Myelodysplastic syndrome is a heterogeneous disease complex which is basically a clonal disorder and has characteristics of cytopenia of one or more cell series in peripheral blood and of dyspoiesis of precursors in bone marrow. Since the nature of this disease in childhood is very different from that in adults, retrospective clinical study was performed with 17 confirmed patients who were admitted to the Department of Pediatrics, Seoul National University Children's Hospital from June 1986 till October 1992. The results were as follows: 1) Themost frequent occurrence was found in preschool age group (76.5%), and male predominance was noted. 2) Hepatosplenomegaly was the most frequent clinical finding, and in view of laboratory findings, anemia waas found in all cases, and leukopenia in 5 cases, thrombocytopenia in 13cases were observed. Bone marrow aspiration revealed dyspoiesis of 3 cell series in almost all cases. The percentage of myeloblasts more than 5% of total bone marrow nucleated cells was seen in 8 cases on bone marrow study. 3) Subtypes of the disease were found to be 3 cases of RA, 6 cases of RAEB, 3 cases of RAEB-T, 4 cases of JCML, and 1 case of monosomy 7 syndrome. Chromosomal study was performed in 8 cases, and 6 of them were detected to be abnormal. 4) Supportive management was performed for almost all cases, and combined therapy with prednisolone and one-alpha for 3 cases, combined chemotherapy with various anticancer drugs for 7 cases, and low dose cytarabine therapy (10 mg/m2/12 hr) for 6 cases were performed. 5) There were 3 cases of drop out, 8 cases followed up on not remitted state, 5 cases of death, and 2 cases followed up on complete remission state. Two cases in complete remission were one of RA patients, and one of JCML patients, to whom prednisolone with one-alpha, and combined chemotherapy with A-Triple-V regimen were applied as treatment modalities, respectively. 6) Average duration of follow up for 10 survival cases was 18.2 months and a significant difference of 2 year survival rate was found in between the group composed of RA, RAEB and the other group composed of remained subtypes.
Adult ; Anemia ; Anemia, Refractory, with Excess of Blasts ; Bone Marrow ; Child* ; Cytarabine ; Drug Therapy ; Follow-Up Studies ; Granulocyte Precursor Cells ; Humans ; Leukemia, Myeloid, Acute ; Leukopenia ; Male ; Monosomy ; Myelodysplastic Syndromes* ; Pediatrics ; Prednisolone ; Preleukemia ; Retrospective Studies ; Seoul ; Survival Rate ; Thrombocytopenia

Hepatoblastoma is a rare malignant liver tumor found in children. Its biological characteristics and prognostic factors have not been well known. We investigated 29 cases of hepatoblastoma, registered in university hospitals in Seoul from 1984 to 1996. By the immunohistochemical method, p53, Waf-1 (p21), bcl-2, heat shock protein 70 (hsp70), c-jun, transforming growth factor-alpha (TGF-alpha) expressions were studied. Those data were compared with clinico-pathologic features; age, sex, tumor size, tumor stage and histologic subtypes. Expression of p53 and bcl-2 were each observed separately in single cases. Expression of c-jun was more frequently noted in patients at higher stages. Expression of TGF-alpha decreased in the order of pure fetal, mixed, embryonal and small cell anaplastic subtypes. Cumulative survival rate was lower in females than in males and in patients with a higher tumor stage. According to histologic subtypes, survival rates decreased in the order of pure fetal, mixed, embryonal and small cell anaplastic subtypes. Survival rate was lower in patients with c-jun expression. Group of TGF-alpha labelling index under 19 showed a lower survival rate than that over 19. In conclusion, we found that tumor associated proteins, c-jun and TGF-alpha, are closely related to the prognosis of hepatoblastoma but p53 and bcl-2 may not be related to it.
Child ; Female ; Hepatoblastoma* ; Hospitals, University ; HSP70 Heat-Shock Proteins ; Humans ; Liver ; Male ; Population Characteristics ; Prognosis ; Seoul ; Survival Rate ; Transforming Growth Factor alpha

OBJECTIVE: The purpose of this study was to evaluate differences in three-dimensional (3D) morphology of the hard palate between Korean adults with and without mild-to-moderate obstructive sleep apnea (OSA) using cone-beam computed tomographic (CBCT) data. METHODS: The protocol for the two-dimensional (2D) and 3D mathematical modeling was established by analyzing CBCT images of 30 adults with OSA and 30 matched controls without OSA, using MIMICS software. The linear and angular measurements were also determined using this software. The measurements were repeated for 30 palates, by the same operator, to assess reliability. RESULTS: The palates of OSA patients were higher in the posterior part and narrower in the anterior-superior part than those of the control group (p < 0.05). The nasal cavities of patients with OSA were narrower (p < 0.05) than those of controls. The increasing angle of the first molar palatal root is a compensation of the upper dental arch to improve occlusion. However, for most palatal measurements, there were no significant differences between the OSA and control groups (p > 0.05). The results of 2D and 3D mathematical models were consistent for linear and angular measurements, indicating that 2D and 3D mathematical modeling of the palate is a reliable methodology. CONCLUSIONS: OSA is a multifactorial disease; the palates of adults with mild-to-moderate OSA do not have specific morphological features distinct from those of healthy controls.
Adult* ; Compensation and Redress ; Cone-Beam Computed Tomography ; Dental Arch ; Humans ; Models, Theoretical ; Molar ; Nasal Cavity ; Palate ; Palate, Hard* ; Sleep Apnea, Obstructive*

