Periodic Fever, Aphthous Stomatitis, Pharyngitis and Cervical Adenitis (PFAPA) Syndrome in Iranian Children  First Report of Iranian Periodic Fever and Autoinflammatory Registry (IPFAIR)

Fatemeh Fereshteh  Mehregan; Zahra  Ahmadinejad; Fatemeh  Tahghighi; Farah  Sabouni; Mohamad-Hassan  Moradinejad; Vahid Ziaee

IJ Pediatrics

The Scientific Journal of Growth & Development Research Center

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Periodic Fever, Aphthous Stomatitis, Pharyngitis and Cervical Adenitis (PFAPA) Syndrome in Iranian Children First Report of Iranian Periodic Fever and Autoinflammatory Registry (IPFAIR)

Author(s):

Fatemeh Fereshteh Mehregan¹,

Zahra Ahmadinejad²,

Fatemeh Tahghighi³,

Farah Sabouni⁴,

Mohamad-Hassan Moradinejad⁵,

Vahid Ziaee^6,*

6, Tel:09121592716

IJ Pediatrics:Vol. 24, issue 5; 598-602

Published online:Oct 24, 2014

Article type:Research Article

Received:Jun 20, 2014

Accepted:Sep 23, 2014

How to Cite:Fatemeh Fereshteh MehreganZahra Ahmadinejad Fatemeh TahghighiFarah SabouniMohamad-Hassan MoradinejadVahid Ziaeeet al.Periodic Fever, Aphthous Stomatitis, Pharyngitis and Cervical Adenitis (PFAPA) Syndrome in Iranian Children First Report of Iranian Periodic Fever and Autoinflammatory Registry (IPFAIR).Iran J Pediatr.24(5):598-602.

Abstract

Objective: The periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome is a nonhereditary idiopathic febrile syndrome belonging to the group of autoinflammatory diseases. No longtime sequel was reported in this disease. Early diagnosis can lead physicians to treatment of this disorder with a short course steroid application and provide satisfaction of the patient’s family. Methods: This study is a prospective review of patients diagnosed with PFAPA syndrome who were registered in Iranian Periodic Fever and Autoinflammatory Registry (IPFAIR) through periodic fever clinic in the Children's Medical Center, Pediatric Center of Excellence in Tehran, Iran from January 2013 to March 2014. Findings: One hundred thirty patients were registered in our databases. Twenty-one (16.1%) patients including 15 males and 6 females had PFAPA. Normal growth was seen in all patients. The median age at onset was 18 months. The mean duration of fever was 4 days and the mean duration of intervals between fever episodes 21 days. Along with fever, all patients had characteristic symptoms. All patients were asymptomatic between fever episodes. Steroid was used in all patients and causing immediate reduction by 84.61%. Two patients received both steroid and colchicine because of their clinical feature and positive laboratory tests for PFAPA and familial Mediterranean fever. No patient received biological therapy or a tonsillectomy. Conclusion: The long diagnostic delay of PFAPA gives cause to concern indicating a need for greater awareness of the disease so that the diagnosis may be made timely.

Keywords

Autoinflammatory Disorders

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