Böbrek ve Üriner Sistemin Doğumsal Anomalileri: 806 Olgunun Analizi

Year 2019, , 284 - 287, 30.09.2019

Ahmet Midhat Elmacı , Muhammet İrfan Dönmez

https://doi.org/10.16899/jcm.597912

Abstract

Öz

Amaç:Böbrek ve üriner sistemin
doğumsal anomalileri (BÜSDA) tanımı, konjenital yapısal anomaliler kullanılan
bir terimdir. Prenatal ultrasonografinin yaygın kullanıma girmesiyle BÜSDA,
çocuk nefroloji ve üroloji vizitlerinin önemli bir bölümünü oluşturmaktadır. Bu retrospektif çalışmada, BÜSDA tanısı ile takip edilen hastaların
analizi ve bu hastalardaki renal hasar varlığı araştırılmıştır.

Gereç ve Yöntem: Kliniğimizde 2012-2018 yılları arasında BÜSDA tanısı ile
takip edilen hastaların dosyaları retrospektif olarak incelendi. Birden fazla
anomalisi olanlar (sendromik olgular vb.) çalışma dışı bırakıldı. Dosyalardan antenatal
tanı varlığı, yaş, cinsiyet, görüntüleme ve sintigrafi sonuçları kaydedildi.
Ultrasonografi ile takip edilen hastalarda gerektiğinde işeme
sistoüretrografisi (İSUG) ve dimerkaptosüksinik asit (DMSA) sintigrafisi
kullanıldı.

Bulgular: Çalışmaya 572 erkek
(%71) ve 234 kız (%29) olmak üzere toplam 806 hasta dahil edildi. Ortanca yaş 2
ay ve ortanca takip süresi 16 ay olarak bulundu. Hastaların 503’ünde (%62.4)
antenatal tanı mevcuttu ve 29 hastada (%3.6) prematür doğum öyküsü mevcuttu.
Üst üriner sistemde en sık görülen anomaliler sırasıyla izole hidronefroz
(%58.2), ektopik böbrek (%7.8) ve at nalı böbrek (%6.7) idi. Alt üriner
sistemde ise konjenital vezikoüreteral reflü (%10.9) ve megaüreterdi (%1.6).
Hastalardan 75 olguda (%9.3) renal skar, 53 olguda ise (%6.6) renal hipodisplazi saptandı.

Tartışma: BÜSDA’nın prenatal tanısı renal fonksiyon kaybına
neden olabilecek problemlerin erken dönemde tanınması şanısını artırmaktadır.
Buna rağmen hastaların ¼’ünden fazlası uzun dönemde tanı almadan kalmaktadır.
BÜSDA’lı çocuklar için, uygun tanı ve tedavi ile renal hasarın ilerlemesini
önlenmesi gereklidir. 

Keywords

BÜSDA, konjenital, anomali, üriner sistem

Congenital Anomalies of the Kidney and the Urinary Tract: Analysis of 806 Cases

Abstract

Objective

Congenital anomalies of the kidney and the
urinary tract (CAKUT) is a term used for a broad spectrum of congenital structural
anomalies. After implementation of prenatal ultrasonography, CAKUT became an
important part of pediatric nephrology and urology visits. The aim of this
retrospective study is to analyze the outcomes as well as prevelance of renal
damage in this group of patients.

Material and Methods

Patients diagnosed with CAKUT between 2012
and 2018 were retrospectively reviewed. Patients with multiple anomalies
(i.e.syndromes) were excluded. Presence of antenatal diagnosis, patient age,
gender, imaging studies and scintigraphy results were noted. Ultrasonography
was used for follow-up imaging, in addition to VCUG and DMSA when needed.

Results

There were 806 patients in our cohort (572
males and 234 females, 71% vs. 29%). Median age was 2 and median follow-up period
was 16 months. Only 3.6% of the patients were prematurely born where antenatal
diagnosis was present in 503 patients (62.4%). Most common anomalies in the
upper urinary tract were isolated hydronephrosis (58.2%), ectopic kidney (7.8%)
and horseshoe kidney (6.7%), respectively. On the other hand, VUR (10.9%) was
the most common abnormality of the lower urinary tract. Renal scarring was
observed in 75 patients (9.3%) while renal hypodysplasia was present in 53
patients (6.6%).

Conclusions

Prenatal diagnosis of CAKUT has increased
early diagnosis of problems that may lead to renal function loss. For children
with CAKUT, proper diagnosis and follow-up is necessary in order to prevent
renal deterioration.

Keywords

CAKUT, congenital, anomaly, urinary system

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