No abstract available.
Dyskeratosis Congenita*

No abstract available.
Leukemia*

Bile* ; Choledochal Cyst* ; Humans ; Infant* ; Peritonitis*

No abstract available.
Leukemia*

No abstract available.
Anemia, Aplastic* ; Bone Marrow Transplantation* ; Bone Marrow* ; Immunomodulation*

The purpose of this study was to evaluate the association between glycated hemoglobin (HbA1c) and all-cause mortality in type 2 diabetes mellitus. We conducted a community-based prospective cohort study of 352 type 2 diabetic patients aged 30-92 who participated in a community diabetes complications screening program in Gokseng-gun, Jeollanamdo, Korea. HbA1c levels were categorized as <6.5%, 6.5-6.9%, 7.0-7.9%, 8.0-8.9% and ≥9.0%. Patients were followed up on for a mean of 6.9 years. The Cox proportional hazards model was used to evaluate the relationship between HbA1c levels and all-cause mortality. During the mean follow-up period of 6.9 years, 77 patients (21.9%) died. A J-shaped association was found between HbA1c and all-cause mortality, with the lowest mortality at 6.5-6.9% of HbA1c levels. Compared to patients with HbA1c of 6.5-6.9%, patients with <6.5%, 7.0-7.9%, 8.0-8.9% and ≥9.0% had an adjusted hazard ratio (95% confidence interval) for all-cause mortality of 1.71 (0.76-3.84), 1.23 (0.53-2.82), 1.32(0.51-3.44) and 2.66 (1.01-7.02), respectively. We found a J-shaped association between glycated hemoglobin and all-cause mortality in Korean type 2 diabetic patients.
Cohort Studies ; Diabetes Complications ; Diabetes Mellitus ; Diabetes Mellitus, Type 2 ; Follow-Up Studies ; Hemoglobin A, Glycosylated ; Humans ; Jeollanam-do ; Korea ; Mass Screening ; Mortality ; Proportional Hazards Models ; Prospective Studies

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

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

PURPOSE: To evaluate the clinical efficacy of amniotic membrane transplantation and limbal-conjunctival autograft for patients with recurrent pterygium or pseudopterygium, the results of 15 eyes of 14 patients were analyzed. METHODS: Total 10 cases of recurrent pterygium and 5 cases of pseudopterygium were surgically managed during June 1998 to July 2000. After excision of pterygium, amniotic membrane was attached on the bare sclera and limbal-conjunctival autograft was transplanted over the amniotic membrane. Mean follow-up period was 47.2 week and all patients were examined for recurrence, which was graded from G0(no vessel) to G3(fibrotic tissue invading the limbus). RESULTS: 12 out of 15 cases(rate 80%) showed no recurrence(grade 0). 1 case of grade 2 recurrence and 2 cases of grade 3(total 3 cases) were observed(rate 20%). Reoperation was performed on 2 cases of grade 3 recurrence. There was no further recurrence necessitating additional reoperation. 1 case of limbal graft was detached and removed. The operation time of this more prolonged than the other procedures, but there were no significant complications. CONCLUSION: Amniotic membrane and limbal-conjunctival autograft for patients with recurrent pterygium or pseudopterygium reconstructed complete ocular surface with less recurrence. Therefore, we recommend this surgical approach as a safe and effective method for the treatment of complicated cases of pterygium.
Amnion* ; Autografts* ; Follow-Up Studies ; Humans ; Pterygium* ; Recurrence ; Reoperation ; Sclera ; Transplants

Malignant growth of the small bowel accounts for approximately 1 to 3 percent of malignant tumors of the gastrointestinal tract. Leiomyosarcomas are the second most common primary tumor of the small bowel, their frequency being one-third to one-half of adenocarcinoma. The common clinical symptoms of leiomyosarcoma of the small intestine are hemorrhage and abdominal pain. Accurate diagnosis cannot be based on solely on the roentgenographic finding, although in certain situations the dignosis of leiomyosarcoma may be suggested strongly. We report a case of bleeding leiomyosarcoma located in the second portion of the duodenum which was first recognized by endoscopic examination and confirmed by explolaparotomy.
Abdominal Pain ; Adenocarcinoma ; Diagnosis ; Duodenum* ; Gastrointestinal Tract ; Hemorrhage ; Intestine, Small ; Leiomyosarcoma*