Erythropoietic protoporphyria(EPP) is an inherited inborn error of porphyrin metabolism caused by decreased activity of the enzyme ferrochelatase. EPP is characterized clinically by photosensitivity to visible light commencing in childhood, and biochemically by elevated red cell free protoporphyrin levels. We report herein a case of EPP which occurred in a 44-year-old man and his family. He had suffered from immediate photosensitivity since he was 4 years old. He was presented with burning, erythema, scars and waxy thickening of the sun-exposed skin. Red cell free protoporphyrin level was elevated and urinary porphyrins were normal. Histopathologically, homogeneous eosinophilic materials that stained with PAS were deposited in perivascular area of upper dermis. He was managed with light restriction and sunscreen.
Adult ; Burns ; Child, Preschool ; Cicatrix ; Dermis ; Eosinophils ; Erythema ; Ferrochelatase ; Humans ; Light ; Metabolism ; Porphyrins ; Protoporphyria, Erythropoietic* ; Skin

BACKGROUND: Intracoronary stenting in large coronary artery with diameters >3 mm has been shown to be beneficial in the treatment of acute or threatened closures complicating balloon angioplasty and in the prevention of restenosis. However, whether equally favorable results are afforded by stent placement in small vessels (<3 mm) remains unclear. Accordingly, we evaluated the safety and feasibility of intracoronary stenting in native coronary vessels less than 2.75 mm in size. METHODS: Between January 1997 and July 1998, seventy eight patients with 81 lesions were treated with 83 stents, regardless of clinical setting. The angiographic criteria for enrollment included at least 70% stenosis and a vessel that reference diameter was less than 2.75 mm. Every patients received aspirin (300 mg qd, indefinitely) and ticlopidine (250 mg bid, one month) and was given a bolus dose of 10,000 U heparin during procedure. RESULTS: Angiographic success was achieved in 80 of 81 attempts (98%). There was one in-hospital death because of pump failure in AMI patient. There was no acute stent thrombosis. At 6 month follow-up, event free survival was achieved in 90% of patients and angiographic restenosis was found in 28% of patients (9/32). CONCLUSION: The present observational study demonstrates that angiography-guided stent placement in coronary artery < 2.75 mm in diameter is safe and effective in conjunction with current stent deplyment technique and antiplatelet protocol.
Angioplasty, Balloon ; Aspirin ; Constriction, Pathologic ; Coronary Artery Disease* ; Coronary Vessels* ; Disease-Free Survival ; Follow-Up Studies* ; Heparin ; Humans ; Observational Study ; Stents* ; Thrombosis ; Ticlopidine

BACKGROUND: The role of cutaneous bacterial flora in eczematous dermatitis has been discussed for decades. Areas of active dermatitis in patients with atopic dermatitis(AD) are known to be heavily colonized with Staphylococcus aureus. The density of S. aureus is correlated with the severity of AD. S. aureus is considered to be an exacerbating factor in this disease. The bacterium interferes in the inflammatory process of atopic dermatitis in various ways, among which the ability to release superantigens in a high percentage of clinical isolates is of great importance. OBJECTIVE: The purpose of this study was to evaluate the microbiology of the skin of the patients with AD and non-atopic dermatitis(NAD). We compared bacterial colonization of AD with those of NAD and healthy controls(HC). METHODS: Cutaneous organisms were obtained using Tape method from the worst lesion and uninvolved forearm skin of 30 patients with AD, 25 patients with NAD and 30 individuals as HC. Bacterial growth was examined in aerobic culture and the counts of colony forming unit(CFU) were estimated. The sensitivity to ten antibiotics of the bacteria isolated was also tested. RESULTS: 1.S. aureus colonizations of the eczematous skin were found in 19 of 30 AD patients(63%) and in 9 of 25 NAD patients(36%). The difference between the two groups was statistically significan t(p=0.076, alpha<0.1). 2.In AD patients, the frequencies of S. aureus colonizations revealed 63%(19 of 30) in the lesion and 60%(18 of 30) in uninvolved skin, which were significantly higher than 33%(10 of 30) of HC(p<0.05). 3.The frequency of S. aureus colonizations in the lesions of NAD patients revealed 36%, which was not significantly higher than 33% of HC(p=0.836). 4.Coagulase negative strains of staphylococcus were found in 20 of 30 HC(66%) and 17 of 25(68%) uninvolved skin of NAD patients(68%) compared with 12 of 30 uninvolved skin of with AD(40%). 5.In AD patients with positive nasal colonization with S. aureus, the frequencies of S. aureus colonization in the lesion and uninvolved skin were increased in significance compared with patients with negative nasal colonization(p<0.05). And total counts of CFU in the lesion and uninvolved skin of AD patients were significantly higher compared with HC(p<0.05). 6.Total counts of CFU correlated with severity of dermatitis. CONCLUSION: Colonization of S. aureus was significnatly increased in the skin of AD patients compared with healthy controls. As the severity of the AD lesions increased, the numbers of S. aureus isolated increased. These results support the role of S. aureus in the AD described in the previous reports. Tape method had the advantage of being inexpensive, quick and straightforward to prepare, easy for the clinician to use and was well tolerated by children. It is hoped that the result of this study and the availability of Tape method in the culture of bacteria of the skin will facilitate and promote investigations of pathophysiology and treatment of eczematous dermatitis as well as AD.
Anti-Bacterial Agents ; Bacteria ; Child ; Colon ; Dermatitis* ; Dermatitis, Atopic* ; Eczema ; Forearm ; Hope ; Humans ; NAD ; Skin ; Staphylococcus ; Staphylococcus aureus ; Superantigens

