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General Pathology

General Pathology. Cellular and Organ Pathology Disorders of Glycogen Degradation. Pathology of Calcification. Jaroslava Dušková Inst. Pathol. ,1st Med. Faculty, Charles Univ. Prague. Glycogen. linear and branched polymer cca 60 000 - D-glucose molecules monoparticles (beta) - muscle

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General Pathology

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  1. General Pathology Cellular and Organ Pathology Disorders of Glycogen Degradation. Pathology of Calcification. Jaroslava Dušková Inst. Pathol. ,1st Med. Faculty, Charles Univ. Prague

  2. Glycogen • linear and branched polymer • cca 60 000 - D-glucose molecules • monoparticles (beta) - muscle • complex particles (alpha) - hepatocyte

  3. EnzymesInvolved in Glycogen Metabolism • g.-synthase - brancher • phophorylase kinase - debrancher • g-6-phosphatase • -glucosidase

  4. Storage Diseases Def.: inborn errors of metabolism (mostly single gene abnormality) leading to an enzyme defect with subsequent accumulation of the substrate (& lack of the product) in tissues or organs„thesaurismoses“

  5. Glycogenosis I – von Gierke E defect - gl-6 - phosphatase Organ damage - liver, kidney

  6. Glycogenosis II – Pompe E -defect - alfa1,4-glycosidase Organ damage - heart

  7. Glycogen Storage Diseases -1.

  8. Glycogen Storage Diseases -2.

  9. Clear Intracellular Vacuoles& adjunct techniques • accumulations of water neg. • lipides SUDAN, OILRED • polysaccharides PAS, A-PAS

  10. Glycogen water soluble easily lost in long lasting water based fixative solutions

  11. Calcification Def.: depositions of Ca (mostly phosphate salts) in tissues or organs Classification: dystrophic metastatic

  12. Calcification - physiology • Matrix vesicles - osteoblasts- nidus calcification • Non collagen proteins - osteopontin, osteonektin, osteokalcin, Gla protein,sialoprotein • Alkalic phosphatase • Phospholipids • Collagen I • Hydroxyapatite

  13. Pathology Conditions with Calcium Deposits Calcification • dystrophic • metastatic Calcinosis • localized • generalized Chondrocalcinosis-pseudogout

  14. Calcification - Microscopy • Basophilic • Von Kossa - Ag impregnation • Alizarine red + • Tetracyclin fluorescence • Polarized light birefringence

  15. Dystrophic Serum Ca level: normal Tissues/Organs status dystrophic changes (necrosis, scar…, low metab. turnover) Metastatic Serum Ca level: Tissues/Organs status normal, local alcalisation (acid secretion - urine, stomach juice, sweat…) Calcification

  16. Dystrophic Calcification

  17. Dystrophic Serum Ca level: normal Tissues/Organs status dystrophic changes (necrosis, scar…, low metab. turnover) Metastatic Serum Ca level: Tissues/Organs status normal, local alcalisation (acid secretion - urine, stomach juice, sweat…) Calcification

  18. Ca Salts in the Calcified Foci • Ca phophate Ca3(PO4)2 • Ca diphosphate (Ca2P2O7) • Hydroxyapatite (Ca5 (PO4)3.OH

  19. Pathology Conditions with Calcium Deposits Calcification • dystrophic • metastatic Calcinosis • localized • generalized Chondrocalcinosis-pseudogout

  20. Dystrophic Calcification • Frequent • necrotic tissue • connective tissue • vessels • kalkospherites • intracellular calcifiction of lysosoms Less frequent tendon, cartilage, elastics, bas. membranes

  21. Calcinosis Forms - localised generalised Localisation– connective tissue, muscles

  22. Chondrocalcinosis crystal deposit disease (pseudogout) pyrophophate and hydroxyapatite deposits localisation– synovial membrane, cortilage, bone

  23. Calcium pyrophosphate deposition disease(Chondrocalcinosis, pseudogout) • Clinic: may simulate different diseases • Arthroscopy - chalky white deposits • Tophaceus deposits with crystalline aggregates • Crystals stain with von Kossa technique • Foreign body type multinucleated giant cell reaction • Deposits are in synovium, cartilage, bone

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