Two Cases of Tuberous Sclerosis Patients with Renal Anomaly / 대한신장학회잡지

Tuberous sclerosis is a rare disease, which occurs sporadically or hereditarily and is recognized by its neurological and dermatological manifestations and may be accompanied with renal anomalies. The classical triad is composed of seizure, mental retardation and adenoma sebaceum on face. We experienced two cases of tuberous sclerosis in sporadic forms by mutation without any familial history which suggests the diseases were occurred by mutation rather than by autosomal dominant inheritance. In the first case, a 24-year-female patient with hypertension and abnormal renal function tests which were noted on the routine prenatal check at 32 weeks of gestation delivered normally at 37 weeks. The daughter of patient had seizure when she was 6 years old and was diagnosed as polycystic kidney disease by abdominal computed tomography. This case developed sporadic form of disease without familial history but, the daughter of patient might inherited by autosomal dominant form. The patient's clinical feature was characterized by history of epilepsy, painless abdominal mass due to polycystic kidney disease, abnormal renal function, skin abnormalites including angiofibroma and shagreen patch. Abdominal computed tomography demonstrated numerous variable sized cysts throughout both kidney. In second case, the patient was a 32-year-female patient complaining of 5kg weight gain, abdominal distension due to palpable masses. Her clinical feature was characterized by bilateral huge renal angiomyolipoma with normal renal function and skin abnormality such as erythematous papule on the face. Abd CT and MRI revealed huge angiomyolipoma of about 15cm X 18.5cm X 30cm and 14.5cm X 18cm X 30cm respectively. We presented the two cases with brief review of the literatures.

A Case of Periosteal New Bone Formation Associated with Behcet? Disease / 대한류마티스학회지

Behcet? disease is a recurring illness characterized by the triple symptom complex of aphthous stomatitis, genital ulceration, and uveitis. The disease is multisystemic disorder with involvement of skin, gastrointestinal tract, blood vessels, central nervous system, joints, and epididymis. Vascular involvement of Behcet? disease affects both arteries and veins and blood vessels of all sizes. Periosteal new bone formation is one feature of hypertrophic osteoarthropathy. Other features are clubbing and arthritis. Periosteal new bone formation on the bones of the lower extremities without other features of hypertrophic osteoarthropathy has been reported in patients with varicose veins, vitamin A intoxication, infantile cortical hyperostosis, tuberous sclerosis, congenital syphilis and chronic renal failure with hyperparathyroidism, and chronic venous insufficiency. However, periosteal new bone formation has not, to our knowledge, been described in patients with Behcet? disease yet although similar findings are occasionally noted in polyarteritis nodosa. We report a case of Behcet? disease complicated by leg swelling with periosteal new bone formation of right lower leg.

The Safety and Feasibility of Transradial Primary Coronary Intervention in Acute Myocardial Infarction

BACKGROUND AND OBJECTIVES: Aggressive antithrombotic therapy and transfemoral primary intervention during acute myocardial infarction (AMI) restricts the patient's movement and may increase the risk of access site bleeding complications, and lengthen the duration of movement restriction and hospital stay. Transradial approach provides less bleeding complications and early ambulation. The purpose of this study is to know whether transradial primary intervention is safe and feasible in the patients with AMI. MATERIALS AND METHOD: From April 1998 to December 1998, transradial primary interventions were performed in the consecutive 28 patients (24 male, 57+/-7 years) by two experienced operators. The results were compared to the results of 44 (15 patients during same period, 29 during previous year) transfemoral primary interventions. RESULTS: 1)The success rates of transradial primary interventions was 93% (26/28) and comparable to 95% (42/44) of transfemoral primary interventions. 2)In transradial group, the time from the arrival of catheterization laboratory to arterial access and to reperfusion, the time from the arrival of emergency room to reperfusion were 8.1+/-3.4 minutes, 22.0+/-5.3 minutes, and 71.7+/-9.2 minutes, respectively and comparable to 9.0+/-3.1 minutes, 21.7+/-5.3 minutes, and 68.9+/-8.1 minutes of transfemoral group, respectively. 3)The complications of the procedure were treated successfully during transradial interventions. 4)In transradial group, puncture site bleeding complications were absent though heparin was continued and mild ambulation was possible early after the procedure. The hospital stay of transradial group was 5.3+/-1.3 days and shorter than 7.7+/-4.2 days of transfemoral group. CONCLUSION: In the low risk patients with AMI, transradial primary intervention might be safe and feasible with acceptable time delay by the experienced operators. It might be effective to reduce access site bleeding complications and to initiate early ambulation, resulting in the shortened hospital stay.