Anomalies Of Urinary Tract Research Articles

SAT-325 RELATIONSHIP AND PATTERN BETWEEN AMBULATORY BLOOD PRESSURE MONITORING PARAMETERS AND ESTIMATED GLOMERULAR FILTRATION RATE IN CHILDREN WITH GLOMERULAR AND NON-GLOMERULAR CONDITIONS

Ambulatory blood pressure monitoring (ABPM) is an important tool for evaluation of blood pressure (BP) among high risk children including those with renal diseases. Our study aimed to describe and compare the ABPM parameters between children with glomerular and non-glomerular renal conditions, and explore the relationship between the various ABPM parameters and the estimated glomerular filtration (eGFR). We performed a cross-sectional review of the office BP records and ABPM reports of children followed up in the paediatric nephrology clinic of Queen Elizabeth Hospital, Hong Kong from year 2013-2017. ABPM reports with <70% success rate were excluded. Among subjects with ≥1 ABPM reports, only the first available report was included for analysis. The renal diagnosis was categorized into glomerular diseases and non-glomerular disease comprising congenital anomalies of kidney and urinary tract (CAKUT), cystic kidney diseases, renovascular hypertension, neurogenic bladder / bladder dysfunction and other conditions. Altogether there were 67 patients included in the final analysis. The median age (interquartile range) of the cohort was 14.5 (5.4) years and the median BMI was 19.3 (5.0) kg/m2. Glomerular diseases accounted for 23.9% of all renal conditions and the median eGFR of the whole cohort was 96.3 (34.6) ml/min/1.73m (Table 1)2. There were 62.7% of subjects having ABPM hypertension (ABPM systolic or diastolic hypertension in either wake or sleep periods), and 40.3% of subjects were non-dippers (nocturnal dipping <10%). Office BP hypertension was found in 35.9% of subjects and 25% of subjects had masked hypertension. Only 16.7% of subjects with ABPM hypertension were receiving anti-hypertensive medications. The baseline characteristics of subjects with glomerular and non-glomerular diseases showed no significant difference, and the ABPM parameters in both wake and sleep periods were comparable between the two groups (Table 2). Most subjects with non-glomerular diagnoses had no hypertension (35.4%), whereas ambulatory hypertension (both office BP hypertension and ABPM hypertension) was the commonest BP category (37.5%) among those with glomerular conditions (p=0.934) (Figure 1). The median eGFR of children with glomerular and non-glomerular diseases showed no significant difference (p=0.538). All systolic or diastolic BP indices and BP loads in wake and sleep periods showed a significantly inverse relationship with the eGFR among children with glomerular diseases (Table 3). The relationship was stronger for glomerular than non-glomerular conditions. A lower eGFR among children with glomerular disease could be observed in those having wake or sleep ABPM hypertension (systolic or diastolic) or ABPM hypertension, as well as non-dippers. And a significant difference could be found among those with wake period diastolic hypertension (p=0.036) and sleep period systolic hypertension (p=0.044). Masked hypertension and non-dippers were common among children with renal conditions and most children with ABPM hypertension were not receiving anti-hypertensives. Both the wake and sleep period ABPM parameters demonstrated a negative correlation with eGFR among children having glomerular diseases at a relatively normal eGFR. The results indicated that regular ABPM should be performed for children with renal conditions, even without chronic kidney disease.

Renal bladder ultrasound in children with urinary tract infection

Introduction. Urinary tract infections (UTI) are a common problem in the pediatric age patients. They can represent the first sign of congenital anomaly of the kidney or urinary tract (CAKUT) in children younger than 2 years, vesicoureteral reflux being the most frequent. Renal bladder ultrasound (RBUS) is the most utilized imaging modality in children with UTI. RBUS is a non-invasive, non-radiating and the least expensive imaging test available. Materials and methods. The present study is a retrospective analysis of patients admitted for UTI in “Grigore Alexandrescu” Clinical Emergency Hospital for Children between 1st January 2015 and 31st December 2017. Results. RBUS was performed in 310/359 cases. Urinary tract anomalies were found in 108 patients. In 67/108 cases upper urinary tract dilations were identified. 31/191 patients (16%) under 2 years of age had upper urinary tract dilations on RBUS, while 36/119 patients (30%) older than 2 years had positive RBUS for upper urinary tract dilations. In 61 cases both RBUS and VCUG were available. 11/29 patients with upper urinary tract dilations on RBUS had VUR on VCUG (8 patients with high grade VUR). 8 patients with normal RBUS had VUR on VCUG (5 patients with high grade VUR). Conclusions. Although RBUS is a non-invasive and non-radiating imaging test, its value when used alone in children with UTI is limited in diagnosing CAKUT, especially VUR.

Congenital Anomalies of the Kidney and Urinary Tract: A Clinical Review.

This review highlights the most common congenital anomalies of the kidney and urinary tract (CAKUT) that are encountered in pediatric practices. CAKUT are the most common cause of prenatally diagnosed developmental malformations and encompass a spectrum of disorders impacting lower urinary tract development as well as kidney development and function. In pediatric and adolescent populations, developmental abnormalities are the leading cause of end-stage kidney disease. The goal of this review is to provide pediatric providers a framework for appropriate clinical management as well as highlight when referral to subspecialty care is needed. While the exact etiologies of CAKUT are not completely defined, new evidence demonstrates that genetic and molecular changes impact embryonic kidney and urinary tract development. As a result, phenotypes and clinical outcomes may be affected. Because pediatric providers provide front-line care to children and adolescents with developmental kidney and urinary tract anomalies, updated knowledge of CAKUT pathogenesis, embryology, clinical management, and patient outcomes is needed. This manuscript reviews CAKUT etiologies and essential diagnostic, prognostic, and management strategies.

