OBJECTIVES: Rapidly progres s ive glomerulonephritis (RPGN) is a clinico- pathologic entity characterized by extens ive crescent formation(usually involving 50% or more of glomeruli) as the principal his tologic finding and a rapid deterioration of kidney function, which can lead to end s tage renal disease within a few weeks. T he etiology and incidence of RPGN has been well defined in Europe and North America, however, there has been no report of a large series in Korea. T he aim of the present s tudy was to analyze the etiology and clinico- pathologic features of 26 patients with RPGN, seen during 1983-1997. METHODS: T wenty-six patients with RPGN(crescents in > 50% of glomeruli) were obs erved during a period of las t 14 years. Male to female ratio was 1:1.4, and the mean age was 30(6-75) years. Mean time from the initial symptoms to the ESRD was 3.1 months . RESULTS: The incidence of RPGN in our series was 2.1% of primary glomerulonephritis. Immunecomplex mediated disease was presented in 14 cases (54%), including 6 sys temic lupus erythematos us, 3 post- streptococcal glomerulonephritis, 3 Henoch- Schonlein purpura, and 2 IgA nephropathy. Pauci- immune disease was presented in 12 cases (46%), including 3 Wegener' s granulomatos is, one necrotizing crescentic glomerulonephritis, and 8 idiopathic crescentic glomerulonephritis. However, there was none of anti-GBM- mediated disease in our s tudy. ANCA were found in 6 patients. All 3 patients with WG were C- ANCA pos itive, whereas one patient with PSGN, necrotizing cres centic GN, and idiopathic crescentic GN were P- ANCA pos itive, respectively. Initial clinical and laboratory features included edema(80%), hypertens ion(72%), oliguria(68%), a decreased renal function(serum creatinine > 5mg/dL, 35%), and gros s hematuria(36%). Renal biopsy showed large crescents more than 80% of the glomeruli in 14 cases (54%) which were predominantly fibrocellular. Fifteen patients (58%) were treated with prednis olone alone, and 12 of them received puls e doses of corticosteroids. Five patients were treated with prednisolone and cyclophos phamide IV pulse. Two cases received plasma exchange. During the mean follow-up of 31+/-37 months, 18 patients (69%) developed inexorable progression of renal failure, three(12%) showed recovery of renal function, and two(8%) showed partial improvement, which is followed by varying degrees of renal insufficiency. During follow-up, three patients died : two from res piratory failure with severe pulmonary hemorrhage and one from opportunistic pulmonary infection during immunosuppressive therapy. Poor prognos is is as sociated with hypertension, increased serum creatinine level at the time of diagnosis, large crescents more than 85% of glomeruli, and glomerular scleros is . CONCLUSION: We conclude that an earlier diagnos is including kidney biopsy and the more aggressive treatment are essential in the management of RPGN.
Adrenal Cortex Hormones ; Antibodies, Antineutrophil Cytoplasmic ; Biopsy ; Creatinine ; Diagnosis ; Europe ; Female ; Follow-Up Studies ; Glomerulonephritis* ; Glomerulonephritis, IGA ; Hemorrhage ; Humans ; Hypertension ; Immune System Diseases ; Incidence ; Kidney ; Kidney Failure, Chronic ; Korea ; Male ; North America ; Plasma Exchange ; Prednisolone ; Purpura ; Renal Insufficiency ; Systemic Vasculitis

No abstract available.
Animals ; Evoked Potentials* ; Rats*

This is to report the technique of reversed iliac leg stent-graft in endovascular treatment for isolated internal iliac artery (IIA) aneurysm, which had significant size discrepancy between the common iliac artery (CIA) and external iliac artery (EIA) in 3 patients from different hospitals. Three patients were a 85- and two 82-year-old men. Treated were right IIA aneurysms, sized 6.5x6.2 cm, 5.0x4.0 cm, and 4.1 cm in longest diameter, respectively. The diameters of the right CIA and right EIA measured 21 mm/11 mm, 15 mm/11 mm, and 20 mm/10 mm, respectively. In all cases, reversed iliac leg stent-grafts were prepared on-site; unsheathed and mounted upside-down manually, and deployed in each right CIA. Post-stent-graft angiograms showed complete exclusion of the aneurysms, except for minimal type 1 endoleak in one case. This technique is a useful treatment option in patients with isolated IIA aneurysm.
Aged, 80 and over ; Aneurysm* ; Endoleak ; Endovascular Procedures ; Humans ; Iliac Aneurysm ; Iliac Artery* ; Leg* ; Male

