General Pathology
-
Upload
beck-weiss -
Category
Documents
-
view
30 -
download
1
description
Transcript of General Pathology
![Page 1: General Pathology](https://reader036.fdocuments.in/reader036/viewer/2022062304/56813507550346895d9c585f/html5/thumbnails/1.jpg)
General Pathology Cellular and Organ Pathology
Disorders of Glycogen Degradation.
Pathology of Calcification.
Jaroslava Dušková
Inst. Pathol. ,1st Med. Faculty, Charles Univ. Prague
![Page 2: General Pathology](https://reader036.fdocuments.in/reader036/viewer/2022062304/56813507550346895d9c585f/html5/thumbnails/2.jpg)
Glycogen linear and branched polymer cca 60 000 - D-glucose
molecules– monoparticles (beta) - muscle– complex particles (alpha) -
hepatocyte
![Page 3: General Pathology](https://reader036.fdocuments.in/reader036/viewer/2022062304/56813507550346895d9c585f/html5/thumbnails/3.jpg)
EnzymesInvolved in Glycogen Metabolism
g.-synthase - brancher phophorylase kinase - debrancher g-6-phosphatase -glucosidase
![Page 4: General Pathology](https://reader036.fdocuments.in/reader036/viewer/2022062304/56813507550346895d9c585f/html5/thumbnails/4.jpg)
Storage DiseasesDef.:
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“
![Page 5: General Pathology](https://reader036.fdocuments.in/reader036/viewer/2022062304/56813507550346895d9c585f/html5/thumbnails/5.jpg)
E defect - gl-6 - phosphatase
Organ damage - liver, kidney
Glycogenosis I – von Gierke
![Page 6: General Pathology](https://reader036.fdocuments.in/reader036/viewer/2022062304/56813507550346895d9c585f/html5/thumbnails/6.jpg)
E -defect - alfa1, 4 - glycosidase Organ damage - heart
Glycogenosis II – Pompe
![Page 7: General Pathology](https://reader036.fdocuments.in/reader036/viewer/2022062304/56813507550346895d9c585f/html5/thumbnails/7.jpg)
Glycogen Storage Diseases -1.
Disease E- def Severity of Disease
Tissues Involved
von Gierke
Glucose-6
phosphatase
Severe Liver,kidney, gut
Pompe 1,4 glucosidase
Lethal Heart (+systemic)
Cori Amylo-1,6 Glucosidase (debrancher)
Lethal Liver (+systemic)
![Page 8: General Pathology](https://reader036.fdocuments.in/reader036/viewer/2022062304/56813507550346895d9c585f/html5/thumbnails/8.jpg)
Glycogen Storage Diseases -2.
Disease E- def Severity of Disease
Tissues Involved
Andersen Amylo-1,4-1,6 transglucosidase (brancher)
Lethal Liver (+systemic)
Mc Ardle Muscle phosphorylase
Mild Skeletal muscle
Hers Liver
phosphorylaseMild Liver
![Page 9: General Pathology](https://reader036.fdocuments.in/reader036/viewer/2022062304/56813507550346895d9c585f/html5/thumbnails/9.jpg)
Clear Intracellular Vacuoles & adjunct techniques
accumulations of water neg. lipides SUDAN, OIL RED polysaccharides PAS, A-PAS
![Page 10: General Pathology](https://reader036.fdocuments.in/reader036/viewer/2022062304/56813507550346895d9c585f/html5/thumbnails/10.jpg)
Glycogen water soluble
easily lost in long lasting water based fixative solutions
![Page 11: General Pathology](https://reader036.fdocuments.in/reader036/viewer/2022062304/56813507550346895d9c585f/html5/thumbnails/11.jpg)
CalcificationDef.:
depositions of Ca (mostly phosphate
salts) in tissues or organs Classification:
dystrophic
metastatic
![Page 12: General Pathology](https://reader036.fdocuments.in/reader036/viewer/2022062304/56813507550346895d9c585f/html5/thumbnails/12.jpg)
Matrix vesicles - osteoblasts- nidus calcification
Non collagen proteins - osteopontin,
osteonektin, osteokalcin, Gla protein, sialoprotein
Alkalic phosphatase Phospholipids Collagen I Hydroxyapatite
Calcification - physiology
![Page 13: General Pathology](https://reader036.fdocuments.in/reader036/viewer/2022062304/56813507550346895d9c585f/html5/thumbnails/13.jpg)
Calcification dystrophic metastatic
