P wave dispersion and QT dispersion in adult Turkish migrants with familial mediterranean fever living in Germany.

BACKGROUND: Familial Mediterranean Fever (FMF) is a hereditary autoinflammatory disease associated with subclinical inflammation, which includes atherosclerosis arising from endothelial inflammation, which in turn increases the risk of atrial or ventricular arrhythmias. Conduction abnormalities can be detected using the electrocardiographic (ECG) indices P and QT dispersion (Pdisp and QTdisp). Currently, it is unknown whether patients with FMF are more likely to have abnormalities of these ECG indices. Moreover, existing studies were conducted in countries with higher FMF prevalence. We therefore perform the first prospective study assessing Pdisp and QTdisp in adult FMF patients in Germany, where prevalence of FMF is low. METHOD: Asymptomatic FMF patients (n=30) of Turkish ancestry living in Germany and age-matched healthy controls (n=37) were prospectively assessed using 12-lead ECG. RESULTS: Patients and controls were comparable in gender and body mass index, and patients had higher erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) and serum amyloid A (SAA) compared to controls (ESR: 23.7±14.3 vs. 16.1±13,3 mm/1(st)h, p=0.03, CRP: 0.73±0.9 vs. 0.26±0.4 g/dl, p=0.01, SAA: 3.14±4,8 vs. 0.37±0.3 mg/dl, p<0.01). No statistically significant difference between patients and controls respectively, for Pdisp (43.7±11.9 vs. 47.1±11.2ms, p=0.23), QTdisp (65.9±12.3 vs. 67.6±12.7 ms, p=0.58) or corrected QTdisp (cQTdisp: 73.9±15.0 vs. 76.0±13.3 ms, p=0.55) was found. No correlation could be found between Pdisp or QTdisp or cQTdisp and any of the biochemical markers of inflammation. CONCLUSION: FMF patients living in Germany show a Pdisp and QTdisp comparable to healthy controls, with no increased risk of atrial or ventricular arrhythmias indicated.

Sonographic assessment of spleen size in Turkish migrants with Familial Mediterranean fever in Germany.

OBJECTIVES: Familial Mediterranean fever (FMF) can be associated with splenomegaly. Prospective quantitative data are lacking. We performed a sonographic assessment of spleen size in patients with FMF and healthy control participants to assess its diagnostic value. METHODS: Patients with FMF according to the criteria of Livneh et al (Arthritis Rheum 1997; 40:1879-1885) who were in an asymptomatic interval and control participants were prospectively included in this study in Germany and underwent sonographic measurement of the spleen as well as a structured interview and a physical examination. Patients and controls were Turkish migrants. RESULTS: Thirty-six patients and 27 controls were included. Patients and controls did not differ significantly in age (mean ± SD, 34.8 ± 9.7 versus 33.3 ± 10.0 years, respectively; P = .56), sex, height, weight, or body mass index (26.7 ± 4.7 versus 26.1 ± 4.3 kg/m(2); P = .63). Spleen size was greater in patients than controls in width (4.3 ± 1.0 versus 3.7 ± 0.7 cm; P = .008) and also length (12.1 ± 1.9 versus 10.5 ± 1.4 cm; P = .001). Twenty-six of 36 patients (72.2%) had a history of appendectomy compared to 3 of 27 controls (11.1%; P < .001). The combination of an enlarged spleen (length >11 cm and/or width >4 cm) gave specificity of 100% (95% confidence interval, 87%-100%) and a positive predictive value of 100% (95% confidence interval, 78%-100%) for the diagnosis of FMF in our study. CONCLUSIONS: Spleen size as evaluated by sonography is larger in patients with FMF compared to healthy controls. Most patients with FMF included in this study had undergone appendectomy. Familial Mediterranean fever should be considered as a differential diagnosis in Turkish migrants in Germany if the spleen is enlarged and a history of appendectomy is reported.

Quality of life in adult patients with Familial Mediterranean fever living in Germany or Turkey compared to healthy subjects: a study evaluating the effect of disease severity and country of residence.

