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Renal agenesis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Shakiba Hassanzadeh, MD[2]

Synonyms and keywords:: Solitary kidney, unilateral renal agenesis, bilateral renal agenesis

Overview

Overview

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Shakiba Hassanzadeh, MD[2]

Overview

Renal agenesis is the congenital failure of embryonic kidney formation and may either be unilateral (congenital absence of one kidney with usually normal and hypertrophied contralateral solitary kidney) or bilateral (congenital absence of both kidneys which is incompatible with life). Renal agenesis may be isolated or associated with other anomalies and syndromes. Renal agenesis is diagnosed with routine screening with fetal ultrasound. Patients with unilateral renal agenesis (URA) have an increased risk for proteinuria, hypertension, and renal insufficiency. The mortality rate of bilateral renal agenesis (BRA) without prenatal therapy with serial amnioinfusion is 100%, however, further studies are required to assess the outcome, risks, and benefits of serial amnioinfusion.

Classification

Renal agenesis may be classified as unilateral renal agenesis(URA) or bilateral renal agenesis (BRA).[1] URA is the congenital absence of one kidney and BRA is the congenital absence of both kidneys which is incompatible with life.[1][2][3][4] Renal agenesis may be isolated or associated with other anomalies and syndromes.[5][6]  

Pathophysiology

Renal agenesis results from the failure of the ureteric bud to form the ureter, renal pelvis and the collecting duct system and the failure of the renal mesenchyme to form nephrons.[7]

Causes

Renal agenesis may be associated with CAKUT (congenital anomalies of the kidney and urinary tract) or extra-renal anomalies, genetic syndromes or chromosomal disorders, and sequences.[6][1][5]

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.[6] The incidence of bilateral renal agenesis (BRA) is approximately 1 in every 3000 pregnancies.[8][9] The mortality rate of bilateral renal agenesis (BRA) without prenatal therapy is 100%.[2]

Screening

Prenatal screening with ultrasound during pregnancy is routinely performed and this has led to an increase in the diagnosis of unilateral renal agenesis (URA).[6]

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.[6] Prognosis of bilateral renal agenesis (BRA) is extremely poor, and the mortality rate is 100% without prenatal therapy with serial amnioinfusion.[2]

Diagnosis

History

Maternal history during pregnancy for uncontrolled diabetes and treatment for hypertension may be important in unilateral renal disease (URA).[10] Checking the family history of patients with URA for URA, CAKUT (congenital anomalies of the kidney and urinary tract), end stage renal disease (ESRD), and consanguinity may be important.[11]

Symptoms and Physical Examination

Follow up with checking of blood pressure (for hypertension) and urinalysis (for proteinuria and renal insufficiency) should be considered in patients with unilateral renal agenesis (URA).[12][13] Checking for other contralateral congenital anomaly of kidney or urinary tract (CAKUT) such as VUR (vesicoureteral reflux) and PUJO (pelviureteric junction obstruction), extra-renal anomalies in different organs (such as cardiac, lung, gastrointestinal, genital, skeleton, and vertebral) , and associated syndromes and sequences (such as VACTERL, CHARGE and Trisomy 18) should be considered when URA is diagnosed.[5][6]

CT

Postnatal abdominal CT scan  in patients with unilateral renal agenesis (URA) shows no renal parenchyma.[13]

MRI

Prenatal MRI may be used for evaluating oligohydramnios, anhydramnios, fetal pulmonary hypoplasia and lung volumes.[14][15][16] Postnatal MRI may be helpful in differentiating unilateral renal agenesis (URA) from multicystic dysplastic kidney (MCDK) or renal ectopia.[17]

Ultrasound

Renal agenesis is diagnosed with routine screening with fetal ultrasound.[6] Renal ultrasonography shows no renal parenchyma in unilateral renal agenesis (URA).[13] Ultrasound findings in bilateral renal agenesis (BRA) may include: absence of fetal kidneys in the renal fossa, empty bladder and anhydramnios after 16 weeks of gestation.[18] Absence of renal arteries on color doppler ultrasonography indicates bilateral renal agenesis (BRA) and may be used as an additive diagnostic tool.[19]

Other Imaging Findings

Postnatal renal scintigraphy in patients with unilateral renal agenesis (URA) shows no renal function and may be helpful in differentiating unilateral renal agenesis (URA) from multicystic dysplastic kidney (MCDK) or renal ectopia.[13] [17]

Other Diagnostic Studies

There are no other diagnostic studies associated with renal agenesis.

