Liver histology as predictor of outcome in patients with acute liver failure.

Acute liver failure (ALF) is a clinical syndrome associated with significant morbidity and mortality with a highly unpredictable outcome. We retrospectively analyzed 71 ALF patients (53 males; mean age = 27.5 ± 15.6 years) that underwent transjugular liver biopsy (TJLB) at our institution. The aims of this study are (i) to report our experience with TJLB in these patients, and (ii) to examine the role of liver histology in predicting their outcome. We also compared the histopathological findings between TJLB and explanted liver specimens in 31 patients who underwent liver transplantation (LT). Biopsy specimens were satisfactory for histopathological analyses in 69 (97.1%) patients, confirmed the clinical diagnosis in 56 (81.2%) patients, and altered the diagnosis in 13 (18.8%) patients. Minor complications were encountered in four (5.6%) patients. Percentage of hepatocyte necrosis was the only histological parameter that has significant discriminatory prognostic value, with no survivors having >75% necrosis without LT. In conclusions, TJLB is a safe technique for obtaining liver tissue in both adult and pediatric patients with ALF. Histological characteristics, mainly etiological diagnosis and degree of hepatocyte necrosis may assist in clinical decision-making for need of LT in these patients.

Chronic HBV with pregnancy: reactivation flare causing fulminant hepatic failure.

Chronic HBV infection is a dynamic state of interaction between HBV, hepatocytes, and the immune system of the host. A series of reactivation flares and remissions may occur due to multiple causes. Among them, spontaneous reactivation and immunosuppressive drugs including steroids or cancer chemotherapy are well known. This is due to immune-mediated destruction of HBV-expressing cells following withdrawal of immunosuppressive effect. Few cases have been reported in females during postpartum period. We report a case of fulminant hepatic failure during pregnancy in a previously unrecognized hepatitis B positive female requiring emergent liver transplantation.

Multiple bile duct hamartomas mimicking diffuse hepatic metastasis: GI image.

Bile duct hamartomas (von Meyenburg complex) are the rare benign neoplasm of the liver due to dysembryogenesis; constituted historically, cystic dilatations of the bile duct encompassed by fibrous stroma. Usually, they are asymptomatic and are not detected on routine radiological examinations including ultrasound or CT scan. Magnetic resonance cholangiography has been suggested as the best investigation for their imaging diagnosis. Their presence can cause diagnostics confusion and complicate the patient's management. We report a 45-year-old female with symptomatic cholelithiasis, whose liver on laparoscopy mimicked multiple hepatic metastases.

Endovascular treatment of hepatic artery thrombosis following liver transplantation.

Hepatic artery thrombosis (HAT) is the most frequent vascular complication following orthotopic liver transplantation. Urgent retransplantation has been considered as the mainstay therapy. Surgical revascularization is an effective alternative in asymptomatic patients. Endovascular therapies including intra-arterial thrombolysis, percutaneous transluminal angioplasty (PTA), and stent placement have shown encouraging results in recent years; however, their use remains controversial because of potential risk of hemorrhage. Until June 2009, 69 cases were published in 16 reports describing therapeutic potential of endovascular modalities. Interventions were performed as early as within 4 h to as late as 120 days in patients ranging from 4 months to 64 years of age. Majority of published reports suggested the use of urokinase. Thrombolysis was successful in 47 out of 69 (68%) patients. Bleeding was the most common complication including fatal intra-abdominal hemorrhage in three patients. Twenty-nine out of 47 (62%) patients underwent further intervention in the form of PTA, stenting, or both. The follow-up patency ranged from 1 month to 26 months. In conclusion, whenever possible, efforts should be made to rescue the liver grafts through urgent revascularization (surgical and/or endovascular) depending on patient's condition and interventional expertise at the transplant center; reserving the option of retransplantation for failure, complications, and cases with severe clinical symptoms or allograft dysfunction.

Continuous intraarterial thrombolysis for early hepatic artery thrombosis following liver transplantation: case report.

Hepatic artery thrombosis remains one of the major causes of graft failure and mortality in liver transplant recipients. Urgent re-transplantation has been considered as mainstay therapy; however, even with re-transplantation mortality of more than 50% has been reported by many series. Early detection on Doppler ultrasonography and subsequent revascularization in asymptomatic patients can avoid graft loss. Endovascular therapy including intra-arterial thrombolysis, percutaneous transluminal angioplasty, and stent placement have shown encouraging results in recent years; nevertheless, their use remains controversial due to potential risk of bleeding. We present a case of early hepatic artery thrombosis following liver transplantation treated successfully with continuous transcatheter intra-arterial thrombolysis using tissue plasminogen activator (t-PA).

Ciliated hepatic foregut cyst: an increasingly diagnosed condition.

