No abstract available.
Child* ; Endoderm* ; Endodermal Sinus Tumor* ; Humans

No abstract available.
Child* ; Drug Therapy ; Humans ; Radiotherapy

No abstract available.
Erythroblastosis, Fetal* ; Infant, Newborn

No abstract available.
Bone Marrow* ; Histiocytes* ; Niemann-Pick Diseases*

BACKGROUND: The testes are one of the most common extramedullary sites of relapse in boys with acute lymphoblastic leukemia(ALL). The reported incidence of isolated testicular relapse varies from 3 to 40%. If these patients are treated exclusively with testicular irradialion, a systemic relapse occurs within a few months. Recently, the use of intensive chemotherapy and testicular irradiation improved the survival rate for boys with testicular leukemia. So, we performed this study to identify clinical manifestations, disease free survival and prognostic factors of testicular leukemia in children. METHODS: We reviewed 33 patients of testicular leukemia among total 410 boys with ALL diagnosed at the Department of Pediatrics, Seoul National University Children's Hospital from Jan. 1970 to Aug. 1996. Testicular leukemia was confirmed by testicular biopsy in all 33 patients. These patients were treated with combined local testicular irradiation(2,400~2,500 cGy/8~12fractions) and systemic chemotherapy. Two patients, in whom testicular relapse was diagnosed before 1979, unilateral orchiectomy of the involved site and testicular irradiation of the opposite site were performed. Probability estimates of disease free survival (DFS) were calculated by the method of Kaplan and Meier, and the relationship of prognostic factors to DFS was compared using the chi-square test in survival analysis. RESULTS: In 410 boys with ALL, testicular leukemia occurred in 33 patients(8%). Of 33 patients, 6 patients presented with testicular involvement at initial diagnosis, 16 patients had testicular relapse while still receiving chemotherapy and 11 patients had testicular relapse 3 to 57 months(median : 15 months) after cessation of chemotherapy. The median age of 33 patients was 7.4 yrs(9 months~18 yrs) and median WBC count 7,600/ L(2,700~270,000/L). All patients presented with painless testicular enlargement and testicular leukemia was confirmed by testicular biopsy. Among 33 patients, 2 had prior CNS relapse and 11 had concomitant bone marrow and/or CNS relapse. Twenty nine patients were treated with combined local testicular irradiation and systemic chemotherapy. Eleven had second relapse(6 bone marrow, 3 CNS, 2 opposite testis). Seventeen have been followed until now: 6 patients on chemotherapy and 11 patients(37.9%) in complete remission for 48.5+/-22.3 months(19~86 months). The 3 year DFS for 29 patients was 55.3%+/-10.1%. The following prognostic factors showed no significant association with DFS in testicular relapse : age and WBC count at initial diagnosis, age at testicular relapse, and concomitant relapse. Whether testicular relapse occurred on initial therapy or off initial therapy has prognostic value in predicting DFS. The 3 year DFS for boys with testicular relapse on and off initial therapy were 40.0%+/-12.9% and 78.8%+/-13.4%, respectively(P: 0.046). CONCLUSION: With the use of chemotherapy and testicular irradiation, prolonged second re mission can be achieved in many patients with testicular leukemia. The patients with testicular relapse off initial therapy fared significantly better than patients on therapy. So, to improve the DFS for boys with testicular leukemia, a better understanding of its biology and prognostic factors is needed.
Biology ; Biopsy ; Bone Marrow ; Child ; Diagnosis ; Disease-Free Survival ; Drug Therapy ; Humans ; Incidence ; Leukemia ; Missions and Missionaries ; Orchiectomy ; Pediatrics ; Precursor Cell Lymphoblastic Leukemia-Lymphoma* ; Recurrence ; Seoul ; Survival Rate ; Testis

