No abstract available.
Humans ; Religion*

Erythropoietic protoporphyria, sometimes also called erythrohepitic protoporphyria or simple protoporphyria, is a heritable detect of heme synthesis in which the last enzyme of the heme synthetic pathway, ferrochelatase(or heme synthetase), is functioning subopt,imally. A 23-year-old male has experi nced erythema and edema on the fae and hands during or immediately after sun exposure, since 3 years of age. The skin lesions have been accompanied by severe itching, a buring sensation and pain. Severe episodes were followed hy head iche and vomiting. We have seen a case of erythropoietic protoporphyria presenting clinically and histo athologically, wit.h a skin lesion on the sun exposured area, and free erthrocyte protoporphyrin serologically.
Edema ; Erythema ; Hand ; Head ; Heme ; Humans ; Male ; Protoporphyria, Erythropoietic* ; Pruritus ; Sensation ; Skin ; Solar System ; Vomiting ; Young Adult

Syphilis is a communicahble disease caused by the motile microaerophilic spirochete Treponema pallidum, which is only a netural pathogen for human. Prevalence of syphilis and other sexually transmitted diseases has traditioially fluctuated with changes in sccia, conditions and sexual behavior. Although sexual contact is the main route of transmission, T. pallidum may also be infected through direct contact with syphilitic lesions, blood transfusion, ingestion of menstrual blood or vaginal secretions, or trsnsplacental transemission. Fomites as the means of transfer is only hypothetical and account for very few, if any, infection. Prosectors, blood handlers and laboratory technicians are at risk for accidental inoculation with infected materials. In extremely unusual circumstances, infection by means of contact with a skin lesion and human bite have been reported. We report two cases of eyphilis following the abnormal imposition of hands, which caused unnecessary erosive trauma with fingernails,
Bites, Human ; Blood Transfusion ; Eating ; Fomites ; Hand* ; Humans ; Laboratory Personnel ; Nails ; Prevalence ; Sexual Behavior ; Sexually Transmitted Diseases ; Skin ; Spirochaetales ; Syphilis* ; Treponema pallidum

One case of malignant acanthosis nigricans associated with internal malignancy has been presented and the suhject of malignant acanthosis nigricans has been revieived. The patient who was 50 year old man had been affected generalized hyperpigmentation with pnpillomatosis especially on forehead, neck, axilla, umbilical area, anogenital area, palm and soles and associated with highly malignant adenocarcinoma of stomach. Diagnosis was confirmed by characteristic clinical appearance, explorative laparatomy and histopathologically
Acanthosis Nigricans* ; Adenocarcinoma ; Axilla ; Diagnosis ; Forehead ; Humans ; Hyperpigmentation ; Middle Aged ; Neck ; Stomach

One case of malignant acanthosis nigricans associated with internal malignany and oral florid papilloma has been presented and reviewed the subject of malignant acanthosis nigricans. The patient who was 34 year old man had been affected generalixed dark brownish hyperpigmentation with papillomatoses especially on forehead, neck, axillae, umbilical area, anogenital area, webs of fingers, palms and soles. And characteristically it was associated with severe florid papilloma on mucous membrane such as buccal mucosa, lip and glans penis. Histopathologically, internal malignancy was confirmed metastatic adenocarcinoma of stomach after autopsy, although previous biopsy finding was squamous cell carcinoma when explorative laparatomy was done. The response to Bleomycin, new antibiotic anticancer drug, followed by intravenous injection is good and satisfying. But the patient died due to sudden pulmonary hemorrhage which are serious side effect of Bleomycin.
Acanthosis Nigricans* ; Adenocarcinoma ; Adult ; Autopsy ; Axilla ; Biopsy ; Bleomycin ; Carcinoma, Squamous Cell ; Fingers ; Forehead ; Hemorrhage ; Humans ; Hyperpigmentation ; Injections, Intravenous ; Lip ; Male ; Mouth Mucosa ; Mucous Membrane ; Neck ; Papilloma* ; Penis ; Stomach

There are two major types of vascular birthmarks, hemangiomas, those demonstrating endothelial hyperplasia, and malformation, those with normal endothelial turnover. Venous malformations have previously been treated by surgical excision, where possible. Although not a panacea for all such tumors, the use of sclerosing agents is decidedly preferable in some cases to extirpation with a scapel. The method involves the direct injection of absolute alcohol into the lesion on the fluoroscopic guide. The effect of treatment is satisfactory and there appears tobe no long term complication. We report 2 cases of surgically irresectable and deep-seated venous malformation which were treated sclerotherapy using ethanol.
Ethanol ; Hemangioma ; Hyperplasia ; Sclerosing Solutions ; Sclerotherapy*

