A Case of Epithelioid Hemangioendothelioma of the Nasal Cavity / 대한이비인후과학회지

Epithelioid hemangioendothelioma (EHE), a rare vascular tumor that is both clinically and histologically an intermediate between angiosarcoma and hemangioma, was first described by Sharon Weiss and Franz Enzinger. It is characterized by proliferation of a distinct type of endothelial cells, which exhibit epithelioid morphology. It typically occurs in the 20-40 age range with no sex predilection, although the overall age range involved is much broader. This indolent tumor is potentially recurrent, but it rarely metastasizes. There are very few cases of EHE of nasal cavity described in the English literature. We describe here a case of EHE on the left middle turbinate of a 17-year-old male who presented with history of intermittent epistaxis.

Headache Characteristics in Rhinologic Patients and the Role of Surgical Treatment

OBJECTIVES: Even though headache is a common symptom in patients with chronic rhinosinusitis (CRS) or nasal septal deviation (NSD), there are very few recent reports investigating headache characteristics in rhinologic patients. Therefore, we investigated the headache characteristics and differences in CRS and NSD patients who were diagnosed by computerized tomography and endoscopic exams. METHODS: We enrolled 257 patients who had undergone nasal and sinus surgery between January 2012 and December 2013. The subjects were divided into a CRS group (n=147) and NSD group (n=110). They were asked to fill out a Sinonasal Outcome Test (SNOT-20) and questionnaire about pre-operation headaches (1 day prior) and post-operation headaches (1 month after) they experienced, to evaluate their sinonasal symptoms and headache characteristics. RESULTS: There was no significant pre-operative difference in rhinologic symptoms and headache characteristics between the CRS and NSD groups. Females experienced more headaches. Both groups reported significant improvements to their headaches after surgery; however, we found no significant differences in the degree of improvement between the two groups. CONCLUSIONS: There was no significant difference in the headache characteristics and the degree of post-operative improvement between the CRS and NSD groups. Surgical treatments appear to reduce headaches in patients with rhinologic diseases.

A Case of Sarcoidosis Presented with Myofasciitis / 대한류마티스학회지

Sarcoidosis is a systemic granulomatous disorder of unknown cause. The isolated noduar type muscular sarcoidosis without other systemic involvement is very rare in Korea. We report a case of sarcoidosis presented with myofasciitis. A 46-year-old man visited hospital with painful nodular swelling on the right arm for 1 month. We performed a humerus MRI and it revealed irregular shaped subcutaneous mass infiltrating along the fascial plane. The biopsy of triceps mass showed non-caseating granuloma of muscle and fascia. To find out the cause of granuloma, we checked chest CT scan and found out some enlargement of mediastinal lymph nodes. The biopsy also demonstrated non-caseating granulomas. It had no cancerous component and culture for microorganisms were all negative. He was diagnosed as sarcoidosis and given NSAID. On follow up evaluation, the size of mass was decreased.

The Prevalence of Gout among Hyperuricemic Population / 대한류마티스학회지

OBJECTIVE: To investigate the prevalence of gout among hyperuricemic Korean people who visited Health Screening Center and to study related factors in the development of gout. METHODS: The medical records of 6,461 people who visited Health Screening Center from March to May in 2002 were reviewed. Five hundred twenty one out of 603 hyperuricemic individuals were interviewed by telephone. Gout was diagnosed according to the clinical criteria of Wallace. Basic demographic and lifestyle variables as well as biochemical data were collected. RESULTS: The prevalence of hyperuricemia was 14.3% in men and 2.2% in women. The prevalence of gout among hyperuricemic population was 16.6% for men and 6.7% for women. The mean age was higher in gout patients compared to the hyperuricemic people without gout, and serum uric acid and creatinine levels were increased in gout patients. The mean systolic blood pressure and the alcohol consumption amount were also increased in gout patients. In the multiple logistic regression analysis, statistically significant related factors for the development of gout among hyperuricemic population were age, uric acid level and alcohol consumption amount. CONCLUSION: The prevalence of gout among hyperuricemic Korean people who visited Health Screening Center was 16.6% for men and 6.7% for women. The possible related factors in the development of gout among hyperuricemic population were age, uric acid level and alcohol consumption amount.

