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Patients with hepatic iron overload who undergo orthotopic liver transplantation (OLT) have a worse 1-year survival than those who undergo transplantation for other indications; the long-term outcome in this population is unknown. The purpose of this study is to report long-term follow-up after OLT in a cohort of patients with hepatic iron overload. Five liver transplant centers in the United States reported follow-up data on 37 patients receiving a first liver transplant who had severe hepatic iron overload in their native livers. Kaplan-Meier 5-year survival among these patients was compared with survival data from all age-matched liver transplantations reported to the United Network for Organ Sharing (UNOS) over the same time period (1987 to 1993). The 5-year survival rate after OLT was 40% in the hepatic iron overload group compared with an overall survival rate of 62% for all patient groups from the UNOS registry (P =.0009). Although sepsis was the cause of 53% of all deaths occurring within the first year after OLT, cardiac complications accounted for 50% of the late mortality in patients with hepatic iron overload. In conclusion, long-term survival after OLT is significantly decreased in patients with hepatic iron overload. Infectious and cardiac complications are the most common causes of death in these patients. Further studies are needed to define the relationship between hepatic iron overload and mortality and to examine the effect of iron depletion on outcome after OLT in this patient population.

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Diabetes mellitus can lead to metabolic changes that alter normal hepatic and biliary function and structure. These alterations in hepatic and biliary function and structure are usually benign, but in certain situations lead to significant, disabling disease. This article reviews the hepatic and biliary complications of diabetes, with emphasis on epidemiology, diagnosis, and management, as well as the glucose intolerance seen in liver disease.

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Diabetic gastroparesis is a common, underrecognized disorder affecting both type I and type II diabetics usually in the setting of other diabetic complications. The pathogenesis of diabetic gastroparesis is poorly understood, but autonomic neuropathy appears to play a major role. The symptoms of gastroparesis are nonspecific, and complications such as hypoglycemia and bezoar formation due to gastric stasis may occur. Diabetic gastroparesis can be assessed by measuring the gastric emptying of solid radiopaque markers or by scintigraphy. Treatment approaches include optimizing glycemic control and the use of prokinetic agents. Combination therapy or the alternating use of prokinetic agents may prove to be beneficial in dealing with drug tachyphylaxis.

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Cirrhotic patients with hereditary hemochromatosis (HHC) have an increased risk of primary liver cancer (PLC). The purpose of this study was to determine the prevalence of primary liver cancer in patients with HHC undergoing orthotopic liver transplantation (OLT). Five liver transplant centers were surveyed; clinical and pathological data on 37 patients with HHC undergoing OLT were retrospectively collected and analyzed. The diagnosis of HHC was established by a combination of serum transferrin-iron saturation, hepatic iron index (HII), and/or pattern of liver iron staining. The diagnosis of HHC had been unsuspected before OLT in 13 of 37 (35%). Primary liver cancer was found in the explants of 10 of 37 patients (27%) and was unsuspected in 7 of 10 (70%); 8 were hepatocellular carcinoma, and 2 were cholangiocarcinoma; foci of hepatocyte dysplasia were found in 6 additional patients. Mean (+/- SEM) hepatic iron content and HII in 20 patients without prior phlebotomy or bleeding were 17.2 mg/g dry weight (+/- 2.9) and 5.5 (+/- 0.8), respectively. The overall 1-year survival rate after OLT in the 37 HHC patients was 58% (v 55% for HHC patients with PLC). We draw the following conclusions: (1) the diagnosis of HHC is often unsuspected before OLT, and HHC should be evaluated pretransplantation by direct and indirect markers; (2) HHC patients undergoing OLT have a high prevalence of primary liver cancer, the majority being unsuspected; and (3) HHC patients have poorer than average survival after OLT, which cannot be explained solely by the presence of concomitant PLC.

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Two patients with liver failure secondary to isoniazid hepatotoxicity were successfully treated with orthotopic liver transplantation. A 49-year-old man received isoniazid prophylaxis for a positive tuberculin test, and a 60-year-old woman was treated for active pulmonary tuberculosis with isoniazid, rifampin, and pyrazinamide. Both patients developed hepatic failure 4 and 1.5 months after initiation of antituberculous drug therapy, respectively. Liver transplantation was performed for progressive hepatic failure and was successful in both patients. The patient with active pulmonary tuberculosis was successfully treated with a modified antituberculous drug regimen while taking standard doses of immunosuppressive drugs after transplantation. In conclusion, liver transplantation is feasible and effective therapy for patients with isoniazid-induced hepatic failure, and active pulmonary tuberculosis may represent a relative rather than absolute contraindication to transplantation.

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Recent preliminary reports suggest a poor outcome of orthotopic liver transplantation for patients with hemochromatosis. We analyzed an institutional experience with orthotopic liver transplantation for hemochromatosis, focusing on factors contributing to increased morbidity and mortality. Between March 1988 and October 1992, nine of 249 adults (3.6%) undergoing orthotopic liver transplantation had hemochromatosis. Mean age was 53 yr (range, 42 to 62 yr), and eight of nine patients were men. The diagnosis of hemochromatosis was based on transferrin saturation > 62% and hepatic iron index > 2.0. Only two patients were known to have hemochromatosis before liver transplantation. All nine patients underwent standard cardiac evaluation before transplantation, and no patient had detectable pre-existing cardiac disease. One patient had a major operative cardiac complication as a result of pulmonary embolism and made a full recovery. Postoperatively, congestive heart failure developed in three patients and four patients had arrhythmias. One patient is undergoing phlebotomy for post-transplant cardiac complications from hemochromatosis. Two patients had primary hepatic tumors in the explant liver. There were four deaths caused by multiorgan failure with congestive heart failure (1), infection (2), and/or malignancy (2). Five patients are alive 3 to 25 mo post-transplant. The actuarial survival of the nine patients was 53% at 25 mo vs. 89% for 18 age- and sex-matched control transplant recipients (p = 0.1) and 81% for all other adult liver transplant recipients (p < 0.01). In five of seven patients, post-transplant liver biopsies revealed hepatic iron accumulation.(ABSTRACT TRUNCATED AT 250 WORDS)

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IgE-mediated acute and late phase reactions associated with occupational metal fume exposure are rare compared with the more common metal fume fever. The latter is a common acute industrial disease caused by inhalation of oxides of metals, especially zinc. It is a flu-like illness and is thought to be self-limited. This article describes the first case, to my knowledge, of an immediate and a late phase reaction (urticaria and angioedema) to zinc fumes, associated with a metal fume fever-like reaction. Hives and angioedema developed immediately and in a delayed fashion in a 34-year-old man after he had welded zinc at his job. There also was an associated metal fume fever-like reaction. The relationship was proved by a challenge test done at home. The patient has been asymptomatic since using complete protective measures while welding zinc. Possible immunologic mechanisms are discussed.

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The hydrolysis of methyl phosphate bound to the triethylenetetramine-cobalt(III) ion is much faster than the hydrolysis of either dimethyl phosphate bound to the same cation or methyl phosphate bound to the pentamminecobalt-(III) ion. The rate enhancement is attributed to bidenate coordination of the methyl phosphate. This feature suggests a pseudorotation mechanism analogous to that proposed by Westheimer for the hydrolysis of ethylene methyl phosphate. Stabilization of bidentate coordination might play a role in metal ion activation of phosphate-transfer enzymes.