We assessed quality of life (QOL) and disease activity in patients with Familial Mediterranean fever (FMF) of Turkish ancestry living in Germany or Turkey and conducted a correlation with FMF disease activity. 40 FMF patients in Turkey (TR), 40 FMF patients in Germany (G) and 40 healthy controls in Germany (C) were included. QOL was evaluated with the short form of the World Health Organisation Quality of Life scale (WHOQOL-BREF). FMF disease activity was examined with the Pras score. Mean age was TR 30.5 ± 10.6, G 35.2 ± 10.2, C 34.6 ± 10.7. Of the 120 participants, 77 were female. FMF patients in TR and G had a significantly decreased QOL physical health domain compared to controls (TR 59.7 ± 18.8, G 60.4 ± 19.4, C 76.5 ± 14.6). Turkish FMF patients had a lower QOL environment domain compared to controls (TR 62.3 ± 17.5, G 69.7 ± 16.5, C 72.3 ± 13.5). In the other QOL domains, no significant differences were found. The differences in QOL were robust to a regression analysis. No significant correlation between QOL and FMF disease activity was found. German FMF patients had longer duration of disease, younger age at onset and longer delay from disease onset to colchicine treatment. A total of 5 of 40 German FMF patients were not taking colchicine (TR:0). Erythrocyte sedimentation rate was lowest in TR with significant difference between TR and G as well as G and C (TR 13.2 ± 10.3, G 27.8 ± 19.4, C 16.3 ± 12.8 mm/h). C-reactive protein did not differ between TR and G. FMF has an important impact on QOL physical health domain. No correlation between FMF disease activity and the WHOQOL-BREF could be found.

Disease severity in adult patients of Turkish ancestry with familial mediterranean fever living in Germany or Turkey. Does the country of residence affect the course of the disease?

BACKGROUND: The environment may affect the course of familial Mediterranean fever (FMF). OBJECTIVE: The objective of this study was to compare disease severity between adult FMF patients in Turkey (TR) and Germany (G). METHODS: Adult FMF patients of Turkish ancestry on colchicine living in Turkey (n = 40) or G (n = 35) were compared. Disease severity, C-reactive protein (CRP), and erythrocyte sedimentation rate were assessed. RESULTS: Groups differed significantly in the following aspects: age at onset of disease (TR: 15.6, G: 10.8 years; P = 0.02), delay between onset and initiation of colchicine treatment (TR: 6.8 years, G: 14.9 years; P < 0.001), female gender (TR: 80%, G: 57.1%; P = 0.04), and duration of disease (TR: 14.4 years, G: 23.4 years; P < 0.001). There was no significant difference in colchicine treatment concerning average dosing and duration of therapy. No significant difference could be found between the 2 groups in CRP and disease severity as assessed by the score of Pras et al. (Am J Med Genet. 1998;75:216-219) even after adjusting for potential confounding variables. Mean erythrocyte sedimentation rate was significantly higher among patients living in G (TR: 13.2 mm/first hour, G: 26.3 mm/first hour; P < 0.001). Among patients living in Germany, there was a significant difference in age at FMF onset depending on their country of birth (born in TR: 14.9 years, born in G: 6.9 years; P = 0.0001). CONCLUSIONS: In adult FMF patients living in Turkey or Germany, no difference in disease activity or CRP could be found. German patients were younger at onset of disease and had a longer delay between onset and initiation of colchicine treatment.

Correlation between acoustic radiation force impulse (ARFI)-based tissue elasticity measurements and perfusion parameters acquired by perfusion CT in cirrhotic livers: a proof of principle.

