Abstract
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.
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Sukthankar S, Watson AR (2000) Unilateral multicystic dysplastic kidney disease: defining the natural history. Anglia Pediatric Nephrourology Group. Acta Paediatr 89:811–813
Hains DS, Bates CM, Ingraham S, Schwaderer AL (2009) Management and etiology of the unilateral multicystic dysplastic kidney: a review. Pediatr Nephrol 24:233–241
Greenbaum LA, Anver ED (2007) Cystic kidney disease. In: Kher K, Schnaper HW, Makker SP (eds) Pediatric nephrology, 2nd edn. Informa, UK, pp 261–273
Winyard P, Chitty L (2001) Dysplastic and polycystic kidneys: diagnosis, association and management. Prenat Diagn 21:924–935
Shaheen IS, Watson AR, Broderick N, Rance C (2005) Multicystic dysplastic kidney and pelviureteric junction obstruction. Pediatr Surg Int 21:282–284
Atiyeh B, Hussmann D, Baum M (1992) Contralateral renal abnormalities in multicystic dysplastic kidney disease. J Pediatr 121:65–67
Narchi H (2005) Risk of Wilms’ tumor with multicystic dysplastic kidney disease: a systematic review. Arch Dis Child 90:147–149
De Oliveria-Filho AG, Carvalho MH, S bargia-Neto L, Miranda ML, Bustorff-Silvia JM, de Oliveria ER (1997) Wilms’ tumor in a prenatally diagnosed multicystic dysplastic kidney. J Urol 158:1926–1927
Oliveira EA, Silva AC, Rabelo EA, Filgueiras FF, Periera AK, Mesquita FM (2002) Spontaneous improvement of hypertension in multicystic dysplastic kidney: a case report. Pediatr Nephrol 17:954–958
Seman T, John U, Blahova K, Vondrichova H, Janda J, Misselwitz J (2001) Ambulatory blood pressure monitoring in children with unilateral multicystic dysplastic kidney. Eur J Pediatr 160:78–83
Rabelo EA, Oliveira EA, Diniz JS, Silva JM, Filgueiras MT, Pezzuti IL, Tatsuo ES (2004) Natural history of multicystic kidney conservatively managed: a prospective study. Pediatr Nephrol 19:1102–1107
Wacksman J, Phipps L (1993) Report of the multicystic kidney registry: preliminary findings. J Urol 150:1870–1872
Aslam M, Watson AR (2006) Unilateral multicystic dysplastic kidney: long-term outcomes. Arch Dis Child 91:820–823
Schwartz GJ, Haycock GB, Edelmann CM Jr, Spitzer A (1976) A simple estimate of glomerular filtration rate in children derived from body length and plasma creatinine. Pediatrics 58:259–263
Han BK, Babcock DS (1985) Sonographic measurements and appearance of normal kidneys in children. AJR Am J Roentgenol 145:611–616
Chen JJ, Zhi J, Mao W, Steinhardt GF (2006) MrNomogram: a Web-based multivariable pediatric renal nomogram. J Pediatr Urol 2:436–438
Scott JE, Hunter EW, Lee RE, Mathews JNS (1990) Ultrasound measurement of renal size in newborn infants. Arch Dis Child 65:361–364
Force T (1987) Report of the second task force report on high blood pressure in children and adolescents. National Heart, Lung, and Blood Institute, Bethesda, Maryland. Pediatrics 79:1–25
Warady BA, Chadha V (2007) Chronic kidney disease in children: the global perspective. Pediatr Nephrol 22:1999–2009
Hogg RJ, Furth S, Lemley KV, Portman R, Schwartz GJ, Coresh J, Balk E, Lau J, Levin A, Kausz AT, Eknoyan G, Levey AS (2003) National Kidney Foundation’s Kidney Disease Outcomes Quality Initiative clinical practice guidelines for chronic kidney disease in children and adolescents: evaluation, classification and stratification. Pediatrics 111:1461–1421
Schwartz GJ, Work DF (2009) Measurement and estimation of GFR in children and adolescents. Clin J Am Soc Nephrol 11:1832–1843
Schreuder MF, Westland R, Van Wijk JA (2009) Unilateral multicystic dysplastic kidney: a meta-analysis of observational studies on the incidence, associated urinary tract malformations and the contralateral kidney. Nephrol Dial Transplant 24:1810–1818