No abstract available.
Pregnancy*

Between 1977 and 1987, 20 patients with mesothelioma were treated at Severance Hospital. Data was gathered from medical charts at the time of hospitalization of mesothelioma patients and from a follow-up questionnaire by mail or telephone. The results acquired were as follows: 1. Among the 20 patients, 11 men and 9 women with mesothelioma were identified. The mean age at hospitalization was 47 years and 11 mesothelioma patients were known or presumed to be dead during the different observation periods. 2. Only one mesothelioma patient had a definite history of occupational asbestos exposure. 3. The sites of orgin of mesothelioma were the pleura(13), peritoneum(2), pericardium(2), mediastinum(2), and pelvis(1). Common symptoms included dyspnea, chest pain, abdominal distension, etc. 4. Pathologically, mesotheliomas were divided into 14 malignant types and 6 benign types ; and histologically, 8 fibrous mesotheliomas and 3 epithelial mesotheliomas were shown. 5. There was a statistically significant difference in survival rate according to pathologic type and smoking status. In the groups with malignant mesothelioma, 50% survival time from first symptoms was 18 months and that from diagnosis was 11 months. Also, 75% survival time from diagnosis was 6 months in the smoking groups and 19 months in the non-smoking groups.
Asbestos ; Chest Pain ; Diagnosis ; Dyspnea ; Female ; Follow-Up Studies ; Hospitalization ; Humans ; Male ; Mesothelioma* ; Postal Service ; Surveys and Questionnaires ; Smoke ; Smoking ; Solitary Fibrous Tumor, Pleural ; Survival Analysis* ; Survival Rate ; Telephone

PURPOSE: This study is designed to evaluate the reliability for studies of tensiomyography (TMG). TMG can evaluate muscle function noninvasively and selectively. METHODS: We measured 12 male volunteers (age, 26.5±7.6 years; height, 175.3±4.7 cm; weight, 78.8±13.3 kg) in this study and measured TMG during three occasions over 3 consecutive days. None of the participants has had any history of neuromuscular disorders or muscle diseases. Vastus lateralis, vastus medialis (VM), rectus femoris (RF) in quadriceps and biceps femoris, semitendinosus in hamstrings muscles were measured. Coefficient of variation (CV%) and intraclass correlation coefficient (ICC) have been calculated about maximal displacement (Dm, mm) and contraction time (Tc, ms) which are main parameters. RESULTS: Most of the ICC of Dm were over 0.8 and the highest among the muscles except both VM. And, most ICC of Tc was lower than Dm except both BF (right, 18.31; left, 15.03). But, the ICC of Tc was lower than Dm except left RF (0.890) and VM (0.859). CONCLUSION: This study has shown that the Dm is high levels of the ICC and CV(%) in thigh muscle except VM. In the future, we plan to establish the method of measurement more clearly for reducing the errors of measurements. The technique of correct palpation of measurable muscles using TMG devices is also necessary.
Humans ; Male ; Methods ; Muscles ; Palpation ; Quadriceps Muscle ; Thigh ; Volunteers

OBJECTIVE: Treatment of intracranial dural arteriovenous fistulas (dAVFs) remains a challenge. However, after introduction of Onyx, transarterial approach is the preferred treatment option in many centers. We report our experience of dAVFs embolization with special emphasis on transarterial approach. METHODS: Seventeen embolization procedures were performed in 13 patients with dAVFs between Jan 2009 and Oct 2014. Clinical symptoms, location and type of fistulas, embolization methods, complications, radiological and clinical outcomes were evaluated using charts and PACS images. RESULTS: All 13 patients had symptomatic lesions. The locations of fistulas were transverse-sigmoid sinus in 6, middle fossa dura in 4, cavernous sinus in 2, and superior sagittal sinus in 1 patient. Cognard types were as follows : I in 4, IIa in 2, IIa+IIb in 5, and IV in 2. Embolization procedures were performed > or =2 times in 3 patients. Nine patients were treated with transarterial Onyx embolization alone. One of these required direct surgical puncture of middle meningeal artery. Complete obliteration of fistulas was achieved in 11/13 (85%) patients. There were no complications except for 1 case of Onyx migration in cavernous dAVF. Modified Rankin scale score at post-operative 3 months were 0 in 11, and 3 in 2 patients. CONCLUSION: Transarterial Onyx embolization can be a first line therapeutic option in patients with dAVFs. However, transvenous approach should be tried first in cavernous sinus dAVF because of the risk of intracranial migration of liquid embolic materials. Furthermore, combined surgical endovascular approach can be considered as a useful option in inaccessible route.
Cavernous Sinus ; Central Nervous System Vascular Malformations* ; Fistula ; Humans ; Meningeal Arteries ; Punctures ; Superior Sagittal Sinus

