Hepatocellular carcinoma in a non-cirrhotic patient with Wilson's disease

We report the exceptional case of hepatocellular carcinoma in a non-cirrhotic patient, whose Wilson's disease was diagnosed at the unusual age of 58 years. The liver histology revealed macrovesicular steatosis with fibrosis, but no cirrhosis. The disease was treated with D-penicillamine for 3 years until acute discomfort in the right upper quadrant led to detection of multifocal hepatocellular carcinoma, which was successfully resected. The histological examination confirmed the malignant nature of the 4 lesions, which were classified according to Edmondson and Steiner as poorly differentiated hepatocellular carcinoma grade 3. The non-tumoral parenchyma showed 80% steatosis with ballooned cells, lobular inflammation, septal fibrosis but no cirrhosis. Hepatocellular carcinoma is rare in Wilson's disease, especially in the absence of cirrhosis. The literature's 28 published cases are reviewed and the contributory role of copper in the hepatocarcinogenic process is discussed.

A Hebrew and English lexicon of the Old Testament : with an appendix containing the biblical Aramaic ; based on the lexicon of William (Wilhelm) Gesenius as transl. by Edward Robinson ; ed. with constant reference to the Thesaurus of Gesenius ... and with authorized use of the latest German ed. of Gesenius's Handwörterbuch über das Alte Testament

Francis Brown with the co-op. of ...

Edward I., 1273 - 1275

ed. by J. M. Rigg

Edward I., 1275 - 1277

ed. by Hilary Jenkinson

Genetic association study of QT interval highlights role for calcium signaling pathways in myocardial repolarization

The QT interval, an electrocardiographic measure reflecting myocardial repolarization, is a heritable trait. QT prolongation is a risk factor for ventricular arrhythmias and sudden cardiac death (SCD) and could indicate the presence of the potentially lethal mendelian long-QT syndrome (LQTS). Using a genome-wide association and replication study in up to 100,000 individuals, we identified 35 common variant loci associated with QT interval that collectively explain ∼8-10% of QT-interval variation and highlight the importance of calcium regulation in myocardial repolarization. Rare variant analysis of 6 new QT interval-associated loci in 298 unrelated probands with LQTS identified coding variants not found in controls but of uncertain causality and therefore requiring validation. Several newly identified loci encode proteins that physically interact with other recognized repolarization proteins. Our integration of common variant association, expression and orthogonal protein-protein interaction screens provides new insights into cardiac electrophysiology and identifies new candidate genes for ventricular arrhythmias, LQTS and SCD.

Long-term outcomes of patients with Wilson disease in a large Austrian cohort

BACKGROUND & AIMS Wilson disease is an autosomal recessive disorder that affects copper metabolism, leading to copper accumulation in liver, central nervous system, and kidneys. There are few data on long-term outcomes and survival from large cohorts; we studied these features in a well-characterized Austrian cohort of patients with Wilson disease. METHODS We analyzed data from 229 patients diagnosed with Wilson disease from 1961 through 2013; 175 regularly attended a Wilson disease outpatient clinic and/or their physicians were contacted for information on disease and treatment status and outcomes. For 53 patients lost during the follow-up period, those that died and reasons for their death were identified from the Austrian death registry. RESULTS The mean observation period was 14.8 ± 11.4 years (range, 0.5-52.0 years), resulting in 3116 patient-years. Of the patients, 61% presented with hepatic disease, 27% with neurologic symptoms, and 10% were diagnosed by family screening at presymptomatic stages. Patients with a hepatic presentation were diagnosed younger (21.2 ± 12.0 years) than patients with neurologic disease (28.8 ± 12.0; P < .001). In 2% of patients, neither symptoms nor onset of symptoms could be determined with certainty. Most patients stabilized (35%) or improved on chelation therapy (26% fully recovered, 24% improved), but 15% deteriorated; 8% required a liver transplant, and 7.4% died within the observation period (71% of deaths were related to Wilson disease). A lower proportion of patients with Wilson disease survived for 20 years (92%) than healthy Austrians (97%), adjusted for age and sex (P = .03). Cirrhosis at diagnosis was the best predictor of death (odds ratio, 6.8; 95% confidence interval, 1.5-31.03; P = .013) and need for a liver transplant (odds ratio, 07; 95% confidence interval, 0.016-0.307; P < .001). Only 84% of patients with cirrhosis survived 20 years after diagnosis (compared with healthy Austrians, P =.008). CONCLUSION Overall, patients who receive adequate care for Wilson disease have a good long-term prognosis. However, cirrhosis increases the risk of death and liver disease. Early diagnosis, at a precirrhotic stage, might increase survival times and reduce the need for a liver transplant.