A 72-year-old diabetic male patient with high grade fever, right upper quadrant abdominal pain and Klebsiella pneumoniae septicemia is reported. He suffered from high fever and abdominal pain in spite of aspiration of liver abscess and antibiotic treatment. A few days later, we found a palpable pulsating abdominal mass on physical examination. Computed tomography and angiography revealed infected abdominal aortic aneurysm associated with pyogenic liver abscess. He was treated by antibiotics and Endovascular stent without surgical resection. He improved without complications and has been followed-up after discharge with excellent condition. To our knowledge, this is the first case of infected abdominal aortic aneurysm as a septic metastatic lesion secondary to liver abscess.
Abdominal Pain ; Aged ; Aneurysm, Infected ; Angiography ; Anti-Bacterial Agents ; Aortic Aneurysm, Abdominal* ; Fever ; Humans ; Klebsiella pneumoniae ; Liver Abscess* ; Liver Abscess, Pyogenic ; Liver* ; Male ; Physical Examination ; Sepsis ; Stents*

Merkel cell carcinoma is an uncommon aggressive primary neuroendocrine neoplasm of the skin. It is more prevalent in elderly females and occurs as a solitary lesion on the face and extremities. The first case was a 65-year old female with an asymptomatic cherry-sized pink nodule with infiltrative base on the left cheek that had gradually enlarged during several months. The second case was an 81-year-old female with a bean-sized pink nodule on the right breast that had rapidly enlarged during 1 month. Microscopically, small tumor cells with scanty cytoplasm and hyperchromatic nuclei arranged in solid sheets and trabecular or cord like pattern in a delicate fibrous matrix in both cases. In immunohistochemical studies, the first case was reactive to NSE, chromogranin and cytokeratin, and the second case to NSE and cytokeratin. Ultrastructurally, the tumor cells showed membrane-bound, round, dense-core granules of neuroendocrine type in the cytoplasm and perinuclear whorls of intermediate filaments. There was no evidence of metastasis in the whole body CT and bone scintigraphy. In both cases, local wide excision was performed.
Aged ; Aged, 80 and over ; Breast ; Carcinoma, Merkel Cell* ; Cheek ; Cytoplasm ; Extremities ; Female ; Humans ; Intermediate Filaments ; Keratins ; Neoplasm Metastasis ; Radionuclide Imaging ; Skin