Treatment

Medical Therapy and Surgery

Patients with unilateral renal agenesis (URA may progress to renal insufficiency that may progress to end stage renal disease (ESRD) and therefore may require medical treatment or renal replacement therapy.[13][4][12] Early diagnosis of unilateral renal agenesis (URA) and immediate intervention is helpful in controlling the progression to renal insufficiency.[13] The mortality rate of bilateral renal agenesis (BRA) without prenatal therapy with serial amnioinfusion, however is 100%, however, further studies are required to assess the outcome, risks and benefits of serial amnioinfusion, infancy dialysis and kidney transplantation.[2]

Primary Prevention

There are no established measures for the primary prevention of renal agenesis.

Secondary Prevention

There are no established measures for the secondary prevention of renal agenesis.

Cost-Effectiveness of Therapy

There is insufficient evidence about the cost-effectiveness of therapy in renal agenesis.

Future or Investigational Therapies

Intervention with serial amnioinfusion in bilateral renal agenesis (BRA) requires further clinical trials, and further research is needed to assess the long-term outcome of infant dialysis and renal transplantation.[2]

References

  1. ↑ 1.0 1.1 1.2 Dias T, Sairam S, Kumarasiri S (2014). “Ultrasound diagnosis of fetal renal abnormalities”. Best Pract Res Clin Obstet Gynaecol. 28 (3): 403–15. doi:10.1016/j.bpobgyn.2014.01.009. PMID 24524801.
  2. ↑ 2.0 2.1 2.2 2.3 2.4 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.
  3. ↑ Robson WL, Leung AK, Rogers RC (1995). “Unilateral renal agenesis”. Adv Pediatr. 42: 575–92. PMID 8540439.
  4. ↑ 4.0 4.1 Woolf AS, Hillman KA (2007). “Unilateral renal agenesis and the congenital solitary functioning kidney: developmental, genetic and clinical perspectives”. BJU Int. 99 (1): 17–21. doi:10.1111/j.1464-410X.2006.06504.x. PMID 16956352.
  5. ↑ 5.0 5.1 5.2 Laurichesse Delmas H, Kohler M, Doray B, Lémery D, Francannet C, Quistrebert J; et al. (2017). “Congenital unilateral renal agenesis: Prevalence, prenatal diagnosis, associated anomalies. Data from two birth-defect registries”. Birth Defects Res. 109 (15): 1204–1211. doi:10.1002/bdr2.1065. PMID 28722320.
  6. ↑ 6.0 6.1 6.2 6.3 6.4 6.5 6.6 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.
  7. ↑ Kerecuk L, Schreuder MF, Woolf AS (2008). “Renal tract malformations: perspectives for nephrologists”. Nat Clin Pract Nephrol. 4 (6): 312–25. doi:10.1038/ncpneph0807. PMID 18446149.
  8. ↑ 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.
  9. ↑ 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.
  10. ↑ Woolf AS, Hillman KA (2007). “Unilateral renal agenesis and the congenital solitary functioning kidney: developmental, genetic and clinical perspectives”. BJU Int. 99 (1): 17–21. doi:10.1111/j.1464-410X.2006.06504.x. PMID 16956352.
  11. ↑ Xu Q, Wu H, Zhou L, Xie J, Zhang W, Yu H; et al. (2019). “The clinical characteristics of Chinese patients with unilateral renal agenesis”. Clin Exp Nephrol. 23 (6): 792–798. doi:10.1007/s10157-019-01704-x. PMID 30734167.
  12. ↑ 12.0 12.1 Argueso LR, Ritchey ML, Boyle ET, Milliner DS, Bergstralh EJ, Kramer SA (1992). “Prognosis of patients with unilateral renal agenesis”. Pediatr Nephrol. 6 (5): 412–6. doi:10.1007/BF00873996. PMID 1457321.
  13. ↑ 13.0 13.1 13.2 13.3 13.4 13.5 Xu Q, Wu H, Zhou L, Xie J, Zhang W, Yu H; et al. (2019). “The clinical characteristics of Chinese patients with unilateral renal agenesis”. Clin Exp Nephrol. 23 (6): 792–798. doi:10.1007/s10157-019-01704-x. PMID 30734167.
  14. ↑ 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.
  15. ↑ 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.
  16. ↑ 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.
  17. ↑ 17.0 17.1 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.
  18. ↑ Sgro M, Shah V, Barozzino T, Ibach K, Allen L, Chitayat D (2005). “False diagnosis of renal agenesis on fetal MRI”. Ultrasound Obstet Gynecol. 25 (2): 197–200. doi:10.1002/uog.1739. PMID 15543544.
  19. ↑ DeVore GR (1995). “The value of color Doppler sonography in the diagnosis of renal agenesis”. J Ultrasound Med. 14 (6): 443–9. doi:10.7863/jum.1995.14.6.443. PMID 7658512.