Severe Chronic Active Epstein-Barr Virus Infection Presenting as Fever of Unknown Origin

Epstein-Barr virus (EBV) is one of eight human herpesvirus. Primary infection with EBV in childhood is generally asymptomatic or mild, however, often causes overt diseases such as infectious mononucleosis (IM) and lymphoproliferative disorder (LPD), the latter occursing in immunologically compromised individuals. Historically, EBV has been considered to be etiologically linked to human malignancies such as EBV genome positive Burkitt's lymphoma and nasopharyngeal carcinoma. Recently, however, another category of EBV-related disease, "chronic active EBV infection", has been made to describe persons without a clearly defined underlying disease. We report 4 cases of patients, presented with episodic fever of unknown origin (FUO), who were diagnosed as severe chronic active EBV infection. A combined application of serology for EBV and in-situ hybridization established the diagnosis of the EBV infection.

Severe Chronic Active Epstein-Barr Virus Infection Presenting as Fever of Unknown Origin

Epstein-Barr virus (EBV) is one of eight human herpesvirus. Primary infection with EBV in childhood is generally asymptomatic or mild, however, often causes overt diseases such as infectious mononucleosis (IM) and lymphoproliferative disorder (LPD), the latter occursing in immunologically compromised individuals. Historically, EBV has been considered to be etiologically linked to human malignancies such as EBV genome positive Burkitt's lymphoma and nasopharyngeal carcinoma. Recently, however, another category of EBV-related disease, "chronic active EBV infection", has been made to describe persons without a clearly defined underlying disease. We report 4 cases of patients, presented with episodic fever of unknown origin (FUO), who were diagnosed as severe chronic active EBV infection. A combined application of serology for EBV and in-situ hybridization established the diagnosis of the EBV infection.

A Case of Weber-Christian Disease with Severe Fatty Liver and Pericardial Effusion / 대한류마티스학회지

Weber-Christian disease (WCD) was first described by Pfeifer in 1892, and more clearly defined by Weber and Christian in the 1920s. It is a process of unknown etiology characterized by recurrent fever and inflammation of the adipose tissue. Pathological studies disclose areas of fat necrosis with an inflammatory infiltrate showing a lobular pattern and the usual presence of macrophages with foamy cytoplasm. The clinical signs include tender, palpable nodules, located mainly in the extremities, and fever, abdominal pain, arthritis and arthralgia and hepatosplenomegaly have also been reported. We present a case of Weber-Christian disease in which the presence of multiple subcutaneous nodules, enophthalmos, fatty liver, pericardial effusion was noticed. Biopsy of the skin showed mixed panniculitis in the subcutaneous fat layer. She responded well to glucocorticoid, colchicine and hydroxychloroquine.

A Case of Postinfectious Glomerulonephritis Following Meningococcal Meningitis / 대한신장학회잡지

A 21-year-old male was presented with sudden headache, fever, petechiae and neck stiffness. The diagnosis of meningococcal meningitis was confirmed by examination of cerebrospinal fluid. The clinical symptoms of the illness were improved after treatment of antibiotics. However the patient developed generalized edema, oliguria, azotemia, and heavy proteinuria in the recovery phase of illness. Low serum C3 level was also noted. A kidney biopsy was performed and showed the features of postinfectious glomerulonephritis and typical subepithelial humps on electron-microscopic examination. His symptoms and laboratory findings were improved, and C3 level returned to normal range after conservative treatment. We suggest that a complement deficiency should be ruled out in patients of glomerulonephritis developed during the recovery phase of meningococcal meningitis. C3 nephritic factor detection and renal biopsy should be carefully considered in these patients.

