A Case of Anomalous Left Coronary Artery Originating from the Right Sinus of the Valsalva Presenting with Syncope / 대한내과학회지

Congenital anomalies of the coronary artery are associated with various symptoms including syncope, myocardial ischemia, and sudden cardiac death. The abnormality depends on the adjacent structure and pathway of the coronary artery. Most patients with an anomalous left coronary artery that arises from a right coronary sinus of the valsalva have no symptoms and are usually diagnosed at autopsy. Therefore, their first symptom might present as sudden death, particularly when the left coronary arterial course is between the aorta and the pulmonary trunk. Symptomatic patients could be diagnosed early with an anomalous coronary artery, and the risk of fatal events could be decreased by surgical correction. Here, we report the case of 62-year-old male who experienced a first episode of syncope with an anomalous left coronary artery arising from the right sinus of the valsalva with a separate orifice from the right coronary artery. He is alive and in good health receiving medical treatment, and has had no medical events for over 2 years.

An Evaluation of Platelet Transfusion Response Using HLA Crossmatch-compatible Donors in Patients with Platelet Refractoriness / 대한진단검사의학회지

BACKGROUND: Majority of immune-mediated platelet refractoriness is caused by HLA alloimmunization and can be effectively managed by HLA-matched platelet transfusions. However, HLA class I-typed large-sized donor registry has not been well established in Korea. We evaluated the effectiveness of platelet transfusion using HLA crossmatch-compatible donors without HLA typing. METHODS: Sixteen patients showing platelet refractoriness to random donor platelets (1 hr corrected count increment [CCI] 60%) were crossmatched with 78 platelet apheresis-eligible donors using National Institute of Health (NIH) and anti-human globulin (AHG) lymphocytotoxicity methods. NIH negative/AHG negative and NIH negative/AHG positive donors were selected as best and second choice donors, respectively. RESULTS: Eleven patients (11/16, 69%) could find NIH-crossmatch negative donors and 27 donors (27/78, 35%) belonged to the best donors. To 8 patients, 32 apheresis platelet products from 19 donors were transfused. The mean 1 hr and 24 hr CCI values from the best donors were significantly higher than those from random donors (17,893 vs 2,358, P=0.003; 8,292 vs -614, P<0.001), whereas such differences were not observed for those from the second choice donors. Platelet storage time was inversely correlated with CCI values and platelets stored < or =10 hr after collection gave significantly higher CCI values. Neither ABO match nor donor status (related vs unrelated) affected the transfusion effectiveness. CONCLUSIONS: Effective post-transfusion platelet increment using HLA crossmatch-compatible donors was attained in patients with platelet refractoriness due to HLA antibodies, and this method can be used effectively where HLA-typed platelet donor registry is not available.

A Case of Diffuse Neonatal Hemangiomatosis Improved with Prednisolone and Interferon alfa - 2a / 대한피부과학회지

Diffuse neonatal hemangiomatosis is a disease often with a fatal outcome, characterized by widespread capillary hemangiomias of the skin and visceral organs. A 6-month-old-girl was diagnosed to have diffuse neonatal hemangiomatosis by her multiple hemangiomas on the skin, liver, brain, lung and oral mucosa. She was treated with prednisolone 1.5-2mg/kg/day for 9 months and combined interferon alfa-2a 3 million unit/m2/day for 5months. During the treatment the number and size of hemangiomas were markedly reduced

Systemic Scleroderma in Childhood / 대한피부과학회지

The onset of systemic scleroderma in the first. decade of lif occurs in less than 1.5% of instances. Systemic sclerodrma in childhood have been characterized by less severe visceral involvement and a benign course We report a boy, aged 7 years, with a history of Raynaid; phenomenon and dysphagia. He had a mask-like face, flexion contracture of hands and knee joints, digital pitting scars, ichthyosiform skin change on leg and hyperpigrnented induration on all extremities and trunk. Antinuclear antibodies were positive in nucleolar type and Scl-70 antiocy was also present. On radiologie study, the pattern of reflux esophagitis and resorption of the listal ends of all phalanges were nated. Histopathological findings were compatible with sclerodma. He was treated with Penicillamine-D and oral corticosteroic with good improvernent.

A case of multiple furunculosis caused by methicillin-resistant staphylococcs aureus / 대한피부과학회지

Staphylococcus aureus is one of many causative agents of the fu uncles. As the S. aureus strains resistant to penicillinase-repsistant penicillins (methicillin-resistant S. aureus, MRSA) are more frequently isolated, their accuiate identification became a very important step in selection of an appropriate therapeutic regimen. We report herein a case of multiple furunculosis caused by metkicillin-resistant S. aureus in a 11-year-old male patient, who was successfully treated with sulbactam/ampicillin and amikacin.

Clinical study on death in Behcet's disease

We were able to identify 7 patients who died of Behcet's disease, among 2,200 patients registered at the Behcet's Disease Specialty Clinic of Severance Hospital from November 1983 to October 1992. Six were male and one female. Age of death was 31 to 55 years with the mean age of 39.1. The age of onset was 24 to 54 years with the mean age of 32.6. The mean duration of illness was 6.5 years. The most frequent initial symptom was oral ulcer, with the most common type being the incomplete type in Shimizu's classification. Positive pathergy tests were found in all the 5 patients in whom the test was performed out of 7 patients. Various modalities of treatments such as oral steroid, colchicine, azathioprine, colectomy, and pacemaker insertion were attempted without outstanding therapeutic effects. The causes of death were gastrointestinal bleeding, bowel perforation, superior and inferior vena cava syndrome, aortic regurgitation, cerebrovascular disease, sepsis, and lung abscess.

Persistent light reaction / 대한피부과학회지

Persistent light reaction is a condition of chronic photodermatitis in which photosensitive reaction persists even after the rernoval of all photosensilizers. A 56-year-old man had experienced a recurrent dermatitis involving primarily the face, neek, forearms and hands for 9 years, this condition was aggravated by sunexposure. Photopatch testing disclosed a strongly positive reaction to chloropromazine, promethazine, acid trichlorocarbanilide, Phototesting also revealed lowered MED with UVA and UVB thar norrmal mean value.