PURPOSE: The purpose of this study was to investigate the etiology of acute pharygotonsillitis in pediatric patients. METHODS: Pharyngeal swabs from patients with acute pharyngotonsillitis were evaluated for viruses and bacterial organisms from March 2010 through March 2011. RESULTS: Of 615 patients, potentially pathogenic bacteria were isolated in 40 (6.5%), viruses were isolated in 310 (50.4%), and no pathogens were isolated in 267 patients (43.4%). Both viral and bacterial pathogens were found in 2 (0.3%). Of 40 patients with bacterial pathogens, group A streptococci were found in 31 (77.5%). Among 310 patients with virus infection, adenovirus was the most frequently recovered (203 patients; 65.5%), followed by rhinovirus (65 patients; 21.0%), enterovirus (43 patients; 13.9%) and coronavirus (18 patients; 5.8%). There were 25 patients who had been coinfected with 2 viruses. In viral pharyngotonsillitis, cough, rhinorrhea, conjunctivitis and diarrhea were prominent. On the other hand, pharyngeal injection and pharyngeal petechiae were prominent in bacterial pharyngotonsillitis. CONCLUSIONS: Virus infection was a big part of acute pharyngotonsillitis and there were differences in clinical manifestations among viral and bacterial infections. Therefore, we need to distinguish between virus infection and bacterial infection using clinical signs for preventing the abuse of antibiotics.
Adenoviridae Infections ; Anti-Bacterial Agents ; Bacteria* ; Bacterial Infections ; Child* ; Conjunctivitis ; Coronavirus ; Cough ; Diarrhea ; Enterovirus ; Hand ; Humans ; Purpura ; Rhinovirus

Ten cases of post-inflammatory aneurysm of the thoracic aorta were studied morphologically using aortic wall and aortic valve tissue resected during the surgical repair of the lesions. Four cases of aortic aneurysm in Behcet's disease showed massive perivascular infiltration of the lymphocytes and plasma cells in adventitia and vasa vasorum. Fibrous obliteration of the vasa vasorum was seen, and neutrophilic infiltration was also associated in two cases with recurrent prosthetic valve failure. Three cases of aortic aneurysm associated with Takayasu's arteritis showed similar histologic features, but giant cells and histiocytic reactions were additional findings. Plasma cell infiltration was more pronounced compared to the Behcet's aortitis. One case of luetic aortitis showed a non-specific chronic inflammatory reaction with florid endothelial cell proliferation. One case of ankylosing spondylitis showed fibrous thickening at the periannular portion of the aortic valve cusps, but this finding was also seen in an aortic valve of a patient with Behcet's disease. One case without any disease association showed similar features of chronic inflammation with granuloma formation. The findings suggest that they have similar histologic features indistinguishable in terms of the clinical disease association, but a granulomatous reaction could be expected in Takayasu's arteritis.
Adult ; Aorta, Thoracic ; Aortic Aneurysm/*etiology/pathology ; Aortitis/*complications/pathology ; Behcet Syndrome/complications/pathology ; Female ; Humans ; Male ; Middle Aged ; Spondylitis, Ankylosing/complications/pathology ; Syphilis/complications/pathology ; Takayasu Arteritis/complications/pathology

Dentatorubro-pallidoluysian atrophy (DRPLA) is a rare neurodegenerative disorder with various clinical phenotypes and has a cytosine-adenine-guanine (CAG) trinucleotide repeat in a gene on chromosome 12. It has been known that trinucleotide repeat disorders show strong inverse correlations between the CAG repeat number and the age of onset and genetic anticipation. The purpose of this study was to investigate whether these observations are applicable to Korean patients. This report involved three Korean families and had on file the history of the 15 affected family mem-bers .Seven of the affected members had the diagnosis of DRPLA which was confirmed by a gene study. We observed inverse correlations between the CAG repeat number and the age of onset and genetic anticipation with high intra- and interfamilial variations. Although our study was in general agreement with previously documented features of DRPLA, some features could not be explained by currently understood pathophysiologic mechanisms.
Age of Onset ; Anticipation, Genetic ; Atrophy* ; Chromosomes, Human, Pair 12 ; Diagnosis ; Genes, vif ; Humans ; Molecular Biology* ; Neurodegenerative Diseases ; Phenotype ; Trinucleotide Repeats