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Biomedical subjects

B van Hoek

Publications and source records attributed to B van Hoek.

16 recordsLinked to original sources

Temporary heterotopic auxiliary liver transplantation for fulminant hepatitis B.

BACKGROUND/AIMS: Orthotopic liver transplantation has been shown to improve survival in fulminant hepatic failure. However, after orthotopic liver transplantation life-long immunosuppression is necessary and graft complications may occur. METHODS: We employed heterotopic auxiliary liver transplantation in a 26-year-old man with fulminant hepatic failure due to hepatitis B virus infection. RESULTS: From a comatose state with seizures and decerebrate posturing, the patient woke up the day after heterotopic auxiliary liver transplantation. The graft functioned sufficiently. After 2 weeks, when peritonitis developed, immunosuppression was stopped since the native liver was recovering as shown by serial HIDA scans, liver biopsies, clotting parameters and serum bilirubin. When severe rejection of the graft developed 2 weeks later, and the peritonitis had been treated successfully, the native liver had recovered sufficiently to allow the graft to be removed. Now, more than 1 year after heterotopic auxiliary liver transplantation, the patient is free from medication and he is immune for hepatitis B virus, his liver tests have returned to normal and he has regained his normal life. CONCLUSIONS: Temporary heterotopic auxiliary liver transplantation for fulminant hepatitis B is feasible.

Acute Disease

Energy expenditure and substrate metabolism in patients with cirrhosis of the liver: effects of the pattern of food intake.

Patients with liver cirrhosis are often undernourished. In healthy subjects, the pattern of food intake is one of the variables that can influence energy balance and substrate metabolism. The short term (two day) effect of the pattern of food intake in patients with cirrhosis and controls was compared. In a respiration chamber, eight patients with cirrhosis of the liver and 23 controls were fed to estimated energy balance in two meals daily ('gorging' pattern) and four to seven meals daily ('nibbling' pattern). Twenty four hour energy expenditure, expressed as a multiple of the sleeping metabolic rate, was reduced in patients with cirrhosis (1.31 (0.03) v 1.44 (0.02) for controls; p < 0.01) because of an increased sleeping metabolic rate per kg fat free mass in these patients. In both patients and controls, the respiratory quotient was significantly lower during the morning preprandial period (9.00-12.00) on the gorging pattern, reflecting a higher oxidation ratio of fat to carbohydrate compatible with a more catabolic state. For patients with cirrhosis, a nibbling pattern of food intake, including a good breakfast and a late evening meal, would be preferable, in order to have shorter episodes of catabolism during the day.

Adult

The role of portosystemic shunts for variceal bleeding in the liver transplantation era.

OBJECTIVE: To review our experience with portosystemic shunts during the era of liver transplantation at the Mayo Clinic to provide insight into the selection of patients for these procedures. DESIGN: We reviewed the charts of a cohort of 57 patients who underwent portosystemic shunting between 1985 and 1990 for the management of variceal bleeding. A follow-up survey by letter and telephone was also conducted. SETTING: The Mayo Clinic, a tertiary referral center. PATIENTS: These patients were not considered transplantation candidates at the time of the shunt because of active alcoholism, extensive portal vein thrombosis, coexistent myelodysplastic syndromes, or malignant neoplasms. INTERVENTION: Portosystemic shunts; the exact type was at the surgeon's discretion. OUTCOME: Survival after shunt surgery among patients with various liver diseases. RESULTS: Twenty-two patients died during follow-up after being shunted for bleeding, and one of the shunted patients subsequently required liver transplantation. Comparison of Kaplan-Meier survival curves between various groups of patients indicated that patients who were actively alcoholic had a poorer chance of survival (P < .003) than did those who were abstinent. Patients with portal vein obstruction or chronic cholestatic liver disease appeared to do better after shunt surgery than did patients with other causes of portal hypertension. Other factors such as age, Child-Pugh score, or presence of malignant neoplasms did not reliably predict outcome from portosystemic shunts. CONCLUSIONS: In well-selected patients who may not be deemed candidates for liver transplantation, portacaval shunts can be effectively employed to prevent bleeding from esophageal varices that are resistant to obliteration by sclerotherapy.

Cause of Death

Ampullary carcinoid and neurofibromatosis: case report and review of the literature.

A 49-year-old woman is reported with an ampullary carcinoid and von Recklinghausen's neurofibromatosis, presenting with melaena. On upper GI-endoscopy a tumour of the ampulla of Vater was seen. Histochemical examination revealed a carcinoid tumour. A review of the literature shows that patients with von Recklinghausen's neurofibromatosis are at increased risk for developing tumours of neuroectodermal origin, with the ampulla of Vater as predilection site. Therefore, early diagnostic evaluation, primarily by gastroduodenoscopy, for malignancies in patients with von Recklinghausen's neurofibromatosis and abdominal discomfort is recommended. Surgical removal is the only therapy so far evaluated.

Ampulla of Vater

Hepatic allograft rejection: new developments in terminology, diagnosis, prevention, and treatment.

