Cases reported "Kidney Failure, Chronic"

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1/638. Acute torsion of the renal transplant after combined kidney-pancreas transplant.

    BACKGROUND: Surgical complications after combined kidney and pancreas transplantation are a major source of morbidity and mortality. Complications related to the pancreas occur with greater frequency as compared to renal complications. The occurrence in our practice of two cases of renal infarction resulting from torsion about the vascular pedicle led to our retrospective review of similar vascular complications after combined kidney and pancreas transplantation. methods: charts were reviewed retrospectively, and two patients were identified who experienced torsion about the vascular pedicle of an intra-abdominally placed renal allograft. RESULTS: Two patients who had received combined intraperitoneal kidney and pancreas transplantation presented at 16 and 11 months after transplant, respectively, with abdominal pain and decreased urine output. One patient had radiological documentation of abnormal rotation before the graft loss; unfortunately, the significance of this finding was missed. diagnosis was made in both patients at laparotomy, where the kidneys were infarcted secondary to torsion of the vascular pedicle. Both patients underwent transplant nephrectomy and subsequently received a successful second cadaveric renal transplant. CONCLUSIONS: The mechanism of this complication is a result of the intra-abdominal placement of the kidney, length of the vascular pedicle, excess ureteral length, and paucity of adhesions secondary to steroid administration. These factors contribute to abnormal mobility of the kidney. Technical modifications such as minimizing excess ureteral length and nephropexy may help to avoid this complication.
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2/638. Renal granulomatous sarcoidosis in childhood: a report of 11 cases and a review of the literature.

    We analysed retrospectively 11 children with renal granulomatous sarcoidosis confirmed by renal histology in order to describe the course and prognosis of the disease. Symptomatic sarcoidosis was diagnosed at a mean age of 10.1 years. Nine children had renal involvement at the time of diagnosis. In the course of the disease, nine patients developed renal failure and mild proteinuria, seven had transient sterile leukocyturia, four showed microscopic haematuria, seven had a urinary concentrating defect, and enlarged kidneys were seen in three patients. One child had hypercalcaemia and hypercalciuria, none had hypertension. light microscopy of the kidney showed interstitial infiltration by mononuclear cells in all children, interstitial fibrosis in nine patients, epithelioid granulomas in seven, tubular involvement in eight, and mild glomerular involvement in seven patients. Renal immunofluorescence was negative. Ten children received prednisone for 1-11 years. After a mean follow up of 5.5 years, three patients had entered end-stage renal failure and one had chronic insufficiency after interruption of medical supervision and prednisone therapy. CONCLUSION: Renal failure, proteinuria, leukocyturia, haematuria, and concentration defect are the prominent features of renal granulomatous sarcoidosis in children. Steroid therapy, adjusted according to disease activity, may prevent end-stage renal failure.
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3/638. cyclosporine disposition and long-term renal function in a 500-pound kidney transplant recipient.

    Patient size has been suggested as a risk factor in kidney transplantation. We have followed a recipient of a cadaver kidney who became massively obese (232 kg, 511 lbs) 5 years posttransplantation. He has maintained stable renal function with no rejection episodes and at 5 years has a measured serum creatinine of 2.2 mg/dL, creatinine clearance 42 mL/min, and urinary protein excretion of 320 mg/24h. Both oral and intravenous cyclosporine (Sandimmune) pharmacokinetic studies were done on a steady-state dose of 150 mg, which represents 0.65 mg/kg per dose. The patient exhibited very high bioavailability, F = 95%, and an oral elimination T1/2 of over 21 hours. These data confirm that stable cyclosporine delivery in very obese recipients can be sustained by dosing normalized to the ideal body weight and trough level monitoring.
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4/638. renal artery rupture secondary to pretransplantation Candida contamination of the graft in two different recipients.

    Infected graft transplantation is an unwelcome complication that may lead to serious consequences in the immunosuppressed host. It can be caused by infection of the donor or by contamination of the organ during harvest, preservation and handling, or at transplantation. With current donor evaluation protocols, the risk of transmitting infections by exogenous contaminated grafts seems to be more frequent than true donor-transmitted infections. Nevertheless, although rare and usually free of clinically significant sequelae, if contamination is by some virulent organisms such as staphylococcus aureus, gram-negative bacilli, or fungi, severe complications may occur. We report the clinical outcome of liver, heart, and kidney recipients from a single donor. Both renal allografts had to be removed because of renal artery rupture secondary to candida albicans infection. Careful donor evaluation before transplantation, unusually early presentation of mycosis leading to anastomotic renal artery disruption, the histopathologic findings of the grafts, and the absence of Candida infection in the liver and heart recipients make us believe that exogenous contamination of the grafts occurred during donor procedure, kidney processing, or at transplantation. In summary, because infected grafts can lead to serious complications, besides careful donor screening, it is important to achieve early recognition of contaminated organs by culturing the perfusate to start specific antibiotic or antifungal therapy after transplantation if necessary and avoid the rare but, in this case, fatal consequences of these infections.
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5/638. diagnosis and management of primary hyperoxaluria type 1 in infancy.

