No abstract available.
Hematuria* ; Humans

A 9-year-old boy showed orofacial dyskinesia and psychic symptoms shortly after open heart surgery with deep hypothemia and cardiopulmonary bypass for congenital cyanotic heart disease. The choreothetosis progressed to be generalized and accompanied by mental deterioration. This involuntary movement partially responded only to repetitive administration of sedatives. After 3 month the patient expired with sudden development of ventricular tachycardia and cardiogenic shock.
Cardiopulmonary Bypass* ; Child ; Dyskinesias ; Heart Diseases ; Humans ; Hypnotics and Sedatives ; Hypothermia* ; Male ; Movement Disorders ; Shock, Cardiogenic ; Tachycardia, Ventricular ; Thoracic Surgery

Severe segmental renal atrophy with loss of parenchymal elements in small kidney is commonly known as segmental hypoplasia. Since the original description in 1929 by Ask-Upmark, the association of hypertension with congenital renal hypoplasis has been well established and there have been several reports. But there was no reported case in Korea. The diseases commonly recognized after the age of 10 years, although a single case report of Ask-Upmark kidney had been reported in a 13 month old boy, who may be the youngest. But our case is 3 month old boy, who was died because of renal failure inspite of adequate treament including peritoneal dialysis.
Atrophy ; Humans ; Hypertension ; Infant ; Kidney* ; Korea ; Male ; Peritoneal Dialysis ; Renal Insufficiency

Serotonin syndrome, an adverse drug reaction, is a consequence of excess serotonergic agonism of central nervous system receptors and peripheral serotonergic receptors. Serotonin syndrome has been associated with large numbers of drugs and drug combinations, and serotonin-norepinephrine reuptake inhibitor-induced serotonin syndrome is rare. It is often described as a sign of excess serotonin ranging from tremor in mild cases to delirium, neuromuscular rigidity, and hyperthermia in life-threatening cases. Diagnosis is based on the symptoms and patient's history, and several diagnostic criteria have been developed. We experienced a rare case of fibromyalgia accompanied by tremor, hyperreflexia, spontaneous clonus, muscle rigidity, and diaphoresis after 10 days of single use of duloxetine 30 mg. Only one case of serotonin syndrome resulting from administration of duloxetine has been reported in Korea, however that case resulted from co-administration of fluoxetine. We report here on this case along with a review of the relevant literature.
Central Nervous System ; Delirium ; Diagnosis ; Drug Combinations ; Drug-Related Side Effects and Adverse Reactions ; Duloxetine Hydrochloride* ; Felodipine ; Fever ; Fibromyalgia* ; Fluoxetine ; Humans ; Korea ; Muscle Rigidity ; Reflex, Abnormal ; Serotonin Syndrome* ; Serotonin* ; Tremor

Idiopathic non-cirrhotic portal hypertension (INCPH) is a disease with an uncertain etiology consisting of non-cirrhotic portal hypertension and portal pressure increase in the absence of liver cirrhosis. In INCPH, patients exhibit normal liver functions and structures. The factors associated with INCPH include the following: Umbilical/portal pyremia, bacterial diseases, prothrombic states, chronic exposure to arsenic, vinyl chloride monomers, genetic disorders, and autoimmune diseases. Approximately 70% of patients present a history of major variceal bleeding, and treatment relies on the prevention of complications related to portal hypertension. Autoimmune disorders associated with INCPH are mainly systemic sclerosis, systemic lupus erythematosus and rheumatoid arthritis. To the best of our knowledge, a case of ankylosing spondylitis (AS) associated with INCPH has not been reported thus far. Therfore, we report our experience of a patient with AS accompanied by INCPH, who showed perisplenic varices with patent spleno-portal axis and hepatic veins along with no evidence of cirrhosis on liver biopsy, and provide a brief literature review.

Idiopathic non-cirrhotic portal hypertension (INCPH) is a disease with an uncertain etiology consisting of non-cirrhotic portal hypertension and portal pressure increase in the absence of liver cirrhosis. In INCPH, patients exhibit normal liver functions and structures. The factors associated with INCPH include the following: Umbilical/portal pyremia, bacterial diseases, prothrombic states, chronic exposure to arsenic, vinyl chloride monomers, genetic disorders, and autoimmune diseases. Approximately 70% of patients present a history of major variceal bleeding, and treatment relies on the prevention of complications related to portal hypertension. Autoimmune disorders associated with INCPH are mainly systemic sclerosis, systemic lupus erythematosus and rheumatoid arthritis. To the best of our knowledge, a case of ankylosing spondylitis (AS) associated with INCPH has not been reported thus far. Therfore, we report our experience of a patient with AS accompanied by INCPH, who showed perisplenic varices with patent spleno-portal axis and hepatic veins along with no evidence of cirrhosis on liver biopsy, and provide a brief literature review.
Arsenic ; Arthritis, Rheumatoid ; Autoimmune Diseases ; Biopsy ; Esophageal and Gastric Varices ; Fibrosis ; Hepatic Veins ; Humans ; Hypertension, Portal ; Liver ; Liver Cirrhosis ; Lupus Erythematosus, Systemic ; Portal Pressure ; Scleroderma, Systemic ; Spondylitis, Ankylosing ; Varicose Veins ; Vinyl Chloride

