No abstract available.
Chorea* ; Humans

BACKGROUND: Childhood myelodysplastic syndrome(MDS) is a heterogenous disease complex which has characteristics of cytopenia in one or more hemopoietic cell lines in peripheral blood and of dysmorphisms of hemopoietic precursors in bone marrow, but quite different from adult one. We experienced 22 patients with childhood myelodysplastic syndrome during 9 years from 1987 to 1995 in the Department of Pediatrics, Inje University College of Medicine, Pusan Paik Hospital, Pusan, Korea and performed a clinical study about them to analyze the clinical and hematological features and treatment outcome retrospectively. METHOD: We analyzed their clinical features including age and sex distribution, chief complaints and physical findings on first admission, distribution of subtypes, hematologic features including initial hemoglobin, total WBC and platelet count, and treatment outcome including leukmic transformation and prognostic scores. RESULTS: 1) Of the total 22 patients, 10 were in 0-4 years of age on first admission, 8 in 5-9, and 4 above 10 years of age. The male : female sex ratio was 2.1 : 1. 2) Of the 22 patients, chief complaints on first admission were bleeding tendency in 9 patients(40.9%), pallor in 8(36.4%) and fever in 3(13.6%), in order, and physical findings on first admission were purpura or ecchymoses in 17(77.3%), anemia in 15(68.2%) and hepatosplenomegaly in 8(36.4%), in order. 3) Hematologic findings on first admission were as follows : hemoglobin levels were below 3 g/dl in 1 patient(4.5%), 3-6 g/dl in 10(45.5%), and 6-9 g/dl in 11(50.0%). Initial WBC counts were below 5,000/mm3 in 13 patients(59.1%), 5,000-10,000/mm3 in 5(22.7%) and above 10,000/mm3 in 4(18.2%). Initial platelet counts were below 20,000/mm3 in 10(45.5%), 20,000-50,000/mm3 in 5(22.7%), 50,000-100,000/mm3 in 5(22.7%) and above 100,000/mm3 in 2(9.1%). 4) Of the 22 patients, 12 patients(54.6%) were RA type, 1(4.6%) RAS, and 3(13.6%) RAEB, RAEB-T and JCML types, respectively. 5) According to prognostic scores by Mufti et al(1986), none were in 'good' group, 17 patients(89.5%) in 'intermediate' group with 39.5 months of mean duration of survival(range 4-95 months) and 2(10.5%) in 'poor' group with 18 months of mean duration of surviral(range 17-19) until the last follow-up. However, the subtypes and clinical status seemed not to be related to the prognostic scores. 6) Sixteen patients were treated with low dose cytosine arabinoside(10 mg/m2/12hrs), of whom 7 patients gained long-standing event-free survival, whose treatment regimen was changed to oral 6-TG about 2 years later. All 3 of JCML were treated with A-Triple-V regimen, one of whom was died of sepsis, one was transformed into AML and died of sepsis, while the remained one gained long-standig event-free survival (62 months). 7) Leukemic transformation into AML occured in 7 patients(RA 1, RAEB 2, RAEB-T 3, JCML 1), 6 of whom were dead, while one gained long-standing event-free survival of 34 months. CONCLUSION: We concluded that RA was the most dominant type among our patients, and the frequency to transform into AML was 31,8%, and 31.9% of the patients had long-term survival, and that reliability of prognostic scoring system by Mufti et al(1986) was not high.
Adult ; Anemia ; Anemia, Refractory, with Excess of Blasts ; Bone Marrow ; Busan ; Cell Line ; Cytosine ; Disease-Free Survival ; Ecchymosis ; Female ; Fever ; Follow-Up Studies ; Hemorrhage ; Humans ; Korea ; Male ; Myelodysplastic Syndromes* ; Pallor ; Pediatrics ; Platelet Count ; Purpura ; Retrospective Studies ; Sepsis ; Sex Distribution ; Sex Ratio ; Treatment Outcome

No abstract available.
Peptic Ulcer* ; Retrospective Studies*

No abstract available.
Child* ; Humans

The aims of the Tharies replacement are to preserve bone stock and provide a more physiological and anatomical replacement using eccentic shells with the hope that long term durability will be improved. 8 cases of THARIES replacement performed between 1981 and 1983 at Hanyang UniversityHospital were reviewed and analyzed by radiography and U.C.L.A. hip rating system. Follow-up ranged from 34 to 54 months and averaged 43.3 months. The result was relatively good in 6 cases except two cases of aseptic loosening without further progression.
Arthroplasty ; Follow-Up Studies ; Hip ; Hope ; Radiography

