Analysis of the therapeutic approach
attack-free, PFAPA
PFAPA SYNDROME, DISEASE, HYPOVITAMINOSIS
Conservative treatment in our PFAPA patients consisted of the episodic administration of prednisone during attacks. We usually started with a dose of prednisone of above 1mg/kg/dose as recommended by the CARRA PFAPA working group (We also assessed the vitamin D levels and hypovitaminosis D as a risk factor for pure response to this treatment. We considered serum concentrations of vitamin D less than 30 mg/L to indicate hypovitaminosis D for this work (as used by most of the authors). We determined the serum concentration of vitamin D in 81 patients (36 girls and 42 boys). There were 42 patients with hypovitaminosis D in our group (51.9%) compared to 39 patients (48.1%) with normal vitamin D concentrations. The mean serum concentration of vitamin D in patients with hypovitaminosis was 19.95 (16.9–25.4) mg/L. In the group with hypovitaminosis D, the male gender predominated over the female (28/42 vs. 14/42; p=0.0368). In patients with hypovitaminosis D, we did not observe any statistically significant differences in the age of onset of the disease, the observed clinical and laboratory parameters, or the therapeutic response to ketotifen (despite concomitant treatment with vitamin D supplements in patients with hypovitaminosis D). All of the results are included in Vitamin D and PFAPA syndrome.CRP, C-reactive protein; IL-6, interleukin-6; N, number of patients.Therapeutic approach in our PFAPA patients.N, number of patients; TE, tonsillectomy. p-value is counted between the genders, p*- value is counted within the groups for the attack-free interval before and after ketotifen treatment.