Nationwide Turkish Cohort Study of Hypophosphatemic Rickets
Makaleler > Tam Makale · 2019
Yazarlar: ZEYNEP ŞIKLAR, SERAP DEMİRCİOĞLU, ABDULLAH BEREKET, Firdevs Bas, TÜLAY GÜRAN, Azad Akberzade, AYHAN ABACI, KORCAN DEMİR, ECE BÖBER, Mehmet Nuri Ozbek, Cengiz Kara, Sukran Poyrazoglu, Murat Aydin, Asli Kardelen, Omer Tarim, ERDAL EREN, NİHAL HATİPOĞLU, Muammer Buyukinan, Nesibe Akyurek, Semra Cetinkaya, Elvan Bayramoglu, BERAY SELVER EKLİOĞLU, Ahmet Ucakturk, Saygin Abali, Ruhsar Damla GÖKŞEN, Yilmaz Kor, Edip Unal, İHSAN ESEN, Ruken Yildirim, Onur Akin, Atilla Cayir, Emine Dilek, BİRGÜL KİREL, AHMET ANIK, Gonul Catli, MERİH BERBEROĞLU
Journal of Clinical Research in Pediatric Endocrinology, SCI Expanded İndekslerine Giren Dergi, Uluslararası, Wos, 2019, cilt 12, ss 150-159
DOI: 10.4274/jcrpe.galenos.2019.2019.0098
Özet
Objective: Hypophosphatemic rickets (HR) is a rare renal phosphate-wasting disorder, which is usually X-linked and is commonly caused by PHEX mutations. The treatment and follow-up of HR is challenging due to imperfect treatment options. Methods: Here we present nationwide initial and follow-up data on HR. Results: year PTH was higher. However, higher treatment doses of phosphate and calcitriol were found in the NC group. Conclusion: HR treatment and follow-up is challenging and our results showed higher treatment doses were associated with NC without any change in serum phosphate levels, suggesting that giving higher doses led to increased phosphaturia, probably through stimulation of fibroblast growth factor 23. However, higher calcitriol doses could improve bone deformities. Safer and more efficacious therapies are needed.