Showing posts with label Periodic Fever Syndrome. Show all posts
Showing posts with label Periodic Fever Syndrome. Show all posts

Thursday, May 30, 2019

Attention to Periodic Fever Syndrome in Children!

PFAPA (periodic fevers with aphthous stomatitis, pharyngitis, and adenitis)

Image source: Everyday Health
Periodic fever syndrome, which usually gives symptoms with a fever of 41 degrees and worries the families very frequently, is common in children aged 2-5 years. Periodic high fever syndrome is the most common symptom with aphthae in the mouth, neck swelling and pharyngitis.

PFAPA Syndrome definition

PFAPA syndrome is an autoinflammatory disease that occurs in childhood with recurrent fever, aphthous stomatitis, pharyngitis and cervical adenitis. The disease occurs mostly under five years of age. The fever episodes of the disease recur at irregular intervals and lasts for 3-4 days on average. Fever is usually above 39 ° C in children and is unresponsive to all antibiotic and antipyretic treatments. All clinical symptoms disappear immediately with single dose of corticosteroids. Permanent treatment option is tonsillo-adenoidectomy in children.

Basic information about PFAPA

• It usually disappears spontaneously in the second decade of life. However, they can continue to adulthood and the attacks become less frequent in this period.
• 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!

• If your child's fever lasts for 3-7 days (average 5 days) and suddenly falls,

• 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!

High fever with unknown etiology is common in infants and children. However, recurrent fever is also frequently seen. The clinical entity characterized by aphthous stomatitis, pharyngitis, cervical adenitis and periodic fever, which were abbreviated as PFAPA syndrome, were first described in 1985 by Marshall et al. The etiology and treatment of PFAPA syndrome is still unknown. However, glucocorticoids are highly effective in controlling symptoms. Cimetidine and steroids are known to reduce disease duration. There are many reports that the symptoms disappeared completely after tonsillectomy. It was observed that the syndrome complex lasted for 4-6 days, and the symptoms completely resolved with corticosteroid use.

Suddenly start attention to the rise of fever!

Many diseases can be seen in children, especially until the age of primary school. Children can have an upper respiratory tract infection on average 9 times a year, and the majority of them are viral. In addition, periodic fever syndromes should be considered in children with frequent febrile diseases, especially if the fever is cyclic. PFAPA syndrome, which is one of the most common syndromes in children with real fever, is; periodic fever, aphthous stomatitis, pharyngitis and adenitis.

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 response of the fever to steroid treatment is considered as a diagnostic criterion. These patients do not usually have beta-hemolytic streptococcal germ production in throat cultures, but reproduction should not mean that this syndrome is not present. At least 3 fever attacks (shorter than 7 days, regular and periodic), pharyngitis and painful throat swelling or aphthous ulcer in the mouth, normal growth and development, presence of healthy looking children between fever episodes, PFAPA should be considered in the presence of. While the fever episodes last for 4-8 years, their frequency decreases as the child gets older and then heals spontaneously.

The attacks are not easy to control!

There is no definitive treatment for the disease. Steroids are used in the treatment of attacks. Although single dose steroid administration improves fever and pharyngitis findings, it does not prevent the next fever. Other drugs used in the treatment are colchicine and cimetidine. In some cases, removal of tonsils can prevent attacks.

Can be mixed with familial mediterranean fever

This disorder can be confused with Familial Mediterranean Fever (FMF), which is considered to be a periodic fever syndrome in children. FMF usually occurs at older ages and attacks occur at irregular intervals. Although the fever varies between 38-40 degrees, it continues for 12-36 hours and falls spontaneously. Chest pain and joint pain can also be seen, especially with abdominal pain. The child seems healthy among the attacks. Because there is a genetic transition, there may be people with similar complaints in the family. The familial Mediterranean fever should be treated as distinct from this disorder. Otherwise, depending on the accumulation of amyloid in the body, especially in the kidney, other internal organs may be damaged.

How is PFAPA diagnosed?

Laboratory tests for PFAPA diagnosis are not available. The disease is diagnosed by symptoms and physical examination. During seizures, things such as white blood cell counts, signs of inflammation, which can be measured by blood tests, increase. Before approving the diagnosis, it is necessary to eliminate other diseases that may have similar symptoms. The response to treatment with steroids may also provide a diagnosis of PFAPA. In cases other than a classical appearance, it is necessary to exclude other causes of recurrent fever, especially if the symptoms started during the first year of life and signs of inflammation increased during seizures.

How is PFAPA treated?

The goal of treatment is to control symptoms during fever bouts, to shorten the duration of seizures and to prevent them from repeating. Fever does not respond very well to acetaminophen and nonsteroidal anti-inflammatory drugs. If a single dose of steroid is given when the symptoms begin, it may significantly shorten and even terminate the duration of the seizure. However, the time between seizures and this treatment may be shortened and the next seizure may come early than expected.

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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