Stephen B. Kurtz

Fulminant Wilson's disease treated with postdilution hemofiltration and orthotopic liver transplantation

A 22-yr-old woman presented with fulminant Wilsons disease. The diagnosis was suspected clinically and was later confirmed with chemical and pathologic studies. She presented with acute hepatic failure, hemolysis, and acute anuric renal failure. Postdilution hemofiltration and continuous arteriovenous hemofiltration with oral D-penicillamine allowed removal of a total of 95,700 micrograms of copper; 78,665 micrograms of the total were removed via postdilution hemofiltration alone. On the 57th day, the patient received successful liver and renal transplants. We found that the determination of serum copper was instrumental in the diagnosis of fulminant Wilsons disease, that postdilution hemofiltration allowed a rapid removal of copper in the presence of renal failure, and that, finally, orthotopic liver transplantation should be performed early in the clinical course of these patients. This patient is the longest survivor of this serious condition.

Postdilution Hemofiltration in the Management of Acute Hepatic Failure: A Pilot Study

We conducted a pilot study to assess the feasibility and efficacy of postdilution hemofiltration (PDHF) in the management of acute hepatic failure. From January 1984 through May 1986, we encountered seven patients with acute hepatic failure and entered these consecutive patients in the study; three had non-A, non-B hepatitis and one each had type B hepatitis, fulminant Wilsons disease (hepatolenticular degeneration), acute allograft (liver) failure, and acute fatty liver of pregnancy. Two of these seven patients were unable to undergo PDHF because of a precarious hemodynamic status. Of the five patients treated with PDHF, four had amelioration of hepatic encephalopathy; in two of these patients, a close temporal relationship was noted between the improvement and the procedure. Four patients had appreciable thrombocytopenia related to PDHF and bleeding complications. Our preliminary results support a possible role for PDHF as a temporary artificial liver support system for patients with acute hepatic failure.

The Impact of Hospital-Acquired Infection on Outcome in Acute Pancreatitis

BACKGROUND & AIMS Little is known regarding the impact of hospital-acquired infection (HAI) in acute pancreatitis (AP). We conducted a population-based assessment of the impact of HAI on outcome in AP. METHODS Patient data were obtained from the Cardinal Health Clinical Outcomes Research Database, a large population-based data set. Cases with principal diagnosis by International Classification of Diseases, ninth revision, clinical modification 577.0 (AP) between January 2004 and January 2005 were identified. These cases were linked with recently reported HAI data collected by the Pennsylvania Health Care Cost Containment Council. Identification of HAI was based on definitions set forth by the National Nosocomial Infection Surveillance System. We conducted a 5:1 multivariate propensity-matched cohort study to determine the independent contribution of HAI to in-hospital mortality, length of stay (LOS), and hospital charges. RESULTS From 177 participating hospitals, there were 11,046 AP cases identified. Eighty-two (0.7%) patients developed an HAI. Mortality in the overall AP population was 1.2% vs 11.4% among 405 matched non-HAI controls vs 28.4% among patients who developed HAI (chi(2) test, P < .0001). Fifteen percent of all deaths was associated with an HAI. Both average LOS and hospital charges were significantly increased among patients with HAI compared with matched non-HAI controls. CONCLUSIONS We determined that HAI had a major impact on mortality in AP. Patients who developed HAI also had significantly increased LOS and hospital charges. These differences were not explained by increased disease severity alone. Reducing HAI is an important step to improving outcome in AP.

Inaccurate Blood Flow Rate During Rapid Hemodialysis

Simulated hemodialysis with isotonic saline was performed to compare the requested blood flow rate (BFR-r) with the actual blood flow rate (BFR-a) delivered during rapid, efficient hemodialysis. Four different blood pumps and blood lines from three different manufacturers were used for the studies. BFR-r was set on each blood pump, and a timed outflow specimen from the dialysis circuit was used to measure the BFR-a delivered. BFR-r values of 200, 350, and 500 mL/min were used; the arterial pressure was set at -50, -250, and -325 mm Hg. BFR was determined every hour for 5 hours. At an arterial pressure of -50 mm Hg, the BFR-a was slightly higher than the BFR-r, and this did not vary over the 5-hour study period. When the arterial pressure was -250 mm Hg, the initial BFR-a was 95% of the BFR-r; at the end of the 5-hour study, this had declined to an average of 87% of the BFR-r. The largest discrepancy between BFR-a and BFR-r was at an arterial pressure of -325 mm Hg; the initial actual values averaged only 90% of the requested, and by the end of the 5-hour study, this value had declined to a mean of 78% of the BFR-r. The use of whole blood with a hematocrit value of 33% and the addition of venous resistance did not significantly affect these results.(ABSTRACT TRUNCATED AT 250 WORDS)

