Periodic fevers with aphthous stomatitis,pharyngitis, and adenitis (PFAPA) |
| |
| Affiliation: | 1. Vascular Medicine Department, Grenoble University Hospital, F-38000 Grenoble, France;2. Inserm ERI17, Grenoble Medical School, F-38000 Grenoble, France;3. Inserm CIC3, Grenoble Clinical Research Center, Grenoble University Hospital, F-38000 Grenoble, France;1. Department of Pediatrics, Nemours/A.I. duPont Hospital for Children, Wilmington, DE;2. Division of Rheumatology, Nemours Center for Cancer and Blood Disorders, Wilmington, DE;3. Nemours Center for Cancer and Blood Disorders, Wilmington, DE;2. CardioVascular Center, Cardiac Surgery, Tufts Medical Center, Boston, MA;3. Department of Anesthesiology, VA Boston Healthcare System, West Roxbury, MA;4. Department of Anesthesiology and Perioperative Medicine, Tufts Medical Center, Boston, MA;5. Department of Anesthesiology, University of Illinois and Jesse Brown VA Medical Center, Chicago, IL |
| |
| Abstract: | ![]()
PFAPA syndrome (acronym of periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis) is the most common cause of periodic fever in childhood. Nowadays, it is considered part of the wide family of the autoinflammatory diseases, but a genetic or molecular marker hasn't been identified yet, therefore, its etiology is still unknown. Diagnosis is essentially based on clinical criteria but, especially in younger children, it is sometimes difficult to differentiate it from other hereditary periodic fever syndromes. Fever attacks in PFAPA have a spontaneous resolution and in a high rate of patients the syndrome ends spontaneously over time. Treatment is still a matter of debate. Usually a single administration of oral corticosteroids aborts attacks. Tonsillectomy may be an alternative option but its role remains to be clarified. |
| |
| Keywords: | |
| 本文献已被 ScienceDirect 等数据库收录！ |
|
