Myositis Ossificans progressiva is a very rare disease and not more than five cases have been reported in this country. The symptoms usually appear before the age of six and no case has ever been reported in medical literature in which patient is older than the age of twenty. This author reports here that a 52 years old male was diagnoed as a myositis ossificans progressiva. The patient developed his symptoms when he was lynched by a club during the Korean conflict at the Korean conflict at the age of thirty. Another interesting fact is that is that his daughter has also developed similar symptoms which appeared following an accident when she was run over by an angry cow at the age of six.
Humans ; Korean War ; Male ; Myositis Ossificans ; Myositis ; Nuclear Family ; Rare Diseases

No abstract available.
Bone Marrow* ; Precursor Cell Lymphoblastic Leukemia-Lymphoma*

PURPOSE: We reviewed 100 cases of HLA-matched sibling allogeneic bone marrow transplantation(allo-BMT) in children and wish to share these results. MEHTODS: One hundred children had undergone allo-BMT from HLA-identical siblings between Nov. 1983 and May 1998. There were 50 males and 50 females with a median age of 10 years and a median follow-up of 38 months. Out of 100 cases, 43 children were transplanted for severe aplastic anemia (SAA), 29 for acute myelogenous leukemia (AML), 18 for acute lymphocytic leukemia (ALL), 8 for chronic myelogenous leukemia (CML) and 2 for hemophagocytic lympho-histiocytosis (HLH). RESULTS: SAA : The 5-year event free survival (EFS) of SAA was 91%. The types of events that occurred were 3 thrombotic thrombocytopenic purpura (TTP), 2 venoocclusive disease (VOD) and 1 rejection. AML : In 25 of 29 cases, the 4-year EFS after allogeneic BMT in first remission was 71%. That of the TBI-based and Busulfan-based group was 44% and 77%, respectively. The most favorable results were observed in the Busulfan-based group in first remission with an EFS of 81% (n=18). The types of events that occurred were 4 TTP, 3 VOD, 2 rejections and 1 relapse. ALL : Five-year EFS of children with complete remission (CR; n=14, 7 CR1, 7 CR2) was 81%. CML : For the 6 children who received transplants while in the first chronic phase, the event free survival was 67%. HLH : Both of the two children with HLH survived 9 months and 24 months after BMT, respectively. Acute GVHD (> or =Grade ll) was observed in 13 children. Chronic GVHD developed in 10 children; 8 cases were localized and 2 were extensive type. CONCLUSION: Allo-BMT can cure children with refractory stem cell disorders. The most important factor that influences survival after transplantation is interval between diagnosis and transplantation for patients with severe aplastic anemia and remission state at transplantation for patients with leu-
Anemia, Aplastic ; Bone Marrow Transplantation* ; Bone Marrow* ; Child* ; Diagnosis ; Disease-Free Survival ; Female ; Follow-Up Studies ; Humans ; Leukemia, Myelogenous, Chronic, BCR-ABL Positive ; Leukemia, Myeloid, Acute ; Male ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Purpura, Thrombotic Thrombocytopenic ; Recurrence ; Siblings* ; Stem Cells

PURPOSE: We reviewed 100 cases of HLA-matched sibling allogeneic bone marrow transplantation(allo-BMT) in children and wish to share these results. MEHTODS: One hundred children had undergone allo-BMT from HLA-identical siblings between Nov. 1983 and May 1998. There were 50 males and 50 females with a median age of 10 years and a median follow-up of 38 months. Out of 100 cases, 43 children were transplanted for severe aplastic anemia (SAA), 29 for acute myelogenous leukemia (AML), 18 for acute lymphocytic leukemia (ALL), 8 for chronic myelogenous leukemia (CML) and 2 for hemophagocytic lympho-histiocytosis (HLH). RESULTS: SAA : The 5-year event free survival (EFS) of SAA was 91%. The types of events that occurred were 3 thrombotic thrombocytopenic purpura (TTP), 2 venoocclusive disease (VOD) and 1 rejection. AML : In 25 of 29 cases, the 4-year EFS after allogeneic BMT in first remission was 71%. That of the TBI-based and Busulfan-based group was 44% and 77%, respectively. The most favorable results were observed in the Busulfan-based group in first remission with an EFS of 81% (n=18). The types of events that occurred were 4 TTP, 3 VOD, 2 rejections and 1 relapse. ALL : Five-year EFS of children with complete remission (CR; n=14, 7 CR1, 7 CR2) was 81%. CML : For the 6 children who received transplants while in the first chronic phase, the event free survival was 67%. HLH : Both of the two children with HLH survived 9 months and 24 months after BMT, respectively. Acute GVHD (> or =Grade ll) was observed in 13 children. Chronic GVHD developed in 10 children; 8 cases were localized and 2 were extensive type. CONCLUSION: Allo-BMT can cure children with refractory stem cell disorders. The most important factor that influences survival after transplantation is interval between diagnosis and transplantation for patients with severe aplastic anemia and remission state at transplantation for patients with leu-
Anemia, Aplastic ; Bone Marrow Transplantation* ; Bone Marrow* ; Child* ; Diagnosis ; Disease-Free Survival ; Female ; Follow-Up Studies ; Humans ; Leukemia, Myelogenous, Chronic, BCR-ABL Positive ; Leukemia, Myeloid, Acute ; Male ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Purpura, Thrombotic Thrombocytopenic ; Recurrence ; Siblings* ; Stem Cells

