Sevgin TANER, Nur Beril ÖZDEN, Banu SARSIK KUMBARACI, Sait ŞEN, Gülizar KOÇ, Taylan Özgür SEZER, Seçil CONKAR TUNÇAY, Su ÖZGÜR, Ahmet KESKİNOĞLU, İpek Kaplan BULUT
The Journal of Pediatric Research - 2026;13(2):133-140
Aim: Ciliopathies are rare genetic diseases referring to a group of syndromic diseases characterized by the deterioration of the structure of the cilia, which may cause kidney failure in childhood. Follow-up of the patients with ciliopathy after kidney transplantation is important for graft survival. Materials and Methods: This study was designed as a retrospective cohort trial. One hundred and fifty-one renal transplanted children (111 were non-ciliopathy and 31 were ciliopathy) were evaluated. Sociodemographic characteristics and clinical information regarding transplantation stage were recorded. Results: The mean age of the 31 patients (16 female/15 male) with the diagnosis of ciliopathy was 11.1+/-3.5 years and their mean follow-up duration was 7.7+/-4.8 years. Four of the patients (12.9%) experienced acute rejection and two patients had graft loss. Eleven patients had polycystic kidney disease, ten patients had cystic dysplasia and ten patients had nephronophthisis as their primary diagnosis. Graft survival rates were similar for transplants from living and cadaveric donors in those patients with ciliopathy. The data of the 31 patients who underwent kidney transplantation with the diagnosis of ciliopathy were compared with the 111 patients with the diagnosis of non-ciliopathy. The rates of hypertension, acute rejection and graft loss were similar in both groups. According to a Kaplan-Meier analysis, the graft and patient survival rates for those patients with ciliopathy and for those with non-ciliopathy were similar (p=0.123, p=0.370). Conclusion: Kidney transplant outcomes of patients with ciliopathy from well-selected living donors in terms of graft and patient survival are favorable.