PURPOSE: To investigate whether liver stiffness measured by acoustic radiation force impulse (ARFI) sonoelastography always correlates with the liver perfusion parameters quantified by perfusion CT in patients with known liver cirrhosis. METHODS: Sonoelastography and perfusion CT were performed in 50 patients (mean age 65.5; range 45-87 years) with liver cirrhosis, who were classified according to Child-Pugh into class A (30/50, 60%), B (17/50, 34%), and C (3/50, 6%). For standardized ARFI measurements in the left liver lobe at a depth of 4 cm, a convex 6-MHz probe was used. CT examinations were performed using 80 kV, 100 mAs, and 50 ml of iodinated contrast agent injected at 5 ml/s. Using standardized region-of-interest measurements, we quantified arterial, portal venous, and total liver perfusion. RESULTS: There was a significant linear correlation between tissue stiffness and arterial liver perfusion (p = 0.015), and also when limiting the analysis to patients with histology (p = 0.019). In addition, there was a positive correlation between the total blood supply (arterial + portal-venous liver perfusion) to the liver and tissue stiffness (p = 0.001; with histology, p = 0.027). Shear wave velocity increased with higher Child-Pugh stages (p = 0.013). CONCLUSION: The degree of tissue stiffness in cirrhotic livers correlates expectedly-even if only moderately-with the magnitude of arterial liver perfusion and total liver perfusion. As such, liver elastography remains the leading imaging tool in assessing liver fibrosis.

Comparison between acoustic radiation force impulse quantification data and perfusion-CT parameters in hepatocellular carcinoma.

OBJECTIVE: To find out, if ultrasound elastography of hepatocellular carcinoma (HCC) can predict patterns of tumor perfusion in volume perfusion computed tomography (VPCT). MATERIAL AND METHODS: 25 consecutive patients (mean age, 68.9; range, 51-85 years) with liver cirrhosis suspected of HCC underwent VPCT and acoustic radiation force impulse (ARFI) elastography the same day. Quantitative elasticity values were registered, while blood flow (BF), blood volume (BV) and hepatic perfusion index (HPI) of the HCC lesions were calculated. Additionally, we identified histologic WHO grading, lesion size and localization. The Siemens Acuson S 3000 HELX-System with Virtual Touch™-Software and Siemens Somatom Definition Flash with Syngo® software were used. RESULTS: A total of 43 HCC lesions were assessed. Mean shear wave velocity was 2.6m/s (range, 1.1-4.3m/s). There was no significant linear correlation between the elasticity values and BF (p=0.751), BV (p=0.426) and HPI (p=0.437). However, elasticity values were higher, the larger the tumor was (p=0.008). Shear wave velocity declined with increasing distance of the HCC to the skin surface (p=0.028) and depending on liver segment. In addition, elasticity values were higher in less differentiated HCCs. This trend was not statistically significant (p=0.842). CONCLUSION: Tissue elasticity in HCC does not correlate with the degree of tumor vascularization, but calculated values are influenced both by the tumor size and localization inside the liver.

Multiparametric imaging for detection and characterization of hepatocellular carcinoma using gadoxetic acid-enhanced MRI and perfusion-CT: which parameters work best?

BACKGROUND: MRI and perfusion-CT (PCT) are both useful imaging techniques for detection and characterization of liver lesions. The aim of this study was to compare the diagnostic accuracy of imaging parameters derived from PCT and gadoxetic acid-enhanced MRI in patients with hepatocellular carcinoma (HCC). METHODS: 36 patients with liver cirrhosis and a total of 67 lesions referred to our hospital for multi-parametric diagnosis of HCC-suspected liver lesions in the setting of liver cirrhosis were prospectively enrolled and underwent PCT and MRI. HCC diagnosis was confirmed either by histology (n = 60) or interval growth (n = 7). For PCT, mean/max blood flow (BF), blood volume (BV), k-trans, arterial liver perfusion (ALP), portal venous perfusion (PVP) and hepatic perfusion index (HPI) were quantified. Two readers identified the lesions based on single maps each being blinded to the number of lesions. MRI-protocol included fat-suppressed T1w-VIBE sequences obtained before, 2, 5, 10 and 20 min after the injection of gadoxetic acid as well as non-enhanced coronal HASTE, axial T1w-VIBE, fat-suppressed T2w-TSE and DWI. Quantitative analysis was performed using enhancement ratios between tumor and liver parenchyma for post-contrast in the hepatobiliary phase (RIRHB), arterial (ERa) and late-venous (ERv) phases as well as signal intensity ratios (liver/parenchyma) on T1w (RIRT1) and T2w (RIRT2). RESULTS: In PCT analysis, all lesions exhibited high BFmax values (63-250 mL/100 g tissue) and were visible on HPI maps with high degrees of arterial blood supply of (HPI > 96%). In MRI, RIRHB was negative in 8/67. 12/67 HCCs were missed on DWI. 46/67 HCCs showed wash-in and 47/67 HCC showed wash-out of contrast agent. 6/67 HCCs were missed on T1w and 11/67 were missed on T2w-sequences when analyzed separately, while analysis of multiparametric MRI combining typical enhancement pattern, visibility on hepatobiliary phase and T1w-images the same number of lesions as PCT irrespective of their size (1-19 cm) were detected. Quantification of early enhancement by ERa or ERv did not improve detection rates. CONCLUSIONS: Perfusion-CT and gadoxetic acid-enhanced MRI were comparable in detecting HCC lesions. For PCT a mean HPI > 96% proved to be a very robust parameter for detection and characterization of HCC.