No abstract available.
Infectious Mononucleosis*

BACKGROUND: HLA-B5102 is a newly approved antigen at the meeting of the WHO Nomenclature Committee held after the Eleventh International Histocompatibility Workshop. It had been called B5l.35 because it was defined by both B5l and B35 antisera. HLA-B5102 antigen cannot be accurately determined by current commercial HLA typing trays. This study was carried out to assess the frequency of HLA-B5102 antigen in Koreans and serological reaction patterns of HLA-B5102 on commercial HLA trays. METHODS: We performed HLA-A, B, C serological typing for 2,000 Koreans registered for KMDP (Korean Marrow Donor Program) donors using the Terasaki Oriental Tray (One Lambda, USA). Selected samples (17/2000) which showed atypical B5 reaction patterns were tested against Japan Central Block HLA Workshop tray to detect the presence of HLA-B5102. RESULTS: HLA-B5102 showed a phenotype (antigen) frequency of 0.45% (9/2000) and an allele frequency of 0.23%. Two locus HLA haplotype and linkage analysis showed that HLA-B5102 was in linkage disequilibrium with HLA-A3l (p<0.01). The serological patterns of HLA-B5102 on Terasaki Oriental Tray were 1) Lot 14, 15 : B5(+), 2) Lot 15 B : B5(+), B35+53(+), and 3) Lot 16 : B5(+), B5l(+), B35+53(+), and therefore could be identified as HLA-B5, B5l, B52, B35 or B53. CONCLUSIONS: The frequency of HLA-B5102 in the Korean population (antigen frequency 0.45%, allele frequency 0.23%) is similar to that of Japanese. The presence of HLA-B5102 can be suspected when atypical BS reaction patterns are encountered in commercial HLA typing trays, and B5 or BSI had better been assigned to these cases when additional confirmatory typing is not available.
Asian Continental Ancestry Group ; Bone Marrow ; Education ; Gene Frequency ; Haplotypes ; Histocompatibility ; Histocompatibility Testing ; HLA-A Antigens ; Humans ; Immune Sera ; Japan ; Linkage Disequilibrium ; Phenotype ; Tissue Donors

BACKGROUND: HLA-B5102 is a newly approved antigen at the meeting of the WHO Nomenclature Committee held after the Eleventh International Histocompatibility Workshop. It had been called B5l.35 because it was defined by both B5l and B35 antisera. HLA-B5102 antigen cannot be accurately determined by current commercial HLA typing trays. This study was carried out to assess the frequency of HLA-B5102 antigen in Koreans and serological reaction patterns of HLA-B5102 on commercial HLA trays. METHODS: We performed HLA-A, B, C serological typing for 2,000 Koreans registered for KMDP (Korean Marrow Donor Program) donors using the Terasaki Oriental Tray (One Lambda, USA). Selected samples (17/2000) which showed atypical B5 reaction patterns were tested against Japan Central Block HLA Workshop tray to detect the presence of HLA-B5102. RESULTS: HLA-B5102 showed a phenotype (antigen) frequency of 0.45% (9/2000) and an allele frequency of 0.23%. Two locus HLA haplotype and linkage analysis showed that HLA-B5102 was in linkage disequilibrium with HLA-A3l (p<0.01). The serological patterns of HLA-B5102 on Terasaki Oriental Tray were 1) Lot 14, 15 : B5(+), 2) Lot 15 B : B5(+), B35+53(+), and 3) Lot 16 : B5(+), B5l(+), B35+53(+), and therefore could be identified as HLA-B5, B5l, B52, B35 or B53. CONCLUSIONS: The frequency of HLA-B5102 in the Korean population (antigen frequency 0.45%, allele frequency 0.23%) is similar to that of Japanese. The presence of HLA-B5102 can be suspected when atypical BS reaction patterns are encountered in commercial HLA typing trays, and B5 or BSI had better been assigned to these cases when additional confirmatory typing is not available.
Asian Continental Ancestry Group ; Bone Marrow ; Education ; Gene Frequency ; Haplotypes ; Histocompatibility ; Histocompatibility Testing ; HLA-A Antigens ; Humans ; Immune Sera ; Japan ; Linkage Disequilibrium ; Phenotype ; Tissue Donors

Malignant small round cell tumor (SRCT) of the thoracopulmonary region appears to originate in the soft tissues of the chest wall or the peripheral lung. A differential diagnosis of poorly differentiated small round cell tumors which include Ewing's sarcoma of bone and soft tissue, embryonal rhabdomyosarcoma, Askin tumor, neuroblastoma, peripheral neuroectodermal tumor, small cell osteogenic sarcoma and lymphoma are after difficult by light microscopy alone. In recent, by the extensive studies electron microscopic examination, histochemical study, immunochemical study, cytogenetics and gene analysis, these tumors may be derived from the primitive and pluripotential cells, differentiating into mesenchymal, epithelial and neural features in variable proportions. Treatment for SRCT of thoracopulmonary regin is not determined because of massive involvement of the lung, pleura or soft tissues of the chest wall resulted in a dismal outcome despite aggressive surgery, irradiation and chemotherapy.
Cytogenetics ; Diagnosis, Differential* ; Drug Therapy ; Lung ; Lymphoma ; Microscopy ; Neuroblastoma ; Neuroectodermal Tumors, Primitive, Peripheral ; Osteosarcoma ; Pleura ; Rhabdomyosarcoma, Embryonal ; Sarcoma, Ewing ; Thoracic Wall

No abstract available.
Burns* ; Hand* ; Humans ; Infant*