Palliative care.
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Biomedical subjects
Publications and source records attributed to Richard D Swartz.
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BACKGROUND: Nephrogenic fibrosing dermopathy is a newly recognized cutaneous fibrosing disorder marked by the acute onset of induration involving the upper and lower limbs in patients with acute or chronic renal failure. The etiology, pathogenesis, associated clinical conditions (other than renal failure), and ultimate course have not been defined in the few cases studied. Presently, there is no effective treatment, and the condition persists in most patients. METHODS: Clinical and histopathologic data on 13 patients from our institution with the diagnosis of nephrogenic fibrosing dermopathy were reviewed. Several clinical and laboratory parameters were examined to see if any were consistently associated with the disease. Biopsy specimens were analyzed to determine if there was a pattern to the evolution of fibrosis in these patients. RESULTS: All 13 patients had renal failure before disease onset: 8 were undergoing chronic hemodialysis, 2 were undergoing chronic peritoneal dialysis, and 3 with acute renal failure had never undergone dialysis before the development of dermopathy. Most patients had other serious underlying medical conditions. Many patients were taking erythropoietin, cyclosporine, or both before the onset of disease. In transplant patients, no histocompatibility antigens were found to be associated with the disease. There were various laboratory abnormalities, but none were consistently associated with the condition. In skin biopsy specimens taken 7 to 180 days after disease onset, there were histopathologic changes suggestive of a tissue reaction to injury, as well as the development of smooth muscle actin-positive myofibroblasts. CONCLUSION: Nephrogenic fibrosing dermopathy is a novel cutaneous fibrosing disorder that is distinguished from other sclerosing or fibrosing skin disorders by distinctive clinical and histopathologic findings occurring in the setting of renal failure. There were no additional clinical risk factors or laboratory findings common to the 13 patients studied, other than renal failure. The resemblance to a tissue injury reaction and the presence of myofibroblasts in the tissue specimens suggest that fibrogenic cytokines may be involved in the evolution of the disease.
Medical professionals in nephrology are trained to think about improving quality and quantity of life with their patients, but only recently are they being urged to consider ways to improve end-of-life care. For many staff, this still feels contradictory. One nephrologist recently told these authors, "I was trained to help people live, not to help them die." The RWJF Promoting Excellence in End-of-Life Care work group has investigated, accumulated, and designed resources to help renal teams improve in this challenging arena. Peer mentoring is one such powerful resource, and, once in operation, is cost free. In conjunction with renal social work and the renal team, peer mentoring is a wonderful program that can decrease anxiety and preserve caring relationships in our burgeoning dialysis communities.
Dialysis access monitoring may help decrease thrombosis-related morbidity. We investigated the effect of time elapsed since an access flow measurement on test accuracy of a novel flow monitoring method called variable flow (VF) Doppler. A retrospective review was conducted in 36 patients with prosthetic grafts for vascular access using access thrombosis as the clinical endpoint. Receiver operator characteristic (ROC) curves and test sensitivity and specificity were determined for various follow-up time intervals. ROC analysis showed increasing test discrimination for shorter time intervals. Sensitivity and specificity for a commonly used surveillance threshold (600 ml/min) showed specificity that was little changed (88-93%) from follow-up time intervals of 15 days to 6 months. However, sensitivity was low (21%) at 6 months, increased to 50% at 2 months, 67% at 1 month, and 100% at 15 days (a single event). Low access blood flow using VF Doppler predicts near-term thrombosis. These data further imply that the discriminative value of access flow monitoring appears to be highly dependent on time from the flow measurement, improving with shorter time intervals from the measurement.