Hepatic allograft rejection remains a major cause of morbidity related to the need for increased immunosuppression and continues to be a principal cause of late failure of the graft. Hepatic allograft rejection is defined on the basis of morphologic findings; cellular rejection is defined as portal or periportal hepatitis with nonsuppurative cholangitis or endotheliitis (or both), and ductopenic rejection is defined as loss of interlobular and septal bile ducts, typically in at least 50% of the portal tracts. The overall incidence of episodes of cellular rejection, which usually occur within the first 2 weeks after liver transplantation, varies from 50 to 100%. Ductopenic rejection occurs in approximately 8% of patients who undergo initial liver transplantation and is usually diagnosed between 6 weeks and 6 months after transplantation. Induction and maintenance immunosuppression with triple-drug (cyclosporine, prednisone, and azathioprine) therapy and other combinations that include antilymphocyte preparations, however, has decreased the incidence of both cellular and ductopenic rejection. In patients experiencing episodes of cellular rejection, high-dose intravenously administered corticosteroid therapy yields the best response and is associated with a lower incidence of ductopenic rejection than is low-dose and orally administered corticosteroid therapy. The correlation between degree of biochemical liver dysfunction and presence of histologic rejection is minimal early after transplantation. Histologic severity of rejection, however, suggests which patients will require more immunosuppression and which patients may need antilymphocyte therapy for controlling the rejection episode. With the availability of new immunosuppressive agents, distinguishing patients at high risk for rejection is important. The goals for use of new immunosuppressive agents and regimens are to improve graft and patient survival, to decrease the incidence of cellular and ductopenic rejection, and to minimize side effects and complications.

Adrenal Cortex Hormones

Prognostic features and role of liver transplantation in severe corticosteroid-treated autoimmune chronic active hepatitis.

To identify prognostic features and to define the role of liver transplantation in severe autoimmune chronic active hepatitis, findings before and after corticosteroid therapy in 111 patients were correlated with outcome and compared with the findings in 24 patients who had been selected independently for liver transplantation. Patients whose condition deteriorated during corticosteroid treatment were younger (32 +/- 3 yr vs. 43 +/- 2 yr; p less than 0.02) than those who experienced remission, but no individual features predicted outcome. Patients in whom therapy failed required longer durations of continuous treatment than did those who experienced remission (60 +/- 14 mo vs. 20 +/- 12 mo; p = 0.001). Of 13 patients who did not experience remission within 4 yr, 9 (69%) ultimately deteriorated. Ascites developed more often in those patients whose therapy failed and who died of liver failure than in counterparts who survived (86% vs. 33%). Patients undergoing transplantation were similar to those whose treatment failed, but they died less frequently (8% vs. 56%, p less than 0.01). Indeed, the 5-yr survival rate after transplantation was comparable to that of patients who had entered remission (92% vs. 100%). Successive biopsy samples failed to disclose recurrent autoimmune hepatitis after transplantation. Human leukocyte antigens A1, B8 occurred more commonly in patients in whom treatment failed or who underwent transplantation (70% vs. 41%, p less than 0.05). We conclude that failure to achieve remission within 4 yr and the human leukocyte antigen A1, B8 phenotype are associated with poor prognosis. Manifestations of liver decompensation, such as ascites, in patients who have been unable to experience remission justify consideration of transplantation.(ABSTRACT TRUNCATED AT 250 WORDS)

Adrenal Cortex Hormones

Current concepts in cell-mediated hepatic allograft rejection leading to ductopenia and liver failure.

Hepatic allograft rejection is presently classified into acute and chronic rejection based on histological features, timing and reversibility. However, because features of both types of rejection can occur at any time, and in many combinations, the terms "acute" and "chronic" seem inappropriate in some instances. Thus the term "cellular rejection" better defines the histological features of portal hepatitis, nonsuppurative destructive cholangitis and endotheliitis, which are independent of time and response to therapy. Similarly, because progressive bile duct destruction leading to a decrease in the number of interlobular and septal bile ducts is the major histological feature of "chronic rejection," the term "ductopenic rejection," defined as the loss of bile ducts in 50% or more of portal tracts independent of time and reversibility, seems more appropriate. The pathogenesis of cell-mediated rejection has not been completely explained; however, direct immunocytic attack on small bile ducts and small arteries appear to be the major feature. The process may lead to bile duct loss ("ductopenia"). The pathogenetic role of foam-cell arteritis resulting in ischemic bile duct injury and the role of humoral mechanisms in causing ductopenic rejection awaits further clarification. In the past, irreversible ductopenic rejection occurred in approximately 10% of all patients who underwent their first liver transplantation; this figure, however, appears to be decreasing. The clinical features of irreversible rejection include persistent and progressive cholestasis; rising serum levels of bilirubin, alkaline phosphatase and gamma-glutamyltransferase; and a decrease in hepatic synthetic function. Ductopenic rejection can occur early (2 to 5 wk after liver transplantation) but most often develops between 6 wk and 6 mo after transplantation.(ABSTRACT TRUNCATED AT 250 WORDS)

Animals

Indomethacin treatment in a patient with lithium-induced polyuria.

Lithium intoxication causes polyuria, central nervous system manifestations, and ultimately stupor progressing to coma. Moreover, polyuria leading to hypernatraemia itself can progress to convulsions and coma. We present a patient with lithium intoxication who remained polyuric, hypernatraemic and somnolent despite normal serum lithium concentrations. After institution of indomethacin orally, polyuria and hypernatraemia disappeared and patient regained consciousness.

Coma

Inadvertent arteriovenous stenting during transjugular intrahepatic portosystemic shunt procedure and the importance of hepatic artery perfusion.

Penetrating the hepatic artery during transjugular intrahepatic portosystemic shunt (TIPS) is a relatively frequent but almost always benign complication. We report a patient in whom the right hepatic artery, originating from the superior mesenteric artery, was inadvertently catheterized and stented. The arteriovenous fistula was treated with a detachable balloon positioned within the hepatic artery. A second TIPS was attempted and successfully created during the same session. The patient died of cardiac failure, attributed to rapid resolution of massive ascites after TIPS with circulatory overload. At autopsy, the liver distal to the arterial occlusion balloon was infarcted, illustrating the importance of hepatic artery perfusion on liver cirrhosis.

Aged