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Biomedical subjects

R R Schade

Publications and source records attributed to R R Schade.

At least 37 records · Page 2Linked to original sources

Hepatocellular carcinoma in Alagille's syndrome: a family study.

Alagille's syndrome is a common form of familial intrahepatic cholestasis. In addition to the hepatobiliary system, many other organ systems are affected. Most of the affected patients survive through adulthood. Hepatic involvement is the cause of death in about one-third of patients. Hepatocellular carcinoma complicating the course of this disease is very rare and has been reported previously in only three cases. We report a family in which three of four siblings with this syndrome developed hepatocellular carcinoma and died as a result of it. None of these children had a liver disease, other than Alagille's syndrome, that could account for the development of such a tumor. This experience suggests that Alagille's syndrome, or at least chronic cholestasis, may be a predisposing factor for the development of hepatocellular carcinoma. Annual determination of alpha-fetoprotein and abdominal computed tomography (CT) scan may detect the development of a hepatocellular carcinoma in such cases while they are still resectable.

Abnormalities, Multiple↗

Kaposi's sarcoma with visceral involvement in a young heterosexual male without evidence of the acquired immune deficiency syndrome.

A case of disseminated Kaposi's sarcoma (KS) in a 21-yr-old white heterosexual male with cryptogenic cirrhosis and no serological or immunological evidence of acquired immune deficiency syndrome (AIDS) is reported. The patient died 2 wk after diagnosis. Postmortem examination showed involvement of lymph nodes, liver, spleen, gastrointestinal tract, and bone marrow. This case demonstrates that Kaposi's sarcoma can occur in a young heterosexual male with normal immune function and in the absence of HIV infection.

Acquired Immunodeficiency Syndrome↗

Bleeding esophageal varices: treatment by sclerotherapy and liver transplantation.

Variceal hemorrhage is frequently a lethal event. Mortality among patients who have bled is high, with survival over the short term of only 25% to 50%. We retrospectively reviewed the records of 177 patients in whom variceal bleeding was treated with variceal sclerosis during a 5-year period from 1981 to 1986. All patients were treated by freehand injection of 25% sodium morrhuate with 35% dextrose, 4 ml per injection, through a fiberoptic endoscope. Of this group, 46 patients were treated with sclerosis followed by liver transplantation (group 1). These were compared to 36 nonalcoholic Child's class B and C patients treated with sclerosis alone (group 2). Survival at 4 years was poor in group 2 (17%). Liver failure and continued gastrointestinal bleeding were the most frequent causes of death. Survival among the liver-transplant group was significantly better (73%, p less than 0.001). Causes of death in this group were primarily due to sepsis, often in the setting of acute graft rejection. Group 1 patients were younger (39.8 +/- 10.8 vs 49.8 +/- 16.5 years, p less than 0.01); this difference is influenced by the deliberate selection of younger patients for liver transplantation. We conclude that sclerotherapy followed by liver transplantation significantly improves survival compared to conventional therapy in selected patients with advanced liver disease and portal hypertension. Donor organ availability will seriously limit the applicability of this approach to patients with bleeding esophageal varices.

Actuarial Analysis↗

Sclerotherapy of esophageal varices: long-term results and determinants of survival.

Between 1980 and 1986, 177 patients underwent sclerotherapy by means of the flexible fiberoptic endoscope for bleeding esophageal varices. Of these, 129 were treated with serial sclerotherapy alone. The remaining 48 patients underwent liver transplantation after sclerotherapy; these are reported separately. Patients were classified by Child's criteria, by the severity of the initial bleeding episode as reflected by the urgency of treatment, and by the nature of the underlying liver disease. Long-term survival rates were markedly influenced by Child's classification, with 83% of the patients in class A, 45% of those in class B, and 20% of those in class C surviving beyond 36 months (p less than 0.001). Urgent treatment was associated with a poorer survival than was elective treatment (p less than 0.001). Survival was not influenced by underlying alcoholic liver disease as compared to a nonalcoholic liver disease. The majority of deaths occurred within the first 100 days after the initial treatment. Child's class B and C patients had the highest early mortality rates, particularly in an acute treatment setting. The most frequent causes of death included progressive liver failure and persistent hemorrhage. Sclerotherapy for bleeding esophageal varices may successfully control hemorrhage, but the influence of this treatment on long-term survival is limited. Hepatic reserve, indicated by Child's classification, is the major determinant of survival. Significant improvements in survival after variceal bleeding are intimately linked to improvement in liver function.

Actuarial Analysis↗

Diagnostic and interventional radiology in liver transplantation.

Diagnostic and interventional radiology play major roles in the evaluation and management of liver transplant patients. This article begins with a discussion of imaging techniques used in the preoperative evaluation of transplant candidates. Surgical approaches and normal posttransplant anatomy are presented. An in-depth discussion of the radiologic evaluation of posttransplantation complications follows, with numerous illustrative cases. Finally, the role of interventional radiology in the nonoperative management of certain complications, especially biliary problems, is discussed.

Humans↗

Medical problems before and after transplantation.

