1/5. 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 = 1keywords = physical (Clic here for more details about this article) |
2/5. Mucosal ulcerations revealing primitive hypereosinophilic syndrome.We report the case of a 27 year-old man developing recurrent oral aphtosis associated with fever and 8 kg of weight loss. Moderate splenomegaly was observed on physical examination and neurological and cardiac examination were normal. Laboratory findings included marked eosinophilia at 3280 giga/l. bone marrow (BM) examination revealed a myeloproliferative syndrome with mature eosinophils. splenectomy was performed because of a suspected nodule on the BM, the histopathology revealed a myeloid metaplasia. The diagnosis of myeloproliferative form of hypereosinophilic syndrome (HES) was made. He was treated with interferon-alfa and hydroxyurea. After two years of treatment he had no ulcer recurrence and eosinophil count was at 180 giga/l. Mucosal manifestations as a prodromal symptom of HES are rare. The histology of the lesions shows numerous eosinophils; immunohistochemical analysis confirms the presence of eosinophil peroxydase, major basic protein and eosinophil derived neurotoxin. A few cases have been described. death occurs 11 months to 5 years after the diagnosis of oral ulcerations. The treatment consists of interferon-alfa and hydroxyurea.- - - - - - - - - - ranking = 11.640999608841keywords = physical examination, physical (Clic here for more details about this article) |
3/5. 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 = 11.640999608841keywords = physical examination, physical (Clic here for more details about this article) |
4/5. 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 = 1keywords = physical (Clic here for more details about this article) |
5/5. "food allergy": Fact or Fiction?Six patients with longstanding physical and mental symptoms who had not been helped by many years of conventional medical investigation and treatment experienced immediate relief of symptoms when they avoided certain foodstuffs. This clinical study supports the view that some foods may cause widespread and disabling symptoms in people who are sensitive to them.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |