Cases reported "Kidney Failure, Chronic"

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1/11. 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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2/11. Mobilization of lead from bone in end-stage renal failure patients with secondary hyperparathyroidism.

    BACKGROUND: It is now recognized that long-term exposure to even low levels of lead may increase bone lead content. lead can then be released in toxicologically significant amounts during critical states of increased bone turnover. methods: Two patients with end-stage renal failure, one on haemodialysis and the other on continuous ambulatory peritoneal dialysis (CAPD), had been exposed to lead and developed secondary hyperparathyroidism. An edetate calcium disodium (EDTA) test was performed in combination with haemofiltration or CAPD before and after parathyroidectomy. RESULTS: Before parathyroidectomy, both patients had low delta aminolaevulinic acid dehydrase (ALA-D) and high concentrations of chelated lead. After parathyroidectomy, there was a dramatic decrease in chelated lead and the ALA-D returned to normal. CONCLUSION: Secondary hyperparathyroidism increases mobilization of bone lead in dialysis patients with an elevated lead burden. This may cause toxic effects.
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3/11. liver-kidney-transplantation in type 1 primary hyperoxaluria: description and comments on a case.

    BACKGROUND: Primary hyperoxaluria leads to oxalosis, a systemic illness with fatal prognosis in uremic youngsters because of systemic complications. Case report: A 14-year old boy with primary type 1 hyperoxaluria who had a long-lasting history of nephrolithiasis and passed from normal renal function to end-stage renal disease within 7 months. MEASUREMENT of alanine: glyoxylate aminotransferase (AGT) catalytic activity in the liver biopsy disclosed very low activity which was not. responsive to pyridoxin., thus the patient entered onto a priority national waiting list for liver-kidney transplantation and a week later received a combined transplant. In order to increase body clearance of oxalate, the patient underwent medical treatment to increase urine oxalate solubility (sodium and potassium citrate oral therapy, magnesium supplementation and increase of diuresis) and intensive dialysis both before and after transplantation. comment: The medical approach to the treatment of this rare illness is discussed. Since the major risk for the grafted kidney is related to the oxalate burden, i.e. oxalate deposition from the body deposits to the kidney that becomes irreversibly damaged, treatment consists of increasing the body clearance of oxalate both by increasing oxalate solubility in the urine and with intensive dialysis performed both before and after combined transplantation. To the same extent (by limiting body oxalate deposits), a relatively early (native GFR 20-25 ml/minute) transplantation is advisable.
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4/11. Practical considerations in dialysis withdrawal: "to have that option is a blessing".

    Cessation of life-support treatment is an appropriate option for situations in which the burdens of therapy substantially outweigh the benefits. Decisions to withdraw dialysis now precede 1 in 4 deaths of patients who have end-stage renal disease. Guidelines have been recently published to assist clinicians in making these complex and emotionally charged determinations, and they include: relying on shared decision making by all participants, obtaining informed consent, estimating the prognosis on dialysis, adopting a systematic approach for conflict resolution of disagreements, honoring advance directives, and ensuring the provision of palliative care. These principles are discussed in relation to an elderly man with dementia whose family decided to terminate maintenance hemodialysis.
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5/11. Treatment of a patient with chronic renal failure with rituximab for a follicular lymphoma: safe and successful option of rituximab therapy.

    A 47-yr-old woman presented a chronic renal failure for 5 yr, with a creatinine clearance of 12 mL/min. In June 2002, she had a right axillary lymph node (of 4 cm diameter). A biopsy revealed a follicular lymphoma (histology: follicular small cleaved-cell). She had Ann Arbor stage III disease, with a high tumor burden according to the GELF criteria. She received rituximab as single first-line treatment (375 mg/m2 by intravenous infusion for a total of four dosages: days 1, 8, 15 and 22). Rituximab therapy was extremely well tolerated, and we obtained a partial response, 4 wk after completing the treatment. In January 2003, she received one maintenance course of rituximab. Six weeks after maintenance therapy, a complete response was achieved.
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6/11. Home nocturnal hemodialysis in children.

    OBJECTIVE: To describe the effect of home nocturnal hemodialysis (NHD) in North American children. STUDY DESIGN: Four teenagers underwent NHD for 8 hours, 6 to 7 nights/week, using either central venous lines or fistulae for periods of 6 to 12 months. blood flow approximated 200 mL/min, and dialysate flow was 300 mL/min; the dialysate contained potassium and phosphate. The procedure was remotely monitored. RESULTS: The children had unrestricted diets and fluid allowance and did not require phosphate binders. Persistent relative hypotension developed in 2 of 4 children. Weekly Kt/V urea values were consistently >10; other biochemical measures varied. quality of life and school attendance improved in 3 of 4 children. The workload and reported emotional burden of NHD was substantial. No significant complications occurred. Dialysate losses of calcium, phosphate and carnitine required supplementation. The annual cost per patient was dollar 64,000 Canadian, which represented a 27% savings compared with thrice weekly in-center hemodialysis. CONCLUSIONS: NHD is feasible in selected children, allows free dietary and fluid intake, and improves patient wellbeing. The burden on the family is substantial, and NHD requires support of a dedicated multidisciplinary team.
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7/11. c-reactive protein and anemia: implications for patients on dialysis.

    c-reactive protein (CRP) is an acute-phase reactant protein that increases significantly in the presence of intercurrent (concurrent) events such as infectious and inflammatory processes. Data indicate that CRP levels correlate with anemia parameters, higher levels being associated with an increased comorbidity burden, lower hemoglobin (Hb) levels, and higher Epoetin alfa dose requirements. This article explores the use of CRP monitoring in patients on dialysis, and the relationship to anemia outcomes.
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8/11. chlorpropamide overdose in renal failure: management with charcoal hemoperfusion.

    A potentially lethal chlorpropamide overdose in a patient with chronic renal failure on long-term hemodialysis was treated by two courses of charcoal hemoperfusion. hemoperfusion shortened the half-life clearance of the drug from a mean value of 93.6 to 3.4 hours. Calculation of the fractional extraction indicated that hemoperfusion reduced the body burden of the drug by 24% and 19% (mean values) during the first and second hours of treatment, respectively. We conclude that charcoal hemoperfusion should be considered a definitive therapeutic option in such cases.
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9/11. Encephalopathy in chronic renal failure responsive to deferoxamine therapy. Another manifestation of aluminum neurotoxicity.

    We describe a patient undergoing chronic hemodialysis who developed a neurologic syndrome consisting of seizures, progressive myoclonus, and mild dementia and who responded to chelation therapy with deferoxamine mesylate. Neither her serum nor bone aluminum concentrations indicated aluminum toxicity. However, the presence of a positive deferoxamine-infusion test was suggestive of an elevated body burden of aluminum. Treatment with deferoxamine resulted in marked clinical improvement in her neurologic status within two months. The utility of using the deferoxamine-infusion test rather than serum aluminum levels in evaluating aluminum toxicity in chronic renal failure is suggested.
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10/11. "Long-term" survival in light-chain myeloma with dialysis therapy alone.

    We report a case of a 59 year old woman who presented in end-stage renal failure with lambda (lambda) light-chain myeloma (LLCM). Despite a large tumour burden, and refusal to accept cytotoxic chemotherapy, she was commenced on continuous ambulatory peritoneal dialysis (CAPD). With dialysis therapy alone she has shown considerable hematological improvement and remains well 18 months after diagnosis. The extremely poor prognosis attributed to light-chain myeloma is largely due to death from uremia. As the natural history of this disease in patients offered dialysis therapy is unknown, dialysis should not automatically be withheld from patients with LLCM.
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