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Biomedical subjects

B Portmann

Publications and source records attributed to B Portmann.

At least 73 records · Page 4Linked to original sources
PubMed1995

Pathology and biopsy diagnosis of the transplanted liver.

Over the past 30 years, liver transplantation has evolved from an experimental therapy to a routine procedure and most pathology textbooks have now a section dedicated to the pathology of liver transplant. Although there remain problems of biopsy interpretation due to the numerous post-transplant complications which can occur singly or in association, the major changes have been well characterized and are reviewed here, a particular attention being given to those features which are unique to or distinctive of the liver allograft. These include the outcome of donor fatty liver, reperfusion damage, massive haemorrhagic necrosis and the patterns of rejections, in particular the rarity and delayed onset of hyperacute rejection and the selective involvement of the small interlobular bile ducts and vascular endothelia in both acute and chronic graft rejection. "Functional" cholestasis with hepatocyte ballooning, cholangiolar cholestasis associated with sepsis and lesions of the larger bile ducts which may result from preservation, immune and/or ischaemic injury and closely resemble the changes observed in primary sclerosing cholangitis are also considered. Later in the post-transplant course, changes due to de novo or recurrent hepatitis have to be distinguished from those of late cellular rejection, protracted biliary complications, lymphoproliferative disorders, disease recurrence, in particular autoimmune chronic hepatitis, primary biliary cirrhosis and sclerosing cholangitis, differential diagnoses which often require interpretation of allograft changes in conjunction with all laboratory and clinical data available.

Bile Ducts↗
PubMed1994

Accuracy of radiology in detection of hepatocellular carcinoma before liver transplantation.

BACKGROUND/AIMS: Recurrence of hepatocellular carcinoma (HCC) after orthotopic liver transplantation (OLT) depends on tumor size and number; the accuracy of radiology in detecting HCC is uncertain. This study compared tumor size at radiological and pathological examination in 30 patients with HCC who underwent OLT. METHODS: Pre-OLT radiology included ultrasonography (US), computerized tomography (CT), and hepatic angiography (HA). Sensitivity of radiology was calculated by comparison with pathology. RESULTS: Radiology missed HCC in two patients but showed five with small (< 4 cm) and eight with large (> 4 cm) lesions. Multicentricity was shown in 15 cases at radiological examination and 24 at pathological examination, including one incidental and 4 of 5 radiologically small HCC. Mean tumor bulk was 3.4, 74, 338, and 1375 cm3, respectively, in patients with incidental, small, large, and multicentric HCC at radiology. Sensitivities of US, CT, and HA in detection of HCC (and multicentricity) were 80% (16.6%), 86.6% (58.3%), and 90% (58.3%). No recurrence was found in patients with incidental or small HCC; even when multicentric. CONCLUSIONS: US, CT, and HA identified patients with small or large tumor bulk, but sensitivity in detecting satellites was poor; their finding in patients with radiologically small or incidental tumors does not affect outcome. The failure of US, CT, and HA to detect satellites must be considered in patients undergoing partial hepatectomy for HCC.

Carcinoma, Hepatocellular↗
PubMed1994

Nodular regenerative hyperplasia of the liver graft after liver transplantation.

Nodular regenerative hyperplasia has not previously been reported in patients who have undergone orthotopic liver transplantation. In this report we describe the clinical, biochemical and histologic findings in nine liver transplant recipients in whom nodular regenerative hyperplasia developed between 6 and 144 mo (median, 64 mo) after transplantation. Six of the patients manifested features of portal hypertension (variceal bleeding, ascites, severe intractable peripheral edema), whereas three patients were asymptomatic at the time of diagnosis. All patients had elevated serum alkaline phosphatase (median, 269 IU; range, 159 to 1182 IU) and gamma-glutamyl transferase levels (median, 247 IU; range 104 to 1513 IU). Other liver function tests remained normal. In five patients, earlier biopsies had identified histological features of venous outflow block, consistent with azathioprine hepatotoxicity. All nine patients had been taking azathioprine up to the time of presentation. After withdrawal of azathioprine, liver function tests improved appreciably in five patients and histologic improvement was documented in four of these who underwent follow-up liver biopsies. In the remaining four patients progressive graft failure ensued and all subsequently underwent repeat liver retransplantation. At a certain stage, nodular regenerative hyperplasia, in the context of a liver graft, becomes an irreversible lesion, emphasizing the need for early identification and withdrawal of the agent.