Schreuder MF, Langemeijer ME, Bokenkamp A, Delemarre-Van de Waal HA, Van Wijk JA (2008) Hypertension and microalbuminuria in children with congenital solitary kidneys. J Paediatr Child Health 44:363–368
Runeberg-Roos P, Saarma M (2007) Neurotrophic factor receptor RET: structure, cell biology and inherited diseases. Ann Med 39:572–580
As W, Price KL, Scambler PJ, Winyard PJD (2004) Evolving concepts in human renal dysplasia. J Am Soc Nephrol 15:998–1007
Rigoli L, Chimenz R, Di Bella C, Cavallaro E, Caruso R, Briuglia FC, Salpietro CD (2004) Angiotensin-converting enzyme angiotensin type 2 receptor gene genotype distributions in Italian children with congenital uropathies. Pediatr Res 56:998–993
Ichikawa II, Kuwayama F, Pope JC 4th, Stephens FD, Miyazaki Y (2002) Paradigm shift from classic anatomic theories to contemporary cell biological views of CAKUT. Kidney Int 61:889–898
Kerecuk L, Sajoo A, Mcgregor L, Berg J, Haq MR, Sebire NJ, Bingham C, Edghill EL, Ellard S, Taylor J, Rigden S, Flinter FA, Woolf AS (2007) Autosomal dominant inheritance of non-syndromic renal hypoplasia and dysplasia: dramatic variation in clinical severity in a single kindred. Nephrol Dial Transplant 22:259–263
Vats A, Ishwad c, Vats KR, Moritz M, Ellis D, Mueller C, Surti U, Parizhskaya MZ, Meza MP, Burke L, Schneck FX, Saxena M, Ferrell R (2003) Steroid-resistant nephrotic syndrome and congenital anomalies of kidneys: evidence of locus on chromosome 13q. Kidney Int 64:17–24
Luque-Mialdea R, Martín-Crespo R, Cebrian J, Moreno L, Carrero C, Fernández A (2007) Does the multicystic dysplastic kidney really involute? The role of the retroperitoneoscopic approach. J Pediatr Urol 3:48–52
Onal B, Kogan B (2006) Natural history of patients with multicystic dysplastic kidney – what follow up is needed? J Urol 176:1607–1611
Hanh-Kieu V, Van Dyke M, Daniels H, Proesmans W (2008) Renal outcome of children with one functioning kidney from birth. A study of 99 patients and a review of the literature. Eur J Pediatr 167:885–890
Brenner BM, Lawler EV, Mackenzie HS (1996) The hyperfiltration theory: a paradigm shift in nephrology. Kidney Int 49:1774–1777
Raymond MH, Goldszer RC, Brenner BM (1984) Hypertension and proteinuria: long-term sequelae of uni-nephrectomy in humans. Kidney Int 25:930–936
Mandell J, Peters CA, Estroff JA, Alfred EN, Benaceraff BR (1993) Human fetal compensatory renal growth. J Urol 150:790–792
Morgensen CE, Christensen CK (1984) Predicting diabetic nephropathy in insulin-dependent patients. N Engl J Med 311:89–93
Wong H, Vivian L, Weiler G, Filler G (2004) Patients with autosomal dominant polycystic kidney disease hyperfiltrate early in their disease. Am J Kidney Dis 43:624–628
Kiyak A, Yilmaz A, Turhan P, Sander S, Aydin G, Aydogan G (2009) Unilateral multicystic dysplastic kidney: single center experience. Pediatr Nephrol 24:99–104
Ylinen E, Ahonen S, Ala-Houhala M, Wikstrom S (2004) Nephrectomy for multicystic dysplastic kidney: if and when? Urology 63:768–772
Wikstad I, Celsi G, Larson L, Herin P, Aperia A (1988) Kidney function in adults with unilateral renal agenesis or nephrectomized in childhood. Pediatr Nephrol 2:177–182
Weinstein A, Goodman TR, Irragori S (2008) Simple multicystic dysplastic kidney disease: endpoints for subspecialty follow-up. Pediatr Nephrol 23:111–116
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Mansoor, O., Chandar, J., Rodriguez, M.M. et al. Long-term risk of chronic kidney disease in unilateral multicystic dysplastic kidney. Pediatr Nephrol 26, 597–603 (2011). https://doi.org/10.1007/s00467-010-1746-0
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DOI: https://doi.org/10.1007/s00467-010-1746-0