Tuberculosis

Pulmonary Infectious Diseases

Pulmonary Infections

Endo-, Myo-, Pericarditis

Coagulation Cascade

For a list of drugs, check out my old post: anticoagulants, fibrinolytics, antiplatelets

Ciliary Dyskinesia

Kartagener Syndrome is an autosomal recessive defect in ciliary genes leading to impaired mucociliary function. This mutation usually affects the dynein arm. As a result, patients with this syndrome present with bronchiectasis and sinusitis because of secretion retention and recurrent infections. This syndrome is also associated with situs inversus, hearing impairment, and male infertility.

Alpha 1 Antitrypsin Deficiency

Alpha 1 Antitrypsin Deficiency can lead to panacinar emphysema. This condition is autosomal recessive (codominant) and caused by SERPINA1 mutations. Normally, alpha-1 antitrypsin is synthesized in the liver and acts in the lungs. It inhibits enzymes like elastase. However when there is an imbalance in antiprotease and protease levels, the elastase in macrophages can destroy basement membranes leading to emphysema. When alpha-1 antitrypsin is not released from the liver, it can cause cirrhosis. The phenotype ZZ is the most severe form of ATT deficiency while the null does not cause hepatitis.

Cystic Fibrosis

One cause of bronchiectasis is cystic fibrosis, an autosomal recessive disease affecting the CFTR gene on chromosome 7. CFTR codes for a Cl- transporter, which leads to lack of Cl- secretion in epithelial cells. As a result, secretions are dry, thick, and difficult to clear. This leads to recurrent sinopulmonary infections and nasal polyps. The most common mutation is delta F508 resulting in a misfolded CFTR protein that is unable to leave the endoplasmic reticulum. This mutation is a missing phenylalanine.

Cystic fibrosis also affects the sweat glands in which the sweat glands are unable to reabsorb the Cl- causing hypertonic sweat. In fact, a pilocarpine test is used to induce sweating in infants if cystic fibrosis is suspected.

Patients with cystic fibrosis also present with pancreatitis, leading to malabsorption of fat soluble vitamins and greasy stools. Patients have meconium ileus (bowel obstruction) because of the thicker mucus secretions in their bowels. This will be seen on radiographs as air fluid levels. In addition, the vas deferens may not be formed. This causes infertility in male patients.

Treatment for patients with cystic fibrosis include Ivacaftor (G551D mutation), antibiotics, pancreatic enzymes, vitamins, and lung transplant.

Asthma Pharmacology

ASTHMA TREATMENT
Step 1: SABA
Step 2: low-dose ICS
Step 3: low-dose ICS + LABA
Step 4: medium-dose ICS + LABA
Step 5: high-dose ICS + LABA
Step 6: high-dose ICS + LABA + oral steroid

consider adding theophylline as alternative for LABA
consider using cromolyn, LTRA, or nedocromil as alternatives for ICS
consider omalizumab for patients with allergies in steps 5 and 6

FOR EMERGENCIES
O2
high-dose SABA
Ipratropium
Oral CST
IV CST
Mg2+

Restrictive Lung Diseases

Restrictive lung diseases come about from poor breathing mechanics or interstitial lung diseases. Poor breathing mechanics result in under-ventilation of lungs. Thus the A-a gradient is normal. Conditions such as ALS, polio, myasthenia gravis, and scoliosis limit the body's ability to take in air. There is no problem with the alveoli. Interstitial lung diseases cause the lungs to become fibrotic and it is the fibrosis and destruction of lung tissue hat leads to less oxygen into the lungs.