nemoci-sympt/PLICNI/alfa-1-antitrypsinova-deficience/zhorusujici-rizikove-faktory
Cathepsin G
Chemicals
Cigarotvý kouř a kouření
- Increasing the inflammatory reaction in the airways
- Cigarette smoke directly inactivates alpha-1 antitrypsin
- By oxidizing essential methionine residues to sulfoxide forms
- Decreasing the enzyme activity by a factor of 2000 [2]
- Accelerates the onset of symptomatic disease by approximately 10 years [4]
- Producing an increase in the number of neutrophils (and neutrophil elastase) in the alveolus [4]
- Inactivating the remaining small amounts of antiprotease [4]
Dust
- Environmental dust [1] [2]
- Alpha1-antitrypsin deficiency and short-term massive pollution in New York City Fire Department (FDNY) rescue workers
- Responding to the World Trade Center (WTC) collapse
- First 4 years after the event found
- Significant accelerated declines in spirometry
- Related to the degree of exposure at the disaster site and to the degree of AATD
- Increased respiratory symptoms [4]
Increase of elastase production in the lung
- Increasing lung degradation
- Specifically, cigarette smoking and infection [1]
Elastase
Neutrophil elastase
- Released during normal phagocytosis of organisms or particles in the alveoli
- Neutrophil elastase is considered the protease largely responsible for alveolar destruction
pancreatic elastase
Polymers of “Z” antitrypsin
- Are chemotactic for neutrophils
- May contribute to local inflammation and tissue destruction in the lung [1]
Proteinase 3
Bromelain a jiné proteolytické enzymy
- Cokoliv co zvyšuje enzymtickou proteolytickou aktivitu v krvi, která není v našem těle kompezována / regulována / adekvátně potlačována je riziko
- Takže bych asi z opatrnosti nedporučovala například užívání Wobenzymu i když to není napsané v jeho seznamu kontraindikací
- Jednou jsem narazila na studii, kde ukázali, že po užívání bromelainu králíci nedokázali udržet vztyčené uši - došlo k úbytku kolagenních vláken
Recruitment of neutrophils to the alveoli
Risk factors for emphysema
- Dusty occupational exposure
- Parental history of chronic-obstructive pulmonary disease (COPD)
- Personal history of asthma
- Chronic bronchitis
- Pneumonia
Toxic gain of function
- Liver disease
- Accumulation within the hepatocyte of un-secreted variant AAT protein
- Only genotypes associated with pathologic polymerization of AAT within the endoplasmic reticulum of hepatocytes produce disease [1]
- Most patients are homozygous for the Z allele
- PI*ZZ)
- Liver disease does not occur in null homozygotes
- Severe AAT deficiency but no intra-hepatocytic accumulation [1]
