Cystic Fibrosis :
Cystic fibrosis is an autosomal recessive genetic disorder which is caused due to mutation of CFTR gene. Most commonly seen in north european descent compared to other race. It is most fatal condition, detected in neonates at birth. It affects lungs , digestive system, pancreas, liver, sweat glands, reproductive system , mainly the cells that produce mucus and sweat. Pancreas is the most common organ affected whereas pulmonary disease is most common cause of mortality. Most common symptoms we see are lung / respiratory infections, shortness of breath, fatigue, fever , oily stool, dehydration, malnutrition, in neonates we seen meconium ileus. In cystic fibrosis the mucus produced is thick and sticky which blocks the affected organ pathway and cause infection, inflammation, fibrosis and cyst. High salt levels are seen in these patient which indicates electrolyte transport abnormality in sweat gland. For diagnostic test blood test, sweat test, genetic testing, radiology imaging, pulmonary function test, sputum culture is advised . Medical management focus mainly on underlying condition of the affected organ, antibiotics coverage against the pathogen detected, maintaining lung function, improve oxygenation, glucocorticoids, bronchodilators,pancreatic enzymes, nutritional measures, hypertonic fluid. New class of treatment called CFTR modulators are been used which help in improving function of CFTR protein is is affected by mutation, lung transplant is also an option which increase life expectancy. High fat diet with supplemental fat soluble vitamins are recommended.