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Classification

Classification

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Shakiba Hassanzadeh, MD[2]

Overview

Renal agenesis may be classified as unilateral renal agenesis (URA) or bilateral renal agenesis (BRA). URA is the congenital absence of one kidney and BRA is the congenital absence of both kidneys which is incompatible with life. Renal agenesis may be isolated or associated with other anomalies and syndromes.

Classification

Renal agenesis may be unilateral or bilateral:[1]

  • Unilateral renal agenesis (URA)
    • Congenital absence of one kidney[2][3]
    • The contra-lateral solitary kidney usually functions normally and may become hypertrophied[4]
  • Bilateral renal agenesis (BRA)

Renal agenesis may be isolated or associated with other anomalies and syndromes.[6][7]  

References

  1. ↑ 1.0 1.1 Dias T, Sairam S, Kumarasiri S (2014). “Ultrasound diagnosis of fetal renal abnormalities”. Best Pract Res Clin Obstet Gynaecol. 28 (3): 403–15. doi:10.1016/j.bpobgyn.2014.01.009. PMID 24524801.
  2. ↑ Robson WL, Leung AK, Rogers RC (1995). “Unilateral renal agenesis”. Adv Pediatr. 42: 575–92. PMID 8540439.
  3. ↑ Woolf AS, Hillman KA (2007). “Unilateral renal agenesis and the congenital solitary functioning kidney: developmental, genetic and clinical perspectives”. BJU Int. 99 (1): 17–21. doi:10.1111/j.1464-410X.2006.06504.x. PMID 16956352.
  4. ↑ Cho JY, Moon MH, Lee YH, Kim KW, Kim SH (2009). “Measurement of compensatory hyperplasia of the contralateral kidney: usefulness for differential diagnosis of fetal unilateral empty renal fossa”. Ultrasound Obstet Gynecol. 34 (5): 515–20. doi:10.1002/uog.7336. PMID 19852048.
  5. ↑ 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.
  6. ↑ Laurichesse Delmas H, Kohler M, Doray B, Lémery D, Francannet C, Quistrebert J; et al. (2017). “Congenital unilateral renal agenesis: Prevalence, prenatal diagnosis, associated anomalies. Data from two birth-defect registries”. Birth Defects Res. 109 (15): 1204–1211. doi:10.1002/bdr2.1065. PMID 28722320.
  7. ↑ 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.

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Pathophysiology

Pathophysiology

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Shakiba Hassanzadeh, MD[2]

Overview

Renal agenesis results from the failure of the ureteric bud to form the ureter, renal pelvis and the collecting duct system and the failure of the renal mesenchyme to form nephrons.

Pathophysiology

  • Unilateral renal agenesis (URA) is the one-sided congenital failure of embryonic kidney formation.[4][5]
  • In URA, the contralateral solitary kidney usually functions normally and may become hyperthrophied.[6]

References

  1. ↑ Schedl A (2007). “Renal abnormalities and their developmental origin”. Nat Rev Genet. 8 (10): 791–802. doi:10.1038/nrg2205. PMID 17878895.
  2. ↑ Kerecuk L, Schreuder MF, Woolf AS (2008). “Renal tract malformations: perspectives for nephrologists”. Nat Clin Pract Nephrol. 4 (6): 312–25. doi:10.1038/ncpneph0807. PMID 18446149.
  3. ↑ Laurichesse Delmas H, Kohler M, Doray B, Lémery D, Francannet C, Quistrebert J; et al. (2017). “Congenital unilateral renal agenesis: Prevalence, prenatal diagnosis, associated anomalies. Data from two birth-defect registries”. Birth Defects Res. 109 (15): 1204–1211. doi:10.1002/bdr2.1065. PMID 28722320.
  4. ↑ Robson WL, Leung AK, Rogers RC (1995). “Unilateral renal agenesis”. Adv Pediatr. 42: 575–92. PMID 8540439.
  5. ↑ Woolf AS, Hillman KA (2007). “Unilateral renal agenesis and the congenital solitary functioning kidney: developmental, genetic and clinical perspectives”. BJU Int. 99 (1): 17–21. doi:10.1111/j.1464-410X.2006.06504.x. PMID 16956352.
  6. ↑ Cho JY, Moon MH, Lee YH, Kim KW, Kim SH (2009). “Measurement of compensatory hyperplasia of the contralateral kidney: usefulness for differential diagnosis of fetal unilateral empty renal fossa”. Ultrasound Obstet Gynecol. 34 (5): 515–20. doi:10.1002/uog.7336. PMID 19852048.

