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Contents 1 Fundamentals of Pathology Pathology .............................. 2 Signs of Death .......................... 5 Methods Equivocal Signs of Death Brain Death Disease ................................ 5 Unequivocal Signs of Death Etiology Causal Pathogenesis Statistics ............................... 5 Formal Pathogenesis Clinical Course Autopsy ................................ 5 2 Cellular Pathology Nuclear Lesions ........................ 6 Inclusions ............................. 12 Forms of Nuclei Amorphous inclusions Chromosomes Crystalline inclusions Particulate inclusions DNA Repair Defects ..................... 6 Xeroderma pigmentosum Oncofetal Lesion ....................... 12 Ribosome-lamellae complexes Chromosome Abnormalities .............. 6 Annulated lamellae layers Change in the Size of the Nucleus ........ 8 Mitochondria-lamellar-layer complexes Relation of Nucleus to Cytoplasm Nuclear Euploidy Lesions of the Smooth Endoplasmic Nuclear Polyploidy Nuclear Aneuploidy Reticulum (SER) ...................... 14 Chromatin Change ...................... 8 Quantitative Change ................... 14 Heterochromatin Condensation Proliferation of SER Dyskaryosis Atrophy of the SER Perinuclear Hyperchromatosis Morphologic Change ................... 14 Karyolysis Formation of Vesicles in the SER Inclusions .............................. 8 „Cytoplasmic Nuclei“ Cytoplasmic inclusions Inclusions ............................. 14 Paraplasmic inclusions Change in the Number of Nuclei ......... 10 Golgi Lesions ......................... 16 Polynuclear Cells Anuclear Cells Atrophy of the Golgi Apparatus .......... 16 Antinuclear Autoantibodies ............. 10 Hypertrophy of the Golgi Apparatus ..... 16 Nucleolar Change ...................... 10 Golgi-associated disorders ............... 16 Inflammatory nucleoli Cholestasis Tumor nucleoli Fatty Liver Giant nucleoli Achondroplasia Alveolar Proteinosis Lesions of the Rough Endoplasmic Reticulum (RER) ...................... 12 Mitochondrial Lesions ................ 18 Quantitative Change ................... 12 Quantitative Change ................... 18 Increase in the number of cisterns Mitochondrial Proliferation Decrease in the number of cisterns Mitochondrial Depletion Morphologic Change ................... 12 Morphologic Change ................... 18 Fragmentation of the RER Megamitochondria Formation of vacuoles in the RER Mitochondrial Swelling Swelling of the RER RER cistern collapse Structural Change in Mitochondrial DNA .. 20 XII Contents Mitochondrial DNA Mutations ........... 20 Lysosomal Phase ....................... 28 Congenital Forms Heterophagy Acquired Forms Autophagy Changes in the Mitochondrial Cristae .... 20 Post-lysosomal Phase ................... 28 Membrane proliferation Lysosomal Lipid Pigments Membrane loss Lysosomal Diseases .................... 30 Inclusions ............................. 20 Delayed Enzyme Release Matrix inclusions Increased Enzyme Release Crista inclusions Lysosomal Enzyme Deficiency Grana inclusions Cytomembrane Lesions ............... 32 Mitochondrial Disorders .............. 22 Apical Cell Edema Disrupted Communication Mitochondrial Myopathies .............. 22 Hypoxia ............................... 22 Receptor Lesions ..................... 32 Receptor dysfunction Hypoxemic Hypoxia Ischemic Hypoxia Cytoskeletal Lesions .................. 34 Hyperglycemic Hypoxia Histotoxic Hypoxia Microtubular Lesions ................... 34 Polymerization Block Mitochondrial Autoantibodies ........... 22 Microfilament Lesions Peroxisomal Lesions .................. 24 Intermediate Filament Lesions ........... 34 Peroxisomal Diseases ................... 24 Actin Bodies Mallory Bodies Acatalasemia Refsum Disease Cerebrohepatorenal Syndrome Adrenoleukodystrophy Systemic Carnitine Deficiency Lysosomal Lesions .................... 26 Prelysosomal Phase .................... 26 Heterophagy Autophagy 3 Connective Tissue Lesions Collagen Lesions ...................... 36 Structure of Amyloid .................... 50 Types of Amyloid ....................... 50 Types of Collagen ...................... 36 AL Amyloid Biosynthesis Disorders .................. 38 AA Amyloid Ehlers-Danlos Syndrome (EDS) AF Amyloid Scurvy AE Amyloid Osteogenesis Imperfecta AS Amyloid Classification of Amyloidoses ............. 52 Fibrosis ............................... 40 Systemic Amyloidosis Sequelae of Fibrosis .................... 40 Localized Amyloidosis Pulmonary Fibrosis Sequelae of Fibrosis .................... 42 Elastin Lesions ........................ 54 Cirrhosis of the Liver Biosynthesis Disorders .................. 54 Collagenolysis Disorders ................ 