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2. Komplikationen

Author

Weber, Jörg R., Jacobi, Christian, Höfner, Elmar, Hund, Ernst, Meyding-Lamadé, Uta, editor, Stangel, Martin, editor, and Weber, Jörg, editor

Published
2021
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3. Chance or challenge, spoilt for choice? New recommendations on diagnostic and therapeutic considerations in hereditary transthyretin amyloidosis with polyneuropathy: the German/Austrian position and review of the literature

Author

Dohrn, Maike F., Auer-Grumbach, Michaela, Baron, Ralf, Birklein, Frank, Escolano-Lozano, Fabiola, Geber, Christian, Grether, Nicolai, Hagenacker, Tim, Hund, Ernst, Sachau, Juliane, Schilling, Matthias, Schmidt, Jens, Schulte-Mattler, Wilhelm, Sommer, Claudia, Weiler, Markus, Wunderlich, Gilbert, and Hahn, Katrin

Published
2021
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5. Neuromuskuläre Erkrankungen

Author

Müllges, Wolfgang, Gold, Ralf, Hansen, Hans-Christian, Anetseder, Martin, Metterlein, Thomas, Müller, Clemens R., Hund, Ernst, Winterholler, Martin, Schwab, Stefan, editor, Schellinger, Peter, editor, Werner, Christian, editor, Unterberg, Andreas, editor, and Hacke, Werner, editor

Published
2015
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7. Disturbances of Water and Electrolyte Balance

Author

Hund, Ernst F., Böhrer, Hubert, Martin, Eike, Hanley, Daniel F., Hacke, Werner, editor, Hanley, Daniel F., editor, Einhäupl, Karl M., editor, Bleck, Thomas P., editor, Diringer, Michael N., editor, and Ropper, Allan H., editor

Published
1994
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8. Life-Threatening Hyperthermic Syndromes

Author

Hund, Ernst F., Lehmann-Horn, Frank, Einhäupl, Karl M., Hacke, Werner, Hacke, Werner, editor, Hanley, Daniel F., editor, Einhäupl, Karl M., editor, Bleck, Thomas P., editor, Diringer, Michael N., editor, and Ropper, Allan H., editor

Published
1994
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9. Chronic Inflammatory Demyelinating Polyneuropathy

Author

Hund, Ernst F., Hartung, Hans-Peter, Ropper, Allan H., Hanley, Daniel F., Hacke, Werner, editor, Hanley, Daniel F., editor, Einhäupl, Karl M., editor, Bleck, Thomas P., editor, Diringer, Michael N., editor, and Ropper, Allan H., editor

Published
1994
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11. The Porphyrias

Author

Hund, Ernst F., Schuchardt, Volker, Hanley, Daniel F., Hacke, Werner, editor, Hanley, Daniel F., editor, Einhäupl, Karl M., editor, Bleck, Thomas P., editor, Diringer, Michael N., editor, and Ropper, Allan H., editor

Published
1994
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12. Nutrition

Author

Schultz, Tricia, Hund, Ernst F., Hanley, Daniel F., Hacke, Werner, editor, Hanley, Daniel F., editor, Einhäupl, Karl M., editor, Bleck, Thomas P., editor, Diringer, Michael N., editor, and Ropper, Allan H., editor

Published
1994
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19. Understanding critical illness myopathy: approaching the pathomechanism

Author

Friedrich, Oliver, Fink, Rainer H. A., and Hund, Ernst

Subjects
Food/cooking/nutrition
Abstract

Myopathies occurring in critically ill patients have gained increasing interest during the past years. For the patient, they represent a crucial factor for prolonged intensive care unit treatment and secondary complications. Critical illness myopathies (CIMs) seem to be related to various pathogenic factors. Among those, the septic inflammatory response syndrome seems to play a major role. It has been suggested that, similar to sepsis-related multiorgan failure, CIM might be considered a failure of the organ muscle. Muscle function might be impaired by proposed 'myotoxic' humoral factors. These could be endogenously produced during the innate immune response to sepsis. This article follows up recent evidence for such active fractions in the blood serum of CIM patients. To explain muscle weakness in CIM, serum fractions acutely modified membrane excitability and subcellular C[a.sup.2+] regulation in an animal model. From the differential serum effects, early-phase CIM seems to involve a reduction in the overall force generation in muscle but also a compensation by the membrane, increasing the excitability. Different animal models will help to elucidate the underlying mechanisms accounting for the specific proteolytic activities found in different forms of CIM. CIM represents a systemic rather than a local disorder. Humoral factors might initiate the local reaction of skeletal muscle clinically seen as muscle weakness, altered excitability, and proteolysis of contractile proteins. Establishing the interactions in the excitation-contraction cascade in CIM is a challenging task, not only to clarify its pathomechanism but also to deduce clinical interventions. J. Nutr. 135: 1813S-1817S, 2005. KEY WORDS: * myopathy * critical illness * skeletal muscle * calcium regulation

