Thomas Shaw-Stiffel

Pathophysiologic observations and histopathologic recognition of the portal hyperperfusion or small-for-size syndrome

In an attempt to more completely define the histopathologic features of the portal vein hyperperfusion or small-for-size syndrome (PHP/SFSS), we strictly identified 5 PHP/SFSS cases among 39 (5/39; 13%) adult living donor liver transplants (ALDLT) completed between 11/01 and 09/03. Living donor segments consisting of 3 right lobes, 1 left lobe, and 1 left lateral segment, with a mean allograft-to-recipient weight ratio (GRWR) of 1.0±0.3 (range 0.6 to 1.4), were transplanted without complications, initially, into 6 relatively healthy 25 to 63-year-old recipients. However, all recipients developed otherwise unexplained jaundice, coagulopathy, and ascites within 5 days after transplantation. Examination of sequential posttransplant biopsies and 3 failed allografts with clinicopathologic correlation was used in an attempt to reconstruct the sequence of events. Early findings included: (1) portal hyperperfusion resulting in portal vein and periportal sinusoidal endothelial denudation and focal hemorrhage into the portal tract connective tissue, which dissected into the periportal hepatic parenchyma when severe; and (2) poor hepatic arterial flow and vasospasm, which in severe cases, led to functional dearterialization, ischemic cholangitis, and parenchymal infarcts. Late sequelae in grafts surviving the initial events included small portal vein branch thrombosis with occasional luminal obliteration or recanalization, nodular regenerative hyperplasia, and biliary strictures. These findings suggest that portal hyperperfusion, venous pathology, and the arterial buffer response importantly contribute to early and late clinical and histopathologic manifestations of the small-for-size syndrome.

Acute Hepatitis C in a Contemporary US Cohort: Modes of Acquisition and Factors Influencing Viral Clearance

BACKGROUND Acute hepatitis C virus (HCV) infection is often asymptomatic; thus, its epidemiology and natural history are difficult to define. METHODS Acute HCV infection was identified on the basis of HCV seroconversion within 1 year (n=45), new anti-HCV seropositivity with clinical acute hepatitis (n=21), or HCV strain sequencing after an iatrogenic exposure (n=1). Risk factors were assessed with a baseline questionnaire, and participants were followed up prospectively with serial measurement of viral loads. RESULTS Of 67 persons with acute HCV infection, most were asymptomatic (64%) and injection drug users (66%). Thirteen had an unknown mode of transmission; of these, 11 reported high-risk sexual behavior. Ten acquired acute HCV infection within 3 months of an iatrogenic exposure; 3 had confirmed iatrogenic infection, and 4 had no other risk factors identified. The spontaneous viral clearance rate after 6 months of infection was 18% (95% confidence interval, 11%-31%). The rate of viral clearance varied significantly by sex (34% vs. 3% for women vs. men; P<.001). CONCLUSIONS High-risk sexual or iatrogenic exposures may be important contemporary risk factors for HCV infection. The spontaneous viral clearance rate (18%) in this contemporary study was similar to that reported for past studies of transfusion-associated HCV infection. Women were more likely to clear acute HCV infection than men.

Budd-Chiari syndrome: in evolution

Budd-Chiari syndrome (BCS) is a rare but potentially life-threatening disorder caused by hepatic venous obstruction, distinct from cardiac causes of hepatic congestion or sinusoidal obstruction syndrome (formerly known as veno-occlusive disease). BCS may be classified as primary or secondary, depending on the underlying process. Most cases of primary BCS are due to an underlying hypercoagulable disorder. A high index of suspicion is required to make the diagnosis. In most case series, chronic, indolent cases of BCS are more common than acute presentations. Doppler ultrasound, magnetic resonance imaging (MRI), and direct venography are useful in confirming the diagnosis. Systemic anticoagulation should be started expeditiously, as long as there are no contraindications. The use of systemic thrombolysis remains controversial. However, thrombolysis may prove effective when it is administered locally following hepatic venoplasty with or without stenting. Guidelines for the management of more complex cases and of patients who fail medical management are currently in evolution. Budd-Chiari syndrome (BCS) is potentially life-threatening, depending on the extent and rapidity of hepatic venous obstruction. A high index of suspicion is required to clinch the diagnosis, since BCS may be quite indolent or even asymptomatic. Doppler ultrasound or magnetic resonance imaging (MRI) is usually definitive. Systemic anticoagulation should be offered to all patients, unless contraindicated. The role of thrombolysis in BCS remains controversial, and thus it should be reserved for cases undergoing hepatic decompression via percutaneous angioplasty. Guidelines for the management of cases who fail standard medical management are currently in evolution.

Adult liver transplant survey: policies towards eligibility criteria in Canada and the United States 2007.

Goals: To assess the current practice patterns of liver transplant centres in Canada and the USA regarding transplant eligibility.

Diffuse Desmoplastic Metastatic Breast Cancer Simulating Cirrhosis with Severe Portal Hypertension: A Case of “Pseudocirrhosis”

Hepatic metastases from breast cancer can occasionally mimic cirrhosis anatomically, in both the presence and the absence of prior systemic chemotherapy [1–3]. We present a case of diffuse desmoplastic metastatic breast carcinoma (Ca) masquerading as cirrhosis that presented with newonset jaundice associated with severe portal hypertension and its sequelae. This case also serves as a background for a discussion of both the role of 18FDG-PET scanning in metastatic breast cancer and the entity of tamoxifen-induced hepatotoxicity.

Liver transplantation in mushroom poisoning.

Liver transplantation plays an important role in the treatment of patients with fulminant hepatic failure (FHF). Early determination of prognosis in cases of FHF is important to allow prompt decision-making regarding the need for liver transplantation. Mushroom poisoning is a rare cause of FHF, and as a result, prognostic criteria are not well recognized. It appears that the severity of coagulopathy and encephalopathy predicts a poor outcome, whereas the degree of bilirubin elevation may not. We present a case of FHF related to mushroom poisoning that required liver transplantation. The clinical presentation, medical management, and prognostic criteria in mushroom poisoning are discussed.

Acute liver failure as an initial manifestation of an infiltrative hematolymphoid malignancy.

The liver is a very common site for metastasis of tumors, with some suggesting hepatic involvement in 36% of all deaths due to cancer (1). In spite of this, liver dysfunction as a result of cancer involvement is rare until the terminal stage of the illness (2). There are sporadic case reports describing the occurrence of acute liver failure (ALF) due to malignant infiltration of the liver as the initial manifestation of disease. Hematologic malignancies are the most common among them, including Hodgkin’s disease (3, 4), non-Hodgkin’s lymphoma (5–9), malignant histiocytosis (10–12), and acute and chronic leukemia (13–15). Even rarer is any other infiltrative disease such as adenocarcinoma, melanoma, and metastatic anaplastic tumors (16–22). Fulminant hepatic failure is defined as significant liver dysfunction, with synthetic dysfunction and encephalopathy, developing within 8 weeks of the onset of symptoms, in the absence of preexisting liver disease (18, 23–27). There are approximately 2000 cases of ALF yearly in the United States, with an overall mortality of 80% (23, 27). It is a severe condition associated with many diseases, most commonly viral and drug-induced hepatitis (23, 24). Less common causes include toxins (carbon tetrachloride, Amanita), vascular events (hepatic ischemia, venoocclusive disease, heatstroke), Wilson’s disease, Reyes syndrome, and acute fatty liver of pregnancy (23). It is rarely associated with infiltrative malignancies and even less likely to be the presenting feature in these cases (6, 9).