Hereditary anhidrotic ectodermal dysplasia is a rare condition characterized by underdeveloped ectodermal structure including the skin, teeth or skin appendages. The patient has characteristic feature of anhidrosis, hypotrichosis and defective dentition. We experienced a case of hereditary anhidrotic ectodermal dysplasia in a l-month-old male infant who had unexplained recurring fever, anhidrosis and characteristic facial feature, so we established the diagnosis with clinical feature and skin biopsy.
Biopsy ; Dentition ; Diagnosis ; Ectoderm ; Ectodermal Dysplasia* ; Fever ; Humans ; Hypohidrosis ; Hypotrichosis ; Infant ; Male ; Skin ; Tooth

BACKGROUND: In an effort t enhance long term patency of coronary bypass grafts, utilization of arterial conduits have been on an icrease. With the same objective, we have been using the right gastroepiploic artery (RGEA) in coronary artery bypass procedures since 1998. The current paper has been undertaken with the aim of assessing the apropriateness, problems, and short term results of using the RGEA as an arterial graft conduit by studying the postoperative clinical results of 100 patients than received coronary artery bypass grafting (CARG) with this artery. MATERIAL AND METHOD: Between May of 1998 and May of 1999, an analysis of the mortality, postoperative myocardial infarction, and the need for IABP insertion as a result of low cardiac output were made between 100 consecutive patients undergoing CABG with the RGEA. There was one postoperative death due to cerebral infarction. Postoperative complications/morbidity comprised myocardial infarction in 2, cerebral infarct in 3, reoperation due to bleeding in 1, mediastinitis in 1, and low cardiac output syndrome necessitating IABP in 3 patients. Complicatons related to harvesting of the arterial grafts were not experienced in any of the patients. CONCLUSION: The results of the current data show that utilization of the RGEA in CABG is not associated with increased mortality/morbidity and demonstrates satisfactory short term results suggesting the usefulnessof this conduit as an arterial graft.
Arteries ; Cardiac Output, Low ; Cerebral Infarction ; Coronary Artery Bypass* ; Coronary Vessels* ; Gastroepiploic Artery* ; Hemorrhage ; Humans ; Mediastinitis ; Mortality ; Myocardial Infarction ; Reoperation ; Transplants

Tympanic membrane perforations are frequently encountered diseases and can be treated with several different methods, induding tympanoplasty with tympanic membrane grafting which requires more profound techniques and clinical care. A fat graft myringoplasty is a cost-effective techniquc managing small tympanic membrane perforations and is used when challenged by a myringoplasty failure, or following tympanostomy tube extrusion. It involves wedging a piece of fat from the ear lobule into the perforation with local anesthesia in a one-day-stay procedure. This study demonstrates the efficacy of this method in a persistent small tympanic membrane perforation.
Anesthesia, Local ; Ear ; Middle Ear Ventilation ; Myringoplasty* ; Transplants* ; Tympanic Membrane ; Tympanic Membrane Perforation ; Tympanoplasty

Thoracoscopic sympathectomy is a common technique used to treat plamar hyperhiodrosis. The complications of thoracoscopic sympathectomy are rare. Recently, we experienced a complex regional pain syndrome(CRPS) after thoracoscopic sympathecotomy in a patient with hyperhidrosis. The treatment of this complication was chemical epidural sympathetic block and conservative pain control. The result of this treatment was good. The patient was recovered after one month.
Humans ; Hyperhidrosis* ; Pain, Postoperative ; Sympathectomy* ; Thoracoscopy

No abstract available.
Female ; Humans ; Ovary*

Leber's Hereditary Optic Neuropathy(LHON) is a maternally inherited disorders that occurs primarily in young males and is characterized by subacute, sequential, bilateral central visual loss, ultimately, optic atrophy. We report 2 cases of molecularly confirmed LHON which reveal 11778 and 14484 mitochondral DNA mutation, respectively but there is no family history of visual loss. So the diagnosis of LHON deserves to be considered in all crypotogenic cases of acute or subacute optic or chiasmal neuropathy. Late or early age at onset, female gender, and a negative family history should not be dissuasive.
Diagnosis ; DNA ; Female ; Humans ; Male ; Molecular Biology* ; Optic Atrophy ; Optic Nerve Diseases*

