PFAPA (periodic fevers with aphthous stomatitis, pharyngitis, and adenitis)
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PFAPA Syndrome definition
Basic information about PFAPA
• Steroids given at the beginning of the seizure usually terminate it, but more frequent seizures may occur.
• Removal of tonsils in children can treat the disease.
• Seizures may affect the quality of life of children and their families and may not go to school. No harm to the health of the child in the long term.
In PFAPA syndrome, apart from periodic fever, membranous pharyngitis, aphthous stomatitis, cervical lymphadenopathy are seen. Neutropenia or increased IgD levels are not observed during the attack.
Pay attention to these symptoms!
• In addition to fever, abdominal pain, nausea, vomiting, sweating, tremors, muscle-bone and joint pain,
• Two-sided swelling of the neck glands,
• If there is growth in the liver and spleen,
• If aphthae have formed in the mouth (These aphthous lesions are usually painless and quickly heal.)
• If the fever is in a sudden tendency and the general condition of the fever is recovering rapidly and is completely normal between the attacks; your child may have PFAPA syndrome. Although there is frequent attack in PFAPA syndrome, the development and growth of the child is not affected. In blood tests, there is an increase in the findings suggestive of severe inflammation. However, microbes cannot be detected in throat cultures or other materials.
The most important feature of PFAPA syndrome is; As the age grows, the interval between the attacks is opened and spontaneous recovery is seen within 4-8 years. The diagnosis of the disease is made by the presence of the above findings after the other causes of recurrent fever, especially the Familial Mediterranean Fever, have been eliminated.
Patients diagnosed with PFAPA syndrome are treated with cortisone therapy at the onset of an episode and the disease is rapidly controlled. With this treatment, most of the time the inter-episode is opened.
In cases of pfapa syndrome, which cannot be controlled by cortisone treatment, adenoidecomy and tonsil operations are also recommended. In cases where the fever rhythmically repeats, it is necessary to carry out the necessary research and to think about the recurrent fever syndromes.
High fever of unknown origin in infants and children!
Suddenly start attention to the rise of fever!
The most prominent feature of this syndrome is cyclic fever attacks. In the child, the fever starts suddenly, it is between 38.9-41 degrees, and the fever between 3-6 days usually does not last longer than 7 days and sometimes heals suddenly. The fever cycle ranges from 26 to 30 days, with an average of 28 days, with an average of 11-12 fever episodes per year. In this picture, the child looks healthy and maintains normal growth and development. Aphthous ulcers in the mouth can be seen in children with fever. Although there is no definitive laboratory diagnosis, it is clinically diagnosed and at least three fever attacks must be documented.
Attacks can last for 4-8 years on average
The attacks are not easy to control!
Can be mixed with familial mediterranean fever
How is PFAPA diagnosed?
How is PFAPA treated?
Drugs such as cimetidine and colchicine can prevent seizures from half to one-third of children if used regularly. In many studies, it has been observed that the removal of tonsils permanently treats PFAPA in 80% of patients, but the role and timing of surgery during this treatment is not yet clear. Generally, if the steroid response is seen to be good, tonsillectomy and adenoidectomy can be planned in the following period.
You can find details about "Periodic Fever Syndrome" and before and after photos at >> PFAPA Syndrome - Symptoms, Causes, Diagnosis, Treatment
Source links >> Outcome of Tonsillectomy in Selected Patients With PFAPA Syndrome ... / Periodic fever, aphthous stomatitis, pharyngitis and adenitis
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