We performed this study to evaluate the therapeutic responses of bone marrow transplantation and immunosuppressive therapy for aplastic anemia in 103 cases (68 severe and 35 moderate). Among them, 15 cases with severe aplastic anemia having HLA-identical sibling donor underwent bone marrow transplantation, and cases(53 severe and 35 moderate cases)without available HLA-identical sibling donor had received immunosuppressive therapy using antilymphocye globulin and cyclosporin-A. The results were as follows; 1) For cases given bone marrow transplantation, successful engraftment was accomplished in all cases, but 2 cases showed rejection and 1 case died of thrombotic thrombocytopenic purpura (TTP), resulting 80% disease-free survival rate. acute graft-versus-host disease above grade II was noted in 2 cases and chronic graft-versus host disease was seen in other 2 cases. The most common pathogenic organism in post-transplantation infection was Herpes zoster (6 cases), and one each cases of Herpes simplex, candidiasis, measles and hemorrhagic cystitis developed. 2) Among 88 cases of receiving immunsouppressive therapy, complete response was seen in 26 cases (29.5%) and partial respones in 37 cases (42.0%), resulting 71.6% of overall response rate and recurrence rate was 19.5% (12 cases). In 53 severe cases, complete response was seen in 9 cases (17.0%) and partial response in 25 cases (47.2%), resulting 64.2% of overall response rate. In 35 moderate cases, complete response was seen in 17 cases (48.6%) and partial response in 12 cases(34.3%), overall response rate was 82.9%. All initial responses were seen within 6 months post-treatment period, and the time interval to initial response was 8 to 162 days (median; 53 days). There was no sex difference concerning response rate, however, partial response was predominant in male and more complete response in female. The complications of immunosuppressive therapy were thrombocytopenia (93.2%), fever (67.7%), hypertension (50.0%), skin eruption (45.8%), gingivitis (17.0%). In conclusion, our date suggest that bone marrow transplantation is preferred initial treatment modality to immunosuppressive therapy in severe aplastic anemia patient with HLA-identical sibling donor, and immunosuppression is a satisfactory alternative therapy for children with severe aplastic anemia in the absence of HLA-identical sibling and for whom with moderate aplastic anemia.
Anemia, Aplastic* ; Bone Marrow Transplantation ; Candidiasis ; Child ; Cystitis ; Disease-Free Survival ; Female ; Fever ; Gingivitis ; Graft vs Host Disease ; Herpes Simplex ; Herpes Zoster ; Humans ; Hypertension ; Immunosuppression ; Male ; Measles ; Purpura, Thrombotic Thrombocytopenic ; Recurrence ; Sex Characteristics ; Siblings ; Skin ; Thrombocytopenia ; Tissue Donors

No abstract available.
Respiratory Syncytial Virus Infections* ; Respiratory Syncytial Viruses*

No abstract available.
Leg* ; Myocutaneous Flap* ; Superficial Back Muscles*

The "flosting knee" is the term applied to the flail knee joint segment resulting from a fracture of the shaft of adiacent metanhysis of the ipsilateralfemur and tibia. The various methods of treatment of the "floating knee" were adapted by many authors according to degree of the comminution, degree of the soft tissue injury, general condition state of the fracture. Authors experienced the floating knee in 52 cases on 51 patients who were treated at Soon Chun Hyang University Hospital in the period of 4 years and 5 months from January 1985 to June 1989 and among them, 45 cases were treated by operative method and 7 cases by conservative methods at least one of the femur and tibia, The results were as follews;1. Floating Knee occured six times more in male and the peak age was third and fourth decades. 2. The most common cause of fractures was traffie aecident and there was difficulty in diagnosis of ligamentous disruption of the ipsilateral knee. 3. The common fracture site were middle one third and the most common fracture shape was comminuted in both femur and tibia. 4. A good or excellent functional result was aohieved in treated with open reduction and rigid fixation of both fracture and there were no significant difference in group of the internal fixation of both femur and tibia. 5. Intramedullary nailing on the femur and plate fixation on the tibia provided rigid fixation of fracture and the it made possible early joint motion exercise and ambulation and the average healing time of fracuture was much shortened. 6. The most common concomitant injury were soft tissue injury and delayed or nonuion and osteomyelitis were developed more frequently in the plate and screw fixation group of the open fracture.
Clinical Study ; Diagnosis ; Femur ; Fracture Fixation, Intramedullary ; Fractures, Open ; Humans ; Joints ; Knee ; Knee Joint ; Ligaments ; Male ; Methods ; Osteomyelitis ; Soft Tissue Injuries ; Tibia ; Walking

