|NEWER METHOD TO DIAGNOSE CYSTIC FIBROSIS|
Cystic fibrosis is an autosomal recessive disease that leads to recurrent bouts of sinusitis and bronchitis due to clogging of the thick mucus in the lungs. It also leads to failure to thrive and diarrhea in children.
Children with CF release abnormally high amounts of salt in their sweat. The traditional screen for CF is a sweat test that measures the amount of chloride in sweat. A high amount is suggestive of CF. In a small percentage of those with the disease the sweat test show nothing.
Now, a test called the nasal potential difference study is available in few centers. It measures the flow of chloride ions into and out of cells controlled by the protein whose deficiency causes CF.
It is a painless one-hour test and performed by placing electrodes on the lining inside the patient's nose, where the chloride channel is similar to that in the lungs. A thin straw like tube is used to expose epithelial cells in this lining to chemical solutions to stimulate the chloride channel function. If the chloride channel is turned on by these solutions, as recorded by a small electrical current through the electrodes, the patient doesn't have CF.
In CF, the earlier the diagnosis, the better the chances of early treatment with antibiotics and anti-inflammatory, slowing the progression of lethal lung damage. Unchecked, the damage will nearly always lead to death or the need for a lung transplant.
Last updated on 13-02-2003
Pediatric Oncall Journal
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