Metabolic Cardiomyopathy （2nd, rev. ed. 2004. XI, 166 p. w. figs. 23,5 cm）

Metabolic Cardiomyopathy （2nd, rev. ed. 2004. XI, 166 p. w. figs. 23,5 cm）

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Full Description


During the last 30 years a great deal of information has accumulated in the field of inherited metabolic diseases. This information provides a new foundation for our understanding of many heart muscle problems and their corresponding clinical disease entities. Drawing on the latest information in the field, Metabolic Cardiomyopathy provides a clear overview of the cardiological manifestations of inherited errors of metabolism. Reviewing current understanding of cardiomyopathies of metabolic origin, the book highlights cardiomyopathy in Fabry disease and congenital disorders of glycosylation (CDG-syndrome). It keeps you up to date on the in the rapidly changing field of cardiomyopathy.

Contents

MORPHOLOGICAL PRESENTATION OF CARDIOMYOPATHY, G. MAILIntroductionDifferential Diagnosis of Hypertrophic CardiomyopathyDifferential Diagnosis of Dilated CardiomyopathyBiopsy TechniquesHistopathological DiagnosticsIndicationSuccess and ComplicationsConclusionsBohlesDevelopmental Aspects of Myocardial Energy MetabolismThe Carnitine Plasma Membrane TransportMyocardial Fatty Acid OxidationCarnitine Plasma Membrane Transport Defect (Systemic Carnitine Deficiency)The Carnitine Palmitoyltransferase (CPT) SystemCPT 1CPT DeficienciesCPT 2 DeficienciesCarnitine Translocase DeficiencySecondary Carnitine DeficiencyPrzyrembel and A.C. SewellLong-Chain Acyl-CoA Dehydrogenase (LCAD) Deficiency3-Hydroxyl-CoA Dehydrogenase (LCHAD) Deficiency2,4 Dicnoyl Reductase DeficeincyTrifunctional Protein DeficiencyVery-Long-Chain Accl-CoA Dehydrogenase (VLCAD) DeficiencyLong-Chain 3-Ketothiolase DeficiencyCARDIOMYOPATHY IN ss-KETOTHIOLASE DEFICIENCY, V. Hesse, A.C. Sewell, H. Bohles, H. Haberland, B. Middleton, B. Fiedler, H. Forster, and W. JamishCase ReportFamily HistoryPatientsNeurological StatusCardiological StatusBiochemical BasesGlycogen Storage DiseasesDisorders with Vacuolar Glycogen StorageClinical AspectsDisorders with Cytosolic Glycogen StorageDisorders with Vacuolar Glycogen StorageSperlCharacteristics of the Oxidative Phoshporylating SystemAging PresbycardiaCardiomyopathies as a Consequence of a Secondary Damage of the OXPHOS System (Secondary Mitochondrial Cardiomyopathy)Primary Mitochondrial CardiomyopathiesClassification of Mitochondrial CardiomyopathiesInheritanceEnzyme DefectsCharacterization of the Gene DefectMitochondrial Syndromes with Heart InvolvementDisturbances of Cardiac Rhythm in Defects of the OXPHOS SystemCARBOHYDRATE-DEFICIENT GLYCOPROTEIN SYNDROME, W. Kienast, F. Walter, and K. HeyneH. Bohles, and T. MarquardtIntroductionCase ReportsDiscussionConclusionBeck, D. Schranz, R. Huth, I. Michel-Behnke, and B-K JungstChanges of the Coronary ArteriesPathoanatomical ResultsClinical Results Discussion Alterations of Other Arterial VesselsArterial HypertensionMyocardial ChangesPathoanatomical ResultsClinical ObservationsChanges in the Conduction SystemAlterations of the Heart ValvesPathoanatomical ResultsClinical FindingsTherapeutical Influence on the Cardiovascular AlterationsBone Marrow TransplantationCardiac Valve ReplacementMortality as a Consequence of Cardiovascular CausesConclusionsIntroductionHeartPericarditisMyocardial InfiltrationHeart Valve CalcificationCor PulmonaleConclusionPHENYLKETONURIA-METABOLIC AND ECHOCARDIOGRAPHIC FINDIGS DURING SODIUM SELENITE THERAPY, F. Kauf, L. Vogt, J. Seidel, K. Winnerfeld, H. Richter, H. Vogl, H. Dawezynski, A. Forberger, and D. SchlenvoigtPatients and MethodsResults and DiscussionFABRY DISEASE - A PROGRESSIVE MULTISYSTEMIC LYSOSOMAL STORAGE DISORDER, M. BeckPathophysiologySymptomsPainSkinAutonomic DysfunctionEyesHeartKidneyCerebrovascular SystemGeneticsTreatmentTHE ANDERSON-FABRY DISEASE ASSOCIATED WITH CARDIOMYOPATHY, C. KampmannIntroductionCardiac ManifestationStructural Changes of the MyocardiumChanges in Systolic FunctionChanges in Diastolic FunctionValvular InvolvementInvolvement of the Conduction SystemCoronary Artery Disease"Cardiac Variant" of AFDGender Related DifferencesOnset and Progression of the CardiomyopathyClinical Features of Cardiac InvolvementCardiac Involvement in Children with AFDNatural History and DeathTreatmentEffects of Enzyme Replacement Therapy on the HeartIndication for TreatmentA.C. SewellDefects in Mitochondrial Long-Chain Fatty Acid OxidationDefects in Carnitine MetabolismDisorders of the Respiratory ChainDisorders of Complex Carbohydrate MetabolismOrganoacidopathiesOthers