ESPN 51th Annual Meeting

ESPN 2018


 
AUTOSOMAL-DOMINANT POLYCYSTIC KIDNEY DISEASE IN CHILDREN: PRELIMINARY RESULTS OF A TURKISH NATIONAL, MULTICENTER STUDY
ELIF BAHAT OZDOGAN 1 HULYA NALCACIOGLU 2 HARIKA ALPAY 3 ISMAIL DURSUN 4 KENAN BEK 5 BELDE KASAP DEMIR 6 DEMET TEKCAN 7 YONCA ACIKGOZ 8 ENGIN MELEK 9 FATMA YAZILITAS 10 MERAL TORUN BAYRAM 11 ZEYNEP YURUK YILDIRIM 12 IBRAHIM GOKCE 3 DILEK YILMAZ 13 SEBAHAT TULPAR 14 GUNES ISIK 1 PELIN ERTAN 15 AYSE AGBAS 16 OZLEM AYDOG 7 FATMA MUTLUBAS 6 ZELAL BIRCAN 5 NESLIHAN CICEK 3 AYSUN KARABAY BAYAZIT 9 ALPER SOYLU 11 EREN SOYALTIN 6 MEHTAP EZEL CELAKIL 5 NURDAN YILDIZ 3

1- KARADENIZ TECHNICAL UNIVERSITY FACULTY OF MEDICINE, PEDIATRIC NEPHROLOGY DDEPARTMENT, TRABZON, TURKEY
2- KAYSERI TRAINING AND RESEARCH HOSPITAL, PEDIATRIC NEPHROLOGY DEPARTMENT, KAYSERI, TURKEY
3- MARMARA UNIVERSITY FACULTY OF MEDICINE, PEDIATRIC NEPHROLOGY DEPARTMENT, ISTANBUL, TURKEY
4- ERCIYES UNIVERSITY FACULTY OF MEDICINE, PEDIATRIC NEPHROLOGY DEPARTMENT, KAYSERI, TURKEY
5- KOCAELI UNIVERSITY FACULTY OF MEDICINE, PEDIATRIC NEPHROLOGY DEPARTMENT, KOCAELI, TURKEY
6- TEPECIK TRAINING AND RESEARCH HOSPITAL, PEDIATRIC NEPHROLOGY DEPARTMENT,IZMIR, TURKEY
7- ONDOKUZ MAYIS UNIVERSITY FACULTY OF MEDICINE, PEDIATRIC NEPHROLOGY DEPARTMENT, SAMSUN, TURKEY
8- SAMSUN TRAINING AND RESEARCH HOSPITAL, PEDIATRIC NEPHROLOGY, SAMSUN, TURKEY
9- CUKUROVA UNIVERSITY, FACULTY OF MEDICINE, PEDIATRIC NEPHROLOGY DEPARTMENT, ADANA, TURKEY
10- DR.SAMI ULUS TRAINING AND RESEARCH HOSPITAL, PEDIATRIC NEPHROLOGY DEPARTMENT, ANKARA, TURKEY
11- DOKUZ EYLUL UNIVERSITY FACULTY OF MEDICINE, PEDIATRIC NEPHROLOGY DEPARTMENT, IZMIR, TURKEY
12- ISTANBUL UNIVERSITY FACULTY OF MEDICINE, PEDIATRIC NEPHROLOGY DEPARTMENT, ISTANBUL, TURKEY
13- ADNAN MENDERES UNIVERSITY FACULTY OF MEDICINE, PEDIATRIC NEPHROLOGY DEPARTMENT, AYDIN, TURKEY
14- BAKIRKOY DR. SADI KONUK TRAINING AND RESEARCH HOSPÄ°TAL, PEDIATRIC NEPHROLOGY UNIT, ISTANBUL, TURKEY
15- CELAL BAYAR UNIVERSITY FACULTY OF MEDICINE, PEDIATRIC NEPHROLOGY DEPARTMENT, MANISA, TURKEY
16- HASEKI TRAINING AND RESEARCH HOSPÄ°TAL, PEDIATRIC NEPHROLOGY UNIT, ISTANBUL, TURKEY
 
Introduction:

 Our aim is to determine demographic, basal clinical, laboratory and radiologic data of pediatric Autosomal-dominant polycystic kidney disease (ADPKD) patients in Turkey. Additionally, we aim to identify the risk factors for progression. 

Material and methods:

 Multicentric, retrospective, observational cohort study. A questionnaire for ADPKD was filled by the pediatric nephrologists based on the patient’s medical records. 

Results:

 277 patients from 29 pediatric nephrology centers were enrolled between January-April 2018. Mean diagnosis age was 7.98 ± 4.63 years. 54.2% were female. Only 30.3% were diagnosed with screening despite 98% had family history. The second most common cause of admission was the incidental finding of cyst on a routine ultrasound (17.7%). 83.3% had bilateral renal cysts detected by ultrasonography. 80.1% had normal renal size and 88.4% had not proteinuria, at diagnosis. 50% had bilaterally >4 cysts. The median largest cyst was 1.2 cm. Mean eGFR was 135 ± 44.5ml/min. 6.9% were hypertensive at baseline. Long-term follow-up was available for 162 patients with a median duration of 3.5 year. The median largest cyst was 1.5 cm and significantly increased (p<0.001) in the last visit. There was no significant difference in eGFR (p=0.49). In the last follow-up, 14.9% had hypertension, 9.4% had proteinuria and 18.4 % were on ACEi treatment. Urological complications were seen in 7.9% of patients. 

Conclusions:

 In this study, data are available from all regions of our country, reflecting the general characteristics of pediatric ADPKD patients.  The number of patients diagnosed by screening is low. Factors (hypertension, type of genetic mutation or urologic complications e.g) which may affect the progression of the disease, should be considered.