BACKGROUND: Ciliated foregut cysts of the liver are rare, with only 96 cases diagnosed since the first description in 1857. They are being increasingly diagnosed recently; the majority of the cases have been reported in the last 15 years. Although they bear a close resemblance to the simple cyst of the liver which has essentially a benign course, ciliated hepatic foregut cysts (CHFCs) can progress to malignancy with devastating consequences. It is imperative that this group of conditions be diagnosed and treated adequately. DATA SOURCES: This review includes discussion of the data from all the 96 reported cases from English and non-English literature. Analysis of the incidence rates, embryogenesis, growth, clinical features, risk of malignancy and the prognosis are highlighted systematically. The roles of various diagnostic modalities including ultrasound, CT, MRI, fine needle aspiration cytology (FNAC), immunohistochemistry and surgery are further discussed. RESULTS: The mean age of patients with CHFC was 48+/-12 years. The male/female ratio was 1.1:1. The majority of patients with CHFC (62%) were asymptomatic, and the common mode of presentation was right upper abdominal pain. The cysts occurred in the left lobe in 51 patients, with sole location in segment IV in 44, and in the right lobe in 26. The average size of the cysts was 3.6+/-2.12 cm. The majority of the cysts were unilocular, and only 7 cases were multilocular. Cyst contents were described as viscous or mucinous in 73 patients, whereas bilious fluid was noted in 3. Large cysts having squamous carcinoma were cited in 3 patients, and 2 had extensive squamous metaplasia without malignancy. Others had benign histopathology. CONCLUSIONS: Clinicians have become increasingly aware of CHFC. Imaging alone is not diagnostic per se, but when considered in the context of the global picture does provide important clues to the diagnosis. FNAC is diagnostic by the presence of the ciliated columnar aspirate but lacks sensitivity. Infantile presentation is usually accompanied by biliary communication and mandates a different surgical approach. The demonstration of malignant transformation in 3 cases and its fatal course emphasizes the need for surgical resection in all cases once the diagnosis is made.

Ciliated hepatic foregut cyst: an increasingly diagnosed condition.

Ciliated hepatic foregut cyst is a rare foregut cystic developmental malformation. It presents as a solitary cystic lesion in segment four of the liver. Histologically, it consists of four distinct layers; namely, the inner ciliate columnar epithelium, subepithelial connective tissue, smooth muscle layer, and an outer fibrous layer. Usually asymptomatic and detected incidentally, other modes of presentation can include portal hypertension, obstructive jaundice, and development of malignancy. We present a case of a young asymptomatic woman with a complex cyst in segment four of the liver, who underwent a laparoscopic resection, focusing our discussion on the review of the literature and the diagnostic dilemma encountered in these rare cases.

Assessing relative risks of infection and rejection: a meta-analysis using an immune function assay.

BACKGROUND: Long-term use of immunosuppressants is associated with significant morbidity and mortality in transplant recipients. A simple whole blood assay that has U.S. Food and Drug Administration clearance directly assesses the net state of immune function of allograft recipients for better individualization of therapy. A meta-analysis of 504 solid organ transplant recipients (heart, kidney, kidney-pancreas, liver and small bowel) from 10 U.S. centers was performed using the Cylex ImmuKnow assay. METHODS: Blood samples were taken from recipients at various times posttransplant and compared with clinical course (stable, rejection, infection). In this analysis, 39 biopsy-proven cellular rejections and 66 diagnosed infections occurred. Odds ratios of infection or rejection were calculated based on measured immune response values. RESULTS: A recipient with an immune response value of 25 ng/ml adenosine triphosphate (ATP) was 12 times (95% confidence of 4 to 36) more likely to develop an infection than a recipient with a stronger immune response. Similarly, a recipient with an immune response of 700 ng/ml ATP was 30 times (95% confidence of 8 to 112) more likely to develop a cellular rejection than a recipient with a lower immune response value. Of note is the intersection of odds ratio curves for infection and rejection in the moderate immune response zone (280 ng/ml ATP). This intersection of risk curves provides an immunological target of immune function for solid organ recipients. CONCLUSION: These data show that the Cylex ImmuKnow assay has a high negative predictive value and provides a target immunological response zone for minimizing risk and managing patients to stability.

Primary biliary cirrhosis in monozygotic and dizygotic twins: genetics, epigenetics, and environment.

BACKGROUND & AIMS: There is growing evidence that the interplay of genetic susceptibility and environmental factors leads to primary biliary cirrhosis (PBC). In particular, family members of an infected individual have up to a 100-fold higher risk of developing PBC. Although concordant rates for identical twins in other autoimmune diseases range between 25% and 50%, there are no such data on PBC. Accordingly, we evaluated the concordance of PBC in a genetically defined population of twin sets and evaluated the clinical characteristics between concordant subjects. METHODS: We identified 16 pairs of twins within a 1400-family cohort followed up by several centers worldwide, evaluated the diagnosis of PBC in all individuals, and determined the zygosity of sets reported as identical by the analysis of 2 highly variable HLA class II regions and 5 short tandem repeats. RESULTS: Eight of 16 sets of twins were monozygotic. In 5 of 8 monozygotic twin sets, both individuals had PBC (pairwise concordance rate, 0.63). Among the dizygotic twins (n = 8), no set was found to be concordant for PBC. Interestingly, the age at onset of disease was similar in 4 of 5 concordant sets of monozygotic pairs; however, there were differences in natural history and disease severity. CONCLUSIONS: The concordance rate of PBC in identical twins is among the highest reported in autoimmunity. However, discordant pairs were identified. The data show not only the role of genetics but also emphasize that either epigenetic factors and/or environment play a critical role.