Renal agenesis overview: Difference between revisions
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=== MRI === | === MRI === | ||
Prenatal [[Magnetic resonance imaging|MRI]] may be used for evaluating [[oligohydramnios]], anhydramnios, [[Fetus|fetal]] [[Lung|pulmonary]] [[hypoplasia]] and [[lung]] volumes. [[Postnatal]] [[Magnetic resonance imaging|MRI]] may be helpful in differentiating unilateral renal agenesis (URA) from [[Multicystic dysplastic kidney|multicystic dysplastic kidney (MCDK)]] or [[renal ectopia]]. | Prenatal [[Magnetic resonance imaging|MRI]] may be used for evaluating [[oligohydramnios]], anhydramnios, [[Fetus|fetal]] [[Lung|pulmonary]] [[hypoplasia]] and [[lung]] volumes.<ref name="pmid24618008">{{cite journal| author=Gęca T, Krzyżanowski A, Stupak A, Kwaśniewska A, Pikuła T, Pietura R| title=Complementary role of magnetic resonance imaging after ultrasound examination in assessing fetal renal agenesis: a case report. | journal=J Med Case Rep | year= 2014 | volume= 8 | issue= | pages= 96 | pmid=24618008 | doi=10.1186/1752-1947-8-96 | pmc=3976151 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24618008 }} </ref><ref name="pmid21795484">{{cite journal| author=Kehl S, Zirulnik A, Debus A, Sütterlin M, Siemer J, Neff W| title=In vitro models of the fetal lung: comparison of lung volume measurements with 3-dimensional sonography and magnetic resonance imaging. | journal=J Ultrasound Med | year= 2011 | volume= 30 | issue= 8 | pages= 1085-91 | pmid=21795484 | doi=10.7863/jum.2011.30.8.1085 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21795484 }} </ref><ref name="pmid11425973">{{cite journal| author=Paek BW, Coakley FV, Lu Y, Filly RA, Lopoo JB, Qayyum A | display-authors=etal| title=Congenital diaphragmatic hernia: prenatal evaluation with MR lung volumetry--preliminary experience. | journal=Radiology | year= 2001 | volume= 220 | issue= 1 | pages= 63-7 | pmid=11425973 | doi=10.1148/radiology.220.1.r01jl4163 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11425973 }} </ref> [[Postnatal]] [[Magnetic resonance imaging|MRI]] may be helpful in differentiating unilateral renal agenesis (URA) from [[Multicystic dysplastic kidney|multicystic dysplastic kidney (MCDK)]] or [[renal ectopia]].<ref name="pmid18690548">{{cite journal| author=Zaffanello M, Brugnara M, Zuffante M, Franchini M, Fanos V| title=Are children with congenital solitary kidney at risk for lifelong complications? A lack of prediction demands caution. | journal=Int Urol Nephrol | year= 2009 | volume= 41 | issue= 1 | pages= 127-35 | pmid=18690548 | doi=10.1007/s11255-008-9437-5 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18690548 }} </ref> | ||
=== Ultrasound === | === Ultrasound === | ||
Revision as of 11:51, 23 July 2020
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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
Prenatal MRI may be used for evaluating oligohydramnios, anhydramnios, fetal pulmonary hypoplasia and lung volumes.[6][7][8] Postnatal MRI may be helpful in differentiating unilateral renal agenesis (URA) from multicystic dysplastic kidney (MCDK) or renal ectopia.[9]
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.
- ↑ Gęca T, Krzyżanowski A, Stupak A, Kwaśniewska A, Pikuła T, Pietura R (2014). "Complementary role of magnetic resonance imaging after ultrasound examination in assessing fetal renal agenesis: a case report". J Med Case Rep. 8: 96. doi:10.1186/1752-1947-8-96. PMC 3976151. PMID 24618008.
- ↑ Kehl S, Zirulnik A, Debus A, Sütterlin M, Siemer J, Neff W (2011). "In vitro models of the fetal lung: comparison of lung volume measurements with 3-dimensional sonography and magnetic resonance imaging". J Ultrasound Med. 30 (8): 1085–91. doi:10.7863/jum.2011.30.8.1085. PMID 21795484.
- ↑ Paek BW, Coakley FV, Lu Y, Filly RA, Lopoo JB, Qayyum A; et al. (2001). "Congenital diaphragmatic hernia: prenatal evaluation with MR lung volumetry--preliminary experience". Radiology. 220 (1): 63–7. doi:10.1148/radiology.220.1.r01jl4163. PMID 11425973.
- ↑ Zaffanello M, Brugnara M, Zuffante M, Franchini M, Fanos V (2009). "Are children with congenital solitary kidney at risk for lifelong complications? A lack of prediction demands caution". Int Urol Nephrol. 41 (1): 127–35. doi:10.1007/s11255-008-9437-5. PMID 18690548.