Worldwide evidence of a unimodal relationship between productivity and plant species richness

The search for predictions of species diversity across environmental gradients has challenged ecologists for decades. The humped-back model (HBM) suggests that plant diversity peaks at intermediate productivity; at low productivity few species can tolerate the environmental stresses, and at high productivity a few highly competitive species dominate. Over time the HBM has become increasingly controversial, and recent studies claim to have refuted it. Here, by using data from coordinated surveys conducted throughout grasslands worldwide and comprising a wide range of site productivities, we provide evidence in support of the HBM pattern at both global and regional extents. The relationships described here provide a foundation for further research into the local, landscape, and historical factors that maintain biodiversity.

Sir Edward Sassoon [Illustration] : (Porträt)

Scan von Monochrom-Mikroform

Professor Cook Wilson [Illustration] : (Porträt)

Alfred A. Wolmark

Sir Edward Sassoon

S. M. Mélamed

Na 1 Nachlass Max Horkheimer, 11 - Korrespondenzen u.a. mit Edward M. David (p. I 6, 1-183)

1 Brief von Fritz Dannenbaum an Max Horkheimer, 26.11.1935; 2 Briefe zwischen F. Darnbacher und Max Horkheimer, 24.01.1936, 22.01.1936; 3 Briefe zwischen dem Dartmouth College Hanover N.H. und Max Horkheimer, 1939-1941. 06.05.1939; 14 Briefe zwischen Edward M. David und Max Horkheimer, 1941-1942; 1 Brief von der Day Cornell University, Ithaca N.Y. an Max Horkheimer, 24.02.1939; 2 Briefe zwischen Margaret Deaner und Max Horkheimer, 01.04.1935, 04.04.1935; 1 Brief von Max Horkheimer an Hanna Deinhard, 05.01.1949; 1 Brief von Deiters an Max Horkheimer, 26.11.1935; 2 Briefe zwischen Laura Demick und Max Horkheimer, 22.12.1941, 29.12.1941; 2 Briefe zwischen Grace Dertz und Max Horkheimer, 18.10.1934, 11.10.1934; 1 Brief von Max Horkheimer an Dekan, 20.06.1940; 1 Brief von Max Dessoir an Max Horkheimer, 29.08.1937; 1 Brief von Max Horkheimer an Deutschland / deutsches Reich Minister für Wissenschaft, Kunst und Volksbildung, Berli, 21.04.1933; 4 Briefe zwischen John Dewey und Max Horkheimer, 1940-1941, 20.02.1941; 1 Brief von Max Horkheimer an Walter Strauss, 20.02.1941; 3 Briefe zwischen Frederic Dewhurst und Max Horkheimer, 1939, 08.02.1939; 5 Briefe zwischen dem Soziograph Jul Diederich und Max Horkheimer, 1934-1938; 1 Brief von Max Horkheimer an Hugo Sinzheimer, 20.10.1934; 15 Briefe zwischen William Dieterle, Charlotte Dieterle und Max Horkheimer, 1940-1944; 1 Brief von der Dillmann-Oberschule Stuttgart an Max Horkheimer, 13.12.1949; 1 Brief vonMax Horkheimer an W. R. Dittmar, 10.02.1938; 2 Briefe zwischen Sofie Doernberg, Paul Doernberg und Margot von Mendelssohn, 1942, 06.04.1942; 4 Briefe zwischen Willy Dörter und Max Horkheimer, 1936-1937, 20.02.1936; 1 Brief von Georg Glaser anMax Horkheimer; 3 Briefe zwischen Hans Venedey und Max Horkheimer, 22.01.1938, 1938; 28 Briefe sowie Zeitungsausschnitte zwischen Juliette Favez und Max Horkheimer, 1934-1938; 1 Brief von Juliette Favez ann karl Dörter, 02.10.1934; 2 Briefe von Max Horkheimer an Hans Klaus Brill, Januar 1938; 3 Briefe zwischen Karl Dörter und Max Horkheimer, 1934-1937, 12.10.1934; 4 Briefe zwischen Andrés Sternheim und Max Horkheimer, 1937, 16.06.1937; 1 Brief von Else Klee an Max Horkheimer, 13.06.1935;

Na 50 Nachlass Arthur Schopenhauer - 'Schopenhauer-Archiv', 625 - Mitteilung von Edward J. Young der für zwei Artikel verantwortlichen Verfasser in der Westminster Review John Oxenford

u.a.: Besuch bei Schopenhauer im Sommer 1856;

Edward Lankow, Hüftbild

Signatur des Originals: S 36/F05138