1/10. Periodic fever and pharyngitis in young children: a new disease for the otolaryngologist?OBJECTIVE: A clinical entity consisting of periodic fever associated with aphthous stomatitis, pharyngitis and cervical adenitis termed "PFAPA syndrome" in young children (<5 years old) may be unfamiliar to otolaryngologists. We present our 5-year experience of PFAPA syndrome. DESIGN: Case series. SETTING: Tertiary academic. patients: A 5-year retrospective chart review for children (<5 years old) who have undergone tonsillectomies with and without adenoidectomies was conducted. medical records from subjects who underwent the procedures for recurrent pharyngitis were reviewed with reference to a history of periodic fever and stomatitis associated with pharyngitis. INTERVENTIONS: tonsillectomy with and without adenoidectomy. MAIN OUTCOME MEASURE: The objective measure was a comparison of the number of visits to the primary care physician for pharyngitis associated with fever in a 3-month period before and after the surgical intervention. The subjective measure was a telephone interview evaluating preoperative and postoperative symptoms. RESULTS: Of the 117 patients identified, 22 (19%) underwent surgery for recurrent pharyngitis. Five subjects (average age, 2.5 years) were identified as having PFAPA syndrome. The average number of preoperative PFAPA-related complaints was 11.6 compared with 0.2 for the number of postoperative PFAPA-related complaints (P=.03). CONCLUSIONS: Our experience suggests that PFAPA syndrome is an uncommon disease. Most of these children have undergone workup(s) for sepsis performed by their pediatricians because of the associated high fever. The clinical history of this cohort was quite distinctive. This small sample suggests a significant decrease if not cessation of pharyngitis following surgical intervention.- - - - - - - - - - ranking = 1keywords = pharyngitis (Clic here for more details about this article) |
2/10. PFAPA syndrome (Periodic Fever, Aphthous stomatitis, pharyngitis, Adenitis).This paper aims to remind paediatric clinicians to suspect and confirm 'PFAPA' syndrome (Periodic Fever, Aphthous stomatitis, pharyngitis and cervical Adenitis syndrome). We report two cases of PFAPA syndrome: a 3-year-old healthy boy with atopic rhinitis and a boy aged 8 years 5 months who simultaneously had lymphocytic vasculitis syndrome treated with immunosuppressive drugs. Both met Marshall's criteria. The literature regarding PFAPA syndrome was complied using a medline search for articles published between 1963 and 1998 and we then reviewed the reference lists of the articles. The medline search revealed 28 cases with available clinical manifestations, management and prognosis. Our study describes two additional cases. We divided the cases into typical (28 cases) and atypical (two cases) PFAPA syndrome. In typical PFAPA, the age of onset was less than 5 years in most cases and the patients presented 4.9 /- 1.4 days of fever (100%), pharyngitis (89.3%), cervical adenitis (72.1%), stomatitis (71.4%), malaise (64.3%), headache (60.7%), abdominal pain (53.6%) and nausea/vomiting (17.9%). Afebrile intervals were 3.2 /- 2.4 months and increased with age. The time from initial onset to final episode was 3 years 7 months /- 3 years 6 months. The total number of episodes was 8.3 /- 2.5 (range 6-14). Effective treatment included steroids, tonsillectomy/adenoidectomy and cimetidine. The general outcome was good. In atypical PFAPF, the clinical manifestations were similar to those of typical PFAPA except that the age of onset was more than 5 years, and life-threatening intestinal perforation happened once in a patient with underlying Fanconi's anaemia. It was concluded that typical PFAPA syndrome is benign and can be diagnosed by detailed history-taking and from physical findings during repeated febrile episodes with tests to rule out other periodic fever syndromes. A review of the literatures since the first report in 1987 has shown that typical PFAPA syndrome is not associated with significant long-term sequelae and has a good response to steroids. One patient with atypical PFAPA, who received low-dose steroids for over 1 year, developed intestinal perforation after an increment of the 7-day steroid dose. If an underlying problem requires long-term immunosuppressive medication, it is wiser to choose cimetidine rather than increasing the steroid dosage to resolve atypical PFAPA.- - - - - - - - - - ranking = 0.1keywords = pharyngitis (Clic here for more details about this article) |
