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Mysterious fevers of unknown origin: Could surgery be a cure?
Date:3/28/2008

introduction to PFAFA was a personal one: His first patient, described as a case report in the paper, was his own daughter, who at 19 months was getting fevers every three weeks. There was nothing to break the fevers, he recalls. She was seen by several experts at Childrens. The workup was always negative.

Searching the medical literature for answers, Licameli found two small case studies in Europe, where doctors diagnosed PFAPA, removed the tonsils and the children got better, and decided to try this approach. It worked, with an immediate cessation of his daughters fever cycles (shes now almost 6), and other doctors began referring patients to him.

The hallmark of PFAFA is high fevers of a cyclical nature parents can often look at their calendars and predict what day the fevers will arrive. There are typically no other symptoms. Some children have sore throat, swollen glands and small mouth ulcers, but these symptoms arent very specific and can be very subtle.

On the face of it, it doesnt seem like a surgical problem, Licameli says. The tonsils and adenoids appear normal, even when you study them pathologically, yet surgery is immediately curative.

Before undergoing surgery, patients are evaluated for any rheumatologic or infectious etiologies that can also cause cyclical fever.

So what causes this condition, and why does surgery work? Licameli suspects the tonsils may harbor a chronic indolent infection to which the immune system is hypersensitive. He and his colleagues at Childrens including specialists in infectious disease, rheumatology and immunology -- plan to pursue these questions further.

In the meantime, they hope that increased physician awareness will allow children struggling with this condition to be offered surgery sooner.


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Contact: Keri Stedman
keri.stedman@childrens.harvard.edu
617-919-3110
Children's Hospital Boston
Source:Eurekalert

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