ESPN 51th Annual Meeting

ESPN 2018


 
CLINICAL COURSE OF AUTOSOMAL DOMINANT AND RECESSIVE POLYCYSTIC KIDNEY DISEASES
ZEHRA AYDIN 1 OZLEM YUKSEL AKSOY 1 SENEM MUT 2 FATMA SEMSA CAYCI 1 UMUT SELDA BAYRAKCI 1

1- ANKARA CHILDRENS HEALTH, HEMATOLOGY-ONCOLOGY TRAINING AND RESEARCH HOSPITAL, DEPARTMENT OF PEDIATRIC NEPHROLOGY
2- ANKARA CHILDRENS HEALTH, HEMATOLOGY-ONCOLOGY TRAINING AND RESEARCH HOSPITAL, DEPARTMENT OF PEDIATRICS
 
Introduction:

Polycystic kidney diseases are hereditary cystic diseases that affect kidney and other organs. The records of the patients who were followed up with diagnosis of polycystic kidney disease were retrospectively reviewed. The aim is to evaluate the clinical features, course of the disease, complications and the treatment  approaches.

Material and methods:

The study group consisted of patients who were diagnosed with ADPKD and ARPKD between January 2011 and January 2017 in our center. Patient records were evaluated for history, physical examination, laboratory results, imaging and follow-up.

Results:

Medical records of 19 patients followed-up with the diagnosis of ADPKD and three patients diagnosed with ARPKD were searched. Male/female ratio was 12/7 among ADPKD group. All of ARPKD patients were female. Mean age of patients were 89.92±64.95 months and 1.53 ±2.14 months in ADPKD and ARPKD groups respectively. The mean follow-up time was 37.83±19.80 months for ADPKD and 51.13±63.99 months for ARPKD. All ARPKD patients were diagnosed in the first years of their lives, and all of them were hypertensive. Only one (5%) of 19 patients was hypertensive in ADPKD group. One of the patients with ARPKD had chronic kidney failure at the time of diagnosis. We did not observe any significant increase in serum creatinine level during follow-up (p=0.2 for ADPKD, p=0.59 for ARPKD). No significant changes were detected between first and last visits in aspects of cyst diameters (p=0.51 for ADPKD, p=0.18 for ARPKD), and kidney sizes (p>0.05). Hepatic fibrosis is detected in two patients in ARPKD group.

Conclusions:

Course of ADPKD is good in children, whereas ARPKD patients must be followed-up closely for complications like hypertension and chronic renal failure.