We treated 11 patients with advanced transitional cell carcinoma of upper urinary tract with adjuvant M-VAC chemotherapy and their median survival time was compared with 9 patients without M-VAC chemotherapy as e historical group. The total number of cycles per each patient ranged from 1 to 5 with a mean of 3.4. Of these patients, 8 patients could be evaluated for response and 4 patients were responded (2 complete and 2 incomplete. response rate 50%). The median duration of response was 26 months for complete responders and 4.5 months for incomplete responders. The median duration of survival for all chemotherapy group, complete responders, progression and historical control group were 22, 23+, 14 and 21 months. respectively. Median survival was 22 months in all 11 patients. 23+ months in clinical responders, 14 months in progression and 21 months in historical control group. Although overall survival was not prolonged significantly in chemotherapy than the historical control group, M-VAC was effective in small proportion of patients (CR: 2/8). The duration of survival of the patients with complete remission was prolonged significantly.
Carcinoma, Transitional Cell* ; Doxorubicin* ; Drug Therapy* ; Humans ; Urinary Tract* ; Vinblastine*

Thrombotic thrombocytopenic purpura (TTP) is frequently associated with small cortical and subcortical infarcts. However, major intracranial artery occlusion is rare. We report a case of progressive middle cerebral artery (MCA) infarction in a patient with TTP. A 76-year-old woman with hypertension was admitted due to right hemiparesis and transcortical motor aphasia. On laboratory findings, she had microangiopathic hemolytic anemia and thrombocytopenia. Brain MRI showed left subcortical multiple infarction, and MR angiogram revealed severe stenosis of left MCA main stem. She was treated with heparin and steroid but a few days later, there was neurologic deterioration attributed to progressive occlusive stroke in the left MCA territory. Follow-up brain CT demonstrated left malignant MCA infarction. TTP may be associated with progressive occlusion of large cerebral artery.
Aged ; Anemia, Hemolytic ; Aphasia, Broca ; Arteries ; Brain ; Cerebral Arteries ; Constriction, Pathologic ; Female ; Follow-Up Studies ; Heparin ; Humans ; Hypertension ; Infarction ; Infarction, Middle Cerebral Artery* ; Magnetic Resonance Imaging ; Middle Cerebral Artery* ; Paresis ; Purpura, Thrombotic Thrombocytopenic* ; Stroke ; Thrombocytopenia

Neurofibromatosis type 1 is the most common neurocutaneous disorders and affects between 1/2,000 and 1/4,500 people. This occurs at any age and is hereditary disease with autosomal dominant fashion. Renovascular hypertension is major form of renal manifestation of the disease. There are few reported cases in Japan and Hungary of Recklinghausen's neurofibromatosis with several glomerular lesions but their relationship is not apparent. A 21-year-old man was admitted to the hospital because of general edema. On admission, the blood pressure was 130/ 80 mmHg and general edema was noted. He had a plexiform neuroma on right flank and multiple cafe- au-lait spots on chest and extremites. Laboratory findings were as follows : Hemoglobin 14.2 g/dL, AST 28 IU/L, ALT 12 IU/L, albumin 1.2 gm/dL, total cholesterol 533 mg/dL, urinary protein 4.0 gm/ day, C3 86.6 mg/dL, C4 19.9 mg/dL, HBs Ag/Ab (+/-), HBe Ag/Ab (+/-), HCV Ab (-), HBV DNA probe 6,000 pg/mL. Renal biopsy was performed and the histological findings were compatible with minimal change disease. The immunohistochemical method revealed that HBsAg was negative. We experienced a case of minimal change disease concurrent with Neurofibromatosis type 1, but their relationship is not clear. We report this case with a brief review.
Biopsy ; Blood Pressure ; Cholesterol ; DNA ; Edema ; Genetic Diseases, Inborn ; Hepatitis B Surface Antigens ; Hepatitis B, Chronic ; Humans ; Hungary ; Hypertension, Renovascular ; Japan ; Nephrosis, Lipoid* ; Neurocutaneous Syndromes ; Neurofibroma, Plexiform ; Neurofibromatoses* ; Neurofibromatosis 1* ; Thorax ; Young Adult

PURPOSE: The aim of this study was to compare the fracture toughness of currently available resin cements for zirconia restorations and evaluate the effect of water storage on fracture toughness of those resin cements. MATERIALS AND METHODS: Single-edge notched specimens (3 mm x 6 mm x 25 mm) were prepared from three currently available dual cure resin cements for zirconia restorations (Panavia F 2.0, Clearfil SA luting and Zirconite). Each resin cement was divided into four groups: immersed in distilled water at 37degrees C for 1 (Control group), 30, 90, or 180 days (n=5). Specimens were loaded in three point bending at a cross-head speed of 0.1 mm/s. The maximum load at specimen failure was recorded and the fracture toughness (K(IC)) was calculated. Data were analyzed using one-way ANOVA and multiple comparison Scheffe test (alpha=.05). RESULTS: In control group, the mean KIC was 3.41 +/- 0.64 MN.m(-1.5) for Panavia F, 2.0, 3.07 +/- 0.41 MN.m(-1.5) for Zirconite, 2.58 +/- 0.30 MN.m(-1.5) for Clearfil SA luting respectively, but statistical analysis revealed no significant difference between them. Although a gradual decrease of K(IC) in Panavia F 2.0 and gradual increases of KIC in Clearfil SA luting and Zirconite were observed with storage time, there were no significant differences between immersion time for each cement. CONCLUSION: The resin cements for zirconia restorations exhibit much higher K(IC) values than conventional resin cements. The fracture toughness of resin cement for zirconia restoration would not be affected by water storage.
Immersion ; Resin Cements ; Resins, Synthetic* ; Water*