August 13, 1999

Student tracks mystery of childhood fever syndrome

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Medical student Kenneth Tyson Thomas is the lead author of a paper describing periodic fever, a chronic condition usually seen in children under age five. (photo by Anne Rayner)

Student tracks mystery of childhood fever syndrome

Fevers. They're as common in young children as scraped knees and runny noses.

But some children have fevers that occur routinely each month like a woman's monthly period. A fourth-year Vanderbilt medical student has been studying the syndrome along with two Vanderbilt University faculty members. Their findings have been published in the July issue of Journal of Pediatrics.

Kenneth Tyson Thomas is the lead author of the article that describes the syndrome, known as Periodic Fever, Aphthous stomatitis, Pharyngitis, and cervical Adenitis (PFAPA). It is a chronic condition usually seen in children under five, characterized by periodic episodes of high fever occurring roughly every four weeks. The fever briskly rises to 103-105 degrees Fahrenheit and lasts three to six days. The child is healthy otherwise – no runny nose or respiratory symptoms. The syndrome is often accompanied by mouth sores, a bright red and inflamed throat, and swollen glands. The fever spontaneously resolves. The study looks at 94 children identified with PFAPA and their long-term follow-up.

Tyson began studying the syndrome during the summer after his first year of medical school. He was encouraged by Dr. Kathryn M. Edwards, professor of Pediatrics who had first described PFAPA with Dr. Alexander R. Lawton, professor of Pediatrics and Dr. Gary Marshall Jr. from the University of Louisville. Dr. Marshall was also a Vanderbilt medical student at the time of the initial description.

To determine whether the patients who were seen over the past 13 years were still experiencing the episodes of fever, follow-up phone calls were made to most of the families during the summer and fall of 1997.

About one third of the parents contacted said the fevers had stopped although the remainder of children were still experiencing the periodic bouts. None of the group had developed any serious diseases. The study determined that the syndrome might persist for several years but seems to have no detrimental long-term health consequences.

"We're hoping this article may help physicians properly diagnose PFAPA. It's more common than people think," Tyson said. "It may take several years to diagnose if physicians are unaware of the syndrome. Frequently many tests are done on the children because their doctors don't want to miss anything. Lots of children in day care have frequent fever and respiratory infections, but this is not the same. It's a monthly bout of fever that parents and children come to expect."

Nothing can cure PFAPA but a small dose of prednisone has shown promise in alleviating the symptoms in some children. However, in some children it has been shown to increase the frequency of the episodes – from 28 days to 14-21 days.

"For some patients it becomes a trade off – shorter duration but a shorter time between episodes," Tyson said.

Tyson said he is hoping the "observational" paper leads to more research on the causes of the syndrome.

"It's very difficult to have a child who is sick every month like these children are. When it hits they are run down, lethargic and fatigued. Some parents told me that they actually plan vacations around the syndrome, knowing about what day it will occur."

An editorial written by a Philadelphia pediatrician accompanied the article in Journal of Pediatrics.

"At last (the researchers) have led the way with good experiential information," said Dr. Sarah S. Long, chief of Infectious Diseases at St. Christopher's Hospital for Children in Philadelphia. "Now, we need good experimental information. Meanwhile upbeat parents and patients with PFAPA syndrome leave our offices with their disability, relieved we recognize the constellation and predict ultimate good health, satisfied with the notion of an upregulated immune system and armed with a prescription of prednisone should an episode land on a birthday party or trip to Disney World."