A novel c.563 T>G, p.L189R lamin A/C mutation in identical twins with dilated cardiomyopathy

No abstract available.

Dyslipidemia and Lipid-Lowering in Patients with High Risk of Cardiovascular Diseases and Their Cardiovascular Outcomes in Korea (ENSURE study): Secondary Prevention in Chronic Stable Angina

BACKGROUND AND OBJECTIVES: The aim of this study was to investigate the status of LDL-cholesterol level and its relationship with subsequent cardiovascular events in Korean patients with chronic stable angina. METHODS: The patients with stable angina were retrospectively and consecutively enrolled from out-patients clinic during 2007-2009. Mean follow-up duration was 3 years. Occurrences of major adverse cardio-cerebrovascular event (MACCE: a composite of death, myocardial infarction, unstable angina, coronary revascularization, cerebrovascular events, peripheral artery disease and aortic disease requiring hospital admission.) were compared by initial LDL-cholesterol levels using Cox proportional-hazards model. RESULTS: 1,683 subjects were enrolled from 9 hospitals. Initial median LDL-cholesterol by tertile was 62.2, 90.2, and 124.0mg/dL respectively, however, the differences in LDL-cholesterol level among initial 3 tertile groups became narrow at 3rd year (67.8, 85.0, and 91.6mg/dL, respectively). MACCE occurred in 138 (8.2%) subjects, including 127 coronary events, 9 cerebrovascular events and 2 peripheral artery disease during the 3-year follow-up. The adjusted hazard ratio for MACCE was 1.02 (95% confidence interval 0.64-1.64) in the middle tertile of LDL-cholesterol, 1.53 (p=0.063, 95% Confidence Interval 0.98-2.40) in the highest tertile of LDL-cholesterol. The newly diagnosed diabetes mellitus was more frequent in subjects with statin treatment than subjects without statin during the 3-year follow-up (1.5% vs 0.6%). CONCLUSION: Increased cardiovascular risk was observed in angina patients with higher initial LDL-cholesterol levels during the 3-year follow-up, although the differences were statistically insignificant.

Pseudoaneurysm with Arteriovenous Fistula after Arthroscopic Procedure: A Rare Complication of Arthroscopy

Pseudoaneurysm with arteriovenous fistula is a rare complication of arthroscopy, and can be diagnosed by ultrasonography, computed tomography, magnetic resonance imaging, or angiography. This condition can be treated with open surgical repair or endovascular repair. We report our experience with the open surgical repair of a pseudoaneurysm with an arteriovenous fistula in a young male patient who underwent arthroscopy five months previously.

Intraoperative Balloon Angioplasty Using Fogarty Artertial Embolectomy Balloon Catheter for Creation of Arteriovenous Fistula for Hemodialysis: Single Center Experience

BACKGROUND: The purpose of this study was to evaluate the use of a Fogarty arterial embolectomy catheter (Fogarty catheter) in intraoperative balloon angioplasty of the cephalic vein, in order to determine its effect on the patency of arteriovenous fistulas (AVFs) created for hemodialysis access. METHODS: A total of 156 patients who underwent creation of an AVF were divided into two groups, based whether a Fogarty catheter was used during AVF creation. Group A (89 patients) comprised the patients who underwent balloon angioplasty with a Fogarty catheter during the operation. Group B (67 patients) included the patients in whom a Fogarty catheter was not used during the operation. Patient records were reviewed retrospectively and documented. The patency rate was determined by the Kaplan-Meier method. RESULTS: The records of 156 patients who underwent the creation of an AVF from January 2007 to October 2011 were included. The mean follow-up duration was 40.2+/-19.4 months (range, 1 to 97 months). The patency rates in group A at 12, 36, and 72 months were 83.9%+/-3.9%, 78.3%+/-4.6%, and 76.3%+/-4.9%, respectively, while the corresponding patency rates in group B were 92.5%+/-3.2%, 82.8%+/-0.5%, and 79.9%+/-5.7%, respectively. The patency rates in group B were found to be slightly higher than those in group A, but the difference was not statistically significant (p=0.356). CONCLUSION: Intraoperative balloon angioplasty of the cephalic vein using the Fogarty catheter is a simple and easily reproducible procedure, and it can be helpful in increasing AVF patency in cases of insufficient runoff or a suboptimal cephalic vein.

