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{{Renal agenesis}}
{{Renal agenesis}}
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== Overview ==
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). Checking for other contralateral [[Congenital disorder|congenital]] [[anomaly]] of [[kidney]] or [[Urinary system|urinary tract]] (CAKUT) such as [[Vesicoureteral reflux|VUR (vesicoureteral reflux)]] and PUJO (pelviureteric junction obstruction), extra-renal [[Anomaly|anomalies]] in different organs (such as [[Heart|cardiac]], [[lung]], [[Gastrointestinal tract|gastrointestinal]], [[Sex organ|genital]], [[skeleton]], and [[Vertebra|vertebral]]) , and associated syndromes and sequences (such as [[VACTERL association|VACTERL]], [[CHARGE syndrome|CHARGE]] and [[Edwards syndrome|Trisomy 18]]) should be considered when URA is diagnosed.


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== Physical Examination ==
Patients with unilateral renal agenesis (URA) have an increased risk for [[proteinuria]], [[hypertension]], and [[renal insufficiency]]. Therefore, patients, even those with normal solitary kidney, should be followed up with:<ref name="pmid1457321">{{cite journal| author=Argueso LR, Ritchey ML, Boyle ET, Milliner DS, Bergstralh EJ, Kramer SA| title=Prognosis of patients with unilateral renal agenesis. | journal=Pediatr Nephrol | year= 1992 | volume= 6 | issue= 5 | pages= 412-6 | pmid=1457321 | doi=10.1007/BF00873996 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1457321 }} </ref><ref name="pmid30734167">{{cite journal| author=Xu Q, Wu H, Zhou L, Xie J, Zhang W, Yu H | display-authors=etal| title=The clinical characteristics of Chinese patients with unilateral renal agenesis. | journal=Clin Exp Nephrol | year= 2019 | volume= 23 | issue= 6 | pages= 792-798 | pmid=30734167 | doi=10.1007/s10157-019-01704-x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=30734167 }} </ref>
 
*[[Blood pressure]]
** Check for [[hypertension]]
 
* Urinalysis
**[[Proteinuria]]
**[[Hematuria]]
**[[Renal insufficiency]]
**[[Hyperuricemia]]
 
URA may be isolated or associated with other anomalies and malformations. Therefore, screening and checking for other contralateral [[Congenital disorder|congenital]] [[anomaly]] of [[kidney]] or [[Urinary system|urinary tract]] (CAKUT), extra-renal [[Anomaly|anomalies]], and associated syndromes and sequences (such as [[VACTERL association|VACTERL]], [[CHARGE syndrome|CHARGE]] and [[Edwards syndrome|Trisomy 18]]) should be considered when URA is diagnosed. Some of the findings that may be important to consider in the [[Physical examination|physical exam]] of URA include:<ref name="pmid28722320">{{cite journal| author=Laurichesse Delmas H, Kohler M, Doray B, Lémery D, Francannet C, Quistrebert J | display-authors=etal| title=Congenital unilateral renal agenesis: Prevalence, prenatal diagnosis, associated anomalies. Data from two birth-defect registries. | journal=Birth Defects Res | year= 2017 | volume= 109 | issue= 15 | pages= 1204-1211 | pmid=28722320 | doi=10.1002/bdr2.1065 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28722320 }} </ref><ref name="pmid23449343">{{cite journal| author=Westland R, Schreuder MF, Ket JC, van Wijk JA| title=Unilateral renal agenesis: a systematic review on associated anomalies and renal injury. | journal=Nephrol Dial Transplant | year= 2013 | volume= 28 | issue= 7 | pages= 1844-55 | pmid=23449343 | doi=10.1093/ndt/gft012 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23449343  }} </ref>
 
* Urinary tract
** Associated CAKUT
***[[Vesicoureteral reflux|VUR (vesicoureteral reflux)]]
*** PUJO (pelviureteric junction obstruction)
***[[Posterior urethral valves|PUV (posterior urethral valves)]]
*** Duplex kidney
***[[Ureterocele]]
***[[Megaureter]]
**[[Urinary tract infection]]
* Associated extra-renal [[Anomaly|anomalies]]
**[[Skull]]
*** Dysraphism parieto-occipital
*** [[Macrocephaly]]
*** [[Anencephaly]]
**[[Vertebral column|Spine]]
***[[Spina bifida]]
***Hemivertebra
***[[Coccyx|Coccygeal]] congenital fistula
***[[Caudal regression syndrome|Caudal regression]]
**[[Skeleton]]
***Radial agenesia
***Hexadactyly
***[[Club foot]]  
***Limb [[anomaly]]  
**[[Face]]
***[[Dysmorphic feature|Dysmorphic]]
***[[Hypertelorism]]
**[[Chest|Thorax]]
***[[Diaphragmatic hernia]]
***Thoracic [[Hemangioma|haemangioma]]  <br />
**[[Heart]]
***[[Atrial septal defect|ASD]]
***[[Ventricular septal defect|VSD]]
***[[Hypoplastic left heart syndrome]]
***[[Transposition of the great vessels|Transposition of great vessels]]
***[[Tetralogy of Fallot]]
***[[Patent ductus arteriosus]]<br />
**[[Lung]]
***Congenital lung cyst
***Agenesis of lung
**[[Gastrointestinal tract]]
***[[Meckel's diverticulum|Meckel’s diverticulum]]
***[[Imperforate anus]]
**Genital system
***Female tract anomalies
****Unicornate [[uterus]]
****Developmental [[ovarian cyst]]
***[[Hypospadias]]
***[[Epispadias]]
***[[Intersexuality|Ambiguous genitalia]]  


==References==
==References==

Latest revision as of 14:22, 1 August 2020

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

Overview

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). 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.

Physical Examination

Patients with unilateral renal agenesis (URA) have an increased risk for proteinuria, hypertension, and renal insufficiency. Therefore, patients, even those with normal solitary kidney, should be followed up with:[1][2]

URA may be isolated or associated with other anomalies and malformations. Therefore, screening and checking for other contralateral congenital anomaly of kidney or urinary tract (CAKUT), extra-renal anomalies, and associated syndromes and sequences (such as VACTERL, CHARGE and Trisomy 18) should be considered when URA is diagnosed. Some of the findings that may be important to consider in the physical exam of URA include:[3][4]

References

  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.
  2. ↑ 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.
  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. ↑ 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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