>0.99 mm). Serum total cholesterolQlC), low density lipoprotein(LDL), high density lipoprotein(HDL), triglyceride(TG) and lipoprotein (a) (LP(a)) were measured and history of hypertension, diabetes mellitus, and smoking were investigated. RESULTS: A significant difference in IMT of the CCA was found between control and CAD group(0.75+/-0.12mm vs. 1.02+/-0.34 mm; p<0.001). Also a significant difference in the existence of plaque(control; 26.1% vs. CAD; 73.7%, p<0.00l) and the number of plaque(control; 0.39+/-0.94 vs. CAD; 2.20+/-1.87 p<0.001) was found. The existence and number of carotid plaque were more conelated with coronary artery stenosis severity than carotid IMT. The sensitivity of IMT for prediction of significant CAD was 42.1%, the specificity 95.7%, the positive predictive value 96%, and the negative predictive value 40%. The sensitivity of plaque presence on the carotid artery for prediction of CAD was 73.7%, the specificity 73.9%, the positive predictive value 87.5% and the negative predictive value 53.1%. Among the risk factors, age and LP(a) were correlated with IMT of CCA, and diabetes, hypertension, age were correlated with the presence of plaque. Smoking and hypertension were correlated with coronary artery disease. CONCLUSION: Carotid atherosclerosis was significantly correlated with severity of coronary atherosclerosis. We therefore suggest that carotid ultrasonography is useful to predict the severity of coronary artery stenosis and that the best index of coronary artery stenosis severity may be carotid plaque rather than carotid intima-media thickness.
Carotid Arteries ; Carotid Artery Diseases ; Carotid Intima-Media Thickness ; Chest Pain ; Coronary Angiography ; Coronary Artery Disease ; Coronary Disease ; Coronary Stenosis* ; Coronary Vessels* ; Diabetes Mellitus ; Humans ; Hypertension ; Lipoprotein(a) ; Male ; Myocardial Ischemia ; Risk Factors ; Sensitivity and Specificity ; Smoke ; Smoking ; Transducers ; Ultrasonography*

Purpose: This study develops a chatbot for school violence prevention (C-SVP) among elementary school students. Methods: Among the analysis, design, development, implementation, and evaluation (ADDIE) models, ADD phases were applied to develop a C-SVP. Students’ learning needs were identified by constructing content with a design that attracted their attention. Subsequently, a formative evaluation was conducted on the developed C-SVP to test its applicability by ten elementary school students targeting the 5th and 6th grades. Results: The chatbot was designed using KakaoTalk and named “School Guardian Angel.” The formative evaluation revealed that the developed C-SVP was easily accessible and useful for elementary school students. Conclusion The developed C-SVP is expected to be effective in preventing violence among elementary school students. However, further research involving children of various age groups is required.

Cerebellar ataxia with neuropathy and vestibular areflexia syndrome (CANVAS) is a slowing progressive ataxic disorder characterized by bilateral vestibulopathy, cerebellar ataxia and somatosensory impairment. Autonomic dysfunction is recently considered as a core feature in CANVAS in addition to these symptoms. In most cases, patients with CANVAS show cerebellar atrophy in brain imaging, but some cases show minimal or no atrophy of cerebellum. Brain (18F)-fluoro-2-deoxy-D-glucose positron emission tomography (¹⁸F-FDG PET) study can be a complimentary tool to diagnosis CANVAS in cases of no structural abnormality such as cerebellar atrophy. Hereby, we present a case of CANVAS with minimal atrophy of cerebellum but showing a prominent hypometabolism in cerebellum, thalamus and posterior cingulate cortex in ¹⁸F-FDG PET.
Atrophy ; Brain ; Cerebellar Ataxia ; Cerebellum ; Diagnosis ; Gyrus Cinguli ; Humans ; Neuroimaging ; Positron-Emission Tomography ; Primary Dysautonomias ; Thalamus ; Vestibular Neuronitis

Focal subarachnoid hemorrhage occasionally presents as transient focal neurologic episodes mimicking transient ischemic attack (TIA). Unless properly diagnosed, it may aggravate cerebral hemorrhage by administering antithrombotic agents. Therefore, clinicians need to be aware that such focal subarachnoid hemorrhage sometimes cannot be detected on noncontrast computed tomography and blood-sensitive magnetic resonance imaging can detect even a small amount of hemorrhage. We describe an 85-year-old woman with focal subarachnoid hemorrhage and possible cerebral amyloid angiopathy who presented transient left arm weakness recurrently, which mimicked TIA.
Aged, 80 and over ; Arm ; Cerebral Amyloid Angiopathy ; Cerebral Hemorrhage ; Female ; Fibrinolytic Agents ; Hemorrhage ; Humans ; Ischemic Attack, Transient ; Magnetic Resonance Imaging ; Subarachnoid Hemorrhage

