Andreas M. Herneth

Wilson's disease in patients presenting with liver disease: A diagnostic challenge

BACKGROUND & AIMS In patients with Wilsons disease presenting with liver involvement, the correct diagnosis is often missed or delayed. The aim of this study was to find an algorithm for diagnosis of this difficult patient group. METHODS Clinical and laboratory findings of 55 patients with Wilsons disease were evaluated at diagnosis before treatment. Presenting symptom was chronic liver disease in 17 patients, fulminant hepatic failure in 5 patients, hemolysis in 3 patients, and neurological disease in 20 patients, and 10 patients were detected by family screening (siblings). Evaluation included neurological and ophthalmologic examination, routine laboratory tests, and parameters of copper metabolism including liver copper content in 43 liver biopsy specimens. RESULTS In the whole group, serum ceruloplasmin level was <20 mg/dL in 73%, urinary copper excretion was increased in 88%, and liver copper content was elevated in 91% at diagnosis. Kayser-Fleischer rings were detected in 55%. In contrast to patients with neurological disease (90% Kayser-Fleischer rings, 85% low ceruloplasmin), only 65% of patients presenting with liver disease were diagnosed by these typical findings. Ceruloplasmin levels were lower in patients with Kayser-Fleischer rings or with neurological disturbances than in patients without these symptoms. CONCLUSIONS The commonly used clinical and laboratory parameters are not sufficient to exclude the diagnosis of Wilsons disease in patients with liver disease of unknown origin.

Apparent Diffusion Coefficient: a quantitative parameter for in vivo tumor characterization

PURPOSE The purpose of the this study was to evaluate the potential of diffusion weighted imaging (DWI) to distinguish different tissue compartments in early, intermediate and advanced tumor stages. MATERIALS AND METHODS Twenty-two male mice were induced with squamous cell tumor (SCCVII) and scanned with a clinical 1.5 T scanner. T1-SE, T2-FSE, diffusion weighted Line-Scan-MRI and contrast enhanced T1-SE were obtained from mice with early (tumor volume 10-100 mm(3)), intermediate (200-600 mm(3)), advanced tumors (600-1000 mm(3)) and tumor necrosis (>1500 mm(3)). The apparent diffusion coefficient (ADC) of different tumor compartments was calculated offline with a pixel-by-pixel method. The animals were sacrificed immediately after scanning and histopathologic correlation was performed. RESULTS In early stages of tumor development, tumors appeared homogeneous on diffusion weighted images with an ADC of 0.64+/-0.06 x 10(-3) mm(2)/s. With tumor progression the ADC in the rim areas of tumor increased significantly (intermediate stage: 0.70+/-0.11 x 10(-3) mm(2)/s; advanced stage: 0.88+/-0.11 x 10(-3) mm(2)/s; tumor necrosis 1.03+/-0.06 x 10(-3) mm(2)/s), whereas the ADC in viable tumor remained constant. Histologically the areas with an increased ADC correlated well with areas of necrosis (reduced cell density). CONCLUSION The ADC is a non-invasive technique to monitor changes in the biological structure of tumor tissue during tumor progression. Thus, DWI is a potential diagnostic tool for in-vivo tissue characterization.

Rotator cuff tears in asymptomatic individuals: a clinical and ultrasonographic screening study.

OBJECTIVE To determine the prevalence and clinical impact of rotator cuff tears in asymptomatic volunteers. MATERIALS AND METHODS Sonographic examinations of the shoulder of 212 asymptomatic individuals between 18 and 85 years old were performed by a single experienced operator. The prevalence and location of complete rotator cuff tears were evaluated. The clinical assessment was based on the Constant Score. Magnetic resonance imaging (MRI) of the shoulder was obtained in those patients where US showed rotator cuff pathology. RESULTS Ultrasound showed a complete rupture of the supraspinatus tendon in 6% of 212 patients from 56 to 83 years of age (mean: 67 years). MRI confirmed a complete rupture of the supraspinatus tendon in 90%. All patients reported no functional deficits, although strength was significantly lower in the patient group with complete supraspinatus tendon tear (P < 0.01). CONCLUSION There is a higher prevalence in older individuals of rotator cuff tendon tears that cause no pain or decrease in activities of daily living.

Imaging articular cartilage defects in the ankle joint with 3D fat-suppressed echo planar imaging: Comparison with conventional 3D fat-suppressed gradient echo imaging

To shorten the examination time for articular cartilage imaging, using a recently developed three‐dimensional (3D) multishot echo planar imaging (EPI) sequence with fat saturated (FS), compared to aconventional 3D fat‐saturated spoiled gradient echo sequence (3D FS GRE).

Magnetic resonance imaging of liver malignancies.

