No abstract available.
Neurofibroma*

No abstract available.
Arthritis, Rheumatoid* ; Humans ; Scleroderma, Localized*

Rothmund-Thomson syndrome is a rare autosomal recessive genodermatosis, which is characterized by poikiloderma and photosensitivity with variable features including alopecia, sparse hair, short stature, skeletal abnormalities, juvenile cataracts, and an increased risk of developing skin and bone malignancies. A 19-year-old man presented with poikilodermatous skin change on the whole body since three months old, and accompanied by photosensitivity, alopecia, and brachydactyly. As far as we know, this is the first case report of Rothmund-Thomson syndrome in Korea.
Alopecia ; Brachydactyly ; Cataract ; Hair ; Humans ; Korea ; Rothmund-Thomson Syndrome* ; Skin ; Young Adult

Two types of urine dipstick assays, Multistix-SG and Comber-9-Test RL, were compared for compatibility, accuracy, specificity and predictive values of a positive and negative test in 501 patients' urine and artificially prepared specimen. We found that the results of semiquantitative tests of Multistix-SG and Comber-9-Test RL performed were statistically similar in patients' specimen. The urinary leukocyte esterase tests of Comber-9-Test RL assays compared with urine sediment microscopy in regard to compatibility, sensitivity, specificity, and predictive values of a positive and negative test 83.7%, 48.1%, 90.3%, 47.4% and 90.1%, respectively. The urinary nitrite tests of Comber-9-Test RL assays compared with urine culture tests, in regard to compatibility, sensitivity, specificity, and predictive values of a positive and negative test were 90.3%, 19.4%, 84.7%, 53.8% and 94.1, respectively. For the urinary protein, the sulfosalicylic acid method was the most sensitive test for any kinds of protein, and Multistix-SG appeared more sensitive than Comber-9-Test RL for the albuminuria. For the urinary bilirubin and glucose, two dipstick assays were similar in their diagnostic efficiency. Finally in the urinary occult blood tests, Comber-9-Test RL assays was more sensitive than Multistix-SG.
Albuminuria ; Bilirubin ; Glucose ; Leukocytes ; Methods ; Microscopy ; Occult Blood ; Sensitivity and Specificity ; Urinalysis

No abstract available.

We analyzed the clinical result of trabeculectomy with amniotic membrane transplantation which has antifibrotic and antiinflammatory action in risky glaucoma patients. Subjects were included four eyes of four patients with neovascular glaucoma, one eye of one patient with primary open angle glaucoma and four eyes of three patients with congenital glaucoma. After limbal based scleral flap was made in triangular shape with 5x4 mm sized, amniotic membrane, sized by 6x6 mm with epithelial side up, was placed on the scleral flap and then sutured to medial and lateral portion at 3 mm superior to base of scleral flap base with 10-0 nylon(2 points). The state of bleb, intraocular pressure and complications were observed at postoperative 1st, 3rd, 6th and 9th week. The IOP was reduced from 33.6+/-11.1 mmHg, to 15.5+/-6.5 mmHg at postoperative one week, 17.0+/-8.3 mmHg at three weeks, 16.9+/-8.1 mmHg at six weeks, 17.1+/-6.4 mmHg at nine weeks. 6 eyes(77.7%)had controlled IOP under 21 mmHg without antiglaucomatous agents at last follow up. Avascular blebs were observed throughout postoperative period in all subjects. Postoperative complications were shallow anterior chamber in one eye at 1st week and low intraocular pressue in one eye at 1st, 3rd week, that resolved at six weeks. Trabeculectomy with amniotic membrane transplantation on the scleral flap appeared to be effective as an augment therapy for filtering surgery in risky glaucoma.
Amnion ; Anterior Chamber ; Blister ; Filtering Surgery ; Follow-Up Studies ; Glaucoma ; Glaucoma, Neovascular ; Glaucoma, Open-Angle ; Humans ; Intraocular Pressure ; Membranes* ; Postoperative Complications ; Postoperative Period ; Trabeculectomy

Microscopic polyangiitis is a systemic small-vessel vasculitis, which may involve multiple organs, but cardiac involvement is relatively rare. We report a case of microscopic polyangiitis with multiple organ involvement, in which myocardial infarction was the early manifestation of the disease. A 53-year-old man presented with sudden papulovesicular eruptions and swellings on the face, posterior neck, dorsa of both hands and fingers, and with diffuse erythematous patches on the back. He had suffered from renal dysfunction, arthralgia, and hypertension for more than 8 years. He had been admitted to the department of cardiology for acute myocardial infarction and had suffered from recurrent gastrointestinal bleeding, renal failure, acute pancreatitis and sepsis during the admission. Histopathologically, small-vessel leukocytoclastic vasculitis with out granuloma was seen. Direct immunofluorescence showed no immune deposite. A high serum level of P-ANCA was detected by ELISA.
Acute Kidney Injury ; Antibodies, Antineutrophil Cytoplasmic ; Arthralgia ; Cardiology ; Enzyme-Linked Immunosorbent Assay ; Fingers ; Fluorescent Antibody Technique, Direct ; Granuloma ; Hand ; Hemorrhage ; Humans ; Hypertension ; Microscopic Polyangiitis* ; Middle Aged ; Myocardial Infarction* ; Neck ; Pancreatitis ; Sepsis ; Vasculitis