Cerebral Venous Sinus Thrombosis (CVST) in children is rare and its cause is multifactorial. The clinical manifestations of CVST vary and may cause long-term neurological sequelae and even death on rare occasion. In this case, a 15 year old boy presented with severe headache and vomiting for 1 day. Brain MRI with venography revealed multiple lesions of CVST in superior sagittal sinus and the left transverse sinus. Anticoagulation therapy was performed for 3 months, which led to the complete resolution in superior sagittal sinus and partial resolution in left transverse sinus.
Adolescent ; Brain ; Child ; Headache ; Humans ; Phlebography ; Sinus Thrombosis, Intracranial ; Superior Sagittal Sinus ; Vomiting

No abstract available.
Animals ; Neurons* ; Rats*

According to the size of nevus, congenital nevomelanocytic nevi(CMN) could be divided as small, medium, and large or giant. Giant CMN is well known as precursor for malignant melanoma, but the risk of malignant transformation in small or medium-sized CMN is the subject of controversy. We report a case of 54-year-old man who was diagnosed as malignant melanoma arising medium -sized CMN on the trunk. Local wide excision was performed but distant metastases were found 5 year later.
Humans ; Melanoma* ; Middle Aged ; Neoplasm Metastasis ; Nevus*

BACKGROUND AND OBJECTIVES: Primary coronary stenting has been shown to be an effective reperfusion therapy for acute myocardial infarction(AMI). However, few data exist regarding long-term follow-up. We examine the long-term clinical and angiographic outcomes after primary coronary stenting in the early setting of AMI. METHODS: Between September 1995 to October 1999, coronary stenting was attempted in 181 consecutive patients admitted with the diagnosis of AMI within 6 hours from the onset of the chest pain. Clinical events, including death, MI, coronary bypass surgery and repeat angioplasty, were recorded for 1 year. Angiogram were obtained at baseline, after stent, at 2 weeks and 6 months. RESULTS: 168 patients(92.8%) of all patients had complete 1 year clinical follow-up. In-hospital deaths occured in 5 patients(3%). One hundred five patients(62.5%) had follow-up angiography at 6.4+/-2.1 months after stent implantation and restenosis occured in 21.9%. Clinical events after 1 year included death in 6.5%, myocardial infarction in 1.2%, bypass surgery in 1.8% and repeat angioplasty in 7.7%. Freedom from any adverse cardiac event at 1 year was 82.2%. CONCLUSION: Primary stenting is safe and feasible in patients of AMI, even in large thrombus containing lesion and results in excellent long-term outcomes.
Angiography ; Angioplasty ; Chest Pain ; Diagnosis ; Follow-Up Studies ; Freedom ; Humans ; Infarction* ; Myocardial Infarction ; Reperfusion ; Stents* ; Thrombosis

BACKGROUND: Elevation in plasma homocysteine has been widely studied as an independent risk factor for atherosclerosis. And epidemiologic studies have demonstrated that the persons who take the folate and vitamin B6 have lower incidence of atherosclerotic vascular disease and lower plasma homocysteine level. But, not yet the effects of vitamin B6 and folate on the level of plasma homocysteine and brachial artery dilation on healthy subjects was not evaluated. METHODS: We evaluated the effects of 50 mg of vitamin B6 and 1 mg of folate on endothelial function, plasma homocysteine levels to one healthy postmenoausal woman and nineteen men in a randomized, double-blind, placebo-controlled, crossover design. RESULTS: In our study, supplement of vitamin B6 and folate significantly lowered plasma homocysteine level (placebo : folate =6.56 +1.55 micromol/L vs. 5.37 +1.04 micromol/L, p=.001). But, there were no statistically significant increament of flow-mediated dilation (FMD) compared to placebo (placebo : folate =5.12 +3.26% vs. 6.69 +2.60%, p=.070) and there were no significant correlation between the improvement of homocysteine level and increament of flow mediated dilation on healthy subjects. CONCLUSION: Compared to persons with absolute or relative hyper-homocysteinemia, our study did not show such favorable effects in healthy persons. So further studies must to be held to discover the effect of folate and vitamine B6 in healthy persons.
Atherosclerosis ; Brachial Artery* ; Cross-Over Studies ; Female ; Folic Acid* ; Homocysteine* ; Humans ; Incidence ; Male ; Plasma* ; Risk Factors ; Vascular Diseases ; Vitamin B 6* ; Vitamins*