Calcinosis localized generalized
Chondrocalcinosis - pseudogout
Pathology Conditions with Calcium Deposits
![Page 14: General Pathology](https://reader036.fdocuments.in/reader036/viewer/2022062304/56813507550346895d9c585f/html5/thumbnails/14.jpg)
Basophilic
Von Kossa - Ag impregnation
Alizarine red +
Tetracyclin fluorescence
Polarized light birefringence
Calcification - Microscopy
![Page 15: General Pathology](https://reader036.fdocuments.in/reader036/viewer/2022062304/56813507550346895d9c585f/html5/thumbnails/15.jpg)
Calcification
Dystrophic Serum Ca level: normal
Tissues/Organs status
dystrophic changes
(necrosis, scar…, low
metab. turnover)
MetastaticSerum Ca level:
Tissues/Organs status
normal, local alcalisation
(acid secretion - urine,
stomach juice, sweat…)
![Page 16: General Pathology](https://reader036.fdocuments.in/reader036/viewer/2022062304/56813507550346895d9c585f/html5/thumbnails/16.jpg)
Ca in mitochondria and GER
in cytoplasm bound to proteins
Released after cell damage
Activaton of protein kinases
Activaton of phospholipid
degradation and loss
Activaton of proteases
Cytoskeletal disassembly
Membrane damage
Phosphorylation of protein
andchromatin
phragmentation
![Page 17: General Pathology](https://reader036.fdocuments.in/reader036/viewer/2022062304/56813507550346895d9c585f/html5/thumbnails/17.jpg)
Necrosis or degeneration
of tissue
Release of enzymes
Breakdown of organic
phophatesAlteration of pH
Increased deposition of calcium
Dystrophic Calcification
![Page 18: General Pathology](https://reader036.fdocuments.in/reader036/viewer/2022062304/56813507550346895d9c585f/html5/thumbnails/18.jpg)
Calcification
Dystrophic Serum Ca level: normal
Tissues/Organs status
dystrophic changes
(necrosis, scar…, low
metab. turnover)
MetastaticSerum Ca level:
Tissues/Organs status
normal, local alcalisation
(acid secretion - urine,
stomach juice, sweat…)
![Page 19: General Pathology](https://reader036.fdocuments.in/reader036/viewer/2022062304/56813507550346895d9c585f/html5/thumbnails/19.jpg)
Ca phophate Ca3(PO4)2
Ca diphosphate (Ca2P2O7)
Hydroxyapatite (Ca5 (PO4)3.OH
Ca Salts in the Calcified Foci
![Page 20: General Pathology](https://reader036.fdocuments.in/reader036/viewer/2022062304/56813507550346895d9c585f/html5/thumbnails/20.jpg)
Calcification dystrophic metastatic
Calcinosis localized generalized
Chondrocalcinosis - pseudogout
Pathology Conditions with Calcium Deposits
![Page 21: General Pathology](https://reader036.fdocuments.in/reader036/viewer/2022062304/56813507550346895d9c585f/html5/thumbnails/21.jpg)
Frequent necrotic tissue connective tissue vessels kalkospherites intracellular calcifiction of lysosoms
Less frequent tendon, cartilage, elastics, bas. membranes
Dystrophic Calcification
![Page 22: General Pathology](https://reader036.fdocuments.in/reader036/viewer/2022062304/56813507550346895d9c585f/html5/thumbnails/22.jpg)
Forms - localised generalised
Localisation – connective tissue,
muscles
Calcinosis
![Page 23: General Pathology](https://reader036.fdocuments.in/reader036/viewer/2022062304/56813507550346895d9c585f/html5/thumbnails/23.jpg)
pyrophophate and hydroxyapatite deposits
localisation – synovial membrane, cortilage, bone
Chondrocalcinosiscrystal deposit disease (pseudogout)
![Page 24: General Pathology](https://reader036.fdocuments.in/reader036/viewer/2022062304/56813507550346895d9c585f/html5/thumbnails/24.jpg)
Calcium pyrophosphate deposition disease(Chondrocalcinosis, pseudogout)
Clinic: may simulate different diseasesArthroscopy - chalky white depositsTophaceus deposits with crystalline aggregatesCrystals stain with von Kossa techniqueForeign body type multinucleated giant cell reactionDeposits are in synovium, cartilage, bone