Four newborn infants with hyperbilirubinemia, caused by ABO blood group incompatibility, were treated with high-dose intravenous immune globulin(IVIG). As soon as the diagnosis was clinically suspected, these infants received conventional treatment including phototherapy and were monitored closely for bilirubin levels. When bilirubin concentrations reached the risk point in spite of phototherapy, IVIG was given at a dose of 1g/kg for 6 hours. In all cases, bilirubin levels declined within 12 hours after LVIG therapy, and to rebound effect was seen, No side effects of IVIG treatment were observed. We suggest that high-dose IVIG therapy may be useful in the treatment of hyperbilirubinemia due to ABO incompatibility, and reduce the need for exchange transfusion.
Bilirubin ; Blood Group Incompatibility ; Diagnosis ; Humans ; Hyperbilirubinemia* ; Hyperbilirubinemia, Neonatal ; Immunoglobulins, Intravenous* ; Infant ; Infant, Newborn ; Phototherapy

Between 1986 and 1990, four children with recurrent CNS leukemia who had previous CNS prophylaxis therapy were treated with intermittent central nervous system irradiation and intrathecal chemotherapy (IIIC). There was no isolated CNS recurrence. One patient died form bone marrow relapse. Three patients are alive without evidence of disease for 3E3/12 year to 3E6/12 year after the diagnosis of recurrence of CNS leukemia. This experience suggests that IIIC may be an effective treatment for preventing the recurrence of CNS leukemia without any serious side effects.
Bone Marrow ; Central Nervous System* ; Child* ; Diagnosis ; Drug Therapy* ; Humans ; Leukemia* ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Recurrence

Schizencephaly, first described by Yakovlev and Wadsworth in 1946, is a rare congenital anomaly characterized by gray matter-lined clefts that extend through the entire cerebral hemisphere, from the ependymal lining of the lateral ventricle to the subarachnoid space. Clinically, patients with schizencephaly present motor dysfunction such as hemiparesis, seizures, and variable developmental delay. Absence of septum pellucidum, gray matter heterotopia, polymicrogyria, and hypoplasia of optic nerves were also frequently found to be associated with schizencephaly. We experienced a case of open-lip schizencephaly in a one-day-old male infant with wide, tense fontanel, which was confirmed by MRI.
Cerebrum ; Humans ; Infant ; Lateral Ventricles ; Lip* ; Magnetic Resonance Imaging ; Male ; Malformations of Cortical Development* ; Optic Nerve ; Paresis ; Seizures ; Septum Pellucidum ; Subarachnoid Space ; Transcutaneous Electric Nerve Stimulation