Clear thermoplastic retainers have been widely used in daily orthodontics; however, they have inherent limitations associated with thermoplastic polymer materials such as dimensional instability, low strength, and poor wear resistance. To solve these problems, we developed a new type of clear orthodontic retainer that incorporates multi-layer hybrid materials. It consists of three layers; an outer polyethylenterephthalate glycol modified (PETG) hard-type polymer, a middle thermoplastic polyurethane (TPU) soft-type polymer, and an inner reinforced resin core. The resin core improves wear resistance and mechanical strength, which prevent unwanted distortion of the bucco-palatal wall of the retainer. The TPU layer absorbs impact and the PETG layer has good formability, optical qualities, fatigue resistance, and dimensional stability, which contributes to increased support from the mandibular dentition, and helps maintain the archform. This new type of vacuum-formed retainer showed improved mechanical strength and rate of water absorption.
Absorption ; Dentition ; Fatigue ; Orthodontic Retainers* ; Orthodontics ; Polymers ; Polyurethanes ; Water

A totally implanted venous access system was inserted in 19 children with cancer. The devices were utilized for the administration of antineoplastic drugs, parenteral fluids, antibiotics, and blood products. Total duration of implantation was 4,046 days for 23 implanted system (range 7-445 days). Complications included cather infection (0.247/100 catheter days), occlusion (0.692/100 catheter days), and dislodgement of needle (0.643/100 catheter days). There were major complications that necessitated removal of catheters, including systemic infections (0.09/100 catheter days) and complete occlusions (0.09/100 catheter days). The system was thought to be safe and convenient in chemotherapy, and permitted full physical activity.
Anti-Bacterial Agents ; Antineoplastic Agents ; Catheters ; Child* ; Drug Therapy ; Humans ; Motor Activity ; Needles ; Prospective Studies*

Hepatoblastoma is a rare pediatric malignancy which frequently presents at an advanced unresectable stage, but improved resectability and survival is being reported with the help of neoadjuvant chemotherapy. Twenty biopsy proven hepatoblastoma patients were diagnosed and managed during the period between January, 1987 and June, 1995. Median age at diagnosis was 13 months (2mo~7yr 10mo) with M : F = 13 : 7. The histologic profile included 13 epithelial (5 fetal, 4 mixed, 1 embryonal, 3 undetermined), and 5 mixed mesenchymal and epithelial types. Chemotherapy effectively reduced the tumor volume with statistical significance (p=0.008) and was able to convert 7 out of 9 initially unresectable cases (78%) to resectable ones. Fourteen operations were done, 12 radical and 2 palliative with or without adjuvant chemotherapy. The whole population was followed with a median duration of 33 months and the median survival in the whole group was 26 months. The curative resection group displayed a 5 year survival rate of 61.1% and none in the non-curative group survived for more than 13 months (p=0.0001). In univariate analysis for prognostic factors, large tumor size at diagnosis and absence of thrombocytopenia was associated with poor survival, but these differences were not statistically significant. Pure fetal histology was not associated with better prognosis and this may be due to a different histologic classification. In this new era of neoadjuvant chemotherapy, the optimal management strategy for hepatoblastoma is still debated with radical surgical resection at earliest possible time being the final goal. For now, individualized approach appears to be the choice.
Biopsy ; Chemotherapy, Adjuvant ; Classification ; Diagnosis ; Drug Therapy ; Hepatoblastoma* ; Humans ; Prognosis* ; Survival Rate ; Thrombocytopenia ; Tumor Burden

