Purpose: To investigate the outcomes of patients who underwent surgical correction of congenital superior oblique muscle paralysis in adulthood. Methods: We retrospectively analyzed the medical records of 35 patients who underwent surgical correction of congenital superior oblique muscle paralysis after the age of 18 years. At the final follow-up, success was defined as residual hypertropia < 4 prism diopters (PD), whereas failure was defined as residual hypertropia ≥ 5 PD or overcorrection. Clinical features were compared between the success and failure groups to identify factors affecting the surgical outcome. At the final follow-up, the resolution of symptoms, such as head tilt and diplopia, was also recorded. Results: Of the 35 patients, 24 (68.6%) and 11 (31.4%) were classified into the success and failure groups, respectively. Preoperative vertical deviation was larger in the failure group compared to the success group (23.1 vs. 15.2 PD, respectively; p < 0.05). Of the 31 patients with preoperative head tilt, head tilt resolved in 29 (93.5%). Of the 19 patients with diplopia, it resolved in all except 1 who was overcorrected. Conclusions The preoperative vertical angle of the superior oblique muscle may affect the outcome of surgery performed in adulthood for correcting congenital superior oblique paralysis. Head tilt, diplopia, and subjective symptoms are typically resolved postoperatively.

Purpose: To report a case of keratitis caused by Brevundimonas diminuta/vesicularis.Case summary: A 20-year-old man presented with decreased visual acuity and glare in his right eye from 2 days earlier. At the first visit, slit lamp examination revealed a 1.5 × 1.5 mm2-sized stromal infiltration, an edematous cornea and endothelial deposits in the center of the right eye. Microbiological examinations were performed from corneal scrapings. Treatment was started with topical eye drops (fortified cefazolin, fortified tobramycin) as empirical antibiotics. After several days, the cultures revealed Brevundimonas diminuta/vesicularis. After treatment with empirical antibiotic eye drops, the symptoms and visual acuity improved. Conclusions We report a case of infectious keratitis caused by Brevundimonas diminuta/vesicularis, which has never previously been reported in the Republic of Korea.

BACKGROUND: Diabetic cardiac autonomic neuropathy (CAN) is one of the important complications of diabetes. It is characterized by reduced heart rate variability (HRV). METHODS: In this randomized, double-blind, placebo-controlled, multicenter trial, 75 patients were randomly assigned to one of two groups. One group (n=41) received α-lipoic acid (ALA) at an oral dose of 600 mg/day for the first 12 weeks and then 1,200 mg/day for the next 12 weeks. The other group (n=34) received placebo treatment for 24 weeks. CAN was assessed by measuring HRVs in people with diabetes. RESULTS: Most of the baseline measures for HRVs were similar between the ALA and placebo groups. Although there were no statistically significant HRV changes in the ALA group compared to the placebo group after 24 weeks of trial, we found a positive tendency in some of the HRV parameters of the ALA group. The standard deviations of normal-to-normal RR intervals in the standing position increased by 1.87 ms in the ALA group but decreased by −3.97 ms in the placebo group (P=0.06). The power spectrum of the low frequency (LF) band in the standing position increased by 15.77 ms² in the ALA group, whereas it declined by −15.04 ms² in the placebo group (P=0.08). The high frequency/LF ratio in the upright position increased by 0.35 in the ALA group, whereas it declined by −0.42 in the placebo group (P=0.06). There were no differences between the two groups regarding rates of adverse events. CONCLUSION: Although a slight improvement tendency was seen in HRV in the ALA group, there were no statistically significant HRV changes in the ALA group compared to the placebo group after 24 weeks of trial. However, the high oral dose of ALA was well-tolerated.
Diabetes Mellitus, Type 2* ; Heart Rate* ; Heart* ; Humans ; Korea* ; Multicenter Studies as Topic ; Posture ; Thioctic Acid

BACKGROUND: Recently, the amiodarone has emerged as a promising antiarrhythmic agent and its efficacy and safety has been widely accepted with many literatures. But there was no general agreement regarding the dosage and indication of intravenous (IV) amiodarone in acute myocardial infarction with life-threatening refractory ventricular tachyarrhythmias. METHOD: From October 1995 through October 1997, we recruited retrospectively 9 patients of acute myocardial infarction who had received IV amiodarone for life-threatening refractory ventricular tachyarrhythmias and analyzed the initial response, adverse effect, and loading dose. RESULTS: 1) Acute efficacy:Eight of 9 patients promptly restored normal sinus rhythm immediately after intravenous amiodarone administration. 2) In-Hospital Mortality:One patients died due to ventricular tachyarrhythmias refractory to aggressive management and 5 in 8 patients who had responded promptly with IV amiodarone discharged alive and other 3 patients died due to cardiogenic shock with normal sinus rhythm. 3) Immediate adverse effects:Five patients experienced immediate adverse effects after IV amiodarone; 3 patients of hypotension, 1 patient of first degree AV block, and the other of Morbitz type 2 AV block. 4) Long term follow-up:Among 5 patients discharged alive, one died as unexpected consequence. Other 4 patients have been still alive without maintenance medication. CONCLUSION: The IV amiodarone for suppression of life-threatening ventricular tachyarrhythmias in patients with acute myocardial infarction seemed to be an effective second-line therapeutic drug and have acceptable adverse effects. In the future, the large scale study regarding the dosage and indication might be warrented.
Amiodarone* ; Atrioventricular Block ; Humans ; Hypotension ; Myocardial Infarction* ; Retrospective Studies ; Shock, Cardiogenic ; Tachycardia*

