ESPE2024 Top 20 Posters Top 20 Posters (19 abstracts)
Investigating the Effects of Familial Mediterranean Fever on Growth and Puberty: Does the Duration of Puberty Change?
Ozge Bayrak Demirel 1 , Selen Duygu Arik 2 , Tugce Kandemir 1 , Ozlem Akgun 2 , Asli Derya Kardelen Al 1 , Melek Yildiz 1 , Sukran Poyrazoglu 1 , Nuray Aktay Ayaz 2 , Firdevs Bas 1 & Feyza Darendeliler 1
1Istanbul University, Istanbul Faculty of Medicine, Department of Pediatric Endocrinology, Istanbul, Turkey. 2Istanbul University, Istanbul Faculty of Medicine, Department of Pediatric Rheumatology, Istanbul, Turkey
Background: Familial Mediterranean Fever (FMF) is the most common hereditary periodic fever syndrome in Turkey. The effects of FMF on growth and puberty characteristics have yet to be investigated. This study aims to describe the features of children with FMF in terms of growth and puberty and compare them with healthy children.
Methods: This retrospective study analyzed the clinical and laboratory records of 140 FMF-diagnosed patients (73 girls, 67 boys), aged 8 to 18 years, under the care of the Divisions of Pediatric Endocrinology and Rheumatology from 2016 to 2024. The international severity score for FMF was assessed during the participants' last visit, and their growth and puberty data were compared with age-matched healthy children.
Results: In girls, the average age of FMF diagnosis and colchicine treatment initiation was 7.2± 3.4 years. 78% had mild disease under treatment. At pubertal onset (11.0± 1.5 years) mean height and BMI SDS were -0.7± 1.1 and 0.3± 1.5, respectively; menarche occurred at 12.3± 1.2 years. Age at onset of puberty was similar to healthy girls but girls with FMF completed puberty later (P < 0.001). Among 36 girls reaching final height, 19 reached mean target height. The mean target height and final height were -0.7± 0.8 and -0.42± 1.1 SDS, respectively. In boys, FMF diagnosis and colchicine treatment initiation was at the age of 6.1± 3.2 years. 96% had mild disease under treatment. At pubertal onset (12.3± 1.2 years) mean height and BMI SDS were -0.1± 1 and 0.1± 1.3, respectively. Although pubertal onset age was similar to healthy children, boys with FMF reached subsequent stages of puberty later (P < 0.05). Among 12 boys reaching final height, 11 achieved mean target height. The median target height and final height were -0.4 (-1.3- 0.1) and 0.33 (-1.6- 2.9) SDS, respectively. Chronological and bone age were compatible in both sexes. Predicted adult height was consistent with target height in girls and higher in boys. No significant correlation was found between the age at diagnosis and treatment initiation and age at onset and tempo of puberty.
Conclusion: Pubertal onset is similar but completion of puberty may be delayed in FMF patients. 63% of patients with FMF reached a final height within target height range.
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