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Causes

Causes

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Shakiba Hassanzadeh, MD[2]

Overview

Renal agenesis may be associated with CAKUT (congenital anomalies of the kidney and urinary tract) or extra-renal anomalies, genetic syndromes or chromosomal disorders, and sequences.

Causes

Some associated anomalies, genetic syndromes and teratogenic causes of renal agenesis include:

Associated Anomalies in Unilateral Renal Agenesis

(Modified table from Unilateral renal agenesis: a systematic review on associated anomalies and renal injury)[1]

Associated CAKUT

(congenital anomalies of the kidney and urinary tract)

(VUR (vesicoureteral reflux), PUV (posterior urethral valves), PUJO (pelviureteric junction obstruction), duplex kidney, ureterocele, and megaureter)

32%
Urinary tract infection 30%
Associated extra-renal anomalies 31%
Female tract anomalies 11%


Extra-renal Anomalies Associated with URA  

(Modified table from Congenital Unilateral Renal Agenesis: Prevalence, Prenatal Diagnosis, Associated Anomalies. Data from Two Birth-Defect Registries)[2]

Type   Extra-Renal Anomalies  
One effected system/organ
Associated Sequences
Associated Genetic Syndromes
Associated Chromosomal Anomalies
Associated Syndromatic Polymalformations (non-identified)
Macrocephaly, hypertelorism 
Anencephaly, caudal regression  
Occipital meningoencephalocele, unicornate uterus  
Heart defect, limb anomaly  
Tetralogy of Fallot, radial agenesia  
Patent ductus arteriosus, cerebellum hypoplasia, supernumerary hemivertebra, tracheolaryngomalacia, Meckel’s diverticulum, agenesis of eyelashes 
Common arterial trunk, ambiguous genitalia, imperforate anus, cystic hygroma  
Dandy-Walker syndrome, VSD  
Radial agenesia, clubhand, hypoplastic left heart syndrome, transposition of great vessels, agenesis of lung, diaphragmatic hernia, polysplenia  
Spina bifida, stenosis of anus  
Vertebral dysplasia, scrotal hypospadias  
Imperforate anus, ambiguous genitalia  
URA or BRA and Some Genetic Disorders  

(Modified table from Ultrasound diagnosis of fetal renal abnormalities)[3]

Syndrome Gene
Acro–renal–ocular syndrome   SALL 4  
Branchio–oto–renal syndrome  
  • EYA1
  • SIX1  
Ectrodactyly–ectodermal dysplasia–cleft syndrome   P63  
Pallistere Hall syndrome   GLI3  
Renal–coloboma syndrome   PAX2  
Townes–Brocks syndrome   SALL1  
Antley–Bixler syndrome  FGFR2  
Fraser syndrome  FRAS1  
Smith–Lemli-Opitz syndrome   DHCR7  
Goltz–Gorlin syndrome   PORCN  
Kallman syndrome   KAL1  
Lenz microphthalmia  BCOR  

Potter syndrome may include bilateral renal agenesis (BRA), pulmonary hypoplasia and oligohydramnios.[4] (For further information about Potter syndrome, click here.)

Teratogenic Causes Associated with Renal Agenesis:[5][6][7]

References

  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.
  2. ↑ Laurichesse Delmas H, Kohler M, Doray B, Lémery D, Francannet C, Quistrebert J; et al. (2017). “Congenital unilateral renal agenesis: Prevalence, prenatal diagnosis, associated anomalies. Data from two birth-defect registries”. Birth Defects Res. 109 (15): 1204–1211. doi:10.1002/bdr2.1065. PMID 28722320.
  3. ↑ Dias T, Sairam S, Kumarasiri S (2014). “Ultrasound diagnosis of fetal renal abnormalities”. Best Pract Res Clin Obstet Gynaecol. 28 (3): 403–15. doi:10.1016/j.bpobgyn.2014.01.009. PMID 24524801.
  4. ↑ 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.
  5. ↑ Boix E, Zapater P, Picó A, Moreno O (2005). “Teratogenicity with angiotensin II receptor antagonists in pregnancy”. J Endocrinol Invest. 28 (11): 1029–31. doi:10.1007/BF03345344. PMID 16483184.
  6. ↑ Nielsen GL, Nørgard B, Puho E, Rothman KJ, Sørensen HT, Czeizel AE (2005). “Risk of specific congenital abnormalities in offspring of women with diabetes”. Diabet Med. 22 (6): 693–6. doi:10.1111/j.1464-5491.2005.01477.x. PMID 15910618.
  7. ↑ Tse HK, Leung MB, Woolf AS, Menke AL, Hastie ND, Gosling JA; et al. (2005). “Implication of Wt1 in the pathogenesis of nephrogenic failure in a mouse model of retinoic acid-induced caudal regression syndrome”. Am J Pathol. 166 (5): 1295–307. doi:10.1016/S0002-9440(10)62349-8. PMC 1606386. PMID 15855632.

