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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