No abstract available.
Epilepsy ; Humans ; Ophthalmoplegia

Orbital solitary fibrous tumor (SFT) has recently been proposed as the encompassing terminology for hemangiopericytoma, giant cell angiofibroma (GCAF), and fibrous histiocytoma of the orbit. The lacrimal gland is a very rare location for both SFT and GCAF. A 39-year-old man presented with a painless left upper eyelid mass. An orbital computed tomography scan identified a 1.1 cm-sized well-defined nodule located in the left lacrimal gland. He underwent a mass excision. Histopathologic examination showed a proliferation of relatively uniform spindle cells with a patternless or focally storiform pattern. Dilated vessels were prominent, but angiectoid spaces lined with giant cells were absent. Floret-type giant cells were mostly scattered in the periphery. The tumor was immunoreactive for CD34 and CD99, but negative for smooth muscle actin and S-100 protein. This is the first Korean case of SFT of the lacrimal gland with overlapping features of GCAF, suggesting a close relationship between the two entities.
Actins ; Angiofibroma ; Eyelids ; Giant Cells ; Hemangiopericytoma ; Histiocytoma, Benign Fibrous ; Lacrimal Apparatus ; Muscle, Smooth ; Orbit ; S100 Proteins ; Solitary Fibrous Tumors

No abstract available.
Humans ; Myotonia Congenita* ; Myotonia*

Chylopericardium is a very rare, yet potentially fatal, complication following intrathoracic surgery, and can further lead to other life-threatening complications such as cardiac tamponade. A 54-year-old female underwent right upper lobectomy for lung cancer. Chylothorax developed on the 2nd postoperative day, and was managed conservatively with dietary modification. On the 9th postoperative day, the patient suddenly developed hypotension and severe cardiac dysfunction requiring cardiopulmonary resuscitation followed by VA ECMO. Transthoracic echocardiography revealed a large amount of pericardial effusion. Prompt pericardiocentesis was performed and the aspirated fluid showed features of chyle. Thoracic duct ligation with pericardial window operation was performed because the daily amount of chyle drained did not decrease after 3 weeks. Here, we review etiologies and therapeutic options of chylopericardial tamponade following intrathoracic surgery, which should not be underestimated even when the patient seems to demonstrate a good recovery.
Cardiac Tamponade ; Cardiopulmonary Resuscitation ; Chyle ; Chylothorax* ; Echocardiography ; Extracorporeal Membrane Oxygenation ; Female ; Food Habits ; Humans ; Hypotension ; Ligation ; Lung Neoplasms ; Middle Aged ; Pericardial Effusion ; Pericardiocentesis ; Thoracic Duct

BACKGROUND: Metabolically healthy obese (MHO) is regarded as a transient concept. We examined the effect of the dynamic change of metabolic health status on the incidence of type 2 diabetes mellitus (T2DM) both in obese and normal weight individuals.METHODS: We analyzed 3,479,514 metabolically healthy subjects aged over 20 years from the Korean National Health Screening Program, who underwent health examination between 2009 and 2010, with a follow-up after 4 years. The relative risk for T2DM incidence until the December 2017 was compared among the four groups: stable metabolically healthy normal weight (MHNW), unstable MHNW, stable MHO, and unstable MHO.RESULTS: During the 4 years, 11.1% of subjects in the MHNW group, and 31.5% in the MHO group converted to a metabolically unhealthy phenotype. In the multivariate adjusted model, the unstable MHO group showed the highest risk of T2DM (hazard ratio [HR], 4.67; 95% confidence interval [CI], 4.58 to 4.77). The unstable MHNW group had a higher risk of T2DM than stable MHO group ([HR, 3.23; 95% CI, 3.16 to 3.30] vs. [HR, 1.81; 95% CI, 1.76 to 1.85]). The stable MHO group showed a higher risk of T2DM than the stable MHNW group. The influence of the transition into a metabolically unhealthy phenotype on T2DM incidence was greater in subjects with aged <65 years, women, and those with weight gain.CONCLUSION: Metabolically healthy phenotype was transient both in normal weight and obese individuals. Maintaining metabolic health was critical for the prevention of T2DM, irrespective of their baseline body mass index.
Body Mass Index ; Cohort Studies ; Diabetes Mellitus, Type 2 ; Female ; Follow-Up Studies ; Healthy Volunteers ; Humans ; Incidence ; Mass Screening ; Obesity ; Phenotype ; Weight Gain