PURPOSE: The results of the bronchial provocation test were different by the method of inhalation, but there was no data in children. We performed the provocation test by 2 different methods such as two-minute tidal breathing versus five vital capacity breathing and compared the datas. METHODS: Methacholine inhalation test was performed for the In 17 patients with suspected bronchial hyperresponsiveness in Pocheon university Bundang Cha hospital, under identical conditions, except for the method of inhalation. All the patients were performed two-minute tidal breathing (2 min-TB) in one test and five vital capacity breathing (5VCB) with interval 1 to 7 days. Baseline and postsaline FEV1 were also measured to compare effects by two inhalation methods. Methacholine PC20 was calculated by linear interpolation between the last two data parts on the dose-response curve, and the cut off value of bronchial asthma was 8 mg/mL. RESULTS: The rate of positive brochial provaction test in 5VCB was 88.24% which was not significantly lower than 70.59% in 2 min-TB. In 12 patients with positive bronchial provocation test in both inhalation methods, the mean PC20 in 5VCB (5VC-PC20) was 4.40 mg/mL, being significantly higher than 1.02 mg/mL in 2 min-TB (2 min-PC20) (P< 0.05). There was a significant positive correlation between 5VC-PC20 and 2 min-PC20 (R=0.76, P< 0.001). CONCLUSION: We conclude from this study that the results of bronchial provocation test in children are different according to methods of inhalation in using the same nebulizer. Therefore, when we analyze the result of bronchial provocation test, we need attention to factors which are nebulizer output and method of inhalation to influence the response to the test.
Asthma ; Bronchial Provocation Tests* ; Child* ; Humans ; Inhalation ; Methacholine Chloride ; Nebulizers and Vaporizers ; Respiration* ; Vital Capacity*

Transesophageal echocardiography(TEE) is a new acoustic window to the cardiac structures and great vessels via retrocardiac esophagus. Because of the close relation between the esophagus and the heart, higher transducer frequencies can be applied, therefore leading to improved resolution and more accurate images than transthoracic echocardiography. We describe our experience with the first 353 awake patients. The procedure was well tolerated by the patients and associated with no major complications, within examination 10 to 20 minutes. Clinical diseases which appear to be suited for TEE includes 1) evaluation of native valve diseases, particulary mitral valve, pathologic valvular abnormalities and color Doppler regurgitant flows ; 2) assesment of prosthetic heart valves to better define malfunction and important pathologic associations ; 3) congenital heart disease, particularly atrial septal defect ; 4) endocarditis and detection of vegetations and complications ; 5) detection of thrombus and tumors, size, location, morphology, stalk ; and 6) aortic pathology including atherosclerosis, aneurysm and dissection. We conclude that transesophageal echocardiography is a new imaging technique that is rapidly evolving into a major tool for general cardiac imaging in a variety pathologic conditions and is a safe and useful tool in patients. Future advances in probe technology will continue to expand its applications.
Acoustics ; Aneurysm ; Atherosclerosis ; Echocardiography ; Echocardiography, Transesophageal* ; Endocarditis ; Equidae ; Esophagus ; Heart ; Heart Defects, Congenital ; Heart Septal Defects, Atrial ; Heart Valves ; Humans ; Mitral Valve ; Pathology ; Thrombosis ; Transducers

To evaluate the implication of vascular factor in normal tension glaucoma, authors performed color doppler imaging[CDI] for ophthalmic artery, central retinal artery and posterior ciliary artery in 21 eyes of 12 patients in normal tension glaucoma and 16 eyes of 11 patients in normal control group. We compared peak-systolic velocity[PSV], end-diastolic velocity[EDV], mean velocity[MV], resistance index[RI] and pulsatile index[PI] between the two groups. We evaluate the difference in parameters according to age, sex, systolic and diastolic blood pressure, past history of diabetes and hypertension, cup-disc ratio and visual field change. In normal tension glaucoma group, PSV, EDV and MV of ophthalmic artery and central retinal artery were significantly decreased and pulsatile index was significantly increased compared to those of normal group. Resistance index was also increased in normal tension glaucoma group, but statistically not significant. All parameters of posterior ciliary artery had no significant difference between the two groups. Age was related positively to pulsatile index and resistance index of central retianl artery and diastolic blood pressure was related positively to resistance index of ophthalmic artery. Cup-disc ratio was related negatively to PSV and EDV of ophthalmic artery. Above results imply that blood flow velocity decreased by increasing resistance factor in normal tension glaucoma, therefore local vascular factor might be related to the development of the normal tension glaucoma and affected by age, blood pressure and cup-disc ratio change.
Arteries ; Blood Flow Velocity ; Blood Pressure ; Ciliary Arteries ; Humans ; Hypertension ; Low Tension Glaucoma* ; Ophthalmic Artery ; Retinal Artery ; Risk Factors* ; Visual Fields