A Four-Year Comparison of Continuous Ambulatory Peritoneal Dialysis and Home Hemodialysis: A Preliminary Report

All patients who received initial dialysis therapy by either continuous ambulatory peritoneal dialysis (CAPD) or home hemodialysis between January 1979 and January 1983 were retrospectively compared for adequacy of dialysis, morbidity, and survival. In this study group, 30 patients had home hemodialysis and 21 patients had CAPD; the mean ages of the patients in these two groups were comparable. Both methods of treatment provided adequate dialysis, as shown by results of serial laboratory studies. The number of days of hospitalization per year at risk was twice as great for the patients on CAPD as for those on home hemodialysis; peritonitis was responsible for this difference. The survival was similar in both groups at 32 months of therapy. Death was clearly related to coexisting morbid events other than dialysis in the home hemodialysis group; however, one of the two deaths in the group on CAPD seemed to be indirectly related to the treatment of peritonitis. These findings suggest that CAPD, when compared with hemodialysis, (1) provides adequate dialysis, (2) is accompanied by greater morbidity (hospitalization), and (3) may introduce a morbid event (peritonitis) that may adversely affect survival.

Erosive Spondyloarthropathy in Long-Term Dialysis Patients: Relationship to Severe Hyperparathyroidism

We describe the development of a destructive, erosive spondyloarthropathy in three long-term dialysis patients (mean duration of dialysis, 96 months). In all three patients, the lesions caused symptomatic vertebral pain and developed during a period of only a few months. All patients had extremely elevated levels of immunoreactive parathyroid hormone, and two patients had evidence of severe hyperparathyroidism on bone biopsy specimens. Two patients who underwent subtotal parathyroidectomy had rapid relief of symptoms and no further radiographic evidence of progression of the spondyloarthropathy. The third patient refused subtotal parathyroidectomy and had pronounced progression of the destructive spondyloarthropathy in the cervical spine. The limited experience of others, along with our currently reported findings, strongly suggests that hyperparathyroidism plays a major role in the development of this disorder. Erosive spondyloarthropathy is increasingly recognized in long-term dialysis patients and may be a unique clinical and radiographic manifestation of severe hyperparathyroidism in this population.

Bilateral Localized Amyloidosis of The Ureter Presenting with Anuria

A patient with bilateral localized amyloidosis of the ureters is described. This is the first case to be reported in which anuria was the presenting symptom, the second case with bilateral involvement and the thirteenth case of amyloidosis localized to the ureter. Careful urologic and hematologic followup is indicated.

Elevated bone aluminum content in dialysis patients without osteomalacia

In almost all dialysis patients, bone aluminum content (BAC) is elevated in comparison with levels in normal subjects. Extremely high BAC (200 micrograms or more of aluminum per gram of bone) is significantly associated with classic aluminum-related osteomalacia. We noted three patients with elevated BAC but without histologic evidence of typical osteomalacia. Two of the patients had moderately severe osteitis fibrosa (hyperparathyroidism), and one patient had mixed uremic bone disease--predominantly hyperparathyroidism but some impairment of bone mineralization as well. As has recently been reported by others, the deferoxamine infusion test yielded unusual results in these patients. On the basis of our observations, we believe that an isolated measurement of BAC to determine whether aluminum-related osteomalacia is present has certain limitations. Aluminum-related bone disease can be accurately diagnosed only with use of bone histomorphometry. Elevated levels of immunoreactive parathyroid hormone may offer protection from the toxic effects of aluminum.

Results of Treatment of Center Hemodialysis Patients

Four hundred eighty-three patients were maintained by hemodialysis in an outpatient hemodialysis center at the Mayo Clinic between 1963 and 1977. Although only 18 patients had experienced a myocardial infarction and 6 had had a cerebral infarction before beginning dialysis, 30 subsequently had acute myocardial infarction and 45 had a stroke. These two complications accounted for 48 of the 98 deaths that occurred during maintenance dialysis. Despite such complications, 183 patients were employed, 124 remained active at home or at school, and 115 were totally disabled. Survival of patients maintained solely by dialysis was 52% at 5 years. For the group as a whole, including patients who received their first allograft, the survival rate at 5 years was 65%.

Deferoxamine-enhanced fecal losses of aluminum and iron in a patient undergoing continuous ambulatory peritoneal dialysis.

Aluminum-associated osteomalacia and transfusion-induced hemosiderosis developed in an anephric patient receiving long-term maintenance treatment with continuous ambulatory peritoneal dialysis. Intravenous administration of 1.0 g of deferoxamine led to marked increases in the fecal elimination of aluminum and iron. Dialysate removal of these same metals also increased but to a lesser extent. This indicates that the biliary/fecal route of excretion may contribute significantly to deferoxamine-induced losses of aluminum and iron in patients undergoing continuous peritoneal dialysis.