Retrospective evaluation of cystic kidney diseases

Cystic kidney diseases range from simple cyst to inherited cystic diseases that can lead to chronic kidney disease (CKD). In our study, it was aimed to retrospectively evaluate the demographic, clinical and laboratory data of our patients diagnosed with cystic kidney diseases. The files of 257 patients who applied to the Pediatric Nephrology Outpatient Clinic of Gaziantep University Medical Faculty Hospital between 2011-2021 due to cystic kidney disease were retrospectively analyzed. From the file records of the patients, diagnosis, gender, age, follow-up period, parental consanguinity, family history of cystic kidney disease, prenatal follow-up status, clinical, laboratory and ultrasonographic features, accompanying urological abnormalities, genetic analysis results, accompanying non-renal findings, dialysis the patient's need and the last condition ınformation of the patient were recorded. Cystic kidney disease in patients examined within the scope of our study, in 257 patients with the most frequent 100 patients (38,9%) multicystic dysplastic kidney (MCDK), 92 patients (35.8%) simple cyst, 21 patients (8.2%) autosomal dominant polycystic kidney disease (ADPCKD),18 patients (7.0%) autosomal recessive polycystic kidney disease (ARPCKD), 14 patients (5.4%) nephronophthisis, 10 patients (3.9%) bardet biedl, of one patient are followed up (0.4%) tuberous sclerosis and medullary sponge kidney. The mean age at diagnosis of the patients was 60.31±60.59 months, and the mean follow-up period was 94.76±61.98 months. 55.6% (n=143) of the patients were male and 44.4% (n=114) were female. Parental consanguinity was found in 113 (44.0%) patients, and prenatal diagnosis was found in 97 (37.7%) patients. When the findings detected during the application and follow-up of the patients are evaluated; Hematuria in 4.7% (n=12) patients, proteinuria in 5.1% (n=13) patients, elevated creatinine in 11.3% (n=29) patients, hypertension in 12.1% (n=31) patients, and 40.5% (n=14) patients urinary tract infection was detected. When the accompanying urological anomalies were evaluated, urological abnormalities were found in a total of 63 (24.51%) patients. The most common findings were kidney stones in 26.9% (n=17), UP stenosis in 25.4% (n=16) and vesicoureteral reflux in 22.2% (n=14). It was determined that 12.8% (n=33) of the patients had CKD in the final state, and 60.6% (n=20) of the patients with CKD needed dialysis. One of these patients with ORPCKD was found to have died due to a brain tumor. Cystic kidney diseases are an important group of diseases that can lead to proteinuria, hypertension and end-stage renal disease. Regular follow-up of pediatric patients with cystic kidney diseases ensures early detection of possible complications. In this case, complications can be treated with early intervention, and the progression of patients to end-stage renal disease can be prevented or delayed with follow-up.