PERIODIC FEVER, APHTHOUS STOMATITIS, PHARYNGITIS AND CERVICAL ADENITIS (PFAPA) SYNDROME IN IRANIAN CHILDREN FIRST REPORT OF IRANIAN PERIODIC FEVER AND AUTOINFLAMMATORY REGISTRY (IPFAIR)

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FERESHTEH MEHREGAN FATEMEH; ZIAEE VAHID; AHMADINEJAD ZAHRA; TAHGHIGHI FATEMEH; SABOUNI FARAH; MORADINEJAD MOHAMAD HASSAN

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Journal: Iranian Journal of Pediatrics Year:2014 | Volume:24 | Issue:5 Page(s): 598-602

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Title

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)

Keywords

PFAPA SYNDROME

PERIODIC FEVER

RECURRENT FEVER

CHILDREN

AUTOINFLAMMATORY DISORDERS

Abstract

Objective: The PERIODIC FEVER, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome is a nonhereditary idiopathic febrile syndrome belonging to the group of auto inflammatory 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.

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APA: Copy

FERESHTEH MEHREGAN, FATEMEH, ZIAEE, VAHID, AHMADINEJAD, ZAHRA, TAHGHIGHI, FATEMEH, SABOUNI, FARAH, & MORADINEJAD, MOHAMAD HASSAN. (2014). PERIODIC FEVER, APHTHOUS STOMATITIS, PHARYNGITIS AND CERVICAL ADENITIS (PFAPA) SYNDROME IN IRANIAN CHILDREN FIRST REPORT OF IRANIAN PERIODIC FEVER AND AUTOINFLAMMATORY REGISTRY (IPFAIR). IRANIAN JOURNAL OF PEDIATRICS, 24(5), 598-602. SID. https://sid.ir/paper/637197/en

Vancouver: Copy

FERESHTEH MEHREGAN FATEMEH, ZIAEE VAHID, AHMADINEJAD ZAHRA, TAHGHIGHI FATEMEH, SABOUNI FARAH, MORADINEJAD MOHAMAD HASSAN. PERIODIC FEVER, APHTHOUS STOMATITIS, PHARYNGITIS AND CERVICAL ADENITIS (PFAPA) SYNDROME IN IRANIAN CHILDREN FIRST REPORT OF IRANIAN PERIODIC FEVER AND AUTOINFLAMMATORY REGISTRY (IPFAIR). IRANIAN JOURNAL OF PEDIATRICS[Internet]. 2014;24(5):598-602. Available from: https://sid.ir/paper/637197/en

IEEE: Copy

FATEMEH FERESHTEH MEHREGAN, VAHID ZIAEE, ZAHRA AHMADINEJAD, FATEMEH TAHGHIGHI, FARAH SABOUNI, and MOHAMAD HASSAN MORADINEJAD, “PERIODIC FEVER, APHTHOUS STOMATITIS, PHARYNGITIS AND CERVICAL ADENITIS (PFAPA) SYNDROME IN IRANIAN CHILDREN FIRST REPORT OF IRANIAN PERIODIC FEVER AND AUTOINFLAMMATORY REGISTRY (IPFAIR),” IRANIAN JOURNAL OF PEDIATRICS, vol. 24, no. 5, pp. 598–602, 2014, [Online]. Available: https://sid.ir/paper/637197/en

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