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Int J Pediatr Otorhinolaryngol. 2014 Jun;78(6):964-8. doi: 10.1016/j.ijporl.2014.03.026. Epub 2014 Apr 1.

Vitamin D levels and effects of vitamin D replacement in children with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome.

Author information

1
Health's Sciences Department, University of Florence, Anna Meyer Children's University Hospital, Florence, Italy. Electronic address: stefano.stagi@yahoo.it.
2
Department of BioMedicine, Section of Rheumatology, Transition Clinic, University of Florence, Florence, Italy.
3
Institute of Pediatrics, Università Cattolica Sacro Cuore, Rome, Italy.

Abstract

BACKGROUND:

The periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome is an autoinflammatory disease characterized by regularly recurrent fever episodes due to seemingly unprovoked inflammation.

OBJECTIVE:

To assess serum 25-hydroxyvitamin D [25(OH)D] concentrations in children with PFAPA syndrome and evaluate longitudinally the effect of wintertime vitamin D supplementation on the disease course.

STUDY DESIGN:

We have evaluated 25 Italian patients (19 males, 6 females, aged 2.4-5.3 years), fulfilling the Euro-Fever PFAPA criteria. For each patient, we recorded demographic and anthropometric data, clinical manifestations, serum calcium, phosphate, and 25(OH)D. After 400 IU vitamin D supplementation during wintertime, clinical and auxological characteristics, calcium, phosphate, and 25(OH)D levels were re-evaluated. Data were compared with a sex- and age-matched control group.

RESULTS:

PFAPA patients showed reduced 25(OH)D levels than controls (p<0.0001). Regarding the effect of seasons on vitamin D, winter 25(OH)D levels were significantly reduced than summer ones (p<0.005). Moreover, these levels were significantly lower than in healthy controls (p<0.005), and correlated with both fever episodes (p<0.005) and C-reactive protein values (p<0.005). After vitamin D supplementation, PFAPA patients showed a significantly decreased number of febrile episodes and modification of their characteristics (mean duration of fever episodes, p<0.05; number of febrile episodes per year p<0.005).

CONCLUSIONS:

Deficient and insufficient vitamin D serum levels were found in most children with PFAPA syndrome, and hypovitaminosis D might be a significant risk factor for PFAPA flares. However, vitamin D supplementation seems to significantly reduce the typical PFAPA episodes and their duration, supporting the role of vitamin D as an immune-regulatory factor in this syndrome.

KEYWORDS:

25-Hydroxyvitamin D; Aphthous stomatitis; Cervical adenitis; Child; PFAPA syndrome; Periodic fever; Pharyngitis

PMID:
24746456
DOI:
10.1016/j.ijporl.2014.03.026
[Indexed for MEDLINE]

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