We report herein two cases of localized curaneous amyloidosis (LCA) associated with neurodermatitis. Both patients suffered from persistent pruritus during several years and had small brown discrete lichenoid papules or neurodermatitis like lesion. Biopsy specimen taken from lichehoid papules revealed amyloid deposition in the papillary dermis. In histologic examination of the neurodermatitis like lesion, hematoxylin eosin stain revealed only chronic eczematous changes but special stain and election microscopic examination revealed amyloid deposition in the papillary dermis. Although neurodermatitis and LCA may be able to develop concomitantly in one patient, it was suggested that neurodermatitis may transfrom into LCA.
Amyloidosis* ; Biopsy ; Dermis ; Eosine Yellowish-(YS) ; Hematoxylin ; Humans ; Neurodermatitis* ; Plaque, Amyloid ; Pruritus

No abstract available.

Intracranial lipomas located in the cerebellopontine angle are extremely rare. These tumors are maldevelopmental lesions which can cause slowly progessive neurological symptoms. The clinical management of these tumours differs significantly from other lesions in this region. A 45 year old man presented with a 3-month history of vertigo and tinnitus in the left ear. In T1-weighted magnetic resonance imaging, the lesion was hyperintense and did not enhance after application of gadolinium. The patient was treated with medical therapy with good response. We present a review of the literature, with particular regard to management. Conservative treatment is recommended and limited surgery is usually indicated if the patients suffer from disabling neurological symptoms and signs e.g., vertigo, nausea, trigeminal neuralgia, facial weakness or facial spasm.
Cerebellopontine Angle ; Ear ; Gadolinium ; Humans ; Lipoma* ; Magnetic Resonance Imaging ; Middle Aged ; Nausea ; Spasm ; Tinnitus ; Trigeminal Neuralgia ; Vertigo

A case of Sturge-Weber Syndrome is presented in a male neonate with severe gangrenous ulcerative stomatitis involving the angiomatous skin lesion and leaving sequele of cleft lip and nasal disfiguration.
Cleft Lip* ; Congenital Abnormalities* ; Gingivitis, Necrotizing Ulcerative ; Humans ; Infant, Newborn ; Male ; Skin ; Sturge-Weber Syndrome*

An open trial was carried out to investigate the efficacy and tolerance of isosorbide 5-mononitrate(Elantan(R)) in 30 patients diagnosed as angina pectoris at Pusan national university hospital. Drugs were given 20mg two times daily for 2 to 3 weeks and the results were assessed in terms of effects on anginal pain and untoward side effects experienced during administration of medication. Treament resulted in an overall improvement in 25 patients(83.4%), complete abolition of anginal attacks in 17 patients(56.7%) and reduction in frequency of attacks in 8 patients(26.7%). So called "Nitrated headache" was observed in 5 patients(16.6%) but improved with proceeding of administration in 3 patients. This trial indicates that isosorbide 5-mononitrate should be efficacious as well as tolerated in patients with coronary heart disease.
Angina Pectoris* ; Busan ; Coronary Disease ; Humans ; Isosorbide*

An open clinical trial was conducted to evaluate the efficacy and tolerability of isradipine in 30 cases (male 16, female 14 cases, average age 52.6+/-7.94) of mild to moderate essential hypertension using 1.25-2.5mg twice a day for 8 weeks of active treatment. Blood pressure was significantly reduced from 168.5+/-14.33/108.3+/-6.37mmHg, 163.7+/-9.74/105.5+/-7.1mmHg to 141.0+/-13.69/92.0+/-9.27mmHg, 138.8+/-13.46/92.3+/-11.16mmHg in sitting and standing position respectively. The extent of reduction was 27.5/16.3mmHg in sitting position and 29.9/13.2mmHg in standing position. This comprised the mean response rate in terms of reduction of DBP of 10mmHg or more being 90% and the normalization rate, deficed as DBP lowering to 90mmHg or below, being 70%. Heart rate, hematology and blood chemistry including blood sugar and lipids were not changed significantly after treatment with isradipine. No significantl side effect was observed except 2 cases of mild transient facial flushing and nausea during the treatment, so could proceed the trial without drug discontinuation in all 30 cases. The results suggest that isradipine is one of the useful and safe drugs in the treatment of mild to moderate essential hypertension.
Blood Glucose ; Blood Pressure ; Chemistry ; Female ; Flushing ; Heart Rate ; Hematology ; Humans ; Hypertension* ; Isradipine* ; Nausea

The so called MOYAMOYA disease is characterized by idiopathic progressive bilateral occlusions of internal carotid arteries near the supraclinoid portion, with a distinctive picture of abnormal net like vessels in the base of the brain. The term MOYAMOYA is a Japanese expression for "PUFF OF A SMOKE", suggested by Suzuki & Takaku in 1969. We have experienced typical 3 cases of so called MOYAMOYA disease during 1979, which were confirmed by bilateral carotid angiography. All 3 cases were involved bilateral sides of the brain and the exact causes were not detected in spite of various laboratory examinations. All of our cases were compatible with the criteria of Moyamoya disease, suggested by Takeuchi in 1978. In addition to the case report a review of history, etiology & pathogenesis of Moyamoya Disease were discussed.
Angiography ; Asian Continental Ancestry Group ; Brain ; Carotid Artery, Internal ; Humans ; Moyamoya Disease*