    We report a case of a 6-month-old infant who presented with failure to thrive due to end-stage renal disease as a result of primary hyperoxaluria type 1. The infant was managed with a combined daily hemodialysis and peritoneal dialysis prescription in order to manage the total body oxalate burden. Medical management included oral pyridoxine, aggressive hydration and nutritional supplementation via an enteral feeding tube. At one year of age the infant underwent a combined liver/kidney transplantation with intra- and daily post-operative hemodialysis to prevent oxalate deposition in the newly transplanted organs. The post-operative course was complicated by gross hematuria and increased hyperoxaluria, requiring an increase in hydration and thiazide diuretics. This infant received a combination of dialysis modalities which was designed to lower the potential oxalate burden prior to transplantation. This case illustrates the difficulty in medical management of an infant pre- and post-combined liver/kidney transplantation.
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6/638. Oxalate kinetics and reversal of the complications after orthotopic liver transplantation in a patient with primary hyperoxalosis type 1 awaiting renal transplantation.

    We present the case of a young woman with end-stage renal disease secondary to primary hyperoxaluria type 1, who after 3 years and 6 months of maintenance hemodialysis, and despite intensification of the dialytic treatment, developed severe livedo reticularis in her extremities leading to ischemic cutaneous ulcerations, necessitating continuous intravenous infusion of narcotics for pain control. She received a liver transplant after native hepatectomy. However, due to positive crossmatch, she could not receive a kidney from that donor. After transplantation, following serial serum oxalate levels, the hemodialysis regimen was safely reduced from 4 h daily to 3 h three times weekly. Over the course of 6 weeks after liver transplantation, her livedo reticularis resolved, the ischemic ulcers markedly improved, she was weaned off all pain medications, and her erythropoietin-resistant anemia resolved. Our results suggest that in patients with primary hyperoxaluria type 1, who have received a liver transplant and are on maintenance hemodialysis, after serial serum oxalate determinations, some may safely be changed to a thrice-weekly maintenance hemodialysis regimen. Moreover, with this regimen the complications of systemic oxalosis can reverse.
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7/638. A morbid course in a girl with mixed connective tissue disease.

    We describe an 18-year-old girl with a 13-year history of mixed connective tissue disease whose clinical course was unique: she ultimately developed end-stage kidney and bowel disease that led to a protracted morbid clinical course. We report this case to alert pediatric nephrologists to the importance of early recognition of possible intestinal disease in these patients. Unfortunately, no therapy is currently known to reverse the pathological process in the bowel, but multiorgan transplantation might be an option if the numerous medical complications of end-stage bowel disease can be successfully controlled.
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8/638. Autosomal dominant polycystic kidney disease associated with familial sensorineural deafness.

    Autosomal dominant polycystic kidney disease (ADPKD) is characterized by both renal and non-renal disorders. Extrarenal involvement includes noncystic manifestations such as cardiovascular abnormalities, colonic diverticula and intracranial aneurysms. Familial sensorineural hearing loss (SNHL) has been included in the definition of Alport's syndrome. However, other types of nephropathy have been occasionally associated with hereditary deafness. The association of ADPKD with hereditary SNHL has not been previously documented. We report a family with ADPKD associated with bilateral sensorineural deafness in a pedigree of four affected members in four generations.
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9/638. Successful living related simultaneous pancreas-kidney transplant between identical twins.

    Simultaneous pancreas-kidney transplant from living donors has been recently proposed as an effective therapeutic option in selected uremic patients with type I diabetes. We report the first simultaneous pancreas-kidney transplant performed between identical twins. Posttransplant, the recipient has been maintained on low dose cyclosporine to avoid recurrent auto-immune insulitis. At the 1-year follow-up, both donor and recipient are well with normal renal function and excellent glucose control. Simultaneous pancreas-kidney transplant between identical twins can be performed successfully using cyclosporine to prevent recurrent auto-immune insulitis.
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10/638. Heavy chain deposition disease: the disease spectrum.

    A 45-year-old white woman was found to have microscopic hematuria during her annual physical examination. After a negative urologic workup, she returned 5 months later with nephrotic syndrome, renal insufficiency, and hypocomplementemia. Renal biopsy showed a nodular sclerosing glomerulopathy that could not be further characterized because of inadequate tissue for immunofluorescence. The patient returned 8 months later with chronic renal failure. A repeat renal biopsy showed deposits composed of immunoglobulin g (IgG) heavy chain and complement components C3 and C1 along glomerular, tubular, and vascular basement membranes, with negativity for kappa and lambda light chains, findings consistent with heavy chain deposition disease (HCDD). The heavy chain subclass was exclusively IgG3. Staining with monoclonal antibodies to epitopes of the constant domains of IgG heavy chain showed a CH1 deletion, indicating a truncated heavy chain. On review of the previously reported cases of HCDD, common clinical presentations include nephrotic syndrome, renal insufficiency, hematuria, and, in some cases, hypocomplementemia. In most patients, the hematologic disorder is mild, without overt myeloma. light microscopy shows a nodular sclerosing glomerulopathy, and heavy chain deposits are detectable within basement membranes throughout the kidney by immunofluorescence and electron microscopy. There is no effective treatment for this condition, and virtually all patients progress to chronic renal failure.
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