Cases reported "Kidney Failure, Chronic"

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1/206. Bone marrow examinations as final clue to diagnosis of hypercalcemia: report of two cases.

    Two young men with severe hypercalcemia in association with renal failure (one acute and one chronic) are reported in whom usual diagnostic tests failed to reveal an etiology, and the final diagnoses were given by bone marrow examinations. Early bone marrow examinations in specific patients with hypercalcemia of undetermined origin sometimes are vital as shown by our two patients.
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2/206. calciphylaxis: one hospital's experience.

    The lives of the ESRD population have been enhanced and prolonged by improved conservative management, efficient dialysis and successful transplantation. However, practitioners are noting some significant morbidity in these long-term surviving nephrology patients. Clinical signs of renal osteodystrophy can be very subtle and insidious. Bone pain, muscle weakness, and pruritis can be vague symptoms. calciphylaxis, which is classified as a skin disorder is an extremely rare, painful, and progressive complication. Typically, the patient presents with vascular calcifications, cutaneous ulcerations and tissue necrosis. The treatment regimen for bone disease in the ESRD population will be reviewed. The symptoms of calciphylaxis, diagnostic tests and prognosis will be discussed. Four case studies describe our recent experience. Emphasis will be on the intense multidisciplinary care required for this devastating and often fatal syndrome.
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3/206. Chronic renal failure causing brown tumors and myelopathy. Case report and review of pathophysiology and treatment.

    Brown tumors (osteoclastomas) are histologically benign lesions that are caused by primary or secondary hyperparathyroidism. Secondary hyperparathyroidism is a frequent complication of chronic renal failure. Skeletal brown tumors are relatively uncommon, and brown tumors that involve the spine are considered very rare. The authors present the case of a 37-year-old woman with systemic lupus erythematosus and hemodialysis-dependent anuric renal failure, in whom spinal cord compression developed due to a brown tumor and pathological fracture at T-9. The patient underwent transthoracic decompressive surgery and spinal reconstruction in which cadaveric femoral allograft and instrumentation were used. Brown tumors of the vertebral column require surgical treatment if medical therapy and parathyroidectomy fail to halt their progression or if acute neurological deterioration occurs. In patients with renal failure bone healing is delayed and there is an increased risk that healing will fail because the metabolic derangements can result in severe osteoporosis. Surgical reconstruction of the spine may require the use of augmentation with instrumentation and aggressive treatment of hyperparathyroidism to achieve successful outcomes.
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4/206. Post transplant CD8 gammadelta T-cell lymphoma associated with human herpes virus-6 infection.

    Gammadelta T-cell lymphoma is a rare T-cell lymphoproliferative disorder that has been reported in both immunocompetent and immunocompromised persons. This report describes a forty eight year old patient who developed gammadelta T-cell lymphoma four years after undergoing living-related kidney transplantation. The lymphoma expressed CD2, CD3, CD7, CD8 and CD56, and the gammadelta T-cell receptor and did not express CD5, CD4 and the alphabeta T-cell receptor. In addition, HHV-6 was cultured from the patient's bone marrow, marking the first time that this virus has been associated with gammadelta T-cell lymphoma. Since all patients with gammadelta T-cell lymphoma described to date have responded poorly to standard combination chemotherapies, the patient was treated with the purine analogue 2-chlorodeoxyadenosine. While he responded transiently to treatment, long term remission was not achieved indicating that additional therapeutic approches still need to be developed, for the management of this disorder.
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5/206. Administration and pharmacokinetics of high-dose cyclophosphamide with hemodialysis support for allogeneic bone marrow transplantation in acute leukemia and end-stage renal disease.

    We report a patient with pre-existing end-stage renal disease (ESRD) who underwent successful matched related donor allogeneic bone marrow transplantation for AML in second complete remission (CR2) using conditioning with high-dose cyclophosphamide (CY, 60 mg/kg/day x 2) and TBI (165 cGy twice daily x 4 days). The timing of hemodialysis after high-dose CY was extrapolated from available data on the pharmacokinetics of high-dose CY and hemodialysis clearance of conventional dose CY and its metabolites. Pharmacokinetic analyses indicated that the elimination of high-dose CY and its alkylating metabolites is impaired in ESRD but is cleared with hemodialysis. The patient's early post-transplant course was uncomplicated, and WBC and platelet engraftment occurred by day 22. bone marrow examination on day 25 showed trilineage engraftment with no AML; cytogenetics showed 100% donor karyotype. The patient remains in remission with 100% donor karyotype at 3 years post transplant. Clinical results indicate that the administration of high-dose CY is feasible with hemodialysis support for patients with ESRD.
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6/206. Localization of a bone imaging agent in a calcified hematoma.