Monitoring Disease Activity in Patients with Aortitis and Chronic Periaortitis Undergoing Immunosuppressive Therapy by Perfusion CT.

RATIONALE AND OBJECTIVES: To evaluate the role of perfusion CT for monitoring inflammatory activity in patients with aortitis and chronic periaortitis undergoing immunosuppressive therapy. MATERIALS AND METHODS: Seventeen symptomatic patients (median age 68.5 years) who underwent perfusion-based computed tomography (CT) monitoring after diagnostic contrast-enhanced CT were retrospectively included in this study. Blood flow (BF), blood volume (BV), volume transfer constant (k-trans), time to peak, and mean transit time were determined by setting circular regions of interest in prominently thickened parts of the vessel wall or perfused surrounding tissue at sites where the perfusion CT color maps showed a maximum BF value. Differences in CT perfusion and, morphological parameters, C-reactive protein (CRP), and erythrocyte sedimentation rate (ESR) were tested for significance during therapy. RESULTS: In all patients BF and BV dropped at second perfusion CT (P < 0.05). In aortitis patients, CRP dropped from 3.86 ± 5.31 mg/dL to 0.9 ± 1.37 mg/dL and in periaortitis patients from 1.78 ± 2.25 mg/dL to 0.79 ± 1.55 mg/dL, whereas ESR dropped from 45.71 ± 37.59 seconds to 8.57 ± 3.1 seconds and 36.78 ± 34.67 seconds to 17.22 ± 21.82 seconds in aortitis and in periaortitis, respectively. CONCLUSIONS: The course of perfusion CT parameters in aortitis and chronic periaortitis undergoing immunosuppressive therapy dropped at different extent after therapy.

Anxiety and depression in adult patients with familial Mediterranean fever: a study comparing patients living in Germany and Turkey.

AIM: To determine the prevalence of anxiety and depression among patients with familial Mediterranean fever (FMF) living in Germany or Turkey a prospective study was conducted. METHODS: Forty FMF patients living in Turkey (T), 40 FMF patients living in Germany (G) and 40 healthy controls living in Germany (C) were included. Patients and controls were of Turkish ancestry. G were compared to T and C. The Hospital Anxiety and Depression Scale (HADS) was used with a cut-off of ≥ 8 for each subdomain score (HADS-A, HADS-D). RESULTS: Baseline characteristics of G were comparable to T and C except for age (T: 30.5 years, G: 35.2 years, C: 34.6 years; T vs. G P = 0.045), duration of disease (T: 14.4 years, G: 24; P < 0.001), C-reactive protein (T: 0.78 mg/dL, G: 0.78 mg/dL, C: 0.35 mg/dL; G vs. C P = 0.03). Prevalence of anxiety was higher in G compared to C (T: 65%, G: 52.5%, C: 22.5%: G vs. C P < 0.05). No difference was found for the prevalence of depression (T: 30%, G: 35%, C: 20%). The association between FMF and anxiety in subjects living in Germany persisted after adjusting for age and gender in a regression analysis and was robust to an adjustment for coexisting depression. Anxiety and depression did not correlate with FMF disease severity assessed with the Pras score. CONCLUSION: Anxiety, but not depression is more common among FMF patients living in Germany compared to healthy controls. No significant difference could be found between FMF patients living in Germany or Turkey concerning the prevalence of anxiety or depression.