MPO-ANCA Associated Rapidly Progressive Glomerulonephritis in A Patient with Mixed Connective Tissue Disease / 대한신장학회지

Mixed connective tissue disease (MCTD) has the clinical feature of other collagen vascular diseases. According to several reports recently published, MCTD sometimes involved kidney as benign course. ANCA associated glomerulonephritis occurred in systemic autoimmune disease such as systemic sclerosis or rheumatoid arthritis, not in MCTD. In this case, as we experienced that MPO-ANCA associated glomerulonephritis arose and proceeded to rapid progressive glomerulonephritis (RPGN) in women with MCTD, we report it with review of reference. A 60-year-old woman was admitted with uremia and joint pain. In physical finding and serum test, she was diagnosed with MCTD according to Khan`s criteria. Also MPO antibody and ANCA were noted in specific immunological test and the histologic findings showed crescentic glomerulonephritis. So we diagnosed RPGN induced by MPO-ANCA associated glomerulonephritis. She was treated with immunosuppressive treatment and uremia was improved. RPGN induced by MPO-ANCA associated glomerulonephritis should be ruled out by prompt renal biopsy and require proper treatment.

T-cell Large Granular Lymphocytic Leukemia: A Case Report

T cell large granular lymphocytic leukemia (T-LGL leukemia) is defined as a clonal proliferative disorder of CD3+ cytotoxic T cells. T-LGL leukemia usually expresses CD3+, CD4-, CD8+, CD16+, CD56- and CD57+ cell markers, and this represents a rearrangement of the T cell receptor (TCR) gene. The clinical course is indolent in most cases, but on rare occasions, when CD3+ and CD56+ are expressed on the leukemic cells, it can be more aggressive. We experienced a patient with T-LGL leukemia and the disease was indolent at the time of diagnosis, and so any specific treatment was not required. Two years after the initial diagnosis, her clinical course became quite aggressive as the CD 56+ cell surface antigen was expressed. We report here on the first case of T-LGL leukemia in Korea and we review the relevant literature.

Secondary Amyloidosis Associated with Multiple Sclerosis

BACKGROUND: Multiple sclerosis (MS) is a demyelinating disease of the central nervous system. Secondary amyloidosis can occur as a complication of chronic systemic inflammatory and infectious diseases. Until now there has been no report of secondary amyloidosis associated with MS. We report herein a case of renal biopsy-proven secondary amyloidosis in a patient with MS. CASE REPORT: A 41-year-old woman with MS was hospitalized due to aggravated quadriparesis and edema in both lower extremities. Laboratory findings showed nephrotic-range proteinuria and hypoalbuminemia. A percutaneous renal biopsy procedure was performed, the results of which revealed secondary amyloid-A-type amyloidosis associated with MS. CONCLUSIONS: This is the first report of secondary amyloidosis associated with MS.

Acute Renal Failure in Coexisting IgA Nephropathy and Acute Interstitial Nephritis: Complete Recovery after Hemodialysis and Steroid Therapy / 대한신장학회지

Acute renal failure in Immunoglobulin A nephropathy (IgAN), a rare event, is associated with acute tubular necrosis mainly induced by intratubular erythrocytic cast and crescentic glomerulonephropathy (rapidly progressive glomerulonephritis) and the severity paralleled to the degree of glomerular damage. The changes are regarded as those of secondary atrophic response to the glomerular lesions. In that case, renal progression correlates more closely with the severity of tubulointerstitial lesions than with the degree of glomerular lesions in IgAN. Rarely, acute tubulointerstitial nephritis (TIN) could develop independently in primary glomerulonephritis. In this case, the severity of tubulointerstitial lesion was out of proportion with damage of glomerular lesion. To the best of our knowledge, we report the first case of a patient with independently developed severe acute TIN complicating IgAN in Korea. A 38-year-old man was admitted with recurrent hematuria. Proteinuria (<1 g) and severe renal failure were noted and hemodialysis was started. In renal biopsy, IgAN associated with acute TIN was diagnosed. He showed good response to steroid therapy and maintained normal renal function after discontinuation of medication.