BACKGROUND: Hereditary spherocytosis(HS) is a clinically and biochemically very heterogeneous disorder The purpose of this study is to detect erythrocyte membrane protein abnormalities by SDS-PAGE and to investigate the frequency of erythrocyte membrane protein defects in hereditary spherocytosis and correlation between some of the hereditary spherocytosis biochemical subsets and the selected clinical phenotype. METHODS: We evaluated the clinical and laboratory characteristics of 14 normal healthy persons and 23 hereditary spherocytosis patients and 8 their family members. The patients were divided into three groups based on clinical and hematological severity(mild, typical, severe). In addition to routine hematologic determlnatlons, osmotic fragility and autohemolysis, RBC membrane protein analysis were performed in all patients by densitometric tracing of SDS-PAGE(sodium dodecyl sulphate polyacrylamide gel electrophoresis) stained by Coomassle blue utilizing both the discontinuous buffer system of Laemmli with acrylamide linear gradient from 4% to 12% and the continuous buffer system of Fairbank with exponential gradient of acrylamide from 3.5% to 17%. RESULTS: 1) The patients could be seperated into three classes of different clinical severity as mild(3 cases), moderate(16 cases) and severe(4 cases) on the clinical feature. 2) Eighteen patients(82.6%) among 23 hereditary spherocytosis revealed abnormal erythrocyte membrane protein and we detected six patients(26.1%) with spectrin deficiency combined with ankyrin reduction, 4 patients(17.4%) with ankyrin deficiency, 4 patients(17.4%) with isolated spectrin deficiency and 3 patients(13.0%) with band 3 deficiency. Five HS patients(21.7%) showed normal RBC membrane protein. 3) Eight HS and their family members showed same RBC membrane protein deficiency. 4) The type and degree of RBC membrane protein reduction were variale with spectrin at 66~94%, with ankyrin at 48~82% of normal levels. These showed that each patient had different clinical severities according to different RBC membrane protein levels and type. CONCLUSION: RBC membrane protein abnormalities were observed in 82.6% of HS patients. The combined spectrin and ankyrin deficiency is the most common molecular defect in HS. The clinical severity and biochemical expression is heterogeneous. SDS-PAGE analysis of RBC membrane protein was provided the diagnosis of RBC membrane defects and basic molecular studies. We believed that the early identification of the biochemical defect responsible for HS is important because it is helpful starling point for the identification of the primary molecular defect, and it could help to anticipate the clinical outcome of the disease. For these reasons, we consider the SDS-PAGE of the red cell membrane to be of crucial importance for a complete evaluation of children with HS. Further studies with more cases would be to clarify the correlation between clinical and biochemical phenotypes.
Acrylamide ; Ankyrins ; Cell Membrane ; Child ; Diagnosis ; Electrophoresis, Polyacrylamide Gel* ; Erythrocyte Membrane* ; Erythrocytes* ; Erythrocytes, Abnormal ; Humans ; Membrane Proteins ; Membranes ; Osmotic Fragility ; Phenotype ; Population Characteristics* ; Spectrin ; Starlings

No abstract available.
alpha-Fetoproteins* ; Mass Screening*

BACKGROUND: An intrathecal injection of a small-dose local anesthetic and an opioid using combined spinal-epidural anesthesia (CSEA) technique is a new trend in regional anesthesia for cesarean section. Prophylactic epidural injection may be useful to complete the new regimen. METHODS: Spinal anesthesia via the CSEA technique was performed with 6 mg 0.5% hyperbaric bupivacaine plus 20ng fentanyl in 75 parturients undergoing cesarean section. group S (study, n = 38) received an epidural injection of 10 ml of 0.25% bupivacaine 5 min after intrathecal injection, and was compared with group C (control, n = 37) in sensory levels and Bromage scores, incidences of side effects such as hypotentsion, pain and discomfort, nausea and vomiting, dizziness, pruritus and shivering. Recovery times from sensory and motor block and the duration of postoperative analgesia were assessed between the groups. RESULTS: Sensory block levels were higher (but remained Analgesia ; Anesthesia* ; Anesthesia, Conduction ; Anesthesia, Spinal ; Bupivacaine* ; Cesarean Section* ; Dizziness ; Female ; Fentanyl ; Humans ; Incidence ; Injections, Epidural ; Injections, Spinal ; Nausea ; Pregnancy ; Pruritus ; Shivering ; Vomiting