BACKGROUND: Pemphigus is a chronic blistering disease of the skin and mucous membranes, with severe morbidity and occasional mortality. It is necessary to investigate the clinical features, treatment and long-term outcome of Korean patients with pemphigus as it is the most common and severe autoimmune bullous disease in Korea. OBJECTIVE: The purpose of this study was to evaluate the clinical features, treatment and prognosis of Korean patients with pemphigus. METHOD: A retrospective analysis was conducted on 124 pemphigus patients seen between 1987 and 2003 at Youngdong Severance Hospital. RESULTS: Out of 124 patients diagnosed with pemphigus during the study period, 62 patients had pemphigus vulgaris (PV), and 62 patients had pemphigus foliaceus (PF). For the 62 PV patients, the mean age of onset was 48.3 years, and the male to female ratio was 1: 1.7. For the 62 PF patients, the mean age of onset was 44.7 years, and the male to female ratio was 1.4: 1. Average initial dose of oral prednisolone was 30.2mg in PV patients and 19.9mg in PF. Fifty six (90%) patients with PV and 38 (61%) patients with PF received immunosuppressive agents. Partial remission was induced in 9.7%, 23.0%, 41.7%, and 75% of PV patients and in 12.9%, 19.0%, 13.6% and 50% of PF patients after 1, 3, 5, and 10 years of diagnosis, respectively. Five (8%) of each PV and PF patients died during the treatment period. CONCLUSION: The clinical manifestations and results of immunofluorescent studies on 124 Korean pemphigus patients were similar to those of previous studies. Contrast to previous studies, the PV to PF ratio was 1: 1, and a male preference was observed on PF. More studies are needed to improve the prognosis and reduce the complications in the treatment of pemphigus patients.
Age of Onset ; Blister ; Diagnosis ; Female ; Humans ; Immunosuppressive Agents ; Korea ; Male ; Mortality ; Mucous Membrane ; Pemphigus* ; Prednisolone ; Prognosis ; Retrospective Studies ; Skin

No abstract available.
Heart Defects, Congenital* ; Plasma*

DSA(Digital Subtractin Angiography) is a valuable diagnostic imaging method in many clinical fields, includingranal-related conditons. Sixty four renal DSA examinations were performed in 59 patients with renal-relateddiseases from Jan. 1984 to Dec. 1985. Summary of these were as follows: 1. Intraarterial (IA)-DSA is performed in6 cases, intravenous(IV)-DSA in 58 cases. In 58 Examinations of IV-DSA, diagnostic image quality is obtained in 51cases(88%). 2. In investigations of a possible renovascular etiology of hypertension, IV-DSA,is a safe, sensitiveand accurate method. On screening for evaluation of renovascular hypertension, RSP should be replaced with IV-DSA,because IV-DSA is moe sensitive and accurate and can detect not only anatomic change of renal arttery but alsofunctional hemodynamic change. 3. IV-DSA is valuable in diseases with morphologic changes of vessels. Incharacterization of a known renal mass, and evaluation of hematuria, suspected aneurym and renal trauma, IV-DSA isvery useful diagnostic imaging modality. 4. In evaluation of potential renal donors, IV-DSA is an accurate andsafe method with 82.4% of accuracy. IV-DSA also is useful in follow-up of allograft recipients. 5. Ininvestigation of diabetic nephropathy, glomerulonephritis, pyelonephritis, IV-DSA is little helpful. 6. Theadvantages of DSA are well known, particularly post-procedure process using computer programs is helpful forobtaining informations of hemodynamic change or time-sequence-curve of density etc. More technical improvementwith this modality is required for improvement of the image quality and resolution. And more accumulation ofclinical experience is required in order to increase the diagnostic accuracy.
Allografts ; Angiography, Digital Subtraction ; Diabetic Nephropathies ; Diagnostic Imaging ; Follow-Up Studies ; Glomerulonephritis ; Hematuria ; Hemodynamics ; Humans ; Hypertension ; Hypertension, Renovascular ; Mass Screening ; Methods ; Pyelonephritis ; Tissue Donors