The causes of pyogenic liver abscess has been known as biliary tract disease or intrabadominal infection but the large proportions of the patients has no apparent underlying disorders. Recently colonic mucosal lesions were reported in patients with cryptogenic liver abscess and it has been suggested that colonic mucosal break may play a role in developing liver abscess in otherwise healthy patients. We experienced a patient of severe recurrent liver abscess complicated with endophthalmitis only 3 months after successful treatment of initial cryptogenic liver abscess and a polypoid colon cancer was discovered by chance. It seems prudent to proceed colonoscopic examination in patients with cryptogenic liver abscess especially when it is recurrent.
Biliary Tract Diseases ; Colon ; Colonic Neoplasms ; Endophthalmitis ; Humans ; Klebsiella pneumoniae ; Liver ; Liver Abscess ; Liver Abscess, Pyogenic ; Recurrence

Thromboembolic events are rare among systemic complications of inflammatory bowel disease; however, they are a significant cause of mortality when they occur. Several reports have considered thromboembolic events in patients with ulcerative colitis presenting with venous or arterial thromboembolism, such as cerebral thrombosis, deep vein thrombosis, pulmonary thromboembolism, portal vein thrombosis, or mesenteric vein thrombosis. However, increased coagulability related to Crohn's disease is extremely rare compared with that of ulcerative colitis. We report a case of a 42-year-old man with complicated portal hypertension that occurred due to extensive portal vein and mesenteric vein thrombosis. He had a monozygotic twin brother who was also in remission with Crohn's disease. The patient showed protein C and protein S deficiencies; however, he recovered with early anticoagulation therapy.
Colitis, Ulcerative ; Crohn Disease ; Humans ; Hypertension, Portal ; Intracranial Thrombosis ; Mesenteric Veins ; Portal Vein ; Protein C ; Protein S ; Pulmonary Embolism ; Siblings ; Thromboembolism ; Thrombosis ; Twins, Monozygotic ; Venous Thromboembolism ; Venous Thrombosis

Following the implementation of a long-term care insurance system for the elderly in Korea, many nursing homes have been established and many more patients than ever before have been living at nursing homes. Despite the fact that this is a high-risk group vulnerable to hip fractures, no study has yet been conducted in Korea on hip fracture incidence rates and prognoses among patients residing at nursing homes. We recently studied 46 cases of hip fracture in nursing homes; more specifically, we investigated the most common conditions under which fractures occur, and examined the degree of recovery of ambulatory ability and the mortality within 1 yr. Among those who had survived after 1 yr, the number of non-functional ambulators increased from 8 hips before hip fracture to 19 hips at final post-fracture follow-up. These individuals showed poor recovery of ambulatory ability, and the number who died within one year was 11 (23.9%), a rate not significantly different from that among community-dwelling individuals. It was evident that hip-joint-fracture nursing home residents survived for similar periods of time as did those dwelling in the community, though under much more uncomfortable conditions. The main highlight of this report is that it is the first from Korea on nursing home residents' ambulatory recovery and one-year mortality after hip fracture. The authors believe that, beginning with the present study, the government should collect and evaluate the number of hips fractured at nursing facilities in order to formulate criteria that will help to enable all patients to select safer and better-quality nursing facilities for themselves or their family members.
Aged ; Aged, 80 and over ; Body Mass Index ; Cerebrovascular Disorders/etiology ; Dementia/etiology ; Female ; Hip Fractures/complications/*epidemiology/mortality ; Humans ; Insurance, Long-Term Care ; Kaplan-Meier Estimate ; Male ; Nursing Homes ; Odds Ratio ; Parkinson Disease/etiology ; Republic of Korea/epidemiology ; Risk Factors

PURPOSE: Mitochondrial diseases are clinically and genetically heterogeneous disorders, which make their exact diagnosis and classification difficult. The purpose of this study was to identify pathogenic mitochondrial DNA (mtDNA) mutations in 2 Korean families with myoclonic epilepsy with ragged-red fibers (MERRF) and Leigh syndrome, respectively. MATERIALS AND METHODS: Whole mtDNAs were sequenced by the method of mtDNA-targeted next-generation sequencing (NGS). RESULTS: Two causative mtDNA mutations were identified from the NGS data. An m.8344A>G mutation in the tRNA-Lys gene (MT-TK ) was detected in a MERRF patient (family ID: MT132), and an m.9176T>C (p.Leu217Pro) mutation in the mitochondrial ATP6 gene (MT-ATP6) was detected in a Leigh syndrome patient (family ID: MT130). Both mutations, which have been reported several times before in affected individuals, were not found in the control samples. CONCLUSION: This study suggests that mtDNA-targeted NGS will be helpful for the molecular diagnosis of genetically heterogeneous mitochondrial diseases with complex phenotypes.
Classification ; Diagnosis ; DNA, Mitochondrial ; Humans ; Leigh Disease* ; MERRF Syndrome* ; Mitochondrial Diseases ; Phenotype

No abstract available.
Codon, Nonsense* ; Epilepsy, Temporal Lobe* ; Temporal Lobe*