The clinical studies were performen on 304 patients with heart disease who had been received corrective heart surgery at Kosin Medical Center from July, 1984 to December, 1991. The results were as follows: 1) Out of 304 patients, 162 cases (53.3%) were male and 142 cases (46.7%) were female and sex ratio was 1.15:1. 35 cases (11.5%) had clinical cyanosis and 269 cases (88.5%) had no evidence of cyanosis. 2) As age distribution of patients, under 2 years, 3~5 years, 6~10 years, 11~15 years, 16~18 years consist of 22.0%, 26.0%, 29.0%, 16.1% and 6.9%, respectively. 3) As disease distribution, out 304 patients, ventricular septal defect (57.9% of all) was the most common disease, and then atrial septal defect (13.2%), tetralogy of Fallot (11.2%), patent ductus arteriosus (9.9%) and pulmonic stenosis (5.3%), in their order. 4) As sex distribution of each disease, ventricular septal defect, atrial septal defect and tetralogy of Fallot were more common in male and patent ductus arteriosus and pulmonic stenosis were more common in female. 5) The most frequent cardiac anomaly associated with ventricular septal defect and atrial septal defect was pulmonic stenosis. In the case of subarterial ventricular septal defect, aortic insufficiency was associated in 13.3%. 6) Respiratory problems (11.3%), tricuspid regurgitation (9.5%), arrhythmia (6.2%) and congestive heart failure (5.8%) were the major complications after surgery. 7) Case fatality reat was 4.4%. Mortality rate in ventricular septal defect, tetralogy of Fallot and tricuspid atresia were 1.7%, 20.6% and 100%, respectively, Majority (75.0%) of expired patients were died within 24 hours after sugery and the cause of death was hypoxia due to low cardiac output syndrome.
Age Distribution ; Anoxia ; Arrhythmias, Cardiac ; Cardiac Output, Low ; Cause of Death ; Cyanosis ; Ductus Arteriosus, Patent ; Female ; Heart Diseases* ; Heart Failure ; Heart Septal Defects, Atrial ; Heart Septal Defects, Ventricular ; Heart* ; Humans ; Male ; Mortality ; Pulmonary Valve Stenosis ; Sex Distribution ; Sex Ratio ; Tetralogy of Fallot ; Thoracic Surgery ; Tricuspid Atresia ; Tricuspid Valve Insufficiency

Dyskeratosis congenita is a rare genodermatosis of ectodermal dysplasia, which is characterized by the diagnostic triad consisting of reticulated hyperpigmentation, dystrophic nails, and leukoplakia. There is a predisposition to malignancy, particularly at sites of leukoplakia. Bone marrow failure can occur in about a half of the cases. A 16-year-old boy was presented with asymptomatic reticulated pigmentation of the neck and nail dystrophy. The patient also had leukoplakia on the tongue, nasolacrimal duct obstruction and cataract. The histopathological findings taken from the reticulated lesion were consistent with poikiloderma atrophicans vasculare. These clinical and histopathological findings were typical features of dyskeratosis congenita.
Adolescent ; Bone Marrow ; Cataract ; Dyskeratosis Congenita* ; Ectodermal Dysplasia ; Humans ; Hyperpigmentation ; Leukoplakia ; Male ; Nasolacrimal Duct ; Neck ; Pigmentation ; Tongue

PURPOSE: This study was designed to determine the outcome and safety of intravenous gamma-globulin(IVGG) retreatment in Kawasaki disease. METHODS: A clinical observation of the therapeutic effects, laboratory findings and echocardiograms was carried out on 72 patients with Kawasaki disease in Kosin University Hospital from 1991 to 1999. 27 patients were treated with 1g/kg/day IVGG for 2 days, 45 patients were treated with 2 g/kg for 10hours. The clinical indication for retreatment was fever. Persistent fever was defined as a temperature> or =38.3degrees C persisting beyond 48hrs after the completion of the infusion. Recrudescent fever was defined as a temperature> or =38.3degrees C for 48hrs after the completion of the infusion, followed by a temperature< or =38.3degrees C. Treatment failure was defined as the development of new coronary artery abnormalities after IVCG treatment in a child with normal baseline echocardiogram. RESULTS: The 5 patients(6.94%) were retreated with IVC.G. Two were retreated due to persistent fever and three due to recrudescent fever. Among retreated patients, one was retreated with 1 g/kg and the others with 2g/kg. Nobody had serious complications, and there was no significantly different clinical charicteristics except for CRP between one course IVGG infusion and retreatment group(P=0.003). Treatment failure was not found in either gr<>up. CONCLUSION: We concluded that the IVGG retreatment of Kwasaki disease may improve the clinical course and coronary artery outcome.
Child ; Coronary Vessels ; Fever ; gamma-Globulins* ; Humans ; Mucocutaneous Lymph Node Syndrome* ; Retreatment* ; Treatment Failure

