Aprile 2001 - Volume XX - numero 4
Focus
Clinica Pediatrica dell’Università di Trieste, IRCCS “Burlo Garofolo”, Trieste
Key words: Recurrent febrile syndromes, Iper-IgD, Mediterranean fever, Cyclic neutropenia, PFAPA (Periodic Fever, Aphtous stomatitis, Pharyngitis and Adenitis)
A literature review is presented covering the recent advances regarding molecular bases and clinical features of recurrent febrile syndromes in childhood. These include hypoimmune (cyclic neutropenia, benign neutropenia) and hyperimmune (mediterranean fever, iper- IgD syndrome, TNFR-associated fever, PFAPA) forms. Cases of cyclic neutropenia, benign neutropenia, mediterranean fever, iper-IgD syndrome and PFAPA are reported.
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Focus
Istituto di Igiene, Clinica Pediatrica, UO di ORL Pediatrica, IRCCS “Burlo Garofolo”, Trieste
Key words: Recurrent pharyngitis, Adenovirus, FPAPA (Periodic Fever, Aphtous stomatitis, Pharyngitis and Adenitis)
20 cases of recurrent pharyngitis, among whom 9 have undergone tonsillectomy, are compared with 9 cases of tonsillar hypertrophy, all of whom had undergone surgery. Adenovirus etiology was confirmed in 8 out of 14 cases belonging to the first group in whom appropriate laboratory examinations could be performed, and in 1 out of 9 cases of the second group. Based on these findings and on a literature review an auto-inflammatory hypothesis of recurrent pharyngitis is proposed. Adenovirus, and EBV in older children could be the sensitising agent. The Authors suggest that recurrent pharyngitis, when criteria of cyclicity, abnormality of inflammatory markers and intercritical normality are satisfied, may not be differentiated from FPAPA syndrome.
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Focus
1Clinica Pediatrica, IRCCS “Burlo Garofolo”, Trieste
Key words: Recurrent pharyngitis, FPAPA (Periodic Fever, Aphtous stomatitis, Pharyngitis and Adenitis)
Through a review of all cases seen during the last 5 years, 20 practicing paediatricians were able to identify 40 cases who satisfy the diagnostic criteria for FPAPA. Recurrence range was between 3 and 6 weeks and overall duration from 1 to 6 years. 12 cases were successfully treated with a single dose of steroids, 12 underwent surgery and were all cured, 1 was treated with inhalatory steroids and recovered. The remaining 12 recovered spontaneously.
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