3/10. Update on treatment of Marshall's syndrome (PFAPA syndrome): report of five cases with review of the literature.Marshall's syndrome or PFAPA (periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis) syndrome is a recently described pediatric periodic disease characterized by recurrent febrile episodes associated with head and neck symptoms. The origin of this syndrome is unknown, and it can last for several years. During healthy periods, patients grow normally. The differential diagnosis includes other diseases characterized by periodic fevers, such as familial mediterranean fever, familial Hibernian fever, hyperglobulinemia D syndrome, Behcet's disease, cyclic neutropenia, juvenile rheumatoid arthritis, and several infectious diseases. Many treatments have been used, with various results, including antibiotics, nonsteroidal anti-inflammatory drugs, acetylsalicylic acid, colchicine, antiviral medicines, steroids, cimetidine, and tonsillectomy. We describe 5 new patients affected by PFAPA syndrome who were observed at the Department of Pediatric Otorhinolaryngology, Spedali Civili, Brescia, italy, from November 2000 to August 2001. All children underwent physical examination, bacterial, fungal, and viral cultures, chest radiography, and several laboratory studies. The patients were treated by successful tonsillectomy, and after a mean follow-up of 10 months, no recurrence was observed. An analysis of the literature is also presented with particular emphasis on the differential diagnosis of this rare illness and the results of the different therapeutic options.- - - - - - - - - - ranking = 0.1keywords = pharyngitis (Clic here for more details about this article) |
4/10. Utility of tonsillectomy in 2 patients with the syndrome of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis.OBJECTIVES: To review the various causes of period fever in childhood, including the syndrome of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA), and to examine the value of tonsillectomy in the treatment of PFAPA syndrome. DESIGN: Retrospective case series. SETTING: Urban and tertiary care referral children's hospital. patients: Two patients who underwent tonsillectomy for presumed recurrent adenotonsillitis were later diagnosed as having PFAPA syndrome.Intervention tonsillectomy. MAIN OUTCOME MEASURE: Frequency of PFAPA symptoms before and after tonsillectomy. RESULTS: No difference was found in the frequency of PFAPA symptoms after tonsillectomy. CONCLUSION: Although a larger series of patients is required, our initial experience suggests that tonsillectomy is not always beneficial for patients with PFAPA syndrome.- - - - - - - - - - ranking = 0.5keywords = pharyngitis (Clic here for more details about this article) |
5/10. PFAPA syndrome: with regard to a case.BACKGROUND: PFAPA syndrome (Periodic Fever, Aphtas, pharyngitis and cervical Adenopathies) is one of the causes of periodic fever in pediatrics and it is characterised by high fever, pharyngitis, cervical adenitis and aphtous stomatitis. Its etiopathogeny is unknown. The diagnosis is clinical and the findings of laboratory are unspecified. One or two doses (1 mg/kg) of oral prednisone are enough for a fast resolution of the clinic. It is a benign syndrome and no sequels have been noticed after its disappearance, usually in four years from its beginning. CLINICAL CASE: We present the case of a 10-year-old patient who has been diagnosed of PFAPA syndrome after 3 years and a half of characteristic clinical bouts, with the fulfilment of diagnostic criteria and after having excluded other entities of similar presentation. CONCLUSIONS: Periodic episodes of high fever, pharyngitis and cervical adenitis with a bad response to the conventional treatment should alert us to the PFAPA syndrome. The recognition of this entity will help us to improve the diagnostic and therapeutical focusing, lowering also the anxiety that these cases produce.- - - - - - - - - - ranking = 0.2keywords = pharyngitis (Clic here for more details about this article) |
6/10. PFAPA syndrome mimicking familial mediterranean fever: report of a Turkish child.The PFAPA (Periodic Fever, Aphthous stomatitis, pharyngitis, Adenitidis) syndrome is characterized by periodic fever, adenitis, pharyngitis, and aphthous stomatitis. Herein, we present a Turkish child with PFAPA syndrome mimicking familial mediterranean fever because of a rare presentation. A 9-year-old boy was admitted with recurrent fever, aphthous stomatitis, sore throat, headache, and general body pains, lasting 2 to 3 days since 3.5 years of age. He was completely symptom-free between the attacks. He was diagnosed as having familial mediterranean fever according to the clinical findings when he was 6 years of age and colchicum tablet was administrated. Despite colchicines therapy for 8 months, his attacks did not subside; therefore, the drug was discontinued. He had high