Complex abnormalities of urinary tract in boy with renal agenesis
 
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1
Katedra i Klinika Pediatrii i Nefrologii Warszawskiego Uniwersytetu Medycznego
 
2
Klinika Chirurgii i Urologii Warszawskiego Uniwersytetu Medycznego
 
3
Zakład Radiologii Pediatrycznej Warszawskiego Uniwersytetu Medycznego
 
 
Corresponding author
Grażyna Jadwiga Krzemień   

Katedra i Klinika Pediatrii i Nefrologii Warszawskiego Uniwersytetu Medycznego, Żwirki i Wigury 63A, 02-091 Warszawa, Polska
 
 
Ann. Acad. Med. Siles. 2017;71:134-138
 
KEYWORDS
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ABSTRACT
Introduction:
Unilateral renal agenesis can occur as an isolated abnormality or can coexist with other anomalies of the urinary tract. Vesicoureteral reflux and hydronephrosis are the most common pathologies of the urinary tract. The less common pathologies are: megaureter, double kidney, ureterocele, ectopic kidney and hypospadiasis.

Case report:
We report a boy with a complex abnormality of the urinary tract: left renal agenesis and right ectopic kidney with megaureter and hydronephrosis, diagnosed at the age of 2.5 years. A routine ultrasonography of the abdomen was done because of the abdominal pain. There were no clinical symptoms associated with the urinary tract before this diagnosis. Based on dynamic scyntigraphy, urography and voiding cystourethrography a ureterovesical junction obstruction was found. At the age of 3 years, the transplantation of the ureter modo Cohen was performed. Despite the operation, hydronephrosis still persisted and the cortex of kidney was continuously getting narrower. At the age of 11.5 years arterial hypertension was diagnosed. At the age of 14.5 years, slightly elevated creatinine and isosthenuria were observed. During those twelve years of follow-up, urinalysis and culture of urine were normal.

Conclusions:
A child with unilateral renal agenesis and obstructive uropathy in the second kidney requires regular follow up with control visits and appropriate tests in outpatient nephrology clinic. Blood pressure monitoring and assesment of kidney function should be monitored. Urinary tract abnormalities in solitary kidney can progress to chronic renal disease and end-stage renal disease.

REFERENCES (16)
1.
Westland R., Schreuder M.F., van Goudoever J.B., Sanna-Cherchi S., van Wijk J.A.E. Clinical Implications of the Solitary Functioning Kidney. Clin. J. Am. Soc. Nephrol. 2014; 9: 978–986.
 
2.
Sarhan O.M., Albedaiwi K., Al Harbi B., Al Otay A., Al Ghanbar M., Nakshababandi Z. Unilateral Renal Agenesis: Necessity of Postnatal Evaluation in a Contemporary Series. Urology 2016; 98: 144–148.
 
3.
Samanas N.B., Commers T.W., Dennison K.L., Harenda Q.E., Kurz S.G., Lachel C.M., Wavrin K.L., Bowler M., Nijman I.J., Guryev V., Cuppen E., Hubner N., Sullivan R., Vezina C.M., Shull J.D. Genetic Etiology of Renal Agenesis: Fine Mapping of Renag1 and Identification of Kit as the Candidate Functional Gene. PloS. One 2015; 10(2): e0118147, doi: 10 1371/journal.pone.0118147.
 
4.
Roodhooft A.M., Birnholz J.C., Holmes L.B.: Familial nature of congenital absence and severe dysgenesis of both kidneys. N. Engl. J. Med. 1984; 310: 1341–1345.
 
5.
Carter C.O., Evans K., Pescia G. A family study of renal agenesis. J. Med. Genet. 1979; 16(3): 178–188.
 
6.
Humbert C., Silbermann F., Morar B., Parisot M., Zarhrate M., Masson C., Tores F., Blanchet P., Perez M.J., Petrov Y., Khau Van Kien P., Roume J., Leroy B., Gribouval O., Kalaydjieva L., Heidet L., Salomon R., Antignac C., Benmerah A., Saunier S., Jeanpierre C. Integrin Alpha 8 Recessive Mutations Are Responsible for Bilateral Renal Agenesis in Human. Am. J. Hum. Genet. 2014; 94(2): 288–294.
 
7.
Westland R., Schreuder M.F., Ket J.C., van Wijk J.A. Unilateral renal agenesis: a systematic review on associated anomalies and renal injury. Nephrol. Dial. Transplant. 2013; 28: 1844–1855.
 
8.
Krzemień G., Roszkowska-Blaim G., Kostro I., Wojnar J., Karpińska M., Sękowska R. Urological anomalies in children with renal agenesis or multicystic dysplastic kidney. J. Appl. Genet. 2006; 47(2): 171–176.
 
9.
Szmigielska A., Księzopolska A., Roszkowska-Blaim M., Brzewski M., Krzemień G. Rare renal ectopia in children – intrathoracic ectopic kidney. Dev. Period Med. 2015; 19(2): 186–188.
 
10.
Hamid R., Wani S., Baba A., Mufti G. Solitary crossed ectopia with vesicoureteric junction obstruction: A rare case report. J. Indian. Assoc. Pediatr Surg. 2015; 20(2): 95–97.
 
11.
Zolotas E., Kirshnan R.G. Subdiaphragmatic Renal Ectopia: Case Report and Review of the Literature. Case Rep. Nephrol. 2016: 1084917. doi: 101155/2016/1084917.
 
12.
Bagheri A., Khorramirouz R., Keihani S., Fareghi M., Kajbafzadeh A.M. Solitary Crossed Renal Ectopia: Concurrence of Posterior Urethral Valvae and Hypospadiasis. Case Rep. Nephrol. 2015: 748139. doi.org/10.1155/2015/748139.
 
13.
Schwaderer A.L., Bates C.M., McHugh K.M., McBride K.L. Renal anomalies in family members of infants with bilateral renal agenesis/adysplasia. Pediatr. Nephrol. 2007; 22(1): 52–56.
 
14.
Burns J.A., Cooper C.S., Austin J.C. Cystic dysplasia of the testis associated with ipsilateral renal agenesis and contralateral crossed actopia. Urology 2002; 60(2): 344xiv–344xv.
 
15.
Kesan K., Gupta R., Gupta A., Kothari P., Ranjan R., Mudkhedkar K., Karkera P. Solitary Crossed Renal Ectopia with Vesicoureteric Reflux Presenting with Impaired Renal Function in Neonate. J. Clin. Neonatal. 2013; 2(3): 140–142.
 
16.
Habib Z., Abudaia J., Bameheriz F., Ahmed S. Fanconi,s anemia with solitary crossed renal ectopia, vesicouretric reflux, and genital abnormalities. Pediatr. Surg. Int. 2000; 16: 136–137.
 
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