Adult↗
PubMed1994

Hepatitis C and E in non-A non-B fulminant hepatic failure: a polymerase chain reaction and serological study.

A significant proportion of patients with fulminant hepatic failure have clinical, biochemical and histological features suggestive of acute viral hepatitis, without serological evidence of either hepatitis A or B. The contribution of hepatitis C to such cases of non-A non-B fulminant hepatic failure is presently uncertain while hepatitis E is well recognized as a cause of fulminant hepatic failure in endemic areas. Nested polymerase chain reaction for detection of both hepatitis C and E virus as well as two serological assays for anti-hepatitis C virus and anti-hepatitis E virus western blotting (both IgG and IgM) were performed on acute sera of 42 consecutive cases of non A, non B-fulminant hepatic failure and on convalescent sera of 17 of 20 patients who underwent orthotopic liver transplantation. Fresh liver tissue, obtained at the time of transplantation, was also studied by polymerase chain reaction in eight cases. Evidence of an acute hepatitis E virus infection (hepatitis E virus RNA amplified from serum by polymerase chain reaction or serum IgM positive to western blot) was found in eight patients. One patient had anti-HCV at presentation but assays on later sera proved negative. Convalescent sera and sera obtained after orthotopic liver transplantation were all negative to both anti-HCV assay systems, but HCV RNA was not found in either serum or liver tissue in any case.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1994

Impact of immunoprophylaxis and patient selection on outcome of transplantation for HBsAg-positive liver recipients.

We have studied the roles of immunoprophylaxis, patient selection policy and coexistent hepatitis D virus infection in the outcome of 56 HBsAg-positive elective liver transplant recipients. Twenty-nine unselected patients not treated with immunoprophylaxis formed group 1 and were compared to a recent consecutive series of 27 patients (group 2) in whom pre-transplant serological status was determined and who received immunoprophylaxis. One-year actuarial HBsAg serological recurrence rates were 48% in group 2 and 90% in group 1 with particular improvement in recipients who were either HBV DNA-negative or who had co-existent hepatitis delta virus infection. One-year patient survival has improved from 62% in group 1 to 86% in group 2 with improvements in hepatitis delta virus-negative and replicating recipients. Patients who have either co-existent hepatitis delta virus infection or are in group 2 have 1-year survival rates comparable to elective HBsAg-negative recipients (19/21 (90%), 22/27 (86%) vs 87%, respectively). In the event of recurrence, severe graft injury is diminished in recent patients and in those with coexistent delta infection who also have lower levels of circulating HBV DNA. Retransplantation for associated graft injury has a poor prognosis irrespective of administration of immunoprophylaxis. In elective liver recipients, immunoprophylaxis and/or hepatitis delta virus infection modulate hepatitis B virus recurrence and associated graft injury with consequent improvement in patient survival.

Adult↗
PubMed1994

Nodular hyperplasia surrounding fibrolamellar carcinoma: a zone of arterialized liver parenchyma.

We report a case of acetaminophen-induced liver necrosis in a 14-year-old girl. At autopsy, a 9 cm subcapsular nodule was present in the right lobe of the liver which showed distinct zonation: a central greyish white area of fibrolamellar carcinoma with a peripheral fleshy, tan-coloured rim ranging from 1 to 2 cm in thickness. This peripheral zone consisted of nodular, hyperplastic parenchyma resembling the changes seen in focal nodular hyperplasia, and stood out from the adjacent necrotic parenchyma. The sparing of this zone from the deleterious effects of acetaminophen provides indirect evidence of a predominantly arterial rather than portal blood supply to this region. The arterial supply was most probably derived from the tumour vasculature and may explain the parenchymal hyperplasia sometimes reported adjacent to a fibrolamellar carcinoma. Awareness of this phenomenon is essential when evaluating a needle biopsy, as sampling of this region may lead to a false negative diagnosis.

Adolescent↗
PubMed1994

FK 506 rescue therapy for intractable liver allograft rejection.