PURPOSE: We investigated the clinical difference between single infection and coinfection with respiratory virus in hospitalized children with acute respiratory tract infections. METHODS: We reviewed 727 patients who were admitted with the diagnosis of acute respiratory infection at the Department of Pediatrics, Bundang Jesaeng Hospital between January and December of 2014. Diagnoses were made using the multiplex reverse transcriptase polymerase chain reaction (RT-PCR) assay targeting 16 viruses in nasopharyngeal swabs. Subjects were classified as the single virus infection and coinfection groups. RESULTS: A total of 439 patients were enrolled; 359 (77.2%) under 24 months. Single virus was detected in 279 (63.6%). Coinfection with multiple virus was detected in 160 (36.4%): 126 (28.7%) with 2 viruses, 30 (6.8%), and 4 (0.9%) with 3 to 4 viruses. Viral coinfection was detected in 28 samples (17.5%), with respiratory syncytial virus (RSV) A and rhinovirus being the most dominating combination. There were no clinical differences between the single infection and coinfection groups, except sputum and the frequency of high RSV load. Sputum was significantly more frequent in the coinfection group (P=0.043), and the frequency of high RSV load was significantly higher in the single infection group (P=0.029). Disease severity (high fever, the duration of fever [≥5 days], and the length of hospital stay [≥5 days], O₂ therapy) did not differ significantly between both groups. RSV was a frequent virus of single infection during winter. Coinfection was most common in winter. CONCLUSION: There were no clinical differences between single infection and coinfection, except sputum and the frequency of high RSV load.
Child ; Child, Hospitalized ; Coinfection* ; Diagnosis ; Fever ; Humans ; Length of Stay ; Multiplex Polymerase Chain Reaction ; Pediatrics ; Respiratory Syncytial Viruses ; Respiratory Tract Infections ; Reverse Transcriptase Polymerase Chain Reaction ; Rhinovirus ; Sputum

No abstract available.
Anemia, Dyserythropoietic, Congenital*

PURPOSE: Proper education of hospice professionals is essential for ensuring quality of end-of-life care. In 2005, 'End-of-life Care Task Force Team' by Ministry of Health and Welfare established '60 hours of hospice education' as basic requirement for hospice professionals. This study is aimed to determine how many of the hospice professionals meet with the criteria and whether there are significant regional variations. METHODS: We analyzed the data from 46 hospice organizations, which submitted the application to the 2008 designation program of Ministry of Health, Welfare, and Family Affairs. Data included details of the educational records of each hospice professionals. RESULTS: Total 673 hospice professionals were included in the analysis. Overall, only 41.5% (279/673) met the requirement. Nurses (46.8%; 177/378) were more likely to meet the requirement than doctors (35.8%; 38/106), social workers (32.0%; 24/75) and clergies (35.1%; 40/114). Hospice professionals of the organizations in metropolitan area received more education than those in small cities or rural area (52.4% vs. 25.0% for doctors, 50.6% vs. 43.9% for nurses, 42.9% vs. 25.5% for social workers). By geographic areas, hospice professionals in southeast regions received less education than other part of Korea (28.1% vs. 43.0~48.8%, respectively). CONCLUSION: Less than half of the Korean hospice professionals has received proper amount of hospice education, and significant regional variations existed. National programs to promote the education of hospice professionals and eliminate its disparities are greatly warranted. Implementation of the 60-hour currirulum for hospice professionals, based on the train-the-trainer model, would be regarded as one potential solution.
Advisory Committees ; Clergy ; Hospices ; Humans ; Korea ; Palliative Care ; Social Workers

PURPOSE: Surgical excision has been the primary treatment for hepatoblastoma and hepat-ocellular carcinoma. However, at presentation, only one third of such tumors are surgically resectable. Without operation, the disease is fatal. Therefore, neoadjuvant chemotherapy has been introduced for conversion of the unresectable tumors into the resectable ones. We studied th e effects of chemotherapy for hepatic malignancy in children. METHODS: Between November 1986 and August 1993, 30 children presented with hepatoblastomas or heptocellular carcinoma, which were diagnosed by histology. We analysed the laboratory findings of hepatic tumors and the outcome of chemotherapy. RESULTS: Laboratory findings revealed mild anemia, elevated SGOT/SGPT, and extremely increased AFP level. Twenty-six among 30 patients entered into surgery or neoadjuvant chemotherapy. Initial complete resection of tumor was attempted in 11 case, and was successful in 9 cases. Fifteen cases with initially unresectable tumors were treated with chemotherapy including cisplatin and/or doxorubicin. Nine of 15 showed significant reduction in tumor size, and delayed resection of the primary lesion was possible. But one case did not respond to chemotherapy, and 5 cases was droped out due to death(n=2) and refusal of chemotherapy(n=3). Twenty p atients were enrolled in survival analysis. Over-all 3 year survival rate was 61%, and 2 year survival rates of hepatoblastoma and hepatocellular carcinoma were 85% and 33% respectively(P=0.06). According to the stage, 2 year survival rate of stage I and III were 87% and 75% respectively. None of patient with metastasis survived at 16 months. Chemotherapy was tolerable in most patients and its principal toxicities were myelosuppression and fever. Three patients developed decreased left ventricular shortening fraction and their cumulative dose of doxorubicin were 771mg/m2, 557mg/ m2, and 390mg/ m2. CONCLUSIONS: Chemotherapy including cisplatin and/or doxorubicin is an effective treatment in inducing surgical resectability in hepatoblastomas which are unresectable at diagnosis.
Anemia ; Carcinoma, Hepatocellular* ; Child* ; Cisplatin ; Diagnosis ; Disulfiram ; Doxorubicin ; Drug Therapy* ; Fever ; Hepatoblastoma* ; Humans ; Neoplasm Metastasis ; Survival Rate