WOW..Why Is Sweat Chloride High In Cystic Fibrosis

Cystic fibrosis the most common lethal genetic disease in Caucasians is characterized by a decreased permeability in sweat gland duct and airway epithelia. The high chloride concentration in the sweat can be used to diagnose people with CF.

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Chloride impermeability in cystic fibrosis.

Why is sweat chloride high in cystic fibrosis. Measurement of the chloride in sweat has been the standard method for diagnosing cystic fibrosis for over 40 years. In those with CF the mutation in the CFTR gene results in a defective function of the CFTR protein in human sweat ducts and results in extremely high sweat sodium and chloride losses typically 3-5 times. Collaco and colleagues demonstrated that the primary source of variation in sweat chloride can be traced to CFTR mutations 3.

It damages the lungs and makes it hard to breathe. CF is a disease that causes mucus build-up in the lungs and other organs. People with cystic fibrosis CF have an elevated level of chloride in their sweat.

The concentration of chloride in sweat is therefore elevated in individuals with cystic fibrosis. 5 This makes for very salty sweat though it. Normally chloride travels in and out of the bodys cells helping.

In cystic fibrosis the CFTR chloride channel is defective and does not allow chloride to be reabsorbed into sweat duct cells. In addition to its proximal relationship to sweat gland dysfunction sweat chloride is not prone to extrinsic influences further strengthening its role as a key biomarker 8. The sweat test detects a higher amount of chloridea component of salt that is made of sodium and chloridein the sweat of people who have cystic fibrosis.

A mutation in the cystic fibrosis transmembrane conductance regulator gene associated with elevated sweat chloride concentrations in the absence of cystic fibrosis. The lack of CFTR function leads to excess chloride in the sweat of people with CF. If your child received a positive NBS and has sweat test results that fall into the intermediate range and one or no CF-causing mutations he or she will be classified as having CF-related metabolic syndrome CRMS also known as CF Screen Positive Inconclusive Diagnosis CFSPID.

The genetic defect in cystic fibrosis affects the way chloride moves in and out of cells and sweat contains chloride in the form of sodium chloride salt. Chloride transport is virtually eliminated because CFTR located on the surface of the cells in the sweat duct is defective. First described in 1959 the sweat test remains the gold standard for the diagnosis of cystic fibrosis7 A positive sweat test is diagnostic in the absence of known entities associated with a false positive test generally rare and clinically distinct conditions8 as long as it is performed by staff experienced in the technique and on an adequate weight of sweat 100 mg.

People with cystic fibrosis CF have an elevated level of chloride in their sweat. A chloride channel helps maintain the proper balance of salt and water within a cell. Chloride is part of the bodys electrolyte balance and combines with sodium to form the salt found in sweat.

It can also lead to frequent infections and malnutrition. People with CF have a high level of chloride in their sweat. Mice do not use sweat glands for thermoregulation precluding a comparison of sweat in normal and CF mice.

San Francisco Press 1982 p. Normally chloride travels in and out of the bodys cells helping to maintain electrical neutrality and water balance. Abnormalities in electrolyte secretion in cystic fibrosis sweat glands due to decreased anion permeability.

Because cystic fibrosis is so common many states include testing for the cystic fibrosis gene as part of the Newborn Screen. Measuring the amount of chloride in sweat is often used during the process of diagnosing CF through a procedure called the sweat test. Since sodium and chloride travel together as one molecule the sodiumsalt ends up excreted in the sweat.

Fluid and Electrolyte Abnormalities in Exocrine Glands in Cystic Fibrosis edited by Quinton PM Martinez RM and Hopfer U. As CF is caused by a faulty gene that controls the movement of chloride and water into and out of cells people with CF often sweat more than people without the condition and this sweat contains high levels of chloride which can crystallise into salt visibly on the skin. Chloride is part of the bodys electrolyte balance and combines with sodium to form the salt found in sweat.

In order to make sweat for this test a colorless odorless chemical and a little electrical stimulation are applied to a small area of an arm or leg. Typical values of 20 or 30 mM in healthy individuals that the sweat chloride concentration is the most reliable single physiological marker of CF. In cystic fibrosis the sweat sodium or chloride.

Consequently more sodium stays in the duct and more chloride remains in the sweat. Sweat sodium concentration is largely genetically determined. With cystic fibrosis salt cannot move as it normally does through the cells that line the sweat duct.

In sweat duct epithelium a decreased Cl- permeability accounts for the abnormally increased salt content of sweat. The salt is so high 100 mM vs. Chloride and potassium channels in cystic fibrosis airway epithelia.

When sweat chloride test results fall between the range of 30-59 mmolL the sweat test is usually repeated. In people with CF there is a problem in moving chloride across cell membranes which means higher concentrations of chloride as salt in sweat compared with those who do not have cystic fibrosis. A mutation in CFTR causes a dysfunction of the salt and water balance.

The CFTR gene is a protein that functions as a chloride channel. This causes dehydration of the secretions thick mucous and excessive loss of salt in sweat.

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