Common variable immunodeficiency (CVID) is a heterogeneous collection of disorders with hypogammaglobulinemia with recurrent bacterial infections and high incidence of autoimmune disorders as its hallmark. We report a 7-year-old girl suffering from CVID with Coombs' test positive hemolytic anemia. She had been relatively well until 23-months old when she was admitted to Taejon St. Mary's Hospital with pneumonia 5 years ago. Afterwards, she had suffered from recurrent otitis media, paranasal sinusitis, bronchitis and pneumonia, experiencing 13 admissions. She was diagnosed as autoimmune hemolytic anemia at 4-years old and had been treated with prednisolone. Laboratory finidings showed hypogammaglobulinemia(gamma-globulin in immunoelectrophoresis 0.04g/dL, IgG 170mg/dL, IgA 31mg/dL, IgM 27.5mg/dL) which was previously within normal limits checked at the age of 3- and 5-years old. Isohemmagglutinins (Anti-A,-B IgM and IgG) and anti-measles IgG, anti-mumps IgG, anti-rubella IgG and anti-HBs antibody along with PPD skin test were all negative. Peripheral lymphocyte subsets revealed as follows : pan T cells (CD3+) 48.6% (normal values : 60-85%), pan B cells (CD19+) 36.7% (8-20%), CD4+ T cells 24.4% (28+/-8%), CD8+ T cells 15.3% (5+/-10%), and CD4/CD8 ratio of 1.6 (0.6-2.8). Proliferations of peripheral blood mononuclear cells induced by various T cell stimulants were all markedly depressed. Chronic paranasal sinusitis and lung parenchymal damages were revealed on computerized tomography and lung scan, and a monthly intravenous immunoglobulin therapy was started.
Agammaglobulinemia ; Anemia, Hemolytic ; Anemia, Hemolytic, Autoimmune* ; B-Lymphocytes ; Bacterial Infections ; Bronchitis ; Child ; Child, Preschool ; Common Variable Immunodeficiency* ; Coombs Test ; Daejeon ; Female ; Humans ; Immunization, Passive ; Immunoelectrophoresis ; Immunoglobulin A ; Immunoglobulin G ; Immunoglobulin M ; Incidence ; Infant ; Ionomycin ; Lung ; Lymphocyte Subsets ; Otitis Media ; Pneumonia ; Prednisolone ; Sinusitis ; Skin Tests ; T-Lymphocytes

Purpose: The present study aims to determine the clinical outcome of kidney transplantation and to provide data of long-term graft and patient survival. Methods: Between 1969 and 2005, 1,500 kidney transplants were performed at the Kangnam st. Mary's hospital. We analyzed the clinical characteristics and outcomes of kidney transplant recipients retrospectively. Results: The mean follow-up period was 112 months. Chronic glomerulonephritis was the leading cause of primary renal diseases, but the proportion of has increased from 1 % before 1985 to 6% afterwards. First renal transplantation was 94.5% (n=1418), and retransplantation was 5.4% (n=82). Type of donor source was mostly living-related, with the recent decrease in the number of living- unrelated donors. Currently, 72l patients are alive with functioning grafts, 297 cases had graft failure, 277 cases died, 205 cases were transferred or lost during follow-up. Main cause of graft failure was chronic allograft nephropathy (n=316). Overall, 1-, 5-, 10-, and 20-year graft survival were 92%, 81%, 66%, and 29% respectively. 1-, 5-, 10-, and 20-year patient survival were 93%, 88%, 81%, and 69% respectively. Conclusion: This review of 36-years experience in a single center showed that the graft survival has improved compared to the initial transplantation era.
Allografts ; Follow-Up Studies ; Glomerulonephritis ; Graft Survival ; Humans ; Kidney Transplantation ; Kidney* ; Korea* ; Retrospective Studies ; Risk Factors ; Tissue Donors ; Transplantation ; Transplants ; Unrelated Donors

PURPOSE: To evaluate the efficacy and safety of preoperative embolization of intrancranial meningioma. MATERIALS AND METHODS: We retrospectively reviewed intrancranial meningioma patients (n=37) who underwentpreoperative embolization. They were categorized into two groups, skull base lesions (n=22) and non-skull baselesions (n=15), according to tumor location. In addition, embolization results were classified by comparisonbetween pre- and post-embolization angiography as complete (residual tumor staining < 10 or 30 %) or incomplete(residual tumor staining >or= 10 or 30%). In each group, estimated blood loss (EBL) was estimated by amount ofintraoperative transfusion with pre- and post-operative hemoglobin level. Tumor resectability was evaluated byfollow-up computed tomography. New symptoms occurring within 24 hours of embolization were considered to be thoseassociated with embolization ; symptoms improved by conservative treatment were regarded as mild, while thoseresulting in new deficits were considered severe. RESULT: In the group with skull base lesions (n=22), completeembolization with the criteria of residual tumor staining of less than 30% was performed in 14 patients(EBL=1770ml;complete surgical removal in nine patients and incomplete removal four). Incomplete embolization wasperformed in eight patients (EBL=3210ml; complete and incomplete removal each in four patients). In the group withnon-skull base lesions, complete embolization with the criteria of residual tumor staining of less than 10% wasperformed in five patients (EBL=970ml) and incomplete embolization in ten (EBL=2260ml). Complete tumor removal waspossible in this group regardless of the completeness of preoperative tumor embolization. In a case ofintraventricular meningioma (3%), intratumoral hemorrage occurred on the day following embolization. Other mildpost-embolization complications occurred in three cases (8%). CONCLUSION: Preoperative embolization can be aneffective and safe procedure for meningioma and may reduce intraoperative blood loss. Its effectiveness may dependon tumor location and the degree of embolization, though the procedure seems to be helpful for lseions at theskull base.
Angiography ; Humans ; Meningioma* ; Neoplasm, Residual ; Retrospective Studies ; Skull Base