Percutaneous balloon valvuloplasty (PBV) was done in 7 adults of pulmonary valvular stenosis, which was diagnosed with catheterization and cine-angiography of rigtht heart, in Seoul National University Hospital from Jan. 1986 to Dec. 1986. The results were as follows : 1) Among the seven cases six were male and the range of age was from 19 to 40 years. Atrial septal defects associated in 2 cases were not significant clinically. 2) The peak systolic gradient from right ventricle to pulmonary artery before the procedure was below 50 mmhg in 1 cases, 50 to 100 mmHg in 1 case, and above 100 mmHg in 5 cases. 3) Before PBV the mean systolic pressure of right ventricle was 121+/-42 mmHg and the peak systolic gradient was 106+/-41 mmHg. After PBV those were 58+/-30 mmHg and 40+/-31 mmHg, revealing significant improvement. 4) Balloon catheter (Medi-tech, 9F) was used in all cases. single balloon of 20 mm outer diameter was applied in 2 cases and double balloons of various sizes were applied in 5 cases in which single balloon showed inadequate dilatation. It is suggested that percutaneous balloon valvuloplasty is safe and effective alternative to surgical valvotomy of pulmonary valvular stenosis in adults.
Adult* ; Balloon Valvuloplasty* ; Blood Pressure ; Catheterization ; Catheters ; Constriction, Pathologic* ; Dilatation ; Heart ; Heart Septal Defects, Atrial ; Heart Ventricles ; Humans ; Male ; Pulmonary Artery ; Seoul

Rhabdomyolysis is defined as skeletal muscle injury with release of muscle cell constituents into the plasma and may lead to acute renal failure secondary to myoglobinuria. The causes of rhabdomyolysis is diverse:alcohol abuse, primary muscle disease, disturbance of muscle metabolism, sustained seizure, infection, drugs, tox ins, trauma, severe exercise, CO intoxication etc. Rhabdomyolysis may cause acute derangement in electrolyte balance and death. It should be diagnosed earlier and managed properly. We experienced a 49 year-old woman developed acute renal failure and myoglobinuria after alcohol drinking. A kidney biopsy revealed acute interstitial nephritis. In the presence of otherwise unexplained acute renal failure in alcoholic patients, rhabdomyolysis should be considered in the differential diagnosis.
Acute Kidney Injury ; Alcohol Drinking* ; Alcoholics ; Biopsy ; Diagnosis, Differential ; Female ; Humans ; Kidney ; Metabolism ; Middle Aged ; Muscle Cells ; Muscle, Skeletal ; Myoglobinuria* ; Nephritis, Interstitial* ; Plasma ; Rhabdomyolysis ; Seizures ; Water-Electrolyte Balance

Secondary intracranial hypertension (IH) is a syndrome with various etiologies, including a brain tumor, head trauma, hypoparathyroidism, hydrocephalus, meningitis, drug use, or venous obstruction. It is reasonably straightforward to diagnose secondary IH induced by these diseases. However, diagnosing secondary IH induced by unpredictable extracranial lesion is difficult, and is based largely on subtle neurological symptoms or signs and usually can be achieved by an extracranial evaluation. Here we describe four cases of secondary IH with unusual causes.
Brain Neoplasms ; Craniocerebral Trauma ; Headache ; Hydrocephalus ; Hypoparathyroidism ; Intracranial Hypertension ; Meningitis ; Spinal Cord Neoplasms

BACKGROUND AND OBJECTIVES: Congenital nystagmus (CN) is an ocular oscillation that usually manifests during early infancy. To report a novel mutation in FERM domain containing 7 (FRMD7) gene in a Korean family with CN. MATERIALS AND METHODS:Genomic DNA was prepared from peripheral blood leukocytes and direct sequencing of the entire coding and adjacent intronic regions was performed to detect sequence variation of FRMD7 gene, where mutations were found recently in patients with familial CN. The family showed an X-linked pattern of inheritance without father-to-son transmission. RESULTS: Three family members with CN exhibited two sequence variations which were a novel mutation (c. 875T>C; Leu292Pro) and a polymorphism (c. 1403G>A; Arg468His, dbSNP rs#6637934). The proband was hemizygous for both variations and his mother and maternal grandmother were heterozygous carriers. CONCLUSION: This study provides an additional evidence for mutations in FRMD7 as a common cause of X-linked CN and expands its mutation spectrum.
Clinical Coding ; DNA ; Humans ; Introns ; Leukocytes ; Mothers ; Nystagmus, Congenital* ; Wills