Kazuto Taniguchi has his expertise in pediatrics and molecular biology, and has passion in improving the children’s health and wellbeing. He was raised in Kyusyu, Japan and became a pediatrician. He completed a PhD at Saga University. He worked at Saga University Community Medical Support Institute. He then moved to the University of Exeter Medical School (UK) as a postdoctoral fellow and studied under Professor Noel G Morgan. He serves as a pediatrician and the vice director of the Tara Town Hospital since returning to Japan.
Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is characterized by periodic high fever lasting 3 to 6 days and recurring regularly every 2 or 8 weeks. The fever is also associated with aphthous stomatitis, pharyngitis, and cervical adenitis. The etiology of PFAPA syndrome remains unclear. Although various treatments, such as tonsillectomy and the use of cimetidine, prednisolone, colchicine, thalidomide, anakinra, and non-steroidal anti-inflammatory drug, have been reported, the effectiveness of these treatments is still controversial. We here show 3 thought-provoking cases. A 1-year-old girl with PFAPA syndrome received oral cimetidine treatment and febrile attacks were reduced. No side effect was detected due to oral cimetidine treatment. Tonsillectomy was successfully performed on a 3-year-old girl and a 6-year-old boy with PFAPA syndrome and febrile attacks were reduced. Cimetidine, which is renowned as therapeutic agent of a gastroduodenal ulcer, is a common H2 antagonist that inhibits suppressor CD8+ T-lymphocyte activation and chemotaxis. Tonsillectomy is generally recommended for patients aged 3 years and older for safety reasons (to avoid postoperative complications), whereas oral medications can be used in younger patients. Our experience shows that oral cimetidine treatment and tonsillectomy are effective against PFAPA syndrome. We suggest that tonsillectomy be considered as early as possible in patients with PFAPA syndrome. We also propose the use of cimetidine to reduce the febrile attacks of PFAPA syndrome until the age of 3 years.