विवरण
The chronic infection and inflammation of cystic fibrosis (CF) in the lung causes a progressive decline of lung function resulting in symptoms like cough and sputum production. There are intermittent episodes of acute worsening of symptoms, more commonly referred to as pulmonary exacerbations.
Today our guest speaker is going to tell us about the cases related to Pulmonary Exacerbation of Cystic Fibrosis.
सारांश
- The standard method for lung biopsy, transbronchial lung biopsy via bronchoscopy, carries a risk of pneumothorax, which is also present in CT-guided biopsies. Cryobiopsy offers a potential advantage by reducing the likelihood of pneumothorax and providing more reliable tissue samples for diagnosis.
- Cystic fibrosis (CF) is a multi-system disorder caused by pathogenic variants in the CFTR gene, located on chromosome 7. Defects in this gene can lead to issues in protein formation, regulation, processing, or conduction. There are six types of CF categorized based on the specific CFTR mutation and how the CFTR protein functions.
- Acute exacerbations in CF are characterized by changes from the patient's baseline, such as increased sputum production, chest congestion, decreased exercise tolerance, or fever. Early treatment of these exacerbations is crucial for improving patient survival. Causes of exacerbations include viruses, bacteria (e.g., *Staphylococcus aureus*, *Burkholderia cepacia* complex, *Pseudomonas aeruginosa*), and, rarely, non-tuberculous mycobacteria. Allergic bronchopulmonary aspergillosis (ABPA) should be suspected if a patient isn't responding to broad-spectrum antibiotics targeting common CF pathogens.
- Treatment focuses on addressing the defective CF gene through gene replacement or protein repair. Other approaches include antibiotics for infection, steroids for inflammation, and lung transplantation as a last resort when standard therapies fail. Standard treatments include medications, chest physical therapy, pulmonary rehabilitation, and surgery.
- Antibiotic choices depend on the specific organism identified in cultures. *Staphylococcus aureus* infections are treated with cephalosporins, while MRSA requires vancomycin or linezolid. *Pseudomonas aeruginosa* infections are treated with piperacillin-tazobactam, ceftazidime, cefepime, or carbapenems. Ciprofloxacin or levofloxacin may be added for resistant cases. Antibiotic duration is typically 7-10 days, but may extend to 14 days for slow responders.
- After IV antibiotic therapy, maintenance therapy may be needed to prevent recurrence. Options include inhaled tobramycin, ibuprofen, macrolides (azithromycin), or nebulized hypertonic saline. Treatment decisions should be tailored to the individual patient and their specific infection.
- Early diagnosis and prompt treatment of exacerbations are vital for improving outcomes in CF patients. Any change in symptoms from baseline should be considered an exacerbation, and treatment should be initiated promptly. In cases of non-response to standard treatment, surgical options, including referral for lung transplant, should be considered.
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