PURPOSE: The purpose of this study was to analyze the CT findings of hepatoblastoma before and afterchemotherapy, and to compare them with surgical and pathologic features. MATERIALS AND METHODS: Twelvehepatoblastoma patients underwent chemotherapy prior to surgery; in all cases, CT scanning was performed beforeand after chemotherapy. We reviewed the findings with special attention to changes in tumor volume, the extent andpattern of contrast enhancement, the extent of low-attenuation area in the tumor, the presence of a septum, andcalcification or ossification within the mass before and after chemotherapy. Post-chemotherapy CT findings werecompared with operative and pathologic findings. RESULTS: After chemotherapy, the volume of the tumor massdecreased in all patients, and the extent of involved segments decreased in nine(75%), the non-enhancing areawithin the mass, on the other hand, increased in nine (75%). On pre-chemotherapy CT, calcifications were detectedin seven patients(58%), and on post-chemotherapy CT, in nine (75%); the extent of calcification increased in sevenpatients. On the basis of CT findings, viable tumor and necrosis areas could not be distinguished. Massivecalcification or an osteoid mixed with loose connective tissue was noted in the mesenchymal component of thetumor; the whirling pattern of enhancement within the area of low density asen on CT scanning corresponded toosteoid mixed with loose connective tissue, which contained rich blood vessels. CONCLUSION: We describe the CTfindings of hepatoblastoma both before and after chemotherapy, highlighting the changes which occurred. Anunderstanding of these changes is helpful for the proper management of this condition.
Blood Vessels ; Connective Tissue ; Drug Therapy* ; Hand ; Hepatoblastoma* ; Humans ; Necrosis ; Tomography, X-Ray Computed ; Tumor Burden

A 3 year and 3 months old boy with recurrent infections since his age of 5 months was presented with clinical data and autopsy findings. He was the 4th product of healthy parents. His elder brother died of recurrent perianal abscess and sepsis at his age of 3 years. His 2nd elder sister died on the 14th day of life probably from the complication of BCG vaccination. Beginnig with perianal abscesses at his age of 5 months, he has been continuously suffering from recurrent infections such as arthritis, ostomyelitis, pneumonia, epididymitis, subcutaneous abscesses and perianal abscesses. In spite of meticulous supportive and aggressive antibiotic therapy persistent positive cultures for staph. Aureus, klebsiella, E. Coli, Enterococcus and coliform bacilli from different sited were noted. Erythrocyte sedimentation rate of 25 to 40 were constant. White cell count varied frem 15500 to 33400 with polymorphonucleocytes predominance. NBT test showed persistent low scoring of 2% throught the course. He finally died of pneumonia and empyema. At postmortem examination, multiple abscesses and grnulomas of right lung and multipe granulomas in the liver, spleen, lymph node, bone, marrow, adrenal gland, kidney and intestinal wass were noted. At microscopic examination histiocytic granulomas with lipid containing histiocyte infiltrations were noted in every organs described including brain.
Abscess ; Adrenal Glands ; Arthritis ; Autopsy ; Blood Sedimentation ; Bone Marrow ; Brain ; Cell Count ; Empyema ; Enterobacteriaceae ; Enterococcus ; Epididymitis ; Granuloma ; Granulomatous Disease, Chronic* ; Histiocytes ; Humans ; Infant ; Kidney ; Klebsiella ; Liver ; Lung ; Lymph Nodes ; Male ; Mycobacterium bovis ; Parents ; Pneumonia ; Sepsis ; Siblings ; Spleen ; Vaccination