Surgery cures mystery fevers

Greg Licameli, MD, with his daughter Claire

Wracked by seizures and a 105-degree fever, 19-month-old Claire is rushed to the emergency room. The hospital runs test after test and specialists are brought in, but no explanation is found. This isn't the first time: The fevers have struck like clockwork every three weeks for the past nine months. Antibiotics and fever reducers have offered no relief.

Claire's father, Greg Licameli, MD,
of Children's Hospital Boston's Otolaryn-gology Department, decided to search the medical literature for answers. He found two reports of European children with unexplained cyclical fevers, some of whom got better after tonsillectomy. Seeing nothing to lose, he decided to try it. Colleague Dwight Jones, MD, removed Claire's tonsils and adenoids and the fevers immediately stopped.

Other doctors began referring patients to Licameli, who's now operated on 60 patients with this mysterious condition, first reported in 1987 and known as PFAPA (periodic fever, aphthous ulcers, pharyngitis and adenitis). In the March Archives of Otolaryngology and Head and Neck Surgery, he describes 27 of these children, the largest published experience to date; 26 had complete fever resolution.

The reason remains a mystery, since the removed tonsils and adenoids show no evidence of infection or any other abnormality. Lica meli suspects the tonsils harbor a chronic, low-level infection to which the immune system overreacts. He and colleagues in Immunology plan to investigate further.
Meanwhile, the referrals keep coming, and desperate families are finding surgery to be life-changing. "I tell parents, 'I don't know why this works, but it has a good chance of ridding your child of fevers,'" Licameli says. "It works in almost every single kid."

iPods OK with pacemakers

Last May, a widely reported study concluded that errant electronic noise from iPods can make cardiac pacemakers malfunction, but this just didn't sound right to cardiac electrophysiologists at Children's. "Many of our pacemaker patients have iPods and other digital music players, and we've never seen any problem," says Charles Berul, MD, director of the Pacemaker Service. "But kids and parents bring up this concern all the time."

So late last year, cardiac fellow Gregory Webster, MD, and the electrophysiology nurses and physicians studied 51 of their own patients, placing each of four digital music players directly over each patient's pacemaker or implantable cardioverter-defibrillator. As reported in the April issue of Heart Rhythm, none of the players interfered with device functioning. Patients' EKGs showed no change in any of 255 separate tests, and no one reported symptoms.

Players did sometimes interfere with communication between the cardiac device and the programmer used to check and recalibrate it, but this interference didn't compromise device function and stopped when the player was moved away.

Berul and Webster feel reassured, but acknowledge that their testing was short-term. As a precaution, they suggest patients keep iPods at least six inches away from their cardiac device.

Your baby's brain on drugs, alcohol and tobacco

The cortical gray matter indicated by arrows appears thinner in the exposed brain than in the control.

More than one million babies born annually in the United States are exposed to cocaine, alcohol or tobacco before birth. Now, an NIH-funded study led by Michael Rivkin, MD, of Neurology, suggests that such exposures may have effects on brain structure that persist into adolescence.

Rivkin and colleagues at Boston Medical Center used volumetric MRI imaging to study the brain structures of 21 young adolescents with prenatal substance exposures and 14 with no exposures. Adolescents exposed prenatally to cocaine, alcohol or cigarettes showed reductions in total brain volume and in gray matter in the brain's cerebral cortex, important in many cognitive functions. There were too few children to find statistically significant effects of any single substance after accounting for other exposures, but the more substances a child was exposed to, the greater the reduction in brain volume.

Especially striking was the finding that prenatal tobacco exposure alone had an effect on brain volume that fell just short of statistical significance. "About 20 percent of women who smoke continue to do so during pregnancy," Rivkin notes.

The study was published in the April Pediatrics. Rivkin and colleagues now hope to get funding to confirm their findings in a larger group of children.