44 Restrictive Dermopathy a1-Antitrypsin Deficiency Senile Elastosis Fibrinoid Necrosis Trichopoliodystrophy Collagen Hyalinization Type V Ehlers-Danlos Syndrome Elastosis ............................... 56 Collagen Vascular Diseases .............. 46 Actinic Elastosis Microfibril Lesions .................... 48 Pseudoxanthoma Elasticum Marfan’s Syndrome Fibroelastosis .......................... 56 Sequelae of Fibroelastosis ................ 56 Amyloidosis ........................... 48 Intimal Fibroelastosis General Definitions ..................... 48 Endocardial Fibroelastosis Contents XIII Elastin Lysis Disorders .................. 58 Sequelae of Mucoid Degeneration ........ 62 Sequelae of Elastin Lysis Disorders ........ 58 Proteoglycan Secretion Disorders ........ 64 Polyarteritis nodosa Temporal Arteritis Cystic Fibrosis .......................... 64 Syphilitic Aortitis Sequelae of Cystic Fibrosis ............... 64 Bronchiectasis Proteoglycan Lesions ................. 60 Cystic fibrosis of the pancreas Meconium ileus Proteoglycan Complexing Disorders ...... 60 Liver cirrhosis Mucoid Degeneration Sequelae of Mucoid Degeneration ........ 60 Proteoglycan Lysis Deficiencies .......... 66 Mucoid Meniscopathy Mucopolysaccharidoses .................. 66 Ganglion Hurler’s Syndrome Cystic Vascular Degeneration Hunter’s Syndrome Mitral Valve Prolapse Syndrome Mucolipidoses .......................... 66 4 Errors of Metabolism: Inorganic Compounds Oxygen .............................. 68 Iron .................................. 72 Acute Generalized Hypoxia .............. 68 Iron Deficiency ........................ 72 Absolute Iron Deficiency Acute Local Hypoxia .................... 69 Relative Iron Deficiency Chronic Hypoxia ....................... 69 Iron Overload ......................... 72 Fatty Degeneration Hemochromatosis Organ Atrophy Secondary Siderosis Interstitial Fibrosis Copper ............................... 74 Calcium .............................. 70 Copper Deficiency ...................... 74 Hypocalcemia ......................... 70 Chronic Copper Deficiency Hypercalcemia ......................... 70 Trichopoliodystrophy Copper Overload ....................... 74 Wilson’s Disease 5 Errors of Metabolism: Organic Compounds Carbohydrates ........................ 76 Malabsorption Syndromes ............... 82 Disorders Associated with Malabsorption ... 82 Gluten Enteropathy Glycogenoses .......................... 76 Whipple Disease Type I Glycogenosis Type II Glycogenosis Fatty Degeneration ..................... 84 Type III Glycogenosis Dietary Steatosis Transport Steatosis Carbohydrate Malabsorption ............ 78 Absorptive Steatosis Lactose Intolerance Retention Steatosis Saccharose-Isomaltose Intolerance Disorders of Fat Catabolism ............. 84 Glucose Synthesis Disorders ............. 78 Starvation Fructose-1,6-Diphosphatase Deficiency Obesity Hereditary Fructose Intolerance Ketonemia Deficient Carbohydrate Metabolism ...... 78 Phytanic Acid Storage Disease Propionic Acidemia Diabetes Mellitus ....................... 78 Methylmalonic Aciduria Type I Diabetes mellitus Type II Diabetes mellitus Hypolipoproteinemia Disorders .......... 86 Sequelae .............................. 80 Abetalipoproteinemia Galactosemia .......................... 80 Analphalipoproteinemia Lipids ................................ 82 Hyperlipoproteinemia Disorders ......... 88 Type I Hyperlipoproteinemia Fat Uptake Disorders ................... 82 Type II Hyperlipoproteinemia XIV Contents Type III Hyperlipoproteinemia Disorders of Amino Acid Metabolism ..... 96 Type IV Hyperlipoproteinemia Phenylketonuria Type V Hyperlipoproteinemia Albinism Familial LCAT Deficiency Alkaptonuria1 Sequelae of Hyperlipoproteinemia ........ 90 Maple Syrup Urine Disease Atherosclerosis Primary Hyperoxaluria Xanthoma Type I Homocystinuria Cystinuria Sphingolipidoses ....................... 92 Cystinosis Gaucher’s Disease Sphingomyelin Lipidosis Urea Synthesis Disorders .............. 100 Krabbe’s Disease Metachromatic Leukodystrophy Nucleotides ......................... 102 Fabry Disease Hyperuricemia ........................ 102 Type I GM Gangliosidosis 2 Primary Gout GM Gangliosidosis 1 Sequelae of Adult Gout Proteins .............................. 96 Secondary Gout Defective Serum Proteins ............... 96 6 Pigment Lesions Exogenous Pigments ................ 104 Defective Bilirubin Conjugation Defective Bilirubin Excretion Cosmetic Pigments .................... 104 Tyrosine-Derivative Pigments ............112 Occupational Toxin Pigments ........... 104 Cutaneous Melanin .....................112 Anthracosis Vitiligo Plumbism Albinism Chalcosis Hemochromatosis Iatrogenic Pigments ................... 104 Addison’s Disease Argyria Phenylketonuria Amalgam Melasma Tetracycline Neuromelanin ..........................114 Carotene Parkinson’s

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