Prevalence and Gender-Related Characteristics of Metabolic Syndrome in Korean Community

OBJECTIVE: There are still a limited number of studies assessing the prevalence of metabolic syndrome in the community. The aim of this study is to investigate the prevalence and gender-related characteristics of metabolic syndrome in Korean community. METHODS: A total of 417 community subjects (mean age was 60.7+/-13.6 years, 35.3% were men) who attended the routine check-up were analyzed. National Cholesterol Education Program-Adult Treatment Panel (NCEP-ATP) III clinical guideline was used to define metabolic syndrome. RESULTS: Metabolic syndrome was diagnosed in 38.1% of study subjects. The prevalence of metabolic syndrome was not different between men and women (men 39.0% vs. women 37.5%, p=0.766). The positive association between age and the prevalence of metabolic syndrome was more pronounced in women (chi2=17.52, p for trend or =50 years). The most prevalent factor of metabolic syndrome was hypertriglyceridemia (49.9%) and hypertension (47.6%) in both genders. Among metabolic syndrome components, central obesity (40.5% vs. 25.2%, p=0.002) and hypertriglyceridemia (54.5% vs. 41.8%, p=0.015) were more prevalent in women than in men, and the prevalence of other components were similar between genders. CONCLUSIONS: In the community, metabolic syndrome was highly prevalent in middle-aged and elderly Korean adult. Age related change in the prevalence of metabolic syndrome was gender specific. Age and gender effects should be considered for the effective control of metabolic syndrome in the community.

Diabetic ketoacidosis as a presenting symptom of complicated pancreatic cancer

No abstract available.

Cardiac Hemangioma: A Case Report

Hemangioma of the heart, presenting as a primary cardiac tumor is extremely rare; it accounts for approximately 2% of all primary resected heart tumors. In our patient, the tumor was located in the orifice of the right lower pulmonary vein. Few cases of cardiac hemangiomas have been reported to arise from the left atrial (LA) wall. Left atrial hemangiomas, especially those attached to the LA wall, may be erroneously diagnosed as myxomas. Cardiac hemangioma is a rare disease; furthermore, a tumor arising from the LA wall and misconceived as a myxoma is extremely rare. We removed a mass misdiagnosed as a myxoma; it was pathologically confirmed to be a cardiac capillary hemangioma. Therefore, we report a rare case of a cardiac hemangioma misconceived as a myxoma; the tumor was removed successfully.

Successful desensitization for cytarabine induced anaphylaxis

Cytarabine is a very important chemotherapeutic agent for leukemia and lymphoma patients and is prescribed more frequently than before. Cytarabine-induced delayed-onset hypersensitivity may rarely present as non-IgE mediated anaphylaxis. However, we do not know yet whether desensitization therapy in adults may be effective in cytarabine-induced delayed-onset anaphylaxis. A 78-year-old woman who was diagnosed with acute myeloblastic leukemia had chemotherapy including cytarabine. In spite of premedication with hydrocortisone and chlorpheniramine, the patient had anaphylaxis a few hours after cytarabine infusion. We decided to perform desensitization therapy. After desensitization therapy using a newly designed protocol, the patient was tolerable to cytarabine infusion without hypotension or high fever. Desensitization therapy was successfully performed on an adult patient with delayed-onset anaphylaxis caused by cytarabine.

Primary Synovial Sarcoma of the Parietal Pleura: A Case Report

Synovial sarcoma is a malignant soft tissue tumor that most commonly occurs in the extremities of young and middle-aged adults, in the vicinity of large joints. Although synovial sarcoma is frequently associated with joints, it may arise in unexpected sites, such as the mediastinum, heart, lung, pleura, or chest wall. Primary synovial sarcoma of the pleura is rare. To date, nearly 36 cases of primary synovial sarcoma of the pleura have been reported since Gaertner et al. published the first case in 1996. The oncologic characteristics, treatment, and prognosis for pleural synovial sarcomas are not well defined because of a paucity of data. However, a multimodal approach, including surgical resection, chemotherapy, and radiotherapy, has generally been suggested. We report the outcome of one patient with primary pleural synovial sarcoma treated with radical resection and adjuvant treatment.

