Merve Aktaş Özgür, Kenan Bek, Mehtap Çelakıl

Agenezi veya Multikistik Displazi: Doğuştan Tek Böbrekli Çocuklarda Hangisi Daha Kötü?

Amaç: Renal agenezi ve Multikistik Displastik Böbrek (MKDB), gelişimsel böbrek kusurlarındandır. Her iki hastalıkta da fonksiyon gören tek böbrek mevcuttur. Yapılan çalışmalarda her iki hastalık ayrı ayrı incelenmiş ve uzun dönem sonuçları paylaşılmıştır.Yöntem: Çalışmaya Kocaeli Tıp Fakültesi Çocuk Nefroloji Polikliniğinden takipli olan 103 tek taraflı renal agenezi, 76 tek taraflı MKDB tanılı toplam 179 hastanın dosyaları geriye dönük olarak tarandı. Hastaların yaş, cinsiyet, ailede üriner sistem anomali öyküsü, antenatal tanı öyküsü, oligohidramnios öyküsü, eşlik eden ek hastalık öyküsü, cerrahi öyküsü ve anne-baba akrabalığı kaydedildi. Renal agenezi ve MKDB hastaları epidemiyolojik, etiyolojik, klinik ve prognostik faktörler açısından karşılaştırıldı.Bulgular: Tanı yaşı MKDB’de ortanca 1 ay (0-156), renal agenezilerde 48 ay (6-96) olarak bulundu (p<0,01). Antenatal tanı MKDB’de %82,7 (62), renal agenezilerde %49,5 (51) idi (p<0,001). Renal agenezilerde kız/erkek oranı 0,56 (37 kız, 66 erkek), multikistik displazilerde 1,02 (38 kız,37 erkek) idi (p<0,05). Her iki grupta da hastalık sol tarafta daha sıktı (MKDB 44 hasta, renal agenezi 67 hasta). Multikistik displastik böbrek tanısı alan 4 (%5,4) hastada doğum sırasında karşı taraftaki böbrekte hipertrofi mevcuttu. Takip eden yıllarda karşı tarafta hipertrofi gelişimi iki grup arasında benzerdi (MKDB %38,7, renal agenezi %46,1). Karşı tarafta hipertrofi başlama zamanı MKDB hastalarında anlamlı derecede daha erkendi (MKDB 32 ay, renal agenezi 72 ay) (p<0,05). Takip süreleri boyunca mikroalbuminüri görülme riski MKDB hastalarında anlamlı derecede yüksekti (renal agenezilerde %14,6, MKDB’de %30,3) (p<0,05).Sonuç: Her iki hastalık da uzun dönem takibi açısından iyi prognozludur. Hastalara rutin voiding sistoüreterografi (VCUG) çekilmesine ve rutin nefrektomi uygulamasına gerek yoktur.

Anahtar Kelimeler:

Multikistik displastik böbrek, renal agenezi, prognoz

Agenesis or Multicystic Dysplasia: Which One is Bad in Children Borned with only One Kidney?

Objective: Renal agenesis and Multicystic Dysplastic Kidney (MCDK) are among the developmental kidney defects. There is only one kidney that functions in both diseases. In these studies, both diseases were examined separately and their long term results were shared.Methods: A total of 179 unilateral renal agenesis and 76 unilateral MCDK patients followed in Kocaeli Pediatric Nephrology Polyclinic were reviewed retrospectively. Renal agenesis and MCDK patients were compared in terms of epidemiological, etiologic, clinical and prognostic factors.Results: The median age at diagnosis was 1 month (0-156) in MCDK and 48 months (6-96) in renal agenesis (p<0.01). Antenatal diagnosis was 82.7% (62) in MCDK and 49.5% (51) in renal agenesis (p<0.001). The female / male ratio was 0.56 (37 females, 66 males) in renal agenesis and 1.02 (38 females, 37 males) in multicystic dysplasias (p<0.05). In both groups, the disease was more frequent on the left side (44 patients with MCDK and 67 patients with renal agenesis). Four (5.4%) patients with MCDK had hypertrophy of the contralateral kidney during delivery. In the following years, the development of contralateral hypertrophy was similar between the two groups (MCDK 38.7%, renal agenesis 46.1%). The onset of hypertrophy on the opposite side was significantly earlier in MCDK patients (MCDK 32 months, renal agenesis 72 months) (p<0.05). The risk of microalbuminuria during follow-up was significantly higher in patients with MCDK (14.6% in renal agenesis and 30.3% in MCDK) (p<0.05).Conclusion: Both diseases have a good prognosis in terms of long-term follow-up. There is no need for routine voiding cystoureterography (VCUG) and routine nephrectomy.