BACKGROUND: The organ transplantation becomes the management of choice for many patients with chronic and life threatening heart, liver, kidney, bone marrow, and pancreatic diseases. A new set of side effects unique to this groups of patients has become recognized. Bone disease is one of these complications. It is well known that there is an interplay between the cells in the bone marrow and the surrounding bone tissue. Marrow stromal cells include the progenitors of the osteoblastic lineage are the sources of effector molecules that support and regulate both hematopoiesis and bone remodeling. But little is known about the effects of myeloablative treatment followed by bone marrow transplantation(BMT) on bone metabolism. METHODS: We have investigated prospectively in 29 patients undergoing BMT(4 autologous, 25 allogenic) for hematologic diseases(19 leukemia, 9 severe aplastic anemia, 1 myelodyspoietic syndrome). Serum concentrations of calcium, phosphorus, creatinine, gonadotropins, sex hormones and biochemical markers of bone turnover(osteocalcin and carboxyterminal cross-linked telopeptide of type I collagen(ICTP)] were measured. The samples were collected before BMT and 1, 2, 3, 4, 12 weeks, 6 months and 1 year thereafter. Bone mineral density was measured with DEXA(Dual Energy X-ray Absorptiometry) before and after 1 year of BMT. RESULTS: 1. ICIP was progressively increased until 4 weeks after BMT when peak values were reached. And then decreased thereafter and basal values were regained after 1 year. Osteocalcin was progressively decreased until 3 weeks after BMT when nadir values were reached. And then increased thereafter and basal values were regained after 3 months. No distinct differences were observed in serum biochemical turnover marker between both sexes and between patients who received total body irradiation and those who did not. 2. Lumbar BMD was 2.1% decreased from 1.113 +/- 0.132 g/cm to 1.089 +/- 0.137 g/cm, and femoral BMD was 6.2% decreased fiom 1.078 +/- 0.156 g/cm to 1.011 +/- 0.157 g/cm. 3. 92% of the women (11/12) became menopausal manifested by high gonadotropin and low estradiol levels immediately after BMT. In contrast to women, gonadotropins and testosterone levels were not changed significantly in men after BMT. CONCLUSION: The rapid impairment of bone formation and also increase in bone resorption, as mirrored by the biochemical markers in this study, might play a role for the post-BMT bone loss. Further studies over many patients with a longer follow up will be needed.
Anemia, Aplastic ; Biomarkers ; Bone and Bones ; Bone Density ; Bone Diseases ; Bone Marrow Transplantation* ; Bone Marrow* ; Bone Remodeling ; Bone Resorption ; Calcium ; Creatinine ; Estradiol ; Female ; Follow-Up Studies ; Gonadal Steroid Hormones ; Gonadotropins ; Heart ; Hematopoiesis ; Humans ; Kidney ; Leukemia ; Liver ; Male ; Metabolism* ; Organ Transplantation ; Osteoblasts ; Osteocalcin ; Osteogenesis ; Pancreatic Diseases ; Phosphorus ; Prospective Studies ; Stromal Cells ; Testosterone ; Transplants ; Whole-Body Irradiation

Salmonella septic arthritis in a healthy, immunocompetent patient is extremely rare. We experienced a case of septic arthritis of the knee caused by Salmonella Group D in a patient with Non-small cell lung cancer. A 43-year-old female receiving steroid therapy for treatment of Non-small cell lung cancer with metastasis to the spinal cord complained of painful swelling of the right knee joint. Culture of synovial fluid obtained by aspiration yielded growth of Salmonella Group D. The patient was treated with ceftriaxone; however, she expired on the ninth day after treatment.
Arthritis ; Arthritis, Infectious ; Carcinoma, Non-Small-Cell Lung ; Female ; Humans ; Knee ; Knee Joint ; Neoplasm Metastasis ; Salmonella ; Salmonella enteritidis ; Spinal Cord ; Synovial Fluid