Before and after liver transplantation in patients, medical complications are frequent. In this article, the occurrence of medical complications in liver transplant patients as well as their management are reviewed.

Acute Kidney Injury↗

Rapid growth of an intact human liver transplanted into a recipient larger than the donor.

Two individuals undergoing orthotopic hepatic transplantation received livers from donors who were on average 10 kg smaller than themselves based on recipient ideal body weight. As a result, the donor livers in these 2 cases were 29%-59% smaller than would be expected had the donor liver and recipient been matched ideally. The liver grafts in the recipients steadily increased in size, as determined by serial computed tomography scanning, to achieve new volumes consistent with those that would have been expected in a normal individual of the recipient's size, sex, and age. Fasting plasma levels of amino acids, glucagon, insulin, and standard liver injury tests were monitored to determine which measure best reflected the changes observed in the size of the grafts over time. No relationship between the changes observed in any of these parameters and hepatic growth was apparent. In both cases, the liver increased in volume at a rate of approximately 70 ml/day. These data demonstrate that a small-for-size liver transplanted into a larger recipient increases in size at a rate of approximately 70 ml/day until it achieves a liver volume consistent with that expected given the recipient's size, age, and sex.

Adolescent↗

Effects of renal impairment on liver transplantation.

To determine the incidence, prevalence, and prognostic value of preoperative and postoperative renal dysfunction occurring in adults undergoing orthotopic liver transplantation, the records of 102 consecutive adults who underwent orthotopic liver transplantation using cyclosporin A were reviewed. Renal dysfunction was defined arbitrarily as an increase in creatinine or blood urea nitrogen, or both, to 1.5 and 50 mg/dl, respectively, in patients previously having normal renal function or a 50% increase in either creatinine or blood urea nitrogen in patients with preexisting renal dysfunction. Twenty-six of the 102 patients had renal dysfunction before orthotopic liver transplantation. Sixty-eight of the 102 patients studied experienced an episode of renal impairment after orthotopic liver transplantation. Forty-nine of these episodes developed early, having occurred within the first 6 days. Late renal impairment occurred in 36 cases at 32 +/- 6 days after orthotopic liver transplantation. Using multivariate analysis, cirrhosis of a noncholestatic nature was found to be an independent predictor of early renal impairment. Trough blood cyclosporin A levels measured by radioimmunoassay were higher in those who experienced early renal impairment or late renal impairment than in those who did not (p less than 0.05). Several factors capable of adversely influencing renal function (nephrotoxic drugs, shock, and graft failure) other than cyclosporin A were present also in half of the patients who developed late renal impairment. Overall, 25 patients died. Multivariate analyses identified serious postoperative infection, graft failure, and preoperative renal dysfunction to be independent predictors of mortality.

Adult↗

Analysis of intestinal lymphocyte subpopulations in patients with acquired immunodeficiency syndrome (AIDS) and AIDS-related complex.

Lymphocyte subpopulations in the intestinal tissues of seven patients with acquired immunodeficiency syndrome (AIDS) or AIDS-related complex (ARC) were studied by immunohistologic technics at two different locations, the small bowel and the rectum. Intraepithelial and lamina propria lymphocyte subsets stained with monoclonal antibodies T11, T4, T8, and B1 were enumerated in the patients and different normal and patient control groups. Intraepithelial T11+ cells were decreased (P less than 0.05) in the small bowel of AIDS and ARC patients, primarily because of the near complete absence of T4+ lymphocytes. In the lamina propria of these patients, a depletion of T4+ cells (P less than 0.05), an increase in T8+ cells (P less than 0.05), and a reversal of the T4/T8 ratio were observed (e.g., the small bowel ratio was 0.1 +/- 0.02 vs. the normal ratio of 2.3 +/- 0.2 and the rectal ratios were 0.2 +/- 0.06 vs. normal 2.6 +/- 0.3). The T-lymphocytes in the intestine of AIDS and ARC patients did not express the receptor for interleukin-2 (IL-2). A near complete absence of T4+ lymphocytes was also seen in lymphoid follicles in the rectum. B1+ cells were not depleted. The reversal of the T4/T8 ratio, which is a hallmark of AIDS, occurs not only in the circulation but also in the gastrointestinal tissues of patients with AIDS and ARC.

AIDS-Related Complex↗

Neurobehavioral correlates of cholestatic and hepatocellular disease: differentiation according to disease specific characteristics and severity of the identified cerebral dysfunction.

Subjects with three different types of hepatic cirrhosis were compared on a battery of neuropsychological tests to determine if cerebral dysfunction varied according to the type of liver disease. It was found that the manifest cognitive impairments varied according to the type and etiology of the liver disease. These findings underscore the sensitivity of neuropsychological tests in the detection of hepatic encephalopathy, particularly in cases where there are no overt clinical signs or symptoms of neurologic disturbance. They also suggest that the nature and severity of the measurable neuropsychological abnormalities may vary within patient populations depending upon the etiology of the individual's liver disease and the specific pathophysiological mechanisms involved in its progression.

Cholangitis↗