Intractable liver allograft rejection remains an important cause of graft loss. In this present study, we evaluated the role of oral FK 506 in 30 rejection episodes resistant to conventional cyclosporin-based triple immunosuppression in a series of 28 patients. Rejection was reversed in 11 (91.7%) of 12 patients for intractable acute rejection and in 10 (58.8%) of 17 patients for chronic rejection. A progressive decline in serum bilirubin was observed within 14 days in those successfully salvaged and a serum bilirubin of less than 200 micromol/l at the time of FK 506 conversion in the chronic rejection subgroup was found to be good predictor of response (specificity 100%, sensitivity 60%). New onset diabetes mellitus (29%) and reversible renal impairment (32%) were the commonest adverse events observed. Eleven (52%) of the responding patients successfully discontinued corticosteroid medication and are currently on FK 506 monotherapy. FK 506 therapy has a significant impact in the control of both intractable acute and chronic allograft rejection with an acceptable toxicity profile.

Acute Disease↗
PubMed1994

Orthotopic liver transplantation for acute hepatic failure in children.

Thirty children received 35 liver transplants for fulminant or late-onset liver failure between March 1988 and May 1993. Aetiology included non-A non-B hepatitis in 12, Wilson's disease in 8, drug-induced hepatic failure in 6, hepatitis B in 1, hepatitis A in 1, tyrosinaemia in 1 and congenital haemochromatosis in 1. Three patients were retransplanted, one each for hepatic artery thrombosis, non-A non-B graft reinfection, and chronic rejection. Two of these three patients received a third transplant for chronic rejection and hepatic artery thrombosis. One patient in the retransplant group survived. Overall, graft and patient survival at a mean follow-up of 17 months were 49% and 57%, respectively. Mortality was related to vascular complications in three patients (hepatic venous obstruction, portal vein thrombosis and hepatic artery thrombosis). Two patients died of primary sepsis (cerebral aspergillosis and cytomegalovirus (CMV) pneumonitis in association with graft-versus-host disease). Systemic sepsis and multiorgan failure was documented as a cause of death in four children and sepsis in association with chronic rejection in a further three patients. One child died of respiratory failure 4 weeks after transplantation. Mortality in eight children less than 2 years was 75% and this was significantly greater than for older children (P < 0.003, Mantel Cox). Earlier referral, even in the absence of a definitive diagnosis and particularly in children under 2 years is advisable and may improve survival.

Adolescent↗
PubMed1994

Emergency liver transplantation after Kasai portoenterostomy.

Three patients with stable liver function after Kasai portoenterostomy developed acute liver failure secondary to liver necrosis. Doppler ultrasound at presentation revealed reversed diastolic hepatic arterial blood flow. Two patients survived after urgent liver transplantation. Liver necrosis should be suspected in children with chronic liver disease presenting with fever and rapidly deteriorating liver function.

Emergencies↗
PubMed1994

Glycogenosis type IV: liver transplant at 12 years.

Hepatomegaly, the presenting feature of type IV glycogen storage disease at 20 months of age, regressed during childhood. The patient remained asymptomatic until 12 years of age when, after an episode of shock, septicaemia, and spontaneous peritonitis, liver transplantation was successfully performed.

Child, Preschool↗
PubMed1994

Fulminant hepatic failure resulting from coexistent Wilson's disease and hepatitis E.

Fulminant hepatic failure resulting from hepatitis E and coexistent Wilson's disease was diagnosed in a six year old girl six weeks after returning from a holiday in India. Wilson's disease was diagnosed on the basis of histological evidence of hepatocellular copper deposition, confirmed by biochemical estimation of liver copper concentration. Although severely damaged, the liver was non-cirrhotic. Hepatitis E virus (HEV) was diagnosed by nested polymerase chain reaction, the specificity of which was confirmed by direct sequencing of amplified DNA. Replication of HEV within the liver at the time of diagnosis was confirmed by selective amplification of the antigenomic strand of the virus obtained from total liver RNA. The patient had an orthotopic liver transplantation without recurrence of hepatitis and remains well at 19 months. Viral excretion, recorded by serial amplification of HEV RNA extracted from stool samples, persisted for 30 days after liver grafting. Severe vitiligo, present preoperatively, dramatically improved after liver grafting and institution of immunosuppressive treatment. This case suggests that viral infection may play a part in the acute decompensation seen in some cases of Wilson's disease.