Cytokine profiles in synovial fluid of rheumatoid arthritis / 대한내과학회지

BACKGROUND: The assay of cytokines and their soluble receptors in the synovial fluid of inflammatory arthropathy may be useful in studying pathogenetic and immunoregulatory mechanisms of different arthritis. The aim of this study is to investigate cytokine profiles in rheumatoid arthritis and to find the characteristic pattern of cytokine concentration in rheumatoid arthritis according to the clinical manifestations. METHODS: We measured the concentration of TNF-alpha, IL-1 beta, IL-2, IL-6, IL-8 and IL-10, soluble TNF receptor I, II, IL-1 soluble receptor 2 and IL-6 soluble receptor in synovial fluid from the patients with rheumatoid arthritis using ELISA method. We compared these data with result from osteoarthritis patients. In rheumatoid arthritis, we investigated differences of cytokine profile according to clinical manifestations such as duration of disease, radiographic bone erosions and existence of rheumatoid factor. RESULTS: All of the concentrations of cytokines except IL-2 were significantly elevated in synovial fluid of rheumatoid arthritis than osteoarthritis. When we grouped RA patients according to existence of rheumatoid factor and compared the concentration of cytokines, there were no significant differences between seropositive and seronegative group. We also compared early and late disease, and erosive and non-erosive group but there were no significant differences in cytokine level. CONCLUSION: Our data support the results from other studies that concentration of pro-inflammatory or anti-inflammatory cytokines were elevated in rheumatoid arthritis than osteoarthritis. However, we cannot find the relationship between clinical findings and cytokine profiles in joint fluid.

Idiopathic Calcium Pyrophosphate Dihydrate (CPPD) Crystal Deposition Disease in a Young Male Patient: A Case Report

Calcium pyrophosphate dihydrate (CPPD) crystal deposition disease is a disease of the elderly and extremely rare in young individuals. If young people develop CPPD crystal deposition disease, it may be associated with metabolic diseases such as hemochromatosis, hyperparathyroidism, hypophosphatasia, hypomagnesemia, Wilson's disease, hypothyroidism, gout, acromegaly, and X-linked hypophosphatemic rickets. Therefore, in young-onset polyarticular CPPD crystal deposition disease, investigation for predisposing metabolic conditions is warranted. We report a case of a young male patient with idiopathic CPPD crystal deposition disease, who did not have any evidences of metabolic diseases after thorough evaluations. As far as we know, this is the first report of a young male patient presented with idiopathic CPPD crystal deposition disease.

Clinical manifestations of gout in Korea / 대한내과학회지

BACKGROUND: The prevalence of gout in Korea seems to be increasing but previous studies were outdated and small in number of cases. We enrolled relatively large number of patients and compared our data with previous studies to provide a better understanding of clinical manifestations of gout in Korean. METHODS: From 1994 to 2000, 372 patients, visited Samsung medical center for gout, were reviewed retrospectively. Age, sex, duration of disease, body mass index, 24 hour urine uric acid level, involved joints, frequency of tophi, bone changes on simple X-ray, associated diseases and complications of treatments were investigated. RESULTS: In 372 patients reviewed, 361 patients were male (97%) and only 11 patients (3%) were female. Age of onset was 46.4 +/- 13.3 years. Most frequently affected joint in first attack was 1st metatarsophalangeal joint (62.2%). Tophi were observed in 20.4% and bony erosions were detected in 34.6% of patients. The most commonly accompanied disease was hypertension (41.1%) and overweight and obesity were noted in 60.7% of patients. Liver function abnormalities associated with allopurinol were noted in 37.7% of patients and after cessation of drug, most of them showed complete recovery. CONCLUSION: Clinical features were similar to those of western countries. It may be due to changes in life style and usage of drugs that affect serum level of uric acid. So more emphasis on life style modification and control of chronic illness will be needed.