Mycophenolate mofetil(MMF) is a new immunosuppressant which non-competitively and reversibly blocks the de novo synthesis of guanine nucleotides required for DNA and RNA synthesis during lymphocyte proliferation. So MMF selectively inhibits lymphocyte proliferation and it has less side-effects than other immunosuppressants. We described 2 cases of generalized pustular psoriasis improved with MMF. We administered MMF combined with cyclosporin in case 1 and acitretin in case 2. Both patients markedly improved with MMF administration 3 days after and had no severe side-effects. Thus the authors thought that MMF appears to be an effective therapeutic alternative in the treatment of generalized pustular psoriasis.
Acitretin ; Cyclosporine ; DNA ; Guanine Nucleotides ; Humans ; Immunosuppressive Agents ; Lymphocytes ; Psoriasis* ; RNA

No abstract available.
Heart Transplantation*

BACKGROUND: Osteoporosis, one of the most common metabolic bone disease, might be influenced by the severity of Parkinson's disease (PD). Objectives : We investigated the relationship between the Bone Mineral Density (BMD) and the severity of PD in postmenopausal and senile women. METHODS: We measured BMD of lumbar spine (L1-L4) by Dual energy X-ray absorptiometry (DEXA; Hologic QDR-4500A). We compared BMD between patient group (30 patients with PD) and control (183 postmenopausal and senile health women). The patients were divided into two groups according to osteoporosis and analyzed the following potential factors influencing BMD in PD; age, duration of symptom, age of onset, Hoehn and Yahr stage (H-Y stage), UPDRS motor score, duration of treatment, body mass index (BMI), dominant symptom such as tremor or rigidity. RESULTS: 1. BMD was significantly decreased with aging (p <0.01) in control group, but BMD tend to decreased with aging in PD (p=0.08). 2. BMD of patient group was significantly lower than that of control group (p<0.001). 3. BMD of osteoporosis group was significantly related to BMI (p<0.05) and conversely related to H-Y staging(p <0.05), UPDRS motor score (p <0.01). 4. However, BMD of osteoporosis group were not related to age, duration of symptom, age of onset, dominant symptom and duration of treatment (p>0.05). CONCLUSION: Osteoporosis is related to H-Y stage, UPDRS motor score and BMI as well as aging in PD.
Absorptiometry, Photon ; Age of Onset ; Aging ; Body Mass Index ; Bone Density* ; Bone Diseases, Metabolic ; Female ; Humans ; Osteoporosis ; Parkinson Disease* ; Spine ; Tremor

BACKGROUND: For the treatment of primary varicose vein of greater saphenous vein(GSV) due to reflux from incompetent saphenofemoral junction(SFJ), several methods such as ligation and stripping, or duplex-guided sclerotherapy have traditionally been introduced. As for sclerotherapy, the recurrence rate has been reported to be very high. In spite of higher success rate, traditional high ligation with stripping of GSV requires several days' admission with general anesthesia and several more days before returning to normal daily life. New modalities, which are less invasive and possibly have lower recurrence to rate with a chance for early ambulation, are being proposed. They are such as endovenous radiofrequency(RF) therapy, endovenous laser therapy, etc. OBJECTIVE: We tried to evaluate the efficacy and safety of endovenous RF therapy as an alternative to high ligation and stripping in case of GSV varicosity in mild to moderate degrees. METHODS: Both GSV varicosities and varicosed tributaries are marked by doppler and/or ultrasonic imaging. A catheter connected to RF generator is inserted proximally through the incision of vein wall percutaneously, usually above or below the knee. After confirming position of the SFJ using ultrasound, the catheter is progressed as far as to 1-2cm below the SFJ. Then, the generator is activated and the catheter withdrawn slowly for complete heating and occlusion of the target vein. Additional ambulatory phlebectomy or sclerotherapy is optional. We wrap the limbs with self-adhesive short stretch bandage for 2-3 days and encourage the patient to walk regularly and wear compressive stocking later. RESULTS: We performed endoluminal RF treatment on 10 legs of 9 patients(5 males and 4 females). Follow-up intervals varied from 2 to 10 months. Patients were generally satisfied with improvement of symptom. On the clinical evaluation with doppler and duplex examination, there were a few blood flows except only some segments of GSV in 7 patients CONCLUSION: Although endoluminal RF therapy may have a somewhat higher recurrence rate than that of traditional high ligation with stripping of GSV, endoluminal RF therapy is expected to have several advantages over traditional surgery, including less invasiveness, no need for admission or general / spinal anesthesia, earlier return to normal daily life, and finally easy performance, especially for the dermatologist.
Anesthesia, General ; Anesthesia, Spinal ; Bandages ; Catheters ; Early Ambulation ; Extremities ; Follow-Up Studies ; Heating* ; Hot Temperature* ; Humans ; Knee ; Laser Therapy ; Leg ; Ligation ; Male ; Recurrence ; Saphenous Vein* ; Sclerotherapy ; Ultrasonography ; Varicose Veins* ; Veins