Periodic fevers
A fever is a temperature of 100.4º F and higher. When your child has a fever, the body works to control the temperature, but the temperature increases for a number of reasons:
- Chemicals, called cytokines and mediators, are produced in the body in response to an invasion from a microorganism, malignancy, or other intruder.
- The body is making more white blood cells to combat intruders which may be present in the body. These cells can "eat-up" an invading organism.
- The body is trying to produce natural antibodies, which fight infection. These antibodies will recognize the infection next time it tries to invade.
- Many bacteria are enclosed in an overcoat-like membrane. When this membrane is disrupted or broken, the contents that escape can be toxic to the body and stimulate the brain to raise the temperature.
What are periodic fevers?
Most commonly, children who have frequent fevers have frequent viral illnesses. As children grow, their immune systems are “learning” to fight infections. Children become exposed to many viruses from daycare attendance, siblings, and other situations in which they are potentially around others carrying different viruses. Infants and young children average 6-10 upper respiratory infections each year. These situations are not considered true “periodic fevers” by doctors.
Occasionally, children have fevers which recurs frequently, almost as if on schedule. There are several genetic (inherited) diseases and other conditions for which no cause has been identified that sometimes cause periodic or recurrent fevers in children. These include:
- The syndrome of periodic fever, aphthous ulcers, pharyngitis, and adenitis (PFAPA).
- Cyclic neutropenia
- Familial Mediterranean fever
- Hyperimmunoglobulinemia D periodic fever syndrome (HIDS)
- TNF-receptor associated periodic fever syndrome (TRAPS)
What is PFAPA?
The acronym PFAPA refers to the syndrome of periodic fever, aphthous ulcers, pharyngitis, and adenitis. Aphthous ulcers are mouth sores very similar to those many people get when they are sick (often called “cold sores”). Pharyngitis is a medical term for “sore throat,” and “adenitis” refers to swollen lymph nodes.
PFAPA often occurs in young children, who develop fever (usually over 102-103 oF) on a fairly regular schedule every 3 to 6 weeks. The fever lasts 2 to 4 days, usually has no symptoms other than the ones described above, and the child quickly returns to normal. Many parents “sense” when their children are about to develop another fever.
PFAPA is not dangerous, and the condition subsides on its own without treatment. However, some treatment options exist, and consultation with an infectious diseases specialist may be needed to diagnose and appropriately treat children with PFAPA.
What are the genetic causes of periodic fevers?
Cyclic neutropenia, familial Mediterranean fever, Hyperimmunoglobulinemia D periodic fever syndrome, and TNF-receptor associated periodic fever syndrome are all examples of inherited diseases which can lead to periodic fevers. These are rare and are often difficult to diagnose. There are treatment options available for some of these diseases. Consultation with an infectious diseases specialist is often required to diagnose and manage these conditions.