Rada Jesic

Neuropsychiatric aspects of treated Wilson's disease ☆

The objective of the current cross-sectional study was to use standardized psychiatric interviews (the Structured Clinical Interview for DSM-IV Axis I Disorders and the Neuropsychiatric Inventory; NPI) in order to better characterize psychiatric symptoms in 50 consecutive, treated and clinically stable patients with Wilsons disease (WD). Nine patients (18%) had one, 7 patients (14%) had two, and 20 (40%) had >or= 3 neuropsychiatric symptoms present. The most often endosed symptoms were anxiety (62%), depression (36%), irritability (26%), as well as disinhibition and apathy (24% each). Twenty two patients (44%) had a score >or= 4 on at least one of the NPI items: again, most frequently anxiety (17 patients; 34%), depression (13 patients; 26%) and apathy (9 patients; 18%). Therefore, even among stable, long-term treated patients with WD approximately 70% experienced psychiatric symptoms.

Diffusion-weighted MRI versus transient elastography in quantification of liver fibrosis in patients with chronic cholestatic liver diseases

PURPOSE To evaluate the diagnostic value of diffusion-weighted magnetic resonance imaging (DWMRI) and transient elastography (TE) in quantification of liver fibrosis in patients with chronic cholestatic liver diseases. MATERIALS AND METHODS Forty-five patients underwent DWMRI, TE, and liver biopsy for staging of liver fibrosis. Apparent diffusion coefficient (ADC) was calculated for six locations in the liver for combination of five diffusion sensitivity values b=0, 50, 200, 400 and 800 s/mm(2). A receiver operating characteristic (ROC) analysis was performed to determine the diagnostic performance of DWMRI and TE. Segmental ADC variations were evaluated by means of coefficient of variation. RESULTS The mean ADCs (× 10(-3)mm(2)/s; b=0-800 s/mm(2)) were significantly different at stage F1 versus F ≥ 2 (p<0.05) and F2 versus F4. However, no significant difference was found between F2 and F3. For prediction of F ≥ 2 and F ≥ 3 areas under the ROC curves were 0.868 and 0.906 for DWMRI, and 0.966 and 0.960 for TE, respectively. The sensitivity and specificity were 90.9% and 89.3% for F ≥ 2 (ADC ≤ 1.65), and 92.3% and 92.1% for F ≥ 3 (ADC ≤ 1.63). Segmental ADC variation was lowest for F4 (CV=9.54 ± 6.3%). CONCLUSION DWMRI and TE could be used for assessment of liver fibrosis with TE having higher diagnostic accuracy and DWMRI providing insight into liver fibrosis distribution.

MR imaging of primary sclerosing cholangitis: additional value of diffusion-weighted imaging and ADC measurement.

Background Primary sclerosing cholangitis (PSC) is a cholestatic liver disease with chronic inflammation and progressive destruction of biliary tree. Magnetic resonance (MR) examination with diffusion-weighted imaging (DWI) allows analysis of morphological liver parenchymal changes and non-invasive assessment of liver fibrosis. Moreover, MR cholangiopancreatography (MRCP), as a part of standard MR protocol, provides insight into bile duct irregularities. Purpose To evaluate MR and MRCP findings in patients with primary sclerosing cholangitis and to determine the value of DWI in the assessment of liver fibrosis. Material and Methods The following MR findings were reviewed in 38 patients: abnormalities in liver parenchyma signal intensity, changes in liver morphology, lymphadenopathy, signs of portal hypertension, and irregularities of intra- and extrahepatic bile ducts. Apparent diffusion coefficient (ADC) was calculated for six locations in the liver for b = 800 s/mm2. Results T2-weighted hyperintensity was seen as peripheral wedge-shaped areas in 42.1% and as periportal edema in 28.9% of patients. Increased enhancement of liver parenchyma on arterial-phase imaging was observed in six (15.8%) patients. Caudate lobe hypertrophy was present in 10 (26.3%), while spherical liver shape was noted in 7.9% of patients. Liver cirrhosis was seen in 34.2% of patients; the most common pattern was micronodular cirrhosis (61.5%). Other findings included lymphadenopathy (28.9%), signs of portal hypertension (36.7%), and bile duct irregularities (78.9%). The mean ADCs (×10-3mm2/s) were significantly different at stage I vs. stages III and IV, and stage II vs. stage IV. No significant difference was found between stages II and III. For prediction of stage ≥II and stage ≥III, areas under receiver-operating characteristic curves were 0.891 and 0.887, respectively. Conclusion MR with MRCP is a necessary diagnostic procedure for diagnosis of PSC and evaluation of disease severity. Moreover, DWI could be used in continuation with standard MR sequences for the evaluation of liver fibrosis stage and distribution.