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Epidemiology and Demographics

Epidemiology and Demographics

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Shakiba Hassanzadeh, MD[2]

Overview

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. The incidence of bilateral renal agenesis (BRA) is approximately 1 in every 3000 pregnancies. The mortality rate of bilateral renal agenesis (BRA) without prenatal therapy is 100 %.

Epidemiology and Demographics

Incidence

  • The general incidence of unilateral renal agenesis (URA) has been reported to be approximately 1 in 2031 individuals, however, the incidence of URA based on prenatal studies alone has been reported to be 1 in 8091.[1]
  • The incidence of bilateral renal agenesis (BRA) is approximately 1 in every 3000 pregnancies.[2][3]

Prevalence

A report showed that the overall prevalence of unilateral renal agenesis (URA) was 4 per 10,000 in all births which included terminations of pregnancy (TOP) and intrauterine deaths (IUD).[4]

Mortality rate

The mortality rate of bilateral renal agenesis (BRA) without prenatal therapy is 100%.[5]

Gender

Males are more commonly affected by unilateral renal agenesis (URA) than females. It has been reported that 63% of the patients with URA are male.[1]

References

  1. ↑ 1.0 1.1 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.
  2. ↑ 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.
  3. ↑ 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.
  4. ↑ Laurichesse Delmas H, Kohler M, Doray B, Lémery D, Francannet C, Quistrebert J; et al. (2017). “Congenital unilateral renal agenesis: Prevalence, prenatal diagnosis, associated anomalies. Data from two birth-defect registries”. Birth Defects Res. 109 (15): 1204–1211. doi:10.1002/bdr2.1065. PMID 28722320.
  5. ↑ 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.

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Screening

Screening

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Shakiba Hassanzadeh, MD[2]

Overview

Prenatal screening with ultrasound during pregnancy is routinely performed and this has led to an increase in the diagnosis of unilateral renal agenesis (URA).

Screening

Prenatal screening with ultrasound during pregnancy is routinely performed and this has led to an increase in the diagnosis of unilateral renal agenesis (URA).[1]

References

  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.

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Natural History, Complications and Prognosis

Natural History, Complications and Prognosis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Shakiba Hassanzadeh, MD[2]

Overview

Complications of unilateral renal agenesis (URA) may include signs of renal injury such as: hypertension, microalbuminuria and chronic kidney disease. Prognosis of bilateral renal agenesis (BRA) is extremely poor, and the mortality rate is 100% without prenatal therapy with serial amnioinfusion.

Natural History, Complications, and Prognosis

Natural History

Patients with unilateral renal agenesis (URA) may usually be asymptomatic but may progress to renal insufficiency and end stage renal disease (ESRD).[1]

Complications

Complications of unilateral renal agenesis (URA) may include signs of renal injury such as:[2]

Prognosis

References

  1. ↑ Xu Q, Wu H, Zhou L, Xie J, Zhang W, Yu H; et al. (2019). “The clinical characteristics of Chinese patients with unilateral renal agenesis”. Clin Exp Nephrol. 23 (6): 792–798. doi:10.1007/s10157-019-01704-x. PMID 30734167.
  2. ↑ 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.
  3. ↑ 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.
  4. ↑ Dias T, Sairam S, Kumarasiri S (2014). “Ultrasound diagnosis of fetal renal abnormalities”. Best Pract Res Clin Obstet Gynaecol. 28 (3): 403–15. doi:10.1016/j.bpobgyn.2014.01.009. PMID 24524801.

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Diagnosis

Diagnosis

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Treatment

Treatment

Medical Therapy | Surgery | Primary Prevention | Secondary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies

Case Studies

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