Clinical Analysis of Radiocephalic Fistula Using Side-to-side Anastomosis with Distal Cephalic Vein Ligation

BACKGROUND: The surgically created arteriovenous fistula has recently been recommended as the best available angioaccess for hemodialysis. Therefore, in this study, we carried out a clinical analysis on surgical procedures in the ligation and division of a distal vein to achieve similar effects as those of vein end-to-arterial side after side-to-side anastomosis. METHODS: We retrospectively reviewed the clinical data of 113 patients who came for an outpatient clinic follow-up to the department of internal medicine of our hospital; these patients were among the 125 patients who underwent radiocephalic arteriovenous fistula (side-to-side anastomosis with distal vein ligation and division) in our hospital in the period from January 2006 to December 2010. RESULTS: The patency rate showed no statistical significance with respect to sex (p=0.775), age (p=0.775), hypertension (p=0.262), diabetes (p=0.929), and cardio-neurovascular disease (p=0.717). Patency rates were 96% for the first month, 93% for the first year, and 90% for the second year for the radiocephalic arteriovenous fistula (side-to-side anastomosis with distal vein ligation and division) performed on the wrist. CONCLUSION: The patency rates revealed favorable results and few postoperative complications as compared to those of previous reports. Therefore, radiocephalic fistula using side-to-side anastomosis with distal cephalic vein ligation is considered a recommendable surgical procedure in the distal part for the hemodialysis of CRF patients.

Primary Surgical Closure Should Be Considered in Premature Neonates with Large Patent Ductus Arteriosus

BACKGROUND: Treatment for patent ductus arteriosus (PDA) in premature infants can consist of medical or surgical approaches. The appropriate therapeutic regimen remains contentious. This study evaluated the role of surgery in improving the survival of premature neonates weighing less than 1,500 g with PDA. MATERIALS AND METHODS: From January 2008 to June 2011, 68 patients weighing less than 1,500 g with PDA were enrolled. The patients were divided into three groups: a group managed only by medical treatment (group I), a group requiring surgery after medical treatment (group II), and a group requiring primary surgical treatment (group III). RESULTS: The rate of conversion to surgical methods due to failed medical treatment was 67.6% (25/37) in the patients with large PDA (> or =2 mm in diameter). The number of patients who could be managed with medical treatment was nine which was only 20.5% (9/44) of the patients with large PDA. There was no surgery-related mortality. Group III displayed a statistically significantly low rate of development of bronchopulmonary dysplasia (BPD) (p=0.008). The mechanical ventilation time was significantly longer in group II (p=0.002). CONCLUSION: Medical treatment has a high failure rate in infants weighing less than 1,500 g with PDA exceeding 2.0 mm. Surgical closure following medical treatment requires a longer mechanical ventilation time and increases the incidence of BPD. Primary surgical closure of PDA exceeding 2.0 mm in the infants weighing less than 1,500 g should be considered to reduce mortality and long-term morbidity events including BPD.

Primary Surgical Closure Should Be Considered in Premature Neonates with Large Patent Ductus Arteriosus