Quality of life in patients with treated and clinically stable Wilson's disease

Health‐related quality of life (HRQoL) in Wilsons disease (WD) has not been extensively studied. Therefore, the purpose of this cross‐sectional study was to identify clinical and demographic factors influencing HRQoL in 60 treated, clinically stable patients with WD using a generic questionnaire, the Medical Outcomes Study Short‐Form 36‐Item Health Survey (SF‐36). The level of disability and grading of WD multisystemic manifestations were assessed by the Global Assessment Scale for WD (GAS for WD). The Mini Mental State Examination (MMSE) and the 21‐item Hamilton Depression Rating Scale (HDRS) scoring were also applied by the same trained interviewers. Lower scores on the SF‐36 domains were found in patients with neurological compared with those with a predominantly hepatic form of WD. The HRQoL of patients with WD and psychiatric symptoms was also lower than that of those without them. Finally, significant inverse correlations were obtained between the various SF‐36 domains and all the following: period of latency from the first symptoms/signs appearance and treatment initiation, MMSE and HDRS scores, and different domains of the GAS for WD.

Erratum to: Hereditary Angioedema Presenting with Recurrent Ascites

Figure 1 of the above cited article was republished from the article ‘‘Ultrasonography in the diagnosis and monitoring of ascites in acute abdominal attacks of hereditary angioneurotic oedema’’, (European Journal of Gastroenterology & Hepatology 2001 13(10):1225–1230), cited as reference 13. We were recently informed that permission had not been granted for use of this figure in our publication. This erratum is to certify that the publisher, Wolters Kluwer, has kindly granted retrospective permission for the publication of their figure in our Digestive Diseases and Sciences publication.

Comparison of standard fibrinogen measurement methods with fibrin clot firmness assessed by thromboelastometry in patients with cirrhosis

BACKGROUND The Clauss fibrinogen method and thrombin clotting time (TCT) are still routinely used in patients with cirrhosis to define fibrinogen concentration and clotting potential. The thromboelastometric functional fibrinogen FIBTEM assay evaluates the strength of fibrin-based clots in whole blood, providing information on both quantitative deficit and fibrin polymerization disorders. OBJECTIVE To compare these three methods of assessing fibrinogen in patients with cirrhosis of different aetiologies, characterized by impairment in fibrinogen concentration as well as functional aberrance. METHODS Sixty patients with alcoholic and 24 patients with cholestatic cirrhosis were included (Child-Pugh score (CPs)A, n=24; B, n=32; C, n=28). All parameters were compared with those from a control group. Maximum clot firmness (MCF) in the FIBTEM test was assessed in regard to its relevance in detection of qualitative fibrinogen disorders in comparison with results obtained by standard measurement methods, i.e. the Clauss fibrinogen method and TCT. RESULTS With increased cirrhosis severity, fibrinogen and FIBTEM-MCF levels significantly declined (p=0.002), while TCT was significantly prolonged (p=0.002). In all CPs groups, fibrinogen strongly correlated with FIBTEM-MCF (r=0.77, r=0.72, r=0.74; p<0.001), while cross-correlations of other assays were highly variable. The prevalence of decreased FIBTEM-MCF values (<9 mm) was significantly higher in advanced CPs categories (p=0.027), whereby the highest prevalence was detected in patients with CPsC (10/16; 62.5%). Nine of the 16 patients with decreased FIBTEM-MCF values had also decreased fibrinogen levels, while in the remaining 7 patients fibrinogen levels were within the reference range, indicating the possible presence of qualitatively altered fibrinogen that could be detected by FIBTEM-MCF. CONCLUSIONS FIBTEM-MCF may be considered as a reliable alternative to standard plasma fibrinogen measurement in cirrhotic patients, especially in evaluating fibrin polymerization disorders in these patients. Further studies are needed to evaluate the usefulness of this assay in predicting bleeding complications in cirrhotic patients as well as monitoring replacement treatment.