Türkan Terkivatan

High prevalence of autoimmune hepatitis among patients with primary sclerosing cholangitis

BACKGROUND/AIMS Traditionally, autoimmune hepatitis (AIH) and primary sclerosing cholangitis (PSC) are regarded as separate disease entities. We report on a group of patients that suggests the existence of an overlap syndrome of the two conditions and on the prevalence of this syndrome among patients with PSC. Furthermore, the impact of the recently revised AIH scoring system for diagnosing AIH in this context was assessed. METHODS Retrospective analysis of consecutive patients of a tertiary referral centre for liver disease with a diagnosis of PSC. RESULTS Diagnosis of the overlap syndrome was established for nine patients (8%) of a total group of 113 PSC patients. Four patients initially presented with features of AIH and in five cases PSC was diagnosed first. All patients responded to immunosuppressive therapy; in three cases long-term remission was achieved. Three patients underwent liver transplantation after 4 months and 7 and 9 years, respectively. The original and revised versions of the AIH scoring system gave essentially the same results in the patients with the PSC-AIH overlap syndrome. CONCLUSIONS Patients with overlapping features of AIH and PSC may be more common than is currently assumed. Recognition of this syndrome is of clinical significance, considering the important therapeutical consequences.

Hepatocellular Adenomas: Correlation of MR Imaging Findings with Pathologic Subtype Classification

PURPOSE To investigate the correlation between magnetic resonance (MR) imaging findings and pathologic subtype classification of hepatocellular adenoma (HCA). MATERIALS AND METHODS This retrospective study was approved by the institutional review board, and the requirement for informed consent was waived. MR imaging studies of 61 lesions (48 patients; median age, 36 years) were available and were independently reviewed by two radiologists. Consensus readings on all morphologic and signal-intensity imaging features were obtained. Previously, these lesions had been classified on the basis of pathologic findings and immunohistochemical analysis. Fisher exact and χ² tests were performed to compare the results between the different subtypes. A Bonferroni correction was applied to correct for multiple testing (α < .0033). RESULTS MR imaging signs of diffuse intratumoral fat deposition were present in seven (78%) of nine liver-fatty acid binding protein (L-FABP)-negative HCAs compared with five (17%) of 29 inflammatory HCAs (P = .001). Steatosis within the nontumoral liver was present in 11 (38%) of 29 inflammatory HCAs compared with none of the L-FABP-negative HCAs (P = .038). A characteristic atoll sign was only seen in the inflammatory group (P = .027). Presence of a typical vaguely defined type of scar was seen in five (71%) of seven β-catenin-positive HCAs (P = .003). No specific MR imaging features were identified for the unclassified cases. CONCLUSION L-FABP-negative, inflammatory, and β-catenin-positive HCAs were related to MR imaging signs of diffuse intratumoral fat deposition, an atoll sign, and a typical vaguely defined scar, respectively. Since β-catenin-positive HCAs are considered premalignant, closer follow-up with MR imaging or resection may be preferred.

Systematic review of haemorrhage and rupture of hepatocellular adenomas.

Although benign in itself, hepatocellular adenoma (HCA) can be complicated by hormone‐induced growth, and subsequent haemorrhage and rupture. The exact risk of haemorrhage and rupture is not known. This systematic review of the literature was carried out with the aim of estimating the risk of haemorrhage and rupture in HCA.

Validation of a liver adenoma classification system in a tertiary referral centre: Implications for clinical practice

BACKGROUND & AIMS A molecular and pathological classification system for hepatocellular adenomas (HCA) was recently introduced and four major subgroups were identified. We aimed to validate this adenoma classification system and to determine the clinical relevance of the subtypes for surgical management. METHODS Paraffin fixed liver tissue slides and resection specimens of patients radiologically diagnosed as HCA were retrieved from the department of pathology. Immunostainings included liver-fatty acid binding protein (L-FABP), serum amyloid A (SAA), C-reactive protein (CRP), glutamine synthetase (GS) and β-catenin. RESULTS From 2000 to 2010, 58 cases (71 lesions) were surgically resected. Fourteen lesions were diagnosed as focal nodular hyperplasia with a characteristic map-like staining pattern of GS. Inflammatory HCA expressing CRP and SAA was documented in 36 of 57 adenomas (63%). Three of these inflammatory adenomas were also β-catenin positive as well as GS positive and only one was CRP and SAA and GS positive. We identified eleven L-FABP-negative HCA (19%) and four β-catenin positive HCA (7%), without expression of CRP and SAA and with normal L-FABP staining, one of which was also GS positive. Six HCA were unclassifiable (11%). In three patients multiple adenomas of different subtypes were found. CONCLUSIONS Morphology and additional immunohistochemical markers can discriminate between different types of HCA in>90% of cases and this classification, including the identification of β-catenin positive adenomas may have important implications in the decision for surveillance or treatment. Interpretation of nuclear staining for β-catenin can be difficult due to uneven staining distribution or focal nuclear staining and additional molecular biology may be required.