Gitelman syndrome is a condition caused by a mutation of the thiazide sensitive Na-Cl cotransporter gene on the distal convoluted tubule. It results in a variety of clinical features, including hypokalemia, hypomagnesemia, hypocalciuria, and metabolic alkalosis. It is often diagnosed in asymptomatic adults presented with unexplained hypokalemia; however, it is sometimes associated with muscular cramps, numbness, fatigue, weakness, or paralysis. We experienced a case of rheumatoid arthritis accompanied by Gitelman syndrome, presented with hand tremor. We diagnosed her using renal clearance study and genetic analysis. Here, we report our experiences regarding this case along with a literature review.
Adult ; Alkalosis ; Arthritis, Rheumatoid* ; Fatigue ; Furosemide ; Genetic Testing ; Gitelman Syndrome* ; Hand ; Humans ; Hypesthesia ; Hypokalemia ; Muscle Cramp ; Paralysis ; Solute Carrier Family 12, Member 3 ; Thiazides ; Tremor

Neuropathic pain is usually managed pharmacologically, rather than with botulinum toxin type A (BTX-A). However, medications commonly fail to relieve pain effectively or have intolerable side effects. We present the case of a 62-year-old man diagnosed with an intracranial chondrosarcoma, which was removed surgically and treated with radiation therapy. He suffered from neuropathic pain despite combined pharmacological therapy with gabapentin, amitriptyline, tramadol, diazepam, and duloxetine because of adverse effects. BTX-A (100 units) was injected subcutaneously in the most painful area in the posterior left thigh. Immediately after the injection, his pain decreased significantly from 6/10 to 2/10 on a visual analogue scale. Pain relief lasted for 12 weeks. This case report describes intractable neuropathic pain caused by a brain tumor that was treated with subcutaneous BTX-A, which is a useful addition for the management of neuropathic pain related to a brain tumor.
Amitriptyline ; Botulinum Toxins* ; Botulinum Toxins, Type A* ; Brain Neoplasms* ; Brain* ; Chondrosarcoma ; Diazepam ; Duloxetine Hydrochloride ; Humans ; Middle Aged ; Neuralgia* ; Thigh ; Tramadol

PURPOSE: This study was undertaken to determine the differences in genotypes of rotavirus and their incidence between patients with acute rotaviral enteritis who suffered neurologic complications and those who did not suffer neurologic complications. METHODS: Among the 82 patients with rotaviral enteritis whose genotype was analyzed, 71 patients were not associated with neurologic complications(neurology(-) group), and eleven patients were associated with neurologic complications(neurology(+) group). Four G genotypes and four P genotypes were determined by reverse trans cription and multiplex polymerase chain reaction. RESULTS: In the neurology(-) group, the number of G4, G3, G2 and G1 was 24(35.3 percent), 23 (33.8 percent), 17(25.0 percent) and 4(5.6 percent), respectively and the number of P6, P4, P8 and P9 was 26(36.1 percent), 23(31.9 percent), 22(30.6 percent) and 1(1.4 percent), respectively. G2P4, and G4P6 were the most frequently found com bination genotypes, respectively. In the neurology(+) group, the number of G2, G3 and G4 was 9(75.0 percent), 2(16.7 percent) and 1(8.3 percent), respectively and the number of P4, P6, P8 and P9 was 8(66.7 percent), 2(16.7 percent), 1(8.3 percent) and 1(8.3 percent), respectively. G2P4 was the most frequently found combination genotype. The incidence of each of the G2, P4 and G2P4 was significantly higher in the neurology(+) group when compared to that of each of the G2, P4, and G2P4 in the neurology(-) group. CONCLUSION: In the patients with acute rotavirus gastroenteritis associated with neurologic complications, the G1 genotype was not found and genotypes of the G2, P4, and G2P4 were more frequently found.
Enteritis* ; Gastroenteritis ; Genotype* ; Humans ; Incidence ; Multiplex Polymerase Chain Reaction ; Rotavirus* ; Seizures

BACKGROUND/AIMS: Although trials have suggested an association between osteoporosis and cardiovascular disease (CVD), the relationship between fracture risk and cardiovascular disease is not well defined. Here, we examined whether subjects with a higher risk of fracture also share an increased likelihood of developing CVD. METHODS: This study included 477 subjects; patients with a history of diabetes, chronic hepatopathy, nephritic syndrome, or any cardiovascular diseases were excluded. We used dual energy X-ray absorptiometry to assess the bone mineral density (BMD) of the lumbar spine and femur, and calculated fracture risk based on the Fracture Risk Assessment (FRAX) score. The Framingham risk score (FRS) was used to estimate cardiovascular risk. RESULTS: Of the 477 subjects, 222 had osteopenia and 150 had osteoporosis; the remaining 105 had a normal BMD. In men, no significant differences were observed in systolic blood pressure (SBP), diastolic blood pressure, low-density lipoprotein, high-density lipoprotein (HDL), and triglyceride (TG) between groups. Men with osteoporosis were generally older, and had significantly higher total cholesterol (TC). In women, age and FRS were significantly higher in the osteoporosis group. In the multivariate analysis, age, SBP, TC, HDL, TG, and FRAX were all significantly associated with FRS. CONCLUSIONS: These data suggest that patients with a higher risk of fracture are also at greater risk of developing CVD, indicating a possible mechanistic link between CVD and osteoporosis.
Absorptiometry, Photon ; Blood Pressure ; Bone Density ; Bone Diseases, Metabolic ; Cardiovascular Diseases ; Cholesterol ; Female ; Femur ; Humans ; Lipoproteins ; Male ; Multivariate Analysis ; Osteoporosis ; Risk Assessment* ; Spine ; Triglycerides