    A patient with chronic renal failure and secondary hyperparathyroidism had iliac bone biopsy. The procedure was complicated by a soft-tissue hematoma, which had calcified. A 3-4-cm palpable mass was visible in the lower left abdominal wall. Intense uptake of 99mTc-HMDP corresponded with the location of the calcified hematoma in this patient.
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7/206. Identification of calcium oxalate deposits in bone by electron diffraction.

    Oxalosis involving bone secondary to prolonged chronic renal failure and long-term dialysis occurred in a living patient. The cystalline deposit in the small fragment of bone was identified by electron diffraction.
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8/206. Reduced size of liquefaction necrosis of mitral annular calcification in chronic renal failure by using low calcium concentration hemodialysis.

    A report is presented of a liquefaction necrosis of mitral annular calcification in a patient with chronic renal failure and secondary hyperparathyroidism who had been managed by hemodialysis for 11 years. The mass was echogenic with an echo-lucent area inside, high density on computed tomography and low intensity on magnetic resonance imaging. The uptake of gallium-67 (67Ga)-citrate and the bone agent technetium-99m-methylene diphosphate (99mTc-MDP) was seen in the mass. These findings were compatible with liquefaction necrosis of the mitral annular calcification. After treatment with low calcium concentration hemodialysis, the size of the mass reduced with disappearance of the echo-lucent area on the echocardiography and there was no uptake of 67Ga-citrate or 99mTc-MDP. Liquefaction necrosis might be the early and reversible form of mitral annular calcification. When a tumorlike echogenic mass at the base of mitral leaflets is seen in patients with predisposing factors for mitral annular calcification, consider the possibility of this specific form of mitral annular calcification in order to avoid any unnecessary surgical intervention.
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9/206. PHEX expression in parathyroid gland and parathyroid hormone dysregulation in X-linked hypophosphatemia.

    X-linked hypophosphatemia (XLH), a renal phosphate (Pi) wasting disorder with defective bone mineralization, is caused by mutations in the PHEX gene (a Pi-regulating gene with homology to endopeptidases on the x chromosome). parathyroid hormone (PTH) status in XLH has been controversial, with the prevailing belief that hyperparathyroidism develops in response to Pi therapy. We report a 5-year-old girl with XLH (patient 1) who had significant hyperparathyroidism at presentation, prior to initiation of therapy. We examined her response to a single oral Pi dose, in combination with calcitriol, and demonstrated a rise in serum concentration of intact PTH, which peaked at 4 h and paralleled the rise in serum Pi concentration. We also present two other patients whose parathyroid glands were analyzed for PHEX mRNA expression following parathyroidectomy. Patient 2 had autonomous hyperparathyroidism associated with chronic renal insufficiency, and patient 3, with XLH, developed autonomous hyperparathyroidism after 8 years of therapy with Pi and calcitriol. Following parathyroidectomy, patient 3 exhibited an increase in both serum Pi concentration and renal Pi reabsorption. The abundance of PHEX mRNA, relative to beta-actin mRNA, in parathyroid glands from patients 2 and 3 was several-fold greater than that in human fetal calvaria, as estimated by ribonuclease protection assay. In summary, we have shown that hyperparathyroidism can be a primary manifestation of XLH and that PHEX is abundantly expressed in the parathyroid gland. Given that PHEX has homology to endopeptidases, we propose that PHEX may have a role in the normal regulation of PTH.
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10/206. Combined histocompatibility leukocyte antigen-matched donor bone marrow and renal transplantation for multiple myeloma with end stage renal disease: the induction of allograft tolerance through mixed lymphohematopoietic chimerism.

    BACKGROUND: Experimental and clinical evidence has demonstrated that the establishment of allogeneic chimerism after bone marrow transplantation may provide donor-specific tolerance for solid organ allografts. methods: Based on the preliminary results of a clinical trial using nonmyeloablative preparative therapy for the induction of mixed lymphohematopoietic chimerism, we treated a 55-year-old woman with end stage renal disease secondary to multiple myeloma with a combined histocompatibility leukocyte antigen-matched bone marrow and renal transplant after conditioning with cyclophosphamide, antithymocyte globulin, and thymic irradiation. RESULTS: The posttransplant course was notable for early normalization of renal function, the absence of acute graft-versus-host disease, and the establishment of mixed lymphohematopoietic chimerism. cyclosporine, which was the only posttransplant immunosuppressive therapy, was tapered and discontinued on day 73 posttransplant. No rejection episodes occurred, and renal function remains normal on day 170 posttransplant (14 weeks after discontinuing cyclosporine). Although there is presently no evidence of donor hematopoiesis, there is evidence of an ongoing antitumor response with a recent staging evaluation showing no measurable urine kappa light chains. The patient remains clinically well and is off all immunosuppressive therapy. CONCLUSION: This is the first report of the deliberate induction of mixed lymphohematopoietic chimerism after a nonmyeloablative preparative regimen to treat a hematological malignancy and to provide allotolerance for a solid organ transplant.
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