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1/99. Renal transplantation in scleroderma.

    Although the outcome of renal transplantation in patients with systemic lupus erythematosus (SLE) has been studied, there are few reports about the outcome of patients with systemic sclerosis who have undergone renal transplantation. We retrospectively collected data from the United Network for Organ Sharing (UNOS) Scientific Renal Transplant Registry from a 10-year period. From 1987 to 1997, 86 patients with systemic sclerosis who had renal transplantation were identified. Of these 86 patients, 70% were women, 86% were Caucasian, and the mean age at transplantation was 50.4 years. The overall mortality was 24% of the patient group; 44% (38/86) of renal grafts failed. First- through fifth-year graft survival rates were 62%, 60%, 57%, 50%, and 47%, respectively. The causes of graft failure could not be ascertained in 24 of 38 patients (63%). Among the known causes, 5 had acute rejection, 4 had chronic rejection, 3 had recurrence of scleroderma, and 1 each had infection and graft thrombosis. Immunosuppressive regimens used in the patients with systemic sclerosis consisted of antilymphocyte globulin in at least 25%. Sixty percent received a combination of steroids, azathioprine, and cyclosporine. The use of cyclosporine was not associated with either improvement of graft survival or an increased rate of graft failure. graft survival at 5 years in patients with systemic sclerosis was comparable to that of patients with SLE who received renal transplantation, according to existent medical literature. Based upon these data, renal transplantation is as effective a treatment for restoring renal function in patients with systemic sclerosis as it is in patients with SLE. Those patients with systemic sclerosis whose renal function did not improve with angiotensin-converting enzyme (ACE)-inhibitor treatments after scleroderma renal crisis should be considered as transplant candidates. Although the data are incomplete, the use of cyclosporine may not confer the advantage of improving graft survival in patients with systemic sclerosis as compared with SLE patients.
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2/99. cunninghamella infection post bone marrow transplant: case report and review of the literature.

    cunninghamella spp., in the class Zygomycete and order mucorales, are unusual opportunistic pathogens that have been identified with increased frequency in immunocompromised patients. Infections with this group of organisms have been seen most frequently in patients with hematologic malignancy. We describe an allogeneic bone marrow recipient who developed fungal pneumonitis and disseminated fungal dermatitis caused by cunninghamella spp. To our knowledge, this is the first reported case of cunninghamella infection in a BMT recipient. The case highlights the mortality associated with opportunistic infections in immunocompromised patients and confirms the risk factors associated with non-candida fungal infections after bone marrow transplantation.
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3/99. The effectiveness of administering a minimal dose of octreotide long-term prior to surgery for insulinoma: report of a case.

    We report herein the case of an 80-year-old woman with insulinoma who was regarded as an unsuitable candidate for immediate surgery due to her advanced age and obesity, for whom octreotide, a long-acting analogue of somatostatin, was used to improve her hypoglycemia and hyperinsulinemia without hyperalimentation. Administering a minimal dose of octreotide for a long period resulted in the improvement of leg edema, weight control, and cardiopulmonary function, and resection of the pancreatic tumor was safely carried out without any complications.
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4/99. Adverse outcome following transfemoral endovascular stent-graft repair of an abdominal aortic aneurysm in a patient with severe liver dysfunction: report of a case.

    We describe herein the case of a patient with severe liver failure in whom an abdominal aortic aneurysm (AAA) extending to the right iliac artery was diagnosed. Because the risk of performing a standard open repair was considered too high in this patient, the aneurysm was uneventfully repaired using a technique of transfemoral endovascular stent-grafting with femorofemoral bypass and occlusion of the left common and right internal iliac arteries. Unfortunately, multiorgan failure associated with adult respiratory distress syndrome (ARDS) and disseminated intravascular coagulation (DIC) suddenly developed on postoperative day (POD) 2 and the patient died on POD 9. Thus, although endovascular stent-grafting is generally assumed to be less invasive and therefore feasible for high-risk patients, patients with severe liver dysfunction may not be suitable candidates.
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5/99. Intraoesophageal rupture of a thoracic aortic aneurysm.