No abstract available.
Cardiomyopathy, Dilated* ; Doxorubicin

PURPOSE: Crohn's disease is a chronic inflammatory disease that despite the advances in medical therapy is difficult to treat requiring surgery in a large proportion of patients as a result of complications. The aim of this study was to assess the clinical features of Crohn's disease patients who underwent one or more surgical interventions during a ten-year period and to establish the indications and surgical procedures in this group of patients. METHODS: The medical records of 22 patients with Crohn's disease, who were treated by surgery at the Ewha Womans University Hospital between October 1993 and February 2004, were reviewed. RESULTS: The male to female ratio was 4.5: 1 and the mean age at surgery was 29.7+/-11.5 years. There were an increasing number of patients who were being treated with surgery particularly male patients. The most common symptom was abdominal pain. The main indications for abdominal surgery were an abdominal obstruction and bowel perforation. Fourteen patients were diagnosed with Crohn's disease after surgery by a pathologic examination. Five patients had a history of taking anti-tuberculous medication. The re- operation rate was 9.1%, and there were no deaths. CONCLUSION: The number of surgical interventions for Crohn's disease patients appears to be increasing due to the growth of cases diagnosed and the change in the alert for Crohn's disease. Therefore, a surgeon must consider the possibility of Crohn's disease when a young male patient presents non-specific symptoms of acute abdominal pain.
Abdominal Pain ; Crohn Disease* ; Female ; Humans ; Male ; Medical Records

OBJETIVE: To elucidate 1) whether there are any differences in the urine concentrations of steroid hormone metabolites between patients with leiomyoma and normal controls 2) the correlation between urinary profiles of steroid hormones and leiomyomas of the uterus according to their type, location, volume, and weight. MATERIALS OF METHODS: The study population consisted of 37 premenopausal patients with uterine leiomyoma and the control group consisted of 25 premenopausal normal volunteer women without uterine leiomyoma. Confirmation of the existence of uterine leiomyoma was done by ultrasonography and histopathological examination after surgery. The volume of the leiomyoma was estimated by trans-abdominal and/or trans-vaginal ultrasonography. The Leiomyomas were divided into 3 types (subserosal, intramural and submucosal). Seventeen patients had subserosal type of leiomyoma, 10 with the intramural type and 10 with the submucosal type. The locations of the leiomyoma were also divided into 3 groups (fundus, body and isthmus). Seventeen patients showed a fundus location, 10 in body, and 10 in isthmus. We compared urinary profiles of the endogenous steroids between patients with leiomyomas and normal controls, and also investigated the relationship between urinary profiles of the endogenous steroids and leiomyomas according to their type, location, volume and weight by using highly sensitive Gas Chromatography-Mass Spectrometry (GC-MS) system. RESULTS: The mean ages of the patients with leiomyomas and the control group were 43.1+/-5.6 and 40.6+/-7.2 years, the weights were 63.4+/-7.3 and 59.4+/-8.1 kg, and their heights were 155.4+/-4.8 and 159.3+/-4.8 cm respectively. Seventeen patients had subserosal, 10 had intramural, and 10 had submucosal leiomyomas. There were 17 patients with leiomyoma located in fundus, 10 in body and 10 in isthmus. 17beta-estradiol, 5-AT, 11-keto ET, 11beta-hydroxy An, 11beta-hydroxy Et, THS, THA, THE, alpha-cortolone, alpha-cortol, beta-cortol, 11beta-OH Et/11beta-OH An and E2/E1 were significantly increased in patients with leiomyoma than in the control group. 17beta-estradiol was significantly increased in the intramural and the submucosal types than in the subserosal type. There was no significant difference in the concentrations of urinary steroids according to the locations of leiomyomas. There was no significant relationship between the concentration of urinary steroids and the volume of the leiomyomas. 17beta-estradiol significantly decreased as the weight of uterus increased (r=-0.322, p=0.04). CONCLUSION: The concentrations of steroid hormone metabolites were generally increased in patients with leiomyoma but were not significantly related to the volume and weight of the leiomyomas. Our study suggests that steroid hormones may be involved in the initiation of leiomyomas but may not be involved in their progression. In addition, the concentrations of steroid hormone metabolites are not related to the leiomyoma type and location.
Female ; Gas Chromatography-Mass Spectrometry ; Healthy Volunteers ; Humans ; Leiomyoma* ; Steroids ; Ultrasonography ; Uterus* ; Weights and Measures

