11. 11
Á1 - AT Deficiency
Emphysema
• Smoking , genetic , loss trou urine
• Elastase Vs Anti elastase
• Functions of Á1 – AT :
• Blocks enzyme action
• Enzymes are present in WBC ,
endothelium, bronchioles. (Elastase,
Chymotrypsin etc)
12. • Elastase Vs A1- antitrypsin
deficiency
12
Tissue
damage
13. 13
Wilsons disease – Cu toxicity
• What are the functions of ceruloplasmin?
• Kayser Fleischer Ring
• Hepato lenticular degeneration
• Lab finding in Wilsons disease ?
22. MENKE KINKY DISEASE
• Even dietary cu is absorbed but cannot
transported vascular space
• Intracellular ATPase defect vs Wilsons hepatic
ATPase
• Kinky hair
22
23. 23
Anaemia –and plasma protein
• Ferritin : storage form
• Apoferritin: (Tf) without iron
• Transferrin: (Tf) transfer form
• CDT: Detection Of Alchol Abuse
Tf
28. • CONSERVATION OF IRON IN THE BODY:
RBC lysis
Hb Hb-Haptoglobin complex taken up by
liver kupffer’s cells Iron re- utilized
Globin removed
Heme Heme-hemopexin complex taken by
Hepatocytes Iron re-utilized
Porphyrin removed Bilirubin
Iron Iron-Transferrin Storage Iron re- utilized