While the pathogenesis of PANDAS remains unclear, “it’s suspected of being an autoimmune disorder brought on by strep in a susceptible host,” Dr. Tunkel said.
Explore This IssueMarch 2010
Treatments for immune dysregulation, such as corticosteroids, plasma exchange and intravenous immunoglobulin (IVIG) infusion, have produced some success, “but the literature is full of conflicting data about how such therapies should be used,” Dr. Tunkel said.
Researchers have developed a mouse model that may help clarify the relationship between strep infection and the symptoms associated with PANDAS.
While research has suggested tonsillectomy might help alleviate symptoms of PANDAS, Dr. Tunkel is skeptical. “It’s hard to recommend tonsillectomy as a treatment,” he said.
Two randomized studies, however, have produced evidence that tonsillectomy may alleviate the symptoms of periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis syndrome (PFAPA).
PFAPA causes cyclic high fevers that last less than 10 days, recur more than six times and are accompanied by pharyngitis, adenitis and sometimes aphthous stomatitis. “Families say they can mark on their calendars when the fevers will recur,” Dr. Tunkel said.
First reported in 1987, the cause of the syndrome remains unknown. “Some think it’s an infectious phenomenon; others believe it’s the result of immune dysregulation,” Dr. Tunkel said.
Corticosteroids effectively quell the fevers but result in more frequent episodes of fever, according to Dr. Tunkel. Cimetidine (Tagamet) has been used with varying results.
A 2009 prospective, randomized study of 39 children with PFAPA found that 63 percent of children who received adenotonsillectomy had no further episodes of fever, compared to only 5 percent of the children who did not undergo surgery. Based on such evidence, Dr. Tunkel said tonsillectomy may well be indicated for children with PFAPA, “but probably not for PANDAS, at least not yet.”
Kenny H. Chan, MD, of Children’s Hospital, Denver, talked about chronic sinusitis in children, which differs in its pathogenesis between children and adults. “In children, you see predominately lymphocytes, while eosinophils are the primary immune cells seen in adults with chronic sinusitis,” he said.
The bacterial slime structure of biofilm, which is found in adults and presumed to be present in children, may account for part of the pathogenesis in children.
“How little we know about the treatment of pediatric rhinosinusistis,” Dr. Chan said. “The effectiveness of treatments using antibiotics is controversial in the literature. Biofilm may be influencing the outcome. Adenoidectomy may have a role in eliminating the biofilm reservoir.”
To highlight some of the issues in pediatric otitis media, Joseph Haddad, Jr., MD, of Columbia University, presented a case study involving a child who was treated for otalgia with amoxicillin. A few weeks later the child developed ear pain and went to a different pediatrician, who treated the otalgia with azithromycin for five days.