(E84.1) Cystic fibrosis with intestinal manifestations

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580 693 in individuals diagnosis cystic fibrosis with intestinal manifestations confirmed

10 360 deaths with diagnosis cystic fibrosis with intestinal manifestations

2% mortality rate associated with the disease cystic fibrosis with intestinal manifestations

Etiology Pathogenesis Clinical Pattern Diagnostics Treatment Prevention

Diagnosis cystic fibrosis with intestinal manifestations is diagnosed Women are 3.58% more likely than Men

279 960

Men receive the diagnosis cystic fibrosis with intestinal manifestations

4 512 (1.6 %)

Died from this diagnosis.

100

300 733

Women receive the diagnosis cystic fibrosis with intestinal manifestations

5 848 (1.9 %)

Died from this diagnosis.

Risk Group for the Disease cystic fibrosis with intestinal manifestations - Men aged 15-19 and Women aged 10-14

In Men diagnosis is most often set at age 0-79

Less common in men the disease occurs at Age 80-95+Less common in women the disease occurs at Age 85-95+

In Women diagnosis is most often set at age 0-84

Disease Features cystic fibrosis with intestinal manifestations

Absence or low individual and public risk

Cystic fibrosis with intestinal manifestations - what does this mean

Cystic fibrosis with intestinal manifestations occurs when a mutation in the cftr gene causes the body to produce abnormal amounts of thick and sticky mucus that builds up in the intestines, blocking the absorption of nutrients and leading to poor growth and development.

What happens during the disease - cystic fibrosis with intestinal manifestations

Cystic fibrosis is an inherited disorder caused by a mutation in the cystic fibrosis transmembrane conductance regulator (cftr) gene. this mutation leads to the production of abnormal cftr proteins, which disrupt the normal function of chloride channels in the epithelial cells of the lungs, pancreas, and intestines. this causes thick mucus to accumulate in the lungs, leading to recurrent pulmonary infections, and in the pancreas, leading to malabsorption and malnutrition. in the intestines, the thick mucus prevents the absorption of vital nutrients, leading to poor growth and diarrhea.

Clinical Pattern

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How does a doctor diagnose

Physical examination
Sweat test
Genetic testing
X-ray imaging
CT scan
MRI scan
Fecal elastase test
Stool culture
Blood tests
Pulmonary function tests

Treatment and Medical Assistance

Main goal of the treatment: To reduce symptoms and improve quality of life.

Regular monitoring of nutritional status
Enzyme replacement therapy
Nutritional supplementation
Pancreatic enzyme replacement therapy
Antibiotic therapy
Airway clearance therapy
Chest physiotherapy
Mucus clearance techniques
Bronchodilators
Corticosteroids
Surgery

21 Days of Hospitalization Required

Average Time for Outpatient Care Not Established

Cystic fibrosis with intestinal manifestations - Prevention

Cystic fibrosis with intestinal manifestations can be prevented by following a healthy lifestyle, including eating a balanced diet, exercising regularly, and avoiding smoking and excessive alcohol consumption. additionally, genetic counseling should be sought for those at risk of developing the condition.

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