Keywords:

Multicystic dysplastic kidney, renal agenesis, prognosis,

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Davidovits M, Cleper R, Eizenberg N, Hocherman O, Mashiach R. Outcomes of prenatally diagnosed solitary functioning kidney during early life. J Perinatol. 2017;37(12):1325-1329. doi:10.1038/jp.2017.143
Schreuder MF. Life with one kidney. Pediatr Nephrol. 2018;3:595-604.
Clinton CM, Chasen ST. Unilateral fetal renal abnormalities: Are they really isolated? J Ultrasound Med. 2016;35(3):561-564. doi:10.7863/ultra.15.05011
Kara A, Gurgoze MK, Aydin M, Koc ZP. Clinical features of children with multicystic dysplastic kidney. Pediatr Int. 2018;60(8):750-754. doi:10.1111/ped.13612
Can FK, Sarıtaş S, Alparslan C, Yavaşcan Ö. Multikistik displastik böbreklerin uzun dönem izlemi: Tek merkez deneyimi. Tepecik Eğit Hast Derg. 2017;27(3):217-221. doi:10.5222/terh.2017.217
Robson WL, Leung AK, Thomason MA. Multicystic dysplasia of the kidney. Clin. Pediatr. (Phila.). 1995;34:32-40. doi:10.1177/000992289503400106
Schreuder M, Westland R, Wijk JAE. Unilateral multicystic dysplastic kidney: a meta-analysis of observational studies on the incidence, associated urinary tract malformations of the contralateral kidney. Nephrol Dial Transplant. 2009;24:1810-1818. doi:10.1093/ndt/gfn777
Evrengül H, Ertan P, Serdaroğlu E, Yüksel S. Clinical characteristics and follow-up results of unilateral renal agenesis and hypoplasia of children. İzmir Dr. Behçet Uz Çocuk Hast. Dergisi. 2016;6(3):185-190. doi:10.5222/buchd.2016.185
Schreuder MF. Unilateral anomalies of kidney development: why is left not right? Kidney Int. 2011;80(7):740-745. doi:10.1038/ki.2011.204
Cochat P, Febvey O, Bacchetta J, Bérard E, Cabrera N, Dubourg L. Towards adulthood with a solitary kidney. Pediatr Nephrol. 2019;34(11):2311-2323. doi:10.1007/s00467-018-4085-1
Grabnar J, Rus RR. Is renal scintigraphy really a necessity in the routine diagnosis of congenital solitary kidney? Pediatric Surgery Internatıonal. 2019;35:729-735. doi:10.1007/s00383-019-04478-1
Merrot T, Lumenta DB, Tercier S, Morisson-Lacombes G, Guys JM, Alessandrini P. Multicystic dysplastic kidney withipsilateral abnormalities of genitourinary tract: Experience inchildren. Urology. 2006;67(3):603-607. doi:10.1016/j.urology.2005.09.062
Cascio S, Paran S, Puri P. Associated urological anomalies in children with unilateral renal agenesis. J Urol. 1999;162(3):1081-1083. doi:10.1097/00005392-199909000-00036 Krzemień G, Roszkowska-Blaim M, Kostro I, Wojnar J, Karpińska M, Sekowska R. Urological anomalies in children with renal agenesis or multicystic dysplastic kidney. J Appl Genet. 2006;47(2):171-176.doi:10.1007/BF03194618
Doğan ÇS, Torun Bayram M. Renal outcome of children with unilateral renal agenesis. Turk J Pediatr. 2013;55(6):612-615.
Mansoor O, Chandar J, Rodriguez MM, et al. Long-term risks of chronic kidney disease in unilateral multicystic dysplastic kidney. Pediatr Nephrol. 2011;26:597-603. doi:10.1007/s00467-010-1746-0
Shirzai A, Yildiz N, Biyikli N, Ustunsoy S, Benzer M, Alpay H. Is microalbuminuria a risk factor for hypertension in children with solitary kidney? Pediatr Nephrol. 2014;29(2):283-288. doi:10.1007/s00467-013-2641-2
Tabel Y, Aksoy Ö, Elmas AT, Çelik SF. Evaluation of hypertension by ambulatory blood pressure monitoring in children with solitary kidney. Blood Press. 2015;24(2):119-123. doi:10.3109/08037051.2014.992194
Aslam M, Watson AR, Trent & Anglia MCDK Study Group. Unilateral multicystic dysplastic kidney: Long term outcomes. Arch. Dis. Child. 2006;91(10):820-823. doi:10.1136/adc.2006.095786
Wang MK, Gaither T, Phelps A, Cohen R, Baskin L. The incidence and durability of compensatory hypertrophy in pediatric patients with solitary kidneys. Urology. 2019;129:188-193. doi:10.1016/j.urology.2019.04.003
Kim S, Chang Y, Lee YR, et al. Solitary kidney and risk of chronic kidney disease. Eur J Epidemiol. 2019;34:879-888. doi:10.1007/s10654-019-00520-7
Sharada S, Vijayakumar M, Nageswaran P, Ekambaram S, Udani A. Multicystic dysplastic kidney: a retrospective study. Indian Pediatr. 2014;51(8):641-643. doi:10.1007/s13312-014-0467-z
Caldeira F, Juvandes C, Pinto M, Braga M, Calhau P. Multicystic dysplastic kidney. In defense of a conservative experience. Acta Med Port. 2011;24(2):549-556.
Kumar B, Upadhyaya VD, Gupta MK, Bharti LK, Rao RN, Kumar S. Early nephrectomy in unilateral multicystic dysplastic kidney in children cures hypertension early: an observation. Eur J Pediatr Surg. 2017;27(6):533-537. doi:10.1055/s-0037-1599837