Tissue-Engineered Human Neocartilage Formation Using Human Adult Auricular Cell Line

The purpose of these studies was an establishment of human auricular chondrocyte cell line using retrovirus mediated v-myc transfer, characterizing the human auricular chondrocyte cell line by type II collagen mRNA expression and transplantation of human auricular cell line into immunological incompetent nude mice to establish neocartilage formation. Also, I evaluated the growth rate of chondrocyte cell line to measure the cellular proliferative potency. I have established the human auricular chondrocyte cell line integrated v-myc and confirmed by v-myc transduced Myc protein expression by immunohistochemistry and immunoblotting study. And, growth rate of established human auricular chondrocyte cell line increased 4 folds times faster than primarily cultured human auricular chondrocyte. The established human auricular chondrocyte had type II collagen mRNA upto 8 months in monolayer culture. And we observed formation of neocartilage on the back of nude mice using chondrocyte cell line/fibrin glue polymer at 12 weeks transplantation.

A Case of Primary Myxoid Liposarcoma of the Neck / 대한이비인후과학회지

Liposarcoma is a relatively common malignant tumor that can be originated from adipose tissue in the body, especially in the lower extremity and in the retroperitoneal space. But it is extremely rare in the head and neck region. Liposarcoma can be classified histopathologically into four subtypes; well differentiated, myxoid, round cell, and pleomorphic type. The prognosis of liposarcoma is known to be generally poor due to the high local recurrence rate in spite of complete resection. We report a case of myxoid liposarcoma of the neck in a 41-year old male, who was treated with primary excision of tumor and postoperative radiation therapy. The histopathology, clinical appearance, management and prognosis of this rare condition are discussed.

Acoustic Parameters for the Early Detection and Differential Diagnosis of Pathologic Voice / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: The survival rate of laryngeal cancer has improved in recent day. But in case of advanced cancer, radical destructive surgery is required for survival. So early detection of laryngeal cancer prevent from this disastrous condition. In this point of view, mass screening test for early detection of laryngeal cancer is necessary. Screening tests using voice has many advantages of being simple, non invasive, and requiring less space. In this system, the most important factor is the selection of acoustic parameters to be used in voice analysis. Thus authors studied the acoustic parameters that can differentiate normal, benign, malignant laryngeal diseases by an acoustic analyzing system and we also checked the availability of parameters. MATERIALS AND METHODS: We evaluated the voice analyzed data from 25 laryngeal malignancy patients, 33 benign laryngeal disease patients, 35 normal control who visited PNUH otolaryngology department from October 1996 to May 1998. A computerized speech lab. 4300B (CSL) was used to carry out the analysis of each voice sample and statistical analysis, ANOVA. Canonical analysis and cumulative frequency curve were used. RESULTS: The statistically significant parameters that can differentiate normal and malignant laryngeal disease groups were 15 parameters and can differentiate normal and benign laryngeal disease group were 9 parameters and that can differentiate benign and malignant laryngeal disease group were 7 parameters. CONCLUSION: We consider that these parameters and detection programs may be effective in development of a screening system using voice only. Developing diagnostic tools and programs would need further study of phonetics and voice engineering.

A Case of Giant Keratoacanthoma of the Lower Lip Treated with Intralesional Methotrexate / 대한이비인후과학회지

Keratoacanthoma(KA) is a rapidly growing benign cutaneous neoplasm, usually occurring on sunexposed sites in elderly people, and it has a close clinical and pathologic resemblance to squamous cell carcinoma. Giant KA of the lower lip is extremely rare. Various treatment modalities have been reported including radiation therapy, systemic oral retinoids, and the intralesional application of 5-fluorouracil(5-FU), methotrexate(MTX) or interferon alpha-2a. Excisional surgery, however, remains the treatment of choice for the majority of KA. The disadvantages of excision are the possible requirement for extensive reconstructive surgery and the resultant cosmetic or functional deficit. Recently, we experienced a case of giant KA of the lower lip in a 60-year-old male, and he was treated successfully with intralesional methotrexate. Now we report the case with a brief review of literature.