TY - JOUR
T1 - Long-term risk of chronic kidney disease in unilateral multicystic dysplastic kidney
AU - Mansoor, Omer
AU - Chandar, Jayanthi
AU - Rodriguez, Maria M.
AU - Abitbol, Carolyn L.
AU - Seeherunvong, Wacharee
AU - Freundlich, Michael
AU - Zilleruelo, Gaston
PY - 2011/4/1
Y1 - 2011/4/1
N2 - The clinical spectrum of renal dysplasia includes the non-functioning multicystic dysplastic kidney (MCDK). We report our experience of the outcome of unilateral MCDK and its contralateral kidney in 101 children with the diagnosis of MCDK from 1985 to 2009. Data collected included urine protein/creatinine ratio, estimated GFR (eGFR), blood pressure, surgical intervention, renal length and abnormalities of the contralateral kidney, and the involution rate. There was a predominance of left-sided MCDK. Diagnosis was made prenatally in 86.7%. Contralateral abnormalities included vesicoureteral reflux (16.8%), UPJ obstruction (4.1%), and megaureter (2.4%). Complete involution of MCDK occurred within 5 years in 60%. Compensatory hypertrophy of the contralateral kidney to >97% occurred in 74.1%. Nephrectomy was performed in 19.8%. There was an increased risk of chronic kidney disease (CKD) stage 2, and hypertension in those with contralateral abnormalities (p=0.0001; p=0.001 respectively). In those without contralateral abnormalities, hyperfiltration with mean eGFR of 149±13 ml/min/1.73 m2 was seen in 32% and proteinuria in 9.8%. There was a significantly inverse relationship between proteinuria and eGFR (p=0.0001). In conclusion, children with contralateral abnormalities are at risk for developing decreased kidney function, whereas a substantial number of patients with no obvious contralateral abnormalities have markers of renal injury. Therefore, systematic follow-up of all patients is recommended.
AB - The clinical spectrum of renal dysplasia includes the non-functioning multicystic dysplastic kidney (MCDK). We report our experience of the outcome of unilateral MCDK and its contralateral kidney in 101 children with the diagnosis of MCDK from 1985 to 2009. Data collected included urine protein/creatinine ratio, estimated GFR (eGFR), blood pressure, surgical intervention, renal length and abnormalities of the contralateral kidney, and the involution rate. There was a predominance of left-sided MCDK. Diagnosis was made prenatally in 86.7%. Contralateral abnormalities included vesicoureteral reflux (16.8%), UPJ obstruction (4.1%), and megaureter (2.4%). Complete involution of MCDK occurred within 5 years in 60%. Compensatory hypertrophy of the contralateral kidney to >97% occurred in 74.1%. Nephrectomy was performed in 19.8%. There was an increased risk of chronic kidney disease (CKD) stage 2, and hypertension in those with contralateral abnormalities (p=0.0001; p=0.001 respectively). In those without contralateral abnormalities, hyperfiltration with mean eGFR of 149±13 ml/min/1.73 m2 was seen in 32% and proteinuria in 9.8%. There was a significantly inverse relationship between proteinuria and eGFR (p=0.0001). In conclusion, children with contralateral abnormalities are at risk for developing decreased kidney function, whereas a substantial number of patients with no obvious contralateral abnormalities have markers of renal injury. Therefore, systematic follow-up of all patients is recommended.
KW - CKD
KW - Contralateral kidney
KW - Hyperfiltration
KW - Multicystic dysplastic kidney
KW - Non-obstructive renal dysplasia
KW - Obstructive renal dysplasia
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U2 - 10.1007/s00467-010-1746-0
DO - 10.1007/s00467-010-1746-0
M3 - Article
C2 - 21240528
AN - SCOPUS:79954448890
VL - 26
SP - 597
EP - 603
JO - Pediatric Nephrology
JF - Pediatric Nephrology
SN - 0931-041X
IS - 4
ER -