BACKGROUND: Treatment for patent ductus arteriosus (PDA) in premature infants can consist of medical or surgical approaches. The appropriate therapeutic regimen remains contentious. This study evaluated the role of surgery in improving the survival of premature neonates weighing less than 1,500 g with PDA. MATERIALS AND METHODS: From January 2008 to June 2011, 68 patients weighing less than 1,500 g with PDA were enrolled. The patients were divided into three groups: a group managed only by medical treatment (group I), a group requiring surgery after medical treatment (group II), and a group requiring primary surgical treatment (group III). RESULTS: The rate of conversion to surgical methods due to failed medical treatment was 67.6% (25/37) in the patients with large PDA (> or =2 mm in diameter). The number of patients who could be managed with medical treatment was nine which was only 20.5% (9/44) of the patients with large PDA. There was no surgery-related mortality. Group III displayed a statistically significantly low rate of development of bronchopulmonary dysplasia (BPD) (p=0.008). The mechanical ventilation time was significantly longer in group II (p=0.002). CONCLUSION: Medical treatment has a high failure rate in infants weighing less than 1,500 g with PDA exceeding 2.0 mm. Surgical closure following medical treatment requires a longer mechanical ventilation time and increases the incidence of BPD. Primary surgical closure of PDA exceeding 2.0 mm in the infants weighing less than 1,500 g should be considered to reduce mortality and long-term morbidity events including BPD.

Subacute thyroiditis presenting as acute psychosis: a case report and literature review

We describe herein an unusual case of subacute thyroiditis presenting as acute psychosis. An 18-year-old male presented at the emergency department due to abnormal behavior, psychomotor agitation, sexual hyperactivity, and a paranoid mental state. Laboratory findings included an erythrocyte sedimentation rate of 36 mm/hr (normal range, 0 to 9), free T4 of 100.0 pmol/L (normal range, 11.5 to 22.7), and thyroid stimulating hormone of 0.018 mU/L (normal range, 0.35 to 5.5). A technetium-99m pertechnetate scan revealed homogeneously reduced activity in the thyroid gland. These results were compatible with subacute thyroiditis, and symptomatic conservative management was initiated. The patient's behavioral abnormalities and painful neck swelling gradually resolved and his thyroid function steadily recovered. Although a primary psychotic disorder should be strongly considered in the differential diagnosis, patients with an abrupt and unusual onset of psychotic symptoms should be screened for thyroid abnormalities. Furthermore, transient thyroiditis should be considered a possible underlying etiology, along with primary hyperthyroidism.

Effect of Granulocyte Colony-Stimulating Factor on the Peripheral Nerves in Streptozotocin-Induced Diabetic Rat

There are controversial reports about the effect of granulocyte colony-stimulating factor (G-CSF) in peripheral nerve protection. Therefore, the present study aimed to investigate the effect of G-CSF on peripheral nerves in streptozotocin (STZ) induced diabetic rats. After STZ or vehicle injection, rats were divided into five groups (n=6) as follows: normal+vehicle, normal+G-CSF (50 microg/kg for 5 days), diabetes mellitus (DM)+vehicle, DM+G-CSF (50 microg/kg for 5 days), and DM+G-CSF extension (50 microg/kg for 5 days and followed by two injections per week up to 24 weeks). Our results showed that the current perception threshold was not significantly different among experimental groups. G-CSF treatment inhibited the loss of cutaneous nerves and gastric mucosal small nerve fibers in morphometric comparison, but statistical significance was not observed. The present results demonstrated that G-CSF has no harmful but minimal beneficial effects with respect to peripheral nerve preservation in diabetic rats.

Total Occlusion of the Abdominal Aorta Caused by Detachment of Cardiac Myxoma

Abdominal aortic occlusion (AAO) caused by detachment of cardiac myxoma (CM) is a very rare complication in patients with CM. Although the nature of CMs has been well established, detachment of CM may cause unexpected serious complications such as vicious embolic events. Actually, in several cases of AAO caused by detachment of CM, it has been reported that CM fragments easily migrated to the brain, heart, and lungs, and caused lifelong neurological complications despite appropriate surgical therapy. Herein, we report a case of a patient with AAO caused by detachment of CM who underwent CM excision and abdominal aortic thromboembolectomy. Additionally, we have presented the preoperative and postoperative images using 64-multidetector computed tomography.

