Prognosis of patients with unilateral renal agenesis

Argueso, Luis R.; Ritchey, Michael L.; Boyle, Emmett T.; Milliner, Dawn S.; Bergstralh, Erik J.; Kramer, Stephen A.

doi:10.1007/bf00873996

Cited by 156 publications

(103 citation statements)

References 16 publications

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“…Compared with a reference group, the risk for an impaired renal outcome was even higher when VUR was present (hazard ratio, 7.50; 95% confidence interval, 2.72 to 20.68). Results from another retrospective study on URA patients showed that 47% of individuals developed hypertension, 19% of individuals had proteinuria, 13% of individuals had an impaired GFR, and 4% of individuals died of renal failure (36). Similar findings were identified in adults with an acquired solitary functioning kidney (37).…”

Section: Clinical Outcomementioning

confidence: 54%

Clinical Implications of the Solitary Functioning Kidney

Westland

Schreuder

Goudoever

et al. 2014

Clinical Journal of the American Society of Nephrology

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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.

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Section: Clinical Outcomementioning

confidence: 54%

Clinical Implications of the Solitary Functioning Kidney

Westland

Schreuder

Goudoever

et al. 2014

Clinical Journal of the American Society of Nephrology

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“…Unilateral renal agenesis is a relatively frequent condition (1:5000) that is usually asymptomatic early in life but may lead to hypertension and proteinuria later in life due to an overall nephron deficit (44).…”

Section: Clinical Considerationsmentioning

confidence: 99%

Plumbing in the embryo: developmental defects of the urinary tracts

Uetani

Bouchard

2009

Clinical Genetics

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Kidney and urinary tract malformations are among the most frequent developmental defects identified in newborns. Ranging from asymptomatic to neonatal lethal, these malformations represent an important clinical challenge. Recent progress in understanding the developmental origin of urinary tract defects in the mouse and other animal models suggests a new framework for the interpretation of these defects in humans. Gene inactivation studies in mice provided invaluable information on the formation of the Wolffian duct, a central component of embryonic renal development, on ureter and kidney induction as well as on distal ureter maturation. All three developmental processes are crucial for normal urinary tract morphogenesis. A failure to complete these developmental steps is responsible for a spectrum of kidney and urinary tract malformations including renal agenesis, renal dysplasia, vesicoureteral reflux, hydroureter, hydronephrosis and ureterocele. Surprisingly, distal ureter maturation, the process by which the ureter is displaced from the Wolffian duct to its final position within the bladder wall, has only recently been characterized at the morphological level. Anomalies in this process are emerging as a major source of urinary tract developmental defects. This review is aimed at bridging the current knowledge on the morphological and molecular events identified in the mouse, together with clinical observations of urinary tract malformation in humans.

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“…8 In one study of unilateral renal agenesis, 4% had died from renal failure and 13% had decreased renal function. 21 During genetic counseling in cases of unilateral malformations, these points should be stressed. Illustrated case 15 had isolated right-sided multicystic dysplastic kidnney (MCDK).…”

Section: 11mentioning

confidence: 99%