Living kidney donors: Impact of age on long-term safety

The safety of older live kidney donors, especially the decline in glomerular filtration rate (GFR) after donation, has been debated. In this study we evaluated long‐term renal outcome in older live kidney donors. From 1994 to 2006 follow‐up data of 539 consecutive live kidney donations were prospectively collected, during yearly visits to the outpatient clinic. Donors were categorized into two groups, based on age: <60 (n = 422) and ≥60 (n = 117). Elderly had lower GFR predonation (80 vs. 96 mL/min respectively, p < 0.001). During median follow‐up of 5.5 years, maximum decline in eGFR was 38%± 9% and the percentage maximum decline was not different in both groups. On long‐term follow‐up, significantly more elderly had an eGFR <60 mL/min (131 (80%) vs. 94 (31%), p < 0.001). However, renal function was stable and no eGFR of less than 30 mL/min was seen. In multivariate analysis higher body mass index (HR 1.09, 95%CI 1.03–1.14) and more HLA mismatches (HR 1.17, 95%CI 1.03–1.34) were significantly correlated with worse graft survival. Donor age did not influence graft survival. After kidney donation decline in eGFR is similar in younger and older donors. As kidney function does not progressively decline, live kidney donation by elderly is considered safe.

Safety and Efficacy of Radiofrequency Ablation for Hepatocellular Adenoma

PURPOSE To investigate the safety and efficacy of radiofrequency (RF) ablation for the treatment of hepatocellular adenoma (HCA). MATERIALS AND METHODS From 2000 to 2009, 170 patients with HCA were referred to a single tertiary hepatobiliary center. Medical records of 18 patients treated with RF ablation were retrospectively analyzed. RESULTS All patients were female, and the majority had a history of hormonal contraceptive use. Ten patients (56%) had multiple HCAs, with a median number of two lesions (range, one to 12) per patient. Median size of HCA at the time of RF ablation was 3.0 cm (range, 0.8-7.3 cm). A total of 45 HCAs were ablated in 32 sessions (open, n = 4; percutaneous, n = 28). RF ablation was complete after the first session in 26 HCAs (57.8%), and the majority of patients underwent multiple RF ablation sessions to fully ablate all HCAs. Major complications developed in two patients. CONCLUSIONS RF ablation can be used effectively in the treatment of HCA. However, multiple sessions are often required, and signs of residual adenoma might persist in some patients despite repetitive treatment. RF ablation might be especially beneficial in cases not amenable to surgery or in patients who would require major hepatic resection otherwise.

Hepatocellular adenoma: cost-effectiveness of different treatment strategies.

PURPOSE To determine the effectiveness, costs, and cost-effectiveness of strategies for the management of hepatocellular adenoma (HA) in women who are otherwise healthy. MATERIALS AND METHODS A Markov model was developed to estimate the quality-adjusted life expectancy (in quality-adjusted life-years [QALYs]), lifetime costs (in 2007 U.S. dollars), and net health benefits (QALY equivalent) of surgery, transarterial embolization (TAE), radiofrequency ablation (RFA), and watchful waiting. Model parameters and their distributions were derived from the literature and the hospital database. RESULTS In patients with HA tumors suitable for RFA, RFA had the highest effectiveness (23.89 QALYs) and lowest costs (

Focal nodular hyperplasia: lesion characteristics on state-of-the-art MRI including dynamic gadolinium-enhanced and superparamagnetic iron-oxide-uptake sequences in a prospective study.

2965). The treatment decision was sensitive to RFA-related mortality. In patients with tumors unsuitable for RFA, watchful waiting combined with TAE in cases of hemorrhage had the highest effectiveness (23.83 QALYs) and lowest costs (

Randomized controlled trial comparing hand-assisted retroperitoneoscopic versus standard laparoscopic donor nephrectomy

8493). The treatment decision was sensitive to probability of tumor growth, probability of hemorrhage, and hemorrhage-related mortality. CONCLUSION According to the model results, the most favorable treatment strategy for patients with small HAs was RFA. In patients with HA unsuitable for RFA, watchful waiting was the optimal strategy.

Can a decision-making model be justified in the management of hepatocellular adenoma?

To image a cohort of patients with pathology‐proven focal nodular hyperplasia (FNH) to assess which characteristics of state‐of‐the‐art magnetic resonance imaging (MRI) of the liver are the most useful for improving the detection and characterization of FNH.