There are many causes of sudden cardiac arrest. The main cause of sudden cardiac death (SCD) is coronary heart disease. However, the frequency of coronary heart disease is much lower in sudden cardiac arrest occurring below the age of 30-40. Congenital anomalous origin of the coronary arteries is a rare, but well-described, cause of myocardial ischemia and sudden death in young adults. Here, we report the case of a 23-year-old man with sudden cardiac arrest due to ventricular fibrillation associated with an anomalous origin of the right coronary artery. The patient was diagnosed using multi-detector computed tomography and successfully treated with surgical correction.
Coronary Disease ; Coronary Vessel Anomalies ; Coronary Vessels ; Death, Sudden ; Death, Sudden, Cardiac ; Humans ; Myocardial Ischemia ; Ventricular Fibrillation ; Young Adult

BACKGROUND: Leptin has been suggested as a possible cause of atherosclerotic disease. The small dense low-density lipoprotein cholesterol (LDL-C) has also been regarded as a new surrogate marker in atherosclerotic disease. The aim of this study was to evaluate the relationship between the leptin concentration and the small dense LDL-C concentration in Korean type 2 diabetic patients. METHODS: One hundred-ninety one type 2 diabetic patients, who did not use any medication that could affect the concentration of lipid such as statin, fibrate, thiazolidinediones and corticosteroid, were enrolled in this study. We analyzed the relationship between leptin, the small dense LDL-C and the other metabolic parameters. RESULTS: The small dense LDL-C concentrations were higher in the group with the highest tertile of the leptin value, both in males and females than those patients in the group with the lowest tertile of the leptin value. The small dense LDL-C concentrations were also higher in the group with the highest tertile of leptin divided by the BMI value both in males and females than those patients in the group with the lowest tertile of the leptin value. The leptin concentration was positively correlated with the small dense LDL-C, total cholesterol, triglyceride, LDL-C, insulin and HOMAIR values after adjusting for age, gender and BMI. CONCLUSION: The association between leptin and small dense LDL-C could be a factor that explains the association between leptin and cardiovascular disease.
Biomarkers ; Cardiovascular Diseases ; Cholesterol ; Cholesterol, LDL* ; Diabetes Mellitus, Type 2 ; Female ; Humans ; Hydroxymethylglutaryl-CoA Reductase Inhibitors ; Insulin ; Leptin* ; Lipoproteins ; Male ; Thiazolidinediones ; Triglycerides

BACKGROUND: Leptin has been suggested as a possible cause of atherosclerotic disease. The small dense low-density lipoprotein cholesterol (LDL-C) has also been regarded as a new surrogate marker in atherosclerotic disease. The aim of this study was to evaluate the relationship between the leptin concentration and the small dense LDL-C concentration in Korean type 2 diabetic patients. METHODS: One hundred-ninety one type 2 diabetic patients, who did not use any medication that could affect the concentration of lipid such as statin, fibrate, thiazolidinediones and corticosteroid, were enrolled in this study. We analyzed the relationship between leptin, the small dense LDL-C and the other metabolic parameters. RESULTS: The small dense LDL-C concentrations were higher in the group with the highest tertile of the leptin value, both in males and females than those patients in the group with the lowest tertile of the leptin value. The small dense LDL-C concentrations were also higher in the group with the highest tertile of leptin divided by the BMI value both in males and females than those patients in the group with the lowest tertile of the leptin value. The leptin concentration was positively correlated with the small dense LDL-C, total cholesterol, triglyceride, LDL-C, insulin and HOMAIR values after adjusting for age, gender and BMI. CONCLUSION: The association between leptin and small dense LDL-C could be a factor that explains the association between leptin and cardiovascular disease.
Biomarkers ; Cardiovascular Diseases ; Cholesterol ; Cholesterol, LDL* ; Diabetes Mellitus, Type 2 ; Female ; Humans ; Hydroxymethylglutaryl-CoA Reductase Inhibitors ; Insulin ; Leptin* ; Lipoproteins ; Male ; Thiazolidinediones ; Triglycerides