fever, a painful cervical lymphadenopathy, aphthous stomatitis, and tonsillo-pharyngitis. The patient was then diagnosed as having PFAPA syndrome. He was given a single dose of prednisolone (0.35 mg/kg/dose). His complaints dramatically and completely disappeared 3 h after administration of the drug. During the 8th month of follow-up, a similar febrile attack lasting only 1 day was noted and it was controlled with a single dose of prednisolone (0.5 mg/kg/day). At this writing the patient is in the 12th month of follow-up, and there have been no symptoms after the second attack. In conclusion, our patient shows that PFAPA syndrome can be confused with familial mediterranean fever. We also would like to emphasize that the typical PFAPA syndrome can be easily diagnosed by detailed history-taking and physical findings.- - - - - - - - - - ranking = 0.2keywords = pharyngitis (Clic here for more details about this article) |
7/10. PFAPA syndrome: a rare cause of periodic fever.PFAPA syndrome (periodic fever, aphthous stomatitis, pharyngitis, adenopathy) is characterized by abrupt onset of fever, malaise, aphthous stomatitis, tonsillitis, pharyngitis and cervical adenopathy. The age of onset of the disease is four years, with a range of 6 months to 7 years. The syndrome is sporadic and nonhereditary. Long-term sequelae do not develop. A nine- year-old boy presented with sore throat, fever and oral aphthae. After taking a throat culture, he was prescribed oral antipyretic and was called for a follow-up visit the next day. As the culture result was negative, he was given a single dose prednisolone with the suspected diagnosis of PFAPA. Twenty-four hours later his temperature was 36.8 degrees C, with all his complaints regressed. Twenty-two days later the patient was again admitted to our hospital with the same complaints. Again, single dose oral prednisolone was given after a throat culture. On the next day the patient was free of all symptoms and the culture was again normal. To our knowledge this is the first PFAPA case report from turkey in the literature.- - - - - - - - - - ranking = 0.2keywords = pharyngitis (Clic here for more details about this article) |
8/10. Tumor necrosis factor receptor-associated periodic syndrome in a young adult who had features of periodic fever, aphthous stomatitis, pharyngitis, and adenitis as a child.Tumor necrosis factor receptor-associated periodic syndrome (TRAPS) was diagnosed in a 22-year-old man after a 1-year history of periodic fever, myalgia, conjunctivitis, cervical lymphadenopathy, and oral ulcers. As a child he had signs and symptoms suggestive of periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome. This report indicates the importance of considering TRAPS as a cause of periodic fever in older children and adults and that TRAPS may present with signs and symptoms suggestive of periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome in young children.- - - - - - - - - - ranking = 0.6keywords = pharyngitis (Clic here for more details about this article) |
9/10. Recurrent aseptic encephalitis in periodic fever, aphthous stomatitis, pharyngitis and adenopathy (PFAPA) syndrome.An 11-year-old boy with episodes of periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) is reported. Two PFAPA episodes were associated with aseptic encephalitis and seizures. Recurrent acute aseptic encephalitis or seizures have never been reported during the febrile episodes of PFAPA. This possible association is discussed within the context of the etiology of PFAPA.- - - - - - - - - - ranking = 0.5keywords = pharyngitis (Clic here for more details about this article) |
10/10. Atypical PFAPA syndrome (periodic fever, aphthous stomatitis, pharyngitis, adenitis) in a young girl with fanconi anemia.PURPOSE: To describe a case of atypical, severe, periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome (PFAPA syndrome) in a patient with fanconi anemia. Important aspects about the PFAPA syndrome and fanconi anemia are reviewed. patients AND methods: An 8-year-old girl with fanconi anemia was noted to have a pattern of periodic fever, stomatitis, and pharyngitis consistent with the diagnosis of PFAPA syndrome, a generally benign disorder. After prednisone treatment for the syndrome, life-threatening intestinal ulceration and perforation developed, which was successfully treated. CONCLUSION: In patients with underlying hematologic disease such as fanconi anemia, PFAPA syndrome may be associated with severe clinical problems in contrast to otherwise normal children with the disorder.- - - - - - - - - - ranking = 0.6keywords = pharyngitis (Clic here for more details about this article) |
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