A Simple Guide To Mucoviscidosis (Cystic Fibrosis), Diagnosis, Treatment And Related Conditions

This book describes Mucoviscidosis (Cystic Fibrosis), Diagnosis and Treatment and Related Diseases

Cystic Fibrosis (Compendium of Medical Poems by Kenneth Kee 2016)

Cystic fibrosis is an inherited recessive genetic disease
Sticky mucus blocked the lungs and airways with ease
It also blocks ducts in the pancreas and digestive tract
As a result the intestines cannot fully absorb proteins and fats

This can result in vitamin deficiency and malnutrition
There will be bulky stools, intestinal gas and constipation
There is also pain or discomfort in the abdomen.
The sticky mucus can cause all forms of digestion problem

Sweating will cause the loss of salt resulting in dehydration
CF also causes infertility in men by blocking the secretions
This disease can make it harder for women to get pregnant.
An early diagnosis and treatment of the illness is very important

Treatment is with inhalers and medicine to open the air tube obstruction
Pancreatic enzymes are given to help the protein and fats digestion
A rare complication is chronic lung obstruction
There may also be poor absorption and nutrition

-An original poem by Kenneth Kee
Cystic Fibrosis is well known throughout the world for its health complications resulting from the production of mucus that is much thicker and more sticky from the exocrine glands in the lungs and digestive system.

This results in blockage of the ducts of the digestive system and airways with abnormal widening (cyst formation) and scarring of the organ tissues (fibrosis) seen especially in the pancreas.

Cystic fibrosis is named due to the cysts and fibrosis in the pancreas.

A better name for this disorder would be mucoviscidosis because of the thick and viscous mucus.

From ancient times, children around the world have been afflicted with mucoviscidosis (cystic fibrosis) that leads to shortened lifespan.

In medieval Europe, these children were thought to be cursed by witches.

Salty skin was a sign of an imminent disease without known cause or cure.

"Woe to the child who tastes salty from a kiss on the brow, for he is cursed and soon will die."

High levels of salt in the sweat of patients with mucoviscidosis indicated an abnormality in electrolyte transport from the sweat gland.

It is suggested that sweat ducts in these patients were not permeable to chloride.

The faulty chloride channel is believed to be situated in the apical membranes of the lung surface or glandular epithelium to explain the respiratory and systemic organ failure linked with mucoviscidosis.

Mucoviscidosis (cystic fibrosis) is an inherited recessive genetic disorder that causes thick, sticky (viscous) mucus to accumulate in the lungs, digestive tract, and other secretary glands such as salivary, sweat, sinuses, liver and sex organs.

Mucoviscidosis is caused by defects in the cystic fibrosis gene, which codes for a protein transmembrane conductance regulator (CFTR) that functions as a chloride channel and is regulated by cyclic adenosine monophosphate (cAMP).

Medicines used to treat patients with mucoviscidosis (cystic fibrosis) may be pancreatic enzyme supplements, multivitamins (fat-soluble vitamins), mucolytics, antibiotics (inhaled, oral, or parenteral), bronchodilators, anti-inflammatory agents, and CFTR potentiators (e.g., ivacaftor) and correctors (e.g., elexacaftor, lumacaftor, tezacaftor).

Cystic fibrosis transmembrane conductance regulator (CFTR) potentiators are the first available treatment that acts on the defective CFTR protein, the underlying cause of cystic fibrosis.

There is no cure for mucoviscidosis

Better medical and surgical treatment methods have improved the mean survival age to 37 in recent years.

TABLE OF CONTENT
Introduction
Chapter 1 Mucoviscidosis (Cystic Fibrosis)

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