Published
2005

20. Real-world outcomes in non-endemic hereditary transthyretin amyloidosis with polyneuropathy: a 20-year German single-referral centre experience

Author

Ungerer, Matthias N., primary, Hund, Ernst, additional, Purrucker, Jan C., additional, Huber, Laura, additional, Kimmich, Christoph, additional, aus dem Siepen, Fabian, additional, Hein, Selina, additional, Kristen, Arnt V., additional, Hinderhofer, Katrin, additional, Kollmer, Jennifer, additional, Schönland, Stefan, additional, Hegenbart, Ute, additional, and Weiler, Markus, additional

Published
2020
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25. Chance or challenge, spoilt for choice? New recommendations on diagnostic and therapeutic considerations in hereditary transthyretin amyloidosis with polyneuropathy: the German/Austrian position and review of the literature

Author

Dohrn, Maike F., primary, Auer-Grumbach, Michaela, additional, Baron, Ralf, additional, Birklein, Frank, additional, Escolano-Lozano, Fabiola, additional, Geber, Christian, additional, Grether, Nicolai, additional, Hagenacker, Tim, additional, Hund, Ernst, additional, Sachau, Juliane, additional, Schilling, Matthias, additional, Schmidt, Jens, additional, Schulte-Mattler, Wilhelm, additional, Sommer, Claudia, additional, Weiler, Markus, additional, Wunderlich, Gilbert, additional, and Hahn, Katrin, additional

Published
2020
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26. Magnetization transfer ratio quantifies polyneuropathy in hereditary transthyretin amyloidosis

Author

Kollmer, Jennifer, primary, Hegenbart, Ute, additional, Kimmich, Christoph, additional, Hund, Ernst, additional, Purrucker, Jan C., additional, Hayes, John M., additional, Lentz, Stephen I., additional, Sam, Georges, additional, Jende, Johann M. E., additional, Schönland, Stefan O., additional, Bendszus, Martin, additional, Heiland, Sabine, additional, and Weiler, Markus, additional

Published
2020
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27. Real-world outcomes in non-endemic hereditary transthyretin amyloidosis with polyneuropathy: a 20-year German single-referral centre experience.

Author

Ungerer, Matthias N., Hund, Ernst, Purrucker, Jan C., Huber, Laura, Kimmich, Christoph, aus dem Siepen, Fabian, Hein, Selina, Kristen, Arnt V., Hinderhofer, Katrin, Kollmer, Jennifer, Schönland, Stefan, Hegenbart, Ute, and Weiler, Markus

Subjects
*CARDIAC amyloidosis, *AMYLOIDOSIS, *TRANSTHYRETIN, *POLYNEUROPATHIES, *NEUROLOGICAL disorders, *BRAIN natriuretic factor
Abstract

Hereditary transthyretin amyloidosis is caused by pathogenic variants in the TTR gene and typically manifests, alongside cardiac and other organ dysfunctions, with a rapidly progressive sensorimotor and autonomic polyneuropathy (ATTRv-PN) leading to severe disability. While most prospective studies have focussed on endemic ATTRv-PN, real-world data on non-endemic, mostly late-onset ATTRv-PN are limited. This retrospective study investigated ATTRv-PN patients treated at the Amyloidosis Centre of Heidelberg University Hospital between November 1999 and July 2020. Clinical symptoms, survival, prognostic factors and efficacy of treatment with tafamidis were analysed. Neurologic outcome was assessed using the Coutinho ATTRv-PN stages, and the Peripheral Neuropathy Disability (PND) score. Of 346 subjects with genetic TTR variants, 168 patients had symptomatic ATTRv-PN with 32 different TTR variants identified. Of these, 81.6% had the late-onset type of ATTRv-PN. Within a mean follow-up period of 4.1 ± 2.8 years, 40.5% of patients died. Baseline plasma N-terminal prohormone of brain natriuretic peptide (NT-proBNP) ≥900 ng/l (HR 3.259 [1.421–7.476]; p =.005) was the main predictor of mortality in multivariable analysis. 64 patients were treated with tafamidis and presented for regular follow-up examinations. The therapeutic benefit of tafamidis was more pronounced when treatment was started early in ATTRv-PN stage 1 (PND scores II vs. I; HR 2.718 [1.258–5.873]; p =.011). In non-endemic, mostly late-onset ATTRv-PN, cardiac involvement assessed by NT-proBNP is a strong prognosticator for overall survival. Long-term treatment with tafamidis is safe and efficacious. Neurologic disease severity at the start of treatment is the main predictor for ATTRv-PN progression on tafamidis. [ABSTRACT FROM AUTHOR]