The histological structure of the liver is complex, consisting of hepatocytes, biliary epithelium, and mesenchymal cells. From this large variety of cells, a broad spectrum of benign and malignant liver lesions in originate. An accurate diagnosis of these lesions is mandatory for choosing an appropriate therapeutic approach. With the recent developments in hardware and software, magnetic resonance imaging (MRI) has emerged as the method of choice in the diagnostic workup of focal liver lesions, in particular in the pretherapeutic stage. The introduction of high-field MRI at 3.0T in the routine workup and the selective use of liver-specific contrast agents, including hepatobiliary and reticuloendothelial agents, have also strengthened the role of MRI in liver imaging. In this overview article, we will review the recent developments in 3.0-T MRI and MRI contrast agents in the diagnostic workup of the most common malignant liver tumors.

Diffusion weighted imaging in osteoradiology.

Diffusion weighted imaging gained attention as an imaging modality, which provides information on the microstructure of a tissue, which can be used for tissue characterization. This is of importance in patients where other diagnostic tools provide equivocal or unspecific information. In addition quantitative diffusion measurements provide objective parameters for unbiased comparison of treatment response, which is mandatory for therapy monitoring. Technical restriction limited the use of Diffusion Weighted Imaging to the brain. However, with the improvement in scanner technology and the availability of new MR sequences investigation of the Muskulo Skeletal System was made possible. We describe the potential of Diffusion Weighted Imaging as a non-invasive technique to evaluate pathological, inflammatory and physiological processes in osteoradiology.

Successful conservative treatment of severe renal candidosis with fungus balls

Abstract Partial fungal obstruction of the renal collecting system is an unusual finding among infants that poses specific management problems. We report a patient with sepsis and fungal infection of the kidneys post surgery who presented with bilateral fungus balls and was successfully managed by conservative measures. Sonography is the imaging technique of choice in the diagnosis and follow-up of such patients. The need for prompt diagnosis in high-risk patients and the role of sonography are discussed.

Role of tryptophan in the elevated serotonin-turnover in hepatic encephalopathy

Summary. The increase of the brain levels of 5-hydroxyindoleacetic acid (5-HIAA) in hepatic encephalopathy (HE) suggests an increased turnover of serotonin (5-HT). To study the role of tryptophan on the increased brain 5-HT metabolism in HE, we attempted to monitor brain levels of tryptophan in rats with thioacetamide-induced acute liver failure by intravenous infu-sion of branched-chain amino acids (BCAA). The effect of this treatment on 5-HT synthesis and metabolism was investigated in five brain areas. BCAA-infusions (1 and 2 gm/kg/24 h) increased the ratio BCAA/aromatic amino acids in plasma two- and fourfold, respectively, and lowered both plasma and brain levels of tryptophan. At the higher BCAA-dose all parameters suggesting an altered brain 5-HT metabolism (increased brain levels of 5-HT and 5-HIAA, increased 5-HIAA/5-HT ratio) were almost completely normalized. These results provide further evidence for the role of tryptophan in the elevation of brain 5-HT metabolism and for a potential role of BCAA in the treatment of HE.

Role of Doppler Sonography in the Evaluation of Accessory Spleens After Splenectomy

Splenectomy influences the Doppler blood flow pattern in the splenic artery. Blood flow in this vessel might return to normal if an accessory spleen increases in size after splenectomy. Our objective was to evaluate the resistive index of the splenic artery depending on the presence or absence of a hypertrophic accessory spleen in splenectomized patients.

Computed tomography scans in the evaluation of patients with olfactory dysfunction.

Background The necessity of computed tomography (CT) scans of the olfactory cleft and the sinuses in patients presenting with olfactory dysfunction is a matter of debate. This study aimed to investigate the significance of CT scans for the diagnosis of olfactory loss and for therapeutic decisions. Methods This retrospective study included 137 consecutive patients presenting with the main complaint of olfactory loss. Standardized history; ear, nose, and throat examination; and olfactory tests were administered. In addition, a CT scan of the nasal sinuses was obtained. The assumed diagnosis (sinonasal disease [SND] or non-SND) was compared with the diagnosis when additionally considering results from the CT scan. Results CT scans suggested SND in 7/101 patients without clinical evidence of its presence. In 12/36 patients with suspected SND-related olfactory loss, no signs of SND were detected in CT scans. Conclusion CT scans are useful to diagnose conductive/inflammatory olfactory loss in patients suspected of non-SND, which may then justify a course with systemic steroids. Alternatively, considering low costs and rare side effects of a short course with steroids in selected patients, CT scans appear to be of clinical significance only when surgical treatment is contemplated or where the use of systemic corticosteroids is not possible.