Base Sequence↗
PubMed1994

Primary amyloidosis and severe intrahepatic cholestatic jaundice.

Liver involvement in systemic amyloidosis is frequent but is rarely of clinical importance. Five patients with severe cholestatic jaundice are described and an additional 20 from published reports are reviewed. The most frequent presenting symptoms were lethargy and abdominal pain, which were present for a median of 11 months before the onset of jaundice. Hepatomegaly, usually marked, was present in 92%, with ascites in 56% of the cases. The serum bilirubin concentration was noticeably high and the serum globulin low. Histology of the liver showed considerable perisinusoidal deposition with a slight predilection for the periportal area. Two patients presented with predominant centrilobular deposition. Congo red staining was not uniformly positive. A variety of treatment regimens was tried but median survival was only three months from the onset of jaundice.

Aged↗
PubMed1993

Monitoring of veno-occlusive disease after bone marrow transplantation by serum aminopropeptide of type III procollagen.

Differential diagnosis of hepatic complications after bone marrow transplantation (BMT) is often difficult. To assess whether serum concentrations of the aminopropeptide of type III procollagen (PIIINP) could facilitate diagnosis, we measured serum PIIINP, corrected for age by conversion to standard deviation scores (SDS), serially after BMT in 27 children. A preliminary study of 11 patients showed that a PNIIINP-SDS cutoff of 8.0 was an optimum for diagnosis of veno-occlusive disease (VOD). PIIINP-SDS was increased above cut-off 1-25 days before the onset of clinical signs in the 12 patients (4 from the preliminary group, the others from a group of 16 studied prospectively) who developed VOD, with subsequent changes in PIIINP-SDS mirroring the course of VOD. By contrast, PIIINP-SDS remained below cut-off in all other patients, including 7 with liver graft-versus-host disease and 3 with drug hepatotoxicity. PIIINP-SDS values greater than 8.0 predict, diagnose, and monitor VOD after BMT.

Bone Marrow Transplantation↗
PubMed1993

Orthotopic liver transplantation for hepatic complications of Wilson's disease.

Thirteen orthotopic liver transplantations were performed in 12 patients for hepatic complications of Wilson's disease between May 1988 and July 1992. Ten patients had fulminant hepatic failure and two chronic liver disease. One patient underwent retransplantation for liver abscess secondary to hepatic artery thrombosis. Nine patients survive at a median follow-up of 18 (range 6-31) months. Three patients have died: two from multiple organ failure and sepsis, one from B cell lymphoma. Postoperative complications included bleeding requiring laparotomy in two patients, renal impairment in five, bacterial septicaemia in three, fungal sepsis in two and acute cellular rejection in six. The nine surviving patients are well with normal liver function test results.

Adolescent↗
PubMed1993

Cyclosporin A treatment in primary biliary cirrhosis: results of a long-term placebo controlled trial.

BACKGROUND: Effective treatment for primary biliary cirrhosis (PBC) resulting in slower progression and improved survival remains elusive. Cyclosporin A (CyA), which has been so effective in preventing human allograft rejection, has shown promise in small numbers of patients in early studies. METHODS: Three hundred forty-nine patients with PBC were randomized to receive CyA, 3 mg.kg-1.day-1, or placebo in a multicenter study with follow-up for 6 years. The end point was death or liver transplantation. RESULTS: Cox multivariate analysis showed time from entry to death or transplantation was significantly prolonged (by up to 50%) in the CyA-treated group. Liver-related mortality was also significantly lower. However, a univariate analysis of survival showed no statistical differences between the two groups. Biochemical liver indices deteriorated more slowly in the CyA-treated group, but serum creatinine concentration was elevated > 150 mumol/L in 9%, necessitating permanent discontinuation in half of these. A reduction in the dose of CyA was required in 11% because of hypertension. CONCLUSIONS: CyA has some therapeutic potential in primary biliary cirrhosis, providing blood pressure and renal function are closely monitored.

Adult↗