PURPOSE: To report a case of monocular elevation deficiency as the presenting manifestation of systemic lupus erythematosus (SLE). CASE SUMMARY: A 23-year-old, otherwise healthy female presented with a 3-day history of vertical diplopia and headache. She had a left hypotropia, which worsened in adduction and supra-duction and a profound inferior oblique underaction (-3). Magnetic resonance imaging showed an enhancement around the left superior oblique muscle and multiple infarctions in the left midbrain. On repetitive serological tests, anemia, lymphopenia, and anti-phospholipid antibody were positive. A presumptive diagnosis was a myositis of left superior oblique muscle and hyper-coagulation related with anti-phospholipid antibody. Two months after high-dose steroid treatment, the vertical diplopia was resolved. Five months later, the left hypotropia recurred as a more severe form with the inability to elevate the left eye in all directions. In addition, the infarction associated with vasculitis recurred in the left midbrain. As the treatment with high-dose steroid failed to relieve her ocular symptoms, recession of the left inferior rectus was performed 8 months later. One month after the surgery, she developed multiple lesions of erythematous nodosa with tenderness. Skin biopsy of the lesion in the fingers showed the histological findings consistent with lupus. CONCLUSIONS: Eye movement abnormality can be an initial manifestation of SLE, which should be considered as a differential diagnosis especially in young female patients.
Anemia ; Biopsy ; Diagnosis ; Diagnosis, Differential ; Diplopia ; Eye Movements ; Female ; Fingers ; Headache ; Humans ; Infarction ; Lupus Erythematosus, Systemic* ; Lymphopenia ; Magnetic Resonance Imaging ; Mesencephalon ; Myositis ; Serologic Tests ; Skin ; Vasculitis ; Young Adult

Coexistence of cranial and spinal subdural hematomas is rare and only a few cases have been reported in the literature. Herein, we report a case of cranial and spinal subdural hematomas after previous head trauma. As the pathogenesis of simultaneous intracranial and spinal subdural hematoma yet remains unclear, we developed an alternative theory to those proposed in the literature for their coexistence, the migration of blood through the subdural space.
Craniocerebral Trauma ; Hematoma, Subdural* ; Hematoma, Subdural, Spinal* ; Subdural Space

A case of ARC syndrome was found in a newborn male with persistent cholestasis. He showed renal dysfunction, failure to thrive, and ichthyosis as well as arthrogryposis. The patient who had a dead sibling with similar symptoms has not been described previously in our country.
Arthrogryposis ; Cholestasis ; Failure to Thrive ; Humans ; Ichthyosis ; Infant, Newborn ; Jaundice, Obstructive* ; Male ; Siblings*

PURPOSE: To compare ocular biometry and refractive results measured using conventional applanation ultrasonography and 3 different optical interferometries, Lenstar LS900(R), AL-Scan(R) and OA-2000(R). METHODS: The biometries of 31 cataractous eyes were measured using ultrasonography, Lenstar LS900(R), AL-Scan(R) or OA-2000(R). The axial length, anterior chamber depth and keratometry were measured. The SRK/T formula was used to calculate intraocular lens power. Two months after cataract surgery, the refractive outcome was determined and results from the 4 different biometry methods were compared. RESULTS: Axial lengths were 23.39 +/- 0.95 mm, 23.42 +/- 0.98 mm, 23.43 +/- 0.98 mm and 23.44 +/- 0.98 mm measured using ultrasonography, Lenstar LS900(R), AL-Scan(R) and OA-2000(R), respectively with no statistically significant differences observed (p = 0.996). The anterior chamber depth and keratometry were 3.14 +/- 0.41 mm, 3.10 +/- 0.38 mm and 3.13 +/- 0.39 mm (p = 0.936) and 44.41 +/- 1.52 D, 44.54 +/- 1.57 D and 44.44 +/- 1.52 D (p = 0.937) for Lenstar LS900(R), AL-Scan(R) and OA-2000(R) respectively. There were no statistically significant differences between the 3 optical devices. The mean absolute error of the 4 different devices were not statistically significant (p = 0.722). CONCLUSIONS: The ocular biometric measurements and prediction of postoperative refraction using ultrasonography, Lenstar LS900(R), AL-Scan(R) or OA-2000(R) showed no significant differences.
Anterior Chamber ; Biometry ; Cataract ; Interferometry* ; Lenses, Intraocular ; Optical Devices ; Refractive Errors ; Ultrasonography*