Renal agenesis overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Renal agenesis is the absence of one (unilateral) or both (bilateral) kidneys at birth. Renal agenesis is a medical condition in which one (unilateral) or both (bilateral) fetal kidneys fail to develop.
It can be associated with RET or UPK3A.[1]
Epidemiology and Demographics
The general incidence of unilateral renal agenesis (URA) has been reported to be approximately 1 in 2031 individuals. Males are more commonly affected by unilateral renal agenesis (URA) than females.[2] The incidence of bilateral renal agenesis (BRA) is approximately 1 in every 3000 pregnancies.[3][4] The mortality rate of bilateral renal agenesis (BRA) without prenatal therapy is 100%.[5]
Natural History, Complications, and Prognosis
Complications of unilateral renal agenesis (URA) may include signs of renal injury such as: hypertension, microalbuminuria and chronic kidney disease.[2] Prognosis of bilateral renal agenesis (BRA) is extremely poor, and the mortality rate is 100% without prenatal therapy with serial amnioinfusion.[5]
Diagnosis
History and Symptoms
Physical Examination
CT
MRI
Ultrasound
Other Imaging Findings
Other Diagnostic Studies
Treatment
References
- ↑ Online Mendelian Inheritance in Man (OMIM) 191830
- ↑ 2.0 2.1 Westland R, Schreuder MF, Ket JC, van Wijk JA (2013). "Unilateral renal agenesis: a systematic review on associated anomalies and renal injury". Nephrol Dial Transplant. 28 (7): 1844–55. doi:10.1093/ndt/gft012. PMID 23449343.
- ↑ Bienstock JL, Birsner ML, Coleman F, Hueppchen NA (2014). "Successful in utero intervention for bilateral renal agenesis". Obstet Gynecol. 124 (2 Pt 2 Suppl 1): 413–5. doi:10.1097/AOG.0000000000000339. PMID 25004316.
- ↑ Isaksen CV, Eik-Nes SH, Blaas HG, Torp SH (2000). "Fetuses and infants with congenital urinary system anomalies: correlation between prenatal ultrasound and postmortem findings". Ultrasound Obstet Gynecol. 15 (3): 177–85. doi:10.1046/j.1469-0705.2000.00065.x. PMID 10846770.
- ↑ 5.0 5.1 Huber C, Shazly SA, Blumenfeld YJ, Jelin E, Ruano R (2019). "Update on the Prenatal Diagnosis and Outcomes of Fetal Bilateral Renal Agenesis". Obstet Gynecol Surv. 74 (5): 298–302. doi:10.1097/OGX.0000000000000670. PMID 31098643.