A Case of Simultaneous Presentation of Thyroid Crisis and Diabetic Ketoacidosis

Two important endocrine emergencies, thyroid crisis and diabetic ketoacidosis (DKA), are uncommon when presented together, but pose serious complications. Without appropriate management, they may result in high mortality. Although several cases of simultaneous presentation of thyroid crisis and DKA have been reported, it is a clinically unusual situation and remains a diagnostic and management challenge in clinical practice. We report rare case with simultaneous presentation of thyroid crisis and DKA without previous warning symptoms. A 23-year-old-woman was brought to the emergency department presenting with acute abdominal pain for one day. She was healthy and there was no personal history of diabetes or thyroid disease. Through careful physical examination and laboratory tests, the patient was diagnosed with thyroid crisis combined with DKA. Concomitance of these two endocrine emergencies led to sudden cardiac arrest, but she was successfully resuscitated. This emphasizes the importance of early recognition and prompt management when the two diseases are presented concomitantly.

Recurrent True Brachial Artery Aneurysm

True aneurysm of the brachial artery is a rare disease entity. The mechanism of aneurysm formation is considered to be compression of the arterial wall, producing contusion of the media and subsequent weakness of the wall and fusiform dilatation. It can be caused by arteriosclerotic, congenital, and metabolic disorders, and can be associated with diseases such as Kawasaki's disease. Doppler ultrasonography, computed tomography, arteriography, and selective upper extremity angiography may be performed for establishing the diagnosis of aneurysm. The best therapeutic option is operative repair, and it should be performed without any delay, in order to prevent upper extremity ischemic or thrombotic sequelae. Here, we report a case of recurrent brachial artery aneurysm with review of the literature.

Chylopericardium Secondary to Lymphangiomyoma - A case report -

Chylopericardium is a rare disease entity characterized by the accumulation of chylous fluid in the pericardial sac. It usually arises from mediastinal neoplasms, thrombosis of the subclavian vein, tuberculosis, nonsurgical trauma, thoracic or cardiac surgery. The spectrum of symptoms for chylopericardium varies from an incidental finding of cardiomegaly to dyspnea, upper abdominal discomfort, cough, chest pain, palpitation, fatigue. However, most of the patients are asymptomatic. The main purpose of treatment of chylopericardium is the prevention of cardiac tamponade and prevention of metabolic, nutritional, and immunological compromise due to chyle leak. Here, we report a case of chylopercardium secondary to lymphangiomyoma with review of the literature.

Removal of an Infected Permanent Pacemaker through a Right Atriotomy without Cardiopulmonary Bypass Via a Right Thoracotomy / 대한흉부외과학회지

A 52-year-old female presented with pain and swelling owing to recurrent inflammation on a generator pocket. She had undergone a permanent pacemaker implantation (DDD type) 7 years previously. We planned to insert a new pacemaker after removal of the previous generator and wires through a surgical approach. However, she had a history of the left modified radical mastectomy (MRM) with radiation therapy for breast cancer. For this patient, it would be difficult to care for the postoperative wound if we approached via the median sternotomy. Therefore, we decided to use a right atrial approach via a right thoracotomy. We removed the previous pacing wires through an atriotomy and inserted a new pacemaker using epicardial pacing leads without cardiopulmonary bypass.

Acute Dermal Capillary Rupture in a Patient with Diabetic Nephropathy / 대한신장학회지

A 46-year-old diabetic woman visited our hospital with generalized edema and high blood pressure. An appropriately sized noninvasive blood pressure cuff was placed on her right arm above the elbow to measure blood pressure. While we were checking her blood pressure, we noticed small red petechial spots distal to the cuff involving her entire right forearm. Her blood pressure was 170/96 mmHg. The laboratory findings showed no abnormality of anticoagulation. The rest of the patient's extremities were not affected. The petechiae on the right arm resolved spontaneously after ten days. The most likely explanation for these petechiae is acute dermal capillaryrupture, which is called the Rumpel- Leede phenomenon. Acute dermal capillary rupture appears as petechiae in an area following application of vascular constriction such as application of tourniquet to draw blood specimen or use of blood pressure cuff due to capillary fragility or abnormal platelets in numbers or in function. This situation has been reported previously in the literature as the Rumpel-Leede phenomenon in association with prolonged noninvasive BP monitoring. In the patient described herein, increased venous pressure from blood pressure measurement and capillary fragility associated with diabetes mellitus may have increased the risk of acute dermal capillary rupture.