Published
2021
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29. Sural nerve injury in familial amyloid polyneuropathy

Author

Kollmer, Jennifer, primary, Sahm, Felix, additional, Hegenbart, Ute, additional, Purrucker, Jan C., additional, Kimmich, Christoph, additional, Schönland, Stefan O., additional, Hund, Ernst, additional, Heiland, Sabine, additional, Hayes, John M., additional, Kristen, Arnt V., additional, Röcken, Christoph, additional, Pham, Mirko, additional, Bendszus, Martin, additional, and Weiler, Markus, additional

Published
2017
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30. Lenalidomide/melphalan/dexamethasone in newly diagnosed patients with immunoglobulin light chain amyloidosis: results of a prospective phase 2 study with long-term follow up

Author

Hegenbart, Ute, primary, Bochtler, Tilmann, additional, Benner, Axel, additional, Becker, Natalia, additional, Kimmich, Christoph, additional, Kristen, Arnt V., additional, Beimler, Jörg, additional, Hund, Ernst, additional, Zorn, Markus, additional, Freiberger, Anja, additional, Gawlik, Marianne, additional, Goldschmidt, Hartmut, additional, Hose, Dirk, additional, Jauch, Anna, additional, Ho, Anthony D., additional, and Schönland, Stefan O., additional

Published
2017
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31. First European consensus for diagnosis, management, and treatment of transthyretin familial amyloid polyneuropathy

Author

Adams, David, Suhr, Ole B., Hund, Ernst, Obici, Laura, Tournev, Ivailo, Campistol, Josep M., Slama, Michel S., Hazenberg, Bouke P., Coelho, Teresa, Adams, David, Suhr, Ole B., Hund, Ernst, Obici, Laura, Tournev, Ivailo, Campistol, Josep M., Slama, Michel S., Hazenberg, Bouke P., and Coelho, Teresa

Abstract

Purpose of review Early and accurate diagnosis of transthyretin familial amyloid polyneuropathy (TTR-FAP) represents one of the major challenges faced by physicians when caring for patients with idiopathic progressive neuropathy. There is little consensus in diagnostic and management approaches across Europe. Recent findings The low prevalence of TTR-FAP across Europe and the high variation in both genotype and phenotypic expression of the disease means that recognizing symptoms can be difficult outside of a specialized diagnostic environment. The resulting delay in diagnosis and the possibility of misdiagnosis can misguide clinical decision-making and negatively impact subsequent treatment approaches and outcomes. Summary This review summarizes the findings from two meetings of the European Network for TTR-FAP (ATTReuNET). This is an emerging group comprising representatives from 10 European countries with expertise in the diagnosis and management of TTR-FAP, including nine National Reference Centres. The current review presents management strategies and a consensus on the gold standard for diagnosis of TTR-FAP as well as a structured approach to ongoing multidisciplinary care for the patient. Greater communication, not just between members of an individual patient's treatment team, but also between regional and national centres of expertise, is the key to the effective management of TTR-FAP., Supplement: 1

Published
2016
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33. Familial amyloidotic polyneuropathy: current and emerging treatment options for transthyretin-mediated amyloidosis

Author

Hund, Ernst

Subjects
The Application of Clinical Genetics, nutritional and metabolic diseases
Abstract

Ernst HundDepartment of Neurology, University of Heidelberg, Heidelberg, GermanyAbstract: Transthyretin familial amyloid polyneuropathy (TTR-FAP) is a fatal clinical disorder characterized by extracellular deposition of abnormal fibrils derived from misfolded, normally soluble transthyretin (TTR) molecules. The disease is most commonly caused by a point mutation within the TTR gene inherited in an autosomal dominant fashion. Over 100 of such mutations have been identified, leading to destabilization of the physiological TTR tetramer. As a result, many monomers originate with a tendency for spontaneous conformational changes and self-aggregation. The main clinical feature of TTR-FAP is progressive sensorimotor and autonomic neuropathy. In the beginning, this polyneuropathy predominantly involves small unmyelinated nerve fibers with the result of dissociated sensory loss disproportionately affecting sensation of pain and temperature. Autonomic neuropathy typically accompanies sensory deficits early in the disease course. The symptoms include orthostatic hypotension, constipation alternating with diarrhea, erectile dysfunction, anhydrosis, and urinary retention or incontinence. Later, involvement of motor fibers causes rapidly progressive weakness and gait disturbances. In addition to the peripheral nervous system, the heart and the gut are frequently affected. Onset of symptoms is bimodal, with one peak at age 33 years (early onset) and another distinct peak in the sixth decade of life (late onset). The course of TTR-FAP is uniformly progressive and fatal. Death occurs an average of 10.8 years after the onset of symptoms in Portuguese patients, and 7.3 years in late-onset Japanese patients. Common causes include cachexia, cardiac failure, arrhythmia, and secondary infections. Liver transplantation is the standard therapy for patients who are in a clinical condition good enough to tolerate this intervention because it stops progression of neuropathy by removing the main source of mutant TTR. Recently, orally administered tafamidis meglumine has been approved by European authorities for treatment of FAP. The substance has been shown to stabilize the TTR tetramer, thereby improving the outcome of patients with TTR-FAP. Various other strategies have been studied in vitro to prevent TTR amyloidosis, including gene therapy, immunization, dissolution of TTR aggregates, and free radical scavengers, but none of them is ready for clinical use so far.Keywords: FAP, transthyretin, amyloidosis, treatment, therapy