    The intraoesophageal rupture of a large thoracic aortic aneurysm is reported in a 49 year old man. He had been hypertensive for some years while the aneurysm increased in size. Although a graft was successfully inserted to repair the leak, infection from the oesophagus with candida albicans, subsequently led to secondary haemorrhage and death 17 days later. A plea is made for the earlier referral of patients with aneurysm prior to rupture, as the operative mortality rises markedly after rupture has occurred and in this case the situation was virtually irreparable.
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6/99. Recurrent medulloblastoma following pediatric double-lung transplant.

    We report a case of recurrent medulloblastoma following successful pediatric double-lung transplant for chemotherapy-induced pulmonary fibrosis. The patient had an apparent 10-year malignancy-free period prior to the transplant. This case demonstrates a potential complication of lung transplantation in individuals with prior malignancies, and questions whether patients with a history of medulloblastoma are suitable candidates for lung transplantation.
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7/99. Transjugular intrahepatic portosystemic shunt for bleeding stomal varices associated with chronic portal vein occlusion: long-term angiographic, hemodynamic, and clinical follow-up.

    The efficacy of the transjugular intrahepatic portosystemic shunt procedure (TIPS) in controlling refractory hemorrhage from stomal varices at the mucocutaneous junction has been previously described, but the durability of this procedure for this indication is unknown. Conservative therapy may be unsuccessful in preventing recurrent hemorrhage. Some authors believe that portosystemic shunting is the treatment of choice for patients with bleeding stomal varices who are good surgical candidates, because of the low incidence of recurrent bleeding and the longest survival. We report the 39-month angiographic and hemodynamic follow-up, and the 48-month clinical follow-up of a patient with refractory hemorrhage from stomal varices and coexistent chronic portal vein occlusion successfully treated with a TIPS procedure.
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8/99. Candidal infections as a cause of recurrent uretero-ileal anastomotic dehiscence.

    Fungal infections are common in immunocompromised patients. The presentation is often subtle and therefore treatment is delayed. Uretero-ileal anastomotic dehiscence due to candidal infection has never been reported before. This case represents an uncommon but potentially life-threatening complication in reconstructive surgery; that is, anastomotic dehiscence due to a unique etiology.
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9/99. De novo fibrillary glomerulopathy in the renal allograft of a patient with systemic lupus erythematosus.

    Lupus glomerulonephritis is a complication of systemic lupus erythematosus, with 10% of the patients developing end-stage renal disease. It is accepted that lupus patients are good candidates for kidney transplantation and that the disease activity is subdued after transplantation due to rigorous immunosuppression, with a low rate of graft loss due to recurrent glomerulonephritis. While recurrent fibrillary glomerulopathy has been reported in renal allografts, de novo disease has not. We report a patient with systemic lupus who underwent a renal transplantation and subsequently lost her allograft due to de novo fibrillary glomerulopathy. Four years after her first kidney transplant, the patient presented with acute deterioration of her renal function. A renal biopsy was performed, and it revealed a focal mesangioproliferative pattern with positive amorphous mesangial immunofluorescence staining for IgG and C3. congo red staining was negative. Electron microscopy demonstrated the presence of randomly oriented nonamyloid fibrils in the mesangiun. The diagnosis of de novo fibrillary glomerulopathy was made. The patient lost her allograft and received a second cadaveric renal transplant 1 year later. She has had a stable renal function since then.
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10/99. Late aortic dissection in a patient with a left ventricular assist device.

    The limited availability of donor hearts means that an increasing number of transplantation candidates are temporarily or permanently supported by mechanical circulatory assist devices. We report a patient undergoing implantation of a Novacor left ventricular assist device who suffered fatal aortic dissection on postoperative day 11 after satisfactory recovery from multiple organ failure. The dissection of the aorta initially presented as an embolic peripheral ischemia. Early complete echocardiography is thus warranted to rule out dissection.
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