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Cystic Fibrosis Symptoms and Complications

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Cystic Fibrosis Symptoms and Complications

Cystic Fibrosis Symptoms and Complications

Sisters with Fibrous-sacks and Symptoms with Complications
Picmonic
Cystic fibrosis is a hereditary disease leading to problems with Cl<sup>-</sup> channels in the body. It is the most common lethal genetic disease in the Caucasion population. Patients develop recurrent pulmonary infections, bronchitis, infertility, pancreatic insufficiency, steatorrhea and malabsorption.
9 KEY FACTS
Recurrent Pulmonary Infections
Recurrent-clock at Lungs with Bacteria

A very serious complication of CF is pulmonary infection. Viscous mucus secretions lead to obstruction and infection, and sometimes abscesses. Staph aureus, H. influenza and Pseudomonas are the most common orgnisms causing disease in CF patients.

Chronic Bronchitis
Crone Broccoli-on-fire

Bronchioles often become distended with thick mucus, with hyperplasia and hypertrophy of the mucus secreting cells. Along with superimposed infections, patients often develop chronic bouts of bronchitis.

Nasal Polyps
Nose Polyps

The pathogenesis of nasal polyps is mostly unknown, but it is understood that in the nasal mucosa oedemetous fluid collects, causing polypoidal change, sometimes leading to metaplastic changes in the epithelium. This is highly associated with CF, along with other pathologies.

Pancreatic Insufficiency
Damaged Pancreas

Due to accumulation and plugging of mucus in the small ducts of the pancreatic exocrine glands, patients may experience pancreatic atrophy and fibrosis. This leads to pancreatic insufficiency and digestive problems.

Intestinal Obstruction
Intestines Obstructed

A common finding in newborns with CF is intestinal obstruction. Meconium ileus is the stasis of newborn's meconium, which is not passed in the first 24-hours of life.

Malabsorption and Diarrhea
Intestinal-mallet and Toilet

Due to pancreatic insufficiency, many patients experience malabsorption and diarrhea, and often steatorrhea.

Vitamin Deficiencies
Vikings Deficient

With impaired pancreatic exocrine function, patients have impaired fat absorption, which leads to avitaminosis and possible deficiencies in fat-soluble vitamins (A, D, E, and K).

Chronic Hepatic Disease
Crone Liver

As bile canaliculi are plugged with mucinous material, portal inflammation, and later, hepatic steatosis may occur. Many patients can develop biliary cirrhosis and diffuse hepatic nodularity.

Infertility in Males
Limp Male-sign

95% of males who survive to adulthood with CF are infertile. This is often because of a congenital bilateral absence of the vas deferens.

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