OBJECTIVE: Few studies on the clinical spectrum of automated pressure-controlled discography (APCD)-defined positive discs have been reported to date. Thus, the present study was undertaken to analyze clinical parameters critical for diagnosis of discogenic pain and to correlate imaging findings with intradiscal pressures and pain responses in patients with APCD-positive discs. METHODS: Twenty-three patients who showed APCD-positive discs were selected for analysis. CT discogram findings and the degrees of nuclear degeneration seen on MRI were analyzed in comparison to changes of intradiscal pressure that provoked pain responses; and clinical pain patterns and dynamic factors were evaluated in relation to pain provocation. RESULTS: Low back pain (LBP), usually centralized, with diffuse leg pain was the most frequently reported pattern of pain in these patients. Overall, LBP was most commonly induced by sitting posture, however, standing was highly correlated with L5/S1 disc lesions (p < 0.01). MRI abnormalities were statistically correlated with grading of CT discogram results (p < 0.05); with most pain response observed in CT discogram Grades 3 and 4. Pain-provoking pressure was not statistically correlated with MRI grading. However, it was higher in Grade 3 than Grade 4. CONCLUSION: APCD-positive discs were demonstrated in patients reporting centralized low back pain with diffuse leg pain, aggravated by sitting and standing. MRI was helpful to assess the degree of nuclear degeneration, yet it could not guarantee exact localization of the painful discs. APCD was considered to be more useful than conventional discography for diagnosis of discogenic pain.
Humans ; Intervertebral Disc ; Leg ; Low Back Pain ; Posture

OBJECTIVES: Methyl-CpG binding protein 2 (MeCP2) is a ubiquitous epigenetic factor that represses gene expression by modifying chromatin. Mutations in the MeCP2 gene cause Rett syndrome, a progressive neurodevelopmental disorder. Recent studies also have shown that MeCP2 plays a role in carcinogenesis. Specifically, functional ablation of MeCP2 suppresses cell growth and leads to the proliferation of cancer cells. However, MeCP2's function in adult tissues remains poorly understood. We utilized a weight matrix-based comparison software to identify transcription factor binding site (TFBS) of MeCP2-regulated genes, which were recognized by cDNA microarray analysis. METHODS: MeCP2 expression was silenced using annealed siRNA in HEK293 cells, and then a cDNA microarray analysis was performed. Functional analysis was carried out, and transcriptional levels in target genes regulated by MeCP2 were investigated. TFBS analysis was done within genes selected by the cDNA microarray analysis, using a weight matrix-based program and the TRANSFAC 6.0 database. RESULTS: Among the differentially expressed genes with a change in expression greater than two-fold, 189 genes were up-regulated and 91 genes were down-regulated. Genes related to apoptosis and cell proliferation (JUN, FOSL2, CYR61, SKIL, ATF3, BMABI, BMPR2, RERE, and FALZ) were highly up-regulated. Genes with anti-apoptotic and anti-proliferative functions (HNRPA0, HIS1, and FOXC1) were down-regulated. Using TFBS analysis within putative promoters of novel candidate target genes of MeCP2, disease-related transcription factors were identified. CONCLUSIONS: The present results provide insights into the new target genes regulated by MeCP2 under epigenetic control. This information will be valuable for further studies aimed at clarifying the pathogenesis of Rett syndrome and neoplastic diseases.
Adult ; Apoptosis ; Binding Sites ; Carcinogenesis ; Carrier Proteins ; Cell Proliferation ; Chromatin ; Epigenomics ; Gene Expression ; HEK293 Cells ; Humans* ; Methyl-CpG-Binding Protein 2 ; Microarray Analysis ; Oligonucleotide Array Sequence Analysis ; Rett Syndrome ; RNA, Small Interfering ; Transcription Factors

OBJECTIVES: Methyl-CpG binding protein 2 (MeCP2) is a ubiquitous epigenetic factor that represses gene expression by modifying chromatin. Mutations in the MeCP2 gene cause Rett syndrome, a progressive neurodevelopmental disorder. Recent studies also have shown that MeCP2 plays a role in carcinogenesis. Specifically, functional ablation of MeCP2 suppresses cell growth and leads to the proliferation of cancer cells. However, MeCP2's function in adult tissues remains poorly understood. We utilized a weight matrix-based comparison software to identify transcription factor binding site (TFBS) of MeCP2-regulated genes, which were recognized by cDNA microarray analysis. METHODS: MeCP2 expression was silenced using annealed siRNA in HEK293 cells, and then a cDNA microarray analysis was performed. Functional analysis was carried out, and transcriptional levels in target genes regulated by MeCP2 were investigated. TFBS analysis was done within genes selected by the cDNA microarray analysis, using a weight matrix-based program and the TRANSFAC 6.0 database. RESULTS: Among the differentially expressed genes with a change in expression greater than two-fold, 189 genes were up-regulated and 91 genes were down-regulated. Genes related to apoptosis and cell proliferation (JUN, FOSL2, CYR61, SKIL, ATF3, BMABI, BMPR2, RERE, and FALZ) were highly up-regulated. Genes with anti-apoptotic and anti-proliferative functions (HNRPA0, HIS1, and FOXC1) were down-regulated. Using TFBS analysis within putative promoters of novel candidate target genes of MeCP2, disease-related transcription factors were identified. CONCLUSIONS: The present results provide insights into the new target genes regulated by MeCP2 under epigenetic control. This information will be valuable for further studies aimed at clarifying the pathogenesis of Rett syndrome and neoplastic diseases.
Adult ; Apoptosis ; Binding Sites ; Carcinogenesis ; Carrier Proteins ; Cell Proliferation ; Chromatin ; Epigenomics ; Gene Expression ; HEK293 Cells ; Humans* ; Methyl-CpG-Binding Protein 2 ; Microarray Analysis ; Oligonucleotide Array Sequence Analysis ; Rett Syndrome ; RNA, Small Interfering ; Transcription Factors