Published
2012

35. In vivo detection of nerve injury in familial amyloid polyneuropathy by magnetic resonance neurography

Author

Kollmer, Jennifer, primary, Hund, Ernst, additional, Hornung, Benjamin, additional, Hegenbart, Ute, additional, Schönland, Stefan O., additional, Kimmich, Christoph, additional, Kristen, Arnt V., additional, Purrucker, Jan, additional, Röcken, Christoph, additional, Heiland, Sabine, additional, Bendszus, Martin, additional, and Pham, Mirko, additional

Published
2014
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36. Familial amyloidotic polyneuropathy: current and emerging treatment options for transthyretin-mediated amyloidosis

Author

Hund,Ernst and Hund,Ernst

Abstract

Ernst HundDepartment of Neurology, University of Heidelberg, Heidelberg, GermanyAbstract: Transthyretin familial amyloid polyneuropathy (TTR-FAP) is a fatal clinical disorder characterized by extracellular deposition of abnormal fibrils derived from misfolded, normally soluble transthyretin (TTR) molecules. The disease is most commonly caused by a point mutation within the TTR gene inherited in an autosomal dominant fashion. Over 100 of such mutations have been identified, leading to destabilization of the physiological TTR tetramer. As a result, many monomers originate with a tendency for spontaneous conformational changes and self-aggregation. The main clinical feature of TTR-FAP is progressive sensorimotor and autonomic neuropathy. In the beginning, this polyneuropathy predominantly involves small unmyelinated nerve fibers with the result of dissociated sensory loss disproportionately affecting sensation of pain and temperature. Autonomic neuropathy typically accompanies sensory deficits early in the disease course. The symptoms include orthostatic hypotension, constipation alternating with diarrhea, erectile dysfunction, anhydrosis, and urinary retention or incontinence. Later, involvement of motor fibers causes rapidly progressive weakness and gait disturbances. In addition to the peripheral nervous system, the heart and the gut are frequently affected. Onset of symptoms is bimodal, with one peak at age 33 years (early onset) and another distinct peak in the sixth decade of life (late onset). The course of TTR-FAP is uniformly progressive and fatal. Death occurs an average of 10.8 years after the onset of symptoms in Portuguese patients, and 7.3 years in late-onset Japanese patients. Common causes include cachexia, cardiac failure, arrhythmia, and secondary infections. Liver transplantation is the standard therapy for patients who are in a clinical condition good enough to tolerate this intervention because it stops progression of neuropathy by removing the mai

Published
2012

42. Indications for Liver Transplantation in Patients with Amyloidosis: A Single-Center Experience with 11 Cases

Author

Singer, Reinhard, primary, Mehrabi, Arianeb, additional, Schemmer, Peter, additional, Kashfi, Arash, additional, Hegenbart, Ute, additional, Goldschmidt, Hartmut, additional, Schönland, Stefan, additional, Kristen, Arnt, additional, Dengler, Thomas, additional, Müller-Schilling, Martina, additional, Sauer, Peter, additional, Dogan, Ayhan, additional, Hund, Ernst, additional, Helmke, Burkhard, additional, Schnabel, Philipp, additional, Altland, Klaus, additional, Linke, Reinhold, additional, Friess, Helmut, additional, Schmidt, Jan, additional, Büchler, Markus W., additional, and Kraus, Thomas W., additional

Published
2005
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43. Indications for High-Dose Chemotherapy with Autologous Stem Cell Support in Patients with Systemic Amyloid Light Chain Amyloidosis

Author

Schonland, Stefan O., primary, Perz, Jolanta B., additional, Hundemer, Michael, additional, Hegenbart, Ute, additional, Kristen, Arnt V., additional, Hund, Ernst, additional, Dengler, Thomas J., additional, Beimler, Jorg, additional, Zeier, Martin, additional, Singer, Reinhard, additional, Linke, Reinhold P., additional, Ho, Anthony D., additional, and Goldschmidt, Hartmut, additional

Published
2005
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