1.	Antitrypsin deffiency

A1AD is due to a mutation in the SERPINA1 gene that results in not enough alpha-1 antitrypsin (A1AT). Risk factors for lung disease include cigarette smoking and environmental dust. The underlying mechanism involves unblocked neutrophil elastase and build up of abnormal A1AT in the liver. Alpha-1 antitrypsin deficiency (A1AD) leads to uninhibited destruction of elastic fibre by elastase; the main result is emphysema. It is autosomal co-dominant, meaning that one defective allele tends to result in milder disease than two defective alleles. The diagnosis is suspected based on symptoms and confirmed by blood tests or genetic tests.

Individuals with A1AD may develop chronic obstructive pulmonary disease (emphysema) during their thirties or forties even without a history of smoking, though smoking greatly increases the risk. Symptoms may include shortness of breath (on exertion and later at rest), wheezing, and sputum production. Symptoms may resemble recurrent respiratory infections or asthma.

More severe forms of A1AD can impaired liver function, leading to cirrhosis and liver failure (15%). In newborns, alpha-1 antitrypsin deficiency can result in early onset jaundice followed by prolonged jaundice. It is a leading reason for liver transplantation in newborns.

Apart from COPD and chronic liver disease, α1-antitrypsin deficiency has been associated with necrotizing panniculitis (a skin condition) and with granulomatosis with polyangiitis in which inflammation of the blood vessels may affect a number of organs but predominantly the lungs and the kidneys.

Treatment of lung disease may include bronchodilators, inhaled steroids, and when infections occur antibiotics. Intravenous infusions of the A1AT protein or in severe disease lung transplantation may also be recommended. In those with severe liver disease liver transplantation may be an option. Avoiding smoking is recommended. Vaccination for influenza, pneumococcus, and hepatitis is also recommended. Life expectancy among those who smoke is 50 years old while among those who do not smoke it is almost normal.



2.	Spirometry and interpretation
3.	Excess Ion gap – Ion Gap and how to calculate it

Sodium - Chloride+Bicarbonate, if potassium: sodium+potassium - chloride+bicarbonate. Because potassium concentrations are low it is done without it. 3-11 normal, >11 bad

The anion gap is affected by changes in unmeasured ions. In uncontrolled diabetes, there is an increase in ketoacids due to metabolism of ketones. Raised levels of acid bind to bicarbonate to form carbon dioxide through the Henderson-Hasselbalch equation resulting in metabolic acidosis. In these conditions, bicarbonate concentrations decrease by acting as a buffer against the increased presence of acids (as a result of the underlying condition). The bicarbonate is consumed by the unmeasured cation(H+) (via its action as a buffer) resulting in a high anion gap.

In general, the HCO3 concentration will fall by an amount equal to the increase in anion gap. When the anion gap isgreater than the fall in plasma HCO3  concentration, the presence of a metabolic alkalosis is suggested.

4.	Base Excess and calculation

Base excess is defined as the amount of strong acid that must be added to each liter of fully oxygenated blood to return the pH to 7.40 at a temperature of 37°C and a pCO2 of 40 mmHg (5.3 kPa).


5.	ABG interpretation
6.	How to assess general x ray (Cardiac and Pulmonary)
7.	COPD(Etiology-Pathophysiology-Diagnostics-Treatment-Complications)


8.	How to differentiate between copd asthma and emphysema
9.	Ecg and Xray
10.	Cor pulomnale and P HTN and associated conditions
11.	NT-Pro-BNB – BNB and normal levels