Clinical Implications of the Solitary Functioning Kidney

Abstract

Congenital anomalies of the kidney and urinary tract are the major cause of ESRD in childhood. Children with a solitary functioning kidney form an important subgroup of congenital anomalies of the kidney and urinary tract patients, and a significant fraction of these children is at risk for progression to CKD. However, challenges remain in distinguishing patients with a high risk for disease progression from those patients without a high risk of disease progression. Although it is hypothesized that glomerular hyperfiltration in the lowered number of nephrons underlies the impaired renal prognosis in the solitary functioning kidney, the high proportion of ipsilateral congenital anomalies of the kidney and urinary tract in these patients may further influence clinical outcome. Pathogenic genetic and environmental factors in renal development have increasingly been identified and may play a crucial role in establishing a correct diagnosis and prognosis for these patients. With fetal ultrasound now enabling prenatal identification of individuals with a solitary functioning kidney, an early evaluation of risk factors for renal injury would allow for differentiation between patients with and without an increased risk for CKD. This review describes the underlying causes and consequences of the solitary functioning kidney from childhood together with its clinical implications. Finally, guidelines for follow-up of solitary functioning kidney patients are recommended.

Kidney, Urinary Bladder, and Ureter

“…In a systematic review of 43 cohorts comprising over 2600 patients with URA, the general incidence of URA was 1 in 2000. Because it is difficult to diagnose, the pre-renal incidence of URA is reported around 1 in 8000 (Westland et al 2013b(Westland et al , 2014. It is suggested that most cases diagnosed as URA are actually cases of renal aplasia, which is not easily detectable on ultrasound (Westland et al 2013b(Westland et al , 2014.…”

“…It should be discriminated from a unilateral nonfunctioning kidney resulting from multicystic renal dysplasia (MCRD); in the latter condition the renal parenchyma exhibits dysplastic and cystic differentiation, with a normally atretic ureter due to abnormal fetal renal development (Schedl 2007;Weber 2012). Its incidence is 1 in 4300 births (Westland et al 2013b(Westland et al , 2014. Fetal ultrasound now enables individuals with a solitary functioning kidney to be identified prenatally.…”

Trajectory of Estimated Glomerular Filtration Rate Predicts Renal Injury in Children with Multicystic Dysplastic Kidney

“…It is thus recommended to perform urinalysis once or twice a year and measure serum creatinine once in 2 years [4]. It is known that compensatory hypertrophy and hyperfiltration lead to glomerulosclerosis and kidney injury.…”

Avaliação Da Função Renal Em Pacientes Portadores De Rim Único

“…Desse modo, houve um aumento significativo de pacientes doadores renais vivos portadores de rim único. Apesar dessas pessoas aparentemente apresentarem uma qualidade de vida semelhante à população geral 1 , há vários estudos indicando haver piora da função renal a longo prazo [2][3][4][5][6] .…”

“…A TFG se altera muito discretamente ao longo do tempo, contudo, há o aparecimento de proteinúria assintomática nesses pacientes. Porém, vale ressaltar que a presença de proteinúria é o sinal mais precoce de lesão glomerular e pode indicar um início de um processo de glomeruloesclerose, sendo mascarado pelos mecanismos compensatórios intra-renais de aumento da TFG, estabilizando a TFG total [1][2][3][4]6,12,13 .…”