PURPOSE: To correlate the mammographic and ultrasonographic findings with the pathologic results in women undergoing hormone replacement therapy (HRT), and to determine the characteristic clinical, mammographic or histologic findings of breast cancer in these patients. MATERIALS AND METHODS: Twenty-five breast lesions in 25 patients aged 44-65 (mean, 55.5) years undergoing HRT were surgically removed due to abnormal mammographic findings or the presence of palpable masses. Mammograms in all patients and ultrasonograms in 23 were retrospectively analyzed in terms of the shape and margin of the mass, and microcalcifications, and the imaging findings were correlated with the pathologic results. As a control group, 45 cancer patients not undergoing HRT were selected. Using the student t test, detection methods, tumor size, mammographic findings, and the proportion of intraductal cancers were compared between the two groups. RESULTS: Surgical excision revealed ten benign lesions (four fibroadenomas and six cases of fibrocystic change) and 15 cancers (three intraductal and twelve invasive ductal cancers). Abnormal findings at mammography were a mass in 16 cases, clustered microcalcifications in seven, and a mass with microcalcifications in two. Mammography showed that all four circumscribed masses were benign. Five of seven ill-defined masses (71%) and all six spiculated masses were malignant. Three of seven cases (43%) with microcalcification, and both with a mass and microcalcification, were malignant. In two cases in which ultrasonography revealed cystic lesions, histologic examination showed that fibrocystic change had occurred. Compared to non-HRT-related cancers, HRT-related cancers were more often detected by mammography (60% vs 16%; p <0.001), smaller (17 mm vs 24 mm, p <0.01), showed microcalcification only (20% vs 13%; p <0.05), and were intraductal (20% vs 7%; p <0.01). CONCLUSION: In patients with HRT, mammographic findings of an ill-defined or spiculated mass, or one with microcalcifications, were associated with breast cancer. Compared to non-HRT-related cancers, breast cancers in patients undergoing HRT tend to manifest more frequently as a mammographic abnormality, and to be intraductal.
Breast Neoplasms ; Breast* ; Female ; Fibroadenoma ; Hormone Replacement Therapy* ; Humans ; Mammography ; Retrospective Studies ; Ultrasonography

Dominantly inherited spinocerebellar ataxias (SCAs) are a group of the heterogenous neurodegenerative diseases that are characterized by chronic progressive cerebellar ataxia associated with various combinations of other neurological signs. Clinical classification is difficult because of the phenotypic overlap. With the evolution of molecular genetics, the loci and mutations for many of the ataxias have been identified, allowing more definitive molecular classification. We experienced 42 years-old man who presented with progressive both lower leg weakness, dysarthria, ataxia, ophthalmoplegia, and nystagmus. The family history was remarkably suspicious. We could not observe the upper extremity weakness, definite evidences of peripheral neuropathy and myopathy in electrodiagnosis. No abnormal findings in blood chemistry and brain MRI. We performed polymerase chain reaction (PCR) and polyacrylamide gel electrophoresis (PAGE) analysis, found that his gene contained expanded CAG repeats (CAG repeat number was 72). Although no effective treatment exists for most the ataxic syndromes, the accurate diagnosis and the genetic counseling are often important to the patient's family for prognostication.
Adult ; Ataxia ; Brain ; Cerebellar Ataxia ; Chemistry ; Classification ; Diagnosis ; Dysarthria ; Electrodiagnosis ; Electrophoresis, Polyacrylamide Gel ; Genetic Counseling ; Humans ; Leg ; Machado-Joseph Disease* ; Magnetic Resonance Imaging ; Molecular Biology ; Muscular Diseases ; Neurodegenerative Diseases ; Ophthalmoplegia ; Peripheral Nervous System Diseases ; Polymerase Chain Reaction ; Spinocerebellar Ataxias* ; Upper Extremity

Ketosis-prone diabetes mellitus (KPD), which is an atypical type of diabetic mellitus with severe β cell dysfunction, is accompanied by ketosis or ketoacidosis without specific preceding factors at diagnosis. KPD shows mixed features of type 1 and type 2 diabetes. In some cases, the recovery of the function of β cells during intensified diabetic management enabled the termination of insulin therapy. The Aβ classification system classifies KPD patients into four distinct subgroups depending upon the presence or absence of β cell autoimmunity and β cell functional reserve and has been recognized as an important tool to predict clinical outcomes. In Korea, several cases of KPD with absence of β cell autoimmunity have been reported. A 60-year-old man presenting with DKA (diabetic ketoacidosis) as the first manifestation of diabetes, was shown to have β cell autoimmunity. A significant improvement in glycemic control was shown as a result of aggressive diabetic management; shortly after an acute episode of DKA, the recovery of β cell functional reserve was confirmed. This result allowed discontinuation of insulin therapy and maintenance of euglycemic status without antidiabetic medication.
Autoimmunity ; Classification ; Diabetes Mellitus, Type 1* ; Diabetes Mellitus, Type 2 ; Diabetic Ketoacidosis ; Diagnosis ; Humans ; Insulin ; Ketosis ; Korea ; Middle Aged