BACKGROUND: Neurofibroma, the hallmark of neurofibromatosis, is a cutaneous or subcutaneous lesion, with a variable clinical presentation. Histologically, neurofibroma consists of proliferation of nerve derived cellular elements, together with an abundant, collagenous extracellular matrix. Specifically, neurofibroma has been shown to contain 30-50% collagen in its matrix. Objective 5. METHODS: We examined the expression of extracellular matrix genes (collagen, fibronectin, laminin), TGF-b mRNA and Ha-ras oncogene mRNA by using Northern and slot-blot hybridization and immunoperoxidase stains. Result: In Northern blot analysis, Ha-ras and TGF-b genes revealed respectively, 8.8kb and 2. 5kb sized mRNA transcripts in neurofibroma. These parameters were normal in the control. The expression of these genes were 1.9, 2.0 fold increased in neurofibroma. In slot-blot analysis, expression of type I collagen showed fibronectin genes to be 2,401+210, 540+43, respectively, in neurofibroma. So there were 3.7 fold, 2.1 fold, differences respectively, compared to the normal control. However, there were no significant changes of type IV collagen and laminin Bl mRNA levels between neurofibroma and normal skin tissues. Irnmunoperoxidase staining by rnonoclonal anti type IV collagen antibody in neurofibroma showed type IV collagen to be diffusely and weakly stained in tissue. On staining by monoclonal anti-laminin antibody, laminin was stained in a matrix and around vessels. CONCLUSION: The increased expression of extracellular matrix genes may suggest that there is a subpopulation of fibroic cells in neurofibroma which are stimulated by TGF-b. Ha-ras genes which might have accumulated with the differentiation of neural tissue may be related to the pathogenesis of neurofibroma tissue formation. Further studies are needed to determine whether the other factors are related to the pathogenesis of neurofibroma.
Blotting, Northern ; Collagen ; Collagen Type I ; Collagen Type IV ; Coloring Agents ; Extracellular Matrix* ; Fibronectins ; Genes, ras* ; Laminin ; Neurofibroma ; Neurofibromatoses* ; RNA, Messenger ; Skin

No abstract available.
Pneumothorax, Artificial* ; Treatment Failure* ; Tuberculosis, Pulmonary*

No abstract available.
Asthma, Occupational*

Neurocutaneous melanosis is a rare congenital phacomatosis, characterized by the presence of multiple pigmented skin nevi at birth. Meningeal melanosis tending to become malignant and seizure. Dandy-Walker syndrome is a developmental disorder of brain characterized by cystic dilatation of the fourth ventricle and agenesis or hypoplasia of the cerebellar vermis. We studied a case of Dandy-Walker syndrome with neurocutaneous giant melanosis in a 3 years old boy.
Brain ; Child, Preschool ; Dandy-Walker Syndrome* ; Dilatation ; Fourth Ventricle ; Humans ; Male ; Melanosis* ; Neurocutaneous Syndromes ; Nevus ; Parturition ; Seizures ; Skin

BACKGROUNDS: It is almost universally accepted that occlusive vascular diseases are best managed by anatomical reconstruction. However, the mortality and the morbidity have limited this operation for patients with high operation risks. In these patients, palliative operations such as extra-anatomic bypass and lumbar sympathectomy, are accepted as useful treatment. MATERIAL AND METHOD: A retrospective study was conducted in 38 patients who underwent palliative operations for occlusive vascular disease at Korea University Guro Hospital between 1996 and 2000. Mean age of the patients was 60.37 +/- 17.65 years, and preoperative diagnoses were atherosclerosis in 32 patients, Buerger's disease in 4 patients, Raynaud's syndrome in 1 patient and SVC syndrome in 1 patient. RESULT: Extra-anatomic bypass(40procedures), lumbar sympathectomy(17), thromboembolectomy(7) and femoral artery graft interposition(1) were performed. Six patients were required reoperation due to graft flow failure or fistula. Three year primary patency rate of entire operations was 78.29 +/- 8.81%, and the correlation between type of operation and patency rate was not statistically significant. CONCLUSION: Palliative operations for occlusive vascular disease are useful treatment in limited patients with high operation risks or limited life expectancy.
Atherosclerosis ; Diagnosis ; Femoral Artery ; Fistula ; Humans ; Korea ; Life Expectancy ; Mortality ; Palliative Care* ; Reoperation ; Retrospective Studies ; Sympathectomy ; Thromboangiitis Obliterans ; Transplants ; Vascular Diseases*

Moyamoya disease is an unusual cerebrovascular disorder characterized by occlusive intimal dysplasia of the distal internal carotid and proximal cerebral arteries as well as other collateral arteries. However,moyamoya diseases are recently being reported as a systemic process.We experienced one case of coronary artery occlusive disease affected by moyamoya disease.The patient was a 35-year-old female,experiencing intermittent NYHA class II dyspnea and exertional chest pain for 6 months and right parest hesi a f or 1 month before admission.Cerebral artery angiogram showed abnormal cerebrovascular systems and confirmed moyamoya disease with cerebral infarction of the left f r ont al l obe. I n cor onar yartery angiogram,left coronary artery was not visualized due to total occlusion of the left main ostium and left coronary blood flow was supplied from normal right coronary artery. CABG was performed with OPCAB.Both internal mammary arteries were used f or LAD and LCx.Intraoperative coronary artery findings showed intimal hyperplasia and no definite thrombi,and nondiseased coronary arteries were good and patent.We concluded that this patient's coronary artery disease was affected by moyamoya disease,and moyamoya disease should be evaluated in the extracerebral cardiovascular system.
Adult ; Arteries ; Cardiovascular System ; Cerebral Arteries ; Cerebral Infarction ; Cerebrovascular Disorders ; Chest Pain ; Coronary Artery Disease* ; Coronary Vessels* ; Dyspnea ; Humans ; Hyperplasia ; Mammary Arteries ; Moyamoya Disease*

The spontaneous regression of herniated cervical discs is not a well established phenomenon. However, we encountered the 3 cases of spontaneous regression of severe radiculopathic herniated cervical discs that were treated using a non-surgical method. Each of the patients were treated with a combination of manipulation, dry needling and analgesics. In each case, the symptoms improved within 12 months of treatment and magnetic resonance imaging (MRI) conducted at that time revealed marked regression of the herniated disc in all cases. These cases provide additional examples of spontaneous regression of herniated cervical discs documented by MRI following non-surgical treatment.
Analgesics ; Humans ; Intervertebral Disc Displacement ; Magnetic Resonance Imaging

BACKGROUND: Proper construction of vascular access and adequate maintenance are essential for the prognosis of the hemodialysis patients. Though arteriovenous fistula using autogenous vessel is the first of choice, the incidence of arteriovenous fistula using artificial graft is gradually increasing. The aim of this study was to analyse the patency rates between autogenous and artificial fistula, among artificial graft types, according to the accompanied disease. MATERIAL AND METHOD: A retrospective study was conducted on 186 patients who underwent 292 arteriovenous fistula operations for hemodialysis at Korea University Guro Hospital between 1996 and 2000. Mean age of the patients was 54.37+/-12.89years, and the male: female ratio 99:87. RESULT: Among 292 operations, there were 156 autogenous fistula and 116 graft fistula. The other 20 operations were thrombectomy, takedown of graft, revision, and balloon dilatation. Patency rates of autogenous fistula were 92.78+/-2.35% at 1 year and 39.03+/-9.08% at 5 years, and those of graft fistula were 96.09+/-2.22% at 1 year and 16.45+/-10.15% at 5 years. However, there was no statistical significance between the two operations. The patients who had hypertension, diabetes or both had no statistical significance in the patency rate compared to that of patients without underlying disease. In addition, the type of graft used did not affect the patency rate. Second operation was needed in 62 patients and third operation in 31 patients, but their patency rate again had no statistical significance compared to that of the first operation. CONCLUSION: The patency of the artificial graft fistula was comparable to the autogenous fistula, but the patency according to types of graft need to be studied further. Furthermore, the underlying diseases did not affect the fistula patency.
Arteriovenous Fistula* ; Dilatation ; Female ; Fistula ; Humans ; Hypertension ; Incidence ; Korea ; Male ; Prognosis ; Renal Dialysis* ; Retrospective Studies ; Thrombectomy ; Transplants

The incidence of colon cancer and cancer-related deaths has been increased in Korea. Because most colon cancers arise from colonic adenomatous polyps, it is important to detect these early and to resect such lesions, and so the incidence of endoscopic polypectomy has increased in Korea since 1970's. At present, conventional colonoscopy is the standard for evaluating the colon, and especially for the screening and treatment of colon tumor. However, the entire colon cannot be visualized during conventional colonoscopy in 5~15% of patients due to a redundant colon, an excessive loop or a history of abdominal surgery. To overcome these difficulties, many radiologic and endoscopic studies have been conducted and there are several recent reports that double balloon enteroscopy has been successfully used in cases of failed conventional colonoscopy. We report here on a case of laterally spreading tumor that was resected with double balloon enteroscopy in a severely redundant colon.
Adenomatous Polyps ; Colon ; Colonic Neoplasms ; Colonoscopy ; Double-Balloon Enteroscopy ; Humans ; Incidence ; Korea ; Mass Screening

PURPOSE: X-linked agammaglobulinemia(XLA) is an immunodeficiency caused by abnormalities in Bruton's tyrosine kinase(Btk), and is characterized by a deficiency of peripheral blood B cells. We studied the cytoplasmic expression of Btk protein and analyzed the Btk gene in peripheral blood mononuclear cells from two siblings and one cousin with XLA, as well as additional family members. METHODS: Btk protein expression was analyzed by flow cytometry. Isolation of the coding sequence of the Btk gene was performed by amplification using the reverse transcription-polymerase chain reaction(RT-PCR) technique. Sequence alterations were screened by the single-stranded conformation polymorphism(SSCP) method and characterized by standard sequencing protocols. RESULTS: Cytoplasmic expression of Btk protein in monocytes was not detected in three patients with XLA. In addition, Btk protein analysis clearly showed cellular mosaicism in monocytes from four obligate carriers, findings further supported by SSCP. A single base pair mutation(T to C) in Btk-exon three, which encodes the PH domain, was identified in four XLA patients. A diagnostic sequencing analysis was established to detect heterozygotic pattern in 4 carrier females. Furthermore, we found significant clinical heterogeneity in individuals with the same gene mutation. CONCLUSION: The implicating genetic alteration provided valuable clues to the pathogenesis of XLA in Korea and the flow cytometric analysis was suggested as a useful tool for rapid detection of XLA patients and carriers. The present study has identified a genetic mutation in the Btk coding region and demonstrated heterogeneity in clinical manifestations among patients with the same mutation. A flow cytometric analysis was found to be informative in establishing a deficiency of Btk protein in both patients and carriers and is recommended as a frontline procedure in the molecular diagnosis and work-up of XLA.
Agammaglobulinemia* ; B-Lymphocytes ; Base Pairing ; Clinical Coding ; Cytoplasm ; Diagnosis ; Female ; Flow Cytometry ; Humans ; Hydrogen-Ion Concentration ; Korea ; Monocytes ; Mosaicism ; Polymorphism, Single-Stranded Conformational ; Population Characteristics ; Protein-Tyrosine Kinases* ; Siblings ; Tyrosine*