Лапароскопическая хирургия опухолей подковообразной почки. Результаты первых 19 операций

Although horseshoe kidney (HSK) is the most common congenital anomaly of the upper urinary tract, renal cell cancer (RCC) in HSK develops extremely rarely. Until 2012 y. there were less than 200 cases of RCC in HSK published in PubMed. Only five cases of laparoscopic partial nephrectomies and some cases of heminephrectomies have been described in PubMed. To conduct a multicenter retrospective analysis of laparoscopic surgery for tumors in HSK. From January 2013 to December 2018 a total of 19 conventional laparoscopic interventions were performed in patients with RCC in HSK, including 1 isthmusectomy, 5 partial nephrectomies and 13 heminefrectomies. In addition, 16 divisions of isthmus were done in 15 patients. The video describing our operation technique is available on: http://youtu.be/nk-WlbjNtIs . There were no conversions to open surgery and mortality as well as intra- and postoperative complications of Clavien grade 3 or higher. Warm ischemia time during partial nephrectomy didnt exceed 19 minutes. Operative time ranged from 110 to 270 min, while max estimated blood loss was 400 ml. All patients were followed for 6 month and no case of disease recurrence or progression was noted. The small number of laparoscopic interventions in patients with RCC in HSK doesnt allow to carry out a proper statistical analysis and draw certain conclusions. We presented the largest experience available in the literature and our results demonstrate the efficacy and safety of conventional laparoscopic technologies in the treatment of RCC in HSK.

Clinicopathological Study of Congenital Anomalies in Urogenital Tract in Infants and Children

Background: Congenital abnormalities and malformations are more frequent in the urinary tract than in any other system of the body. The present study was conducted to evaluate incidence of congenital anomalies in urogenital tract in infants and children. Subjects and Methods: The present study was conducted on 55 cases age ranged 6 months to 8 years of congenital anomalies of urogenital tract which were admitted in department of Pediatric surgery ward of Kamla Nehru hospital, Bhopal during 1 year period. Type of anamoly was also recorded. In all patients, thorough clinical examination was performed. USG was done in all cases to confirm the cases. Results: Out of 1500 pediatric admissions, 56 (3.6%) had anomalies in urinary tract. In males, maximum lesions were hyposposdias seen in 13 followed by VUR & PUV in 12 patients and phimosis in 6 and 1 case of diphallia was found. In females, maximum lesions were congenital hydronephnosis, pelvic kidney, horse shoe kidney, ectopic ureter and ectopia vesicae seen in 1 patient each. Increased frequency of micturition was seen in 16, urine through ureter opening was seen in 12, dribbling of urine in 15 and suprapubic swelling was observed in 8 cases. USG revealed congenital hydronephnosis in 2, PUJ obstruction in 2, pelvic kidney in 2, ectopia vesicae in 2, ureterocele in 2. Conclusion: Authors found that maximum cases were of Hyposposdias followed by VUR & PUV and phimosis.

Laparoscopy-assisted ureterostomy— technique

Urinary tract anomalies in children at times pose challenges in which immediate urinary diversion is required before definitive reconstruction. Open cutaneous ureterostomy technique is a well-established approach for this scenario. We describe the laparoscopy-assisted alternative. Transperitoneal laparoscopy is performed with three ports. One trans-umbilical 5mm port for vision and two 3mm working instruments, one of which is through the proposed ureterostomy site minimising scarring. The position of the third port is carefully checked both externally and internally. Laparoscopic identification of the dilated ureter is quick and easy. The redundant ureter is mobilised and excess excised if needed. The ureteric loop is then exteriorised and the ureterostomy fashioned externally. Proximal and distal patency is checked both externally and laparoscopically. Nineteen patients underwent the procedure for primary VUR (n=6), neuropathic bladder 7, posterior urethral valve 4, and vesico-ureteric obstruction 2. The mean operative time was 40 minutes. the patients received three doses of perioperative antibiotics and were discharged in 72 hours (mean). At three months, clinical and ultrasound review have shown satisfactory and stable upper tracts and no episodes of UTI. The laparoscopic view gives the benefit of direct visualisation and identification with adequate dissection in order to bring the ureter to the surface in a tensionless manner. A redundant ureter can be easily addressed to correct tortuosity or redundancy appropriately. Bladder drainage can be ensured with careful dissection of the distal ureter and inspection at the end of the procedure. Laparoscopy-Assisted ureterostomy formation is easy and quick to perform. It is feasible even in the smallest of babies, assures the shortest segment of ureter to the bladder providing prompt drainage in a refluxing ureterostomy setting. Our early experience is promising with potential benefits.

Risk factors for development of urinary tract infection in children with nephrolithiasis.

Nephrolithiasis is one of the causes of urinary tract infection (UTI). In this study, we investigated risk factors for UTI in children with nephrolithiasis. The data from the patients with nephrolithiasis were evaluated in this retrospective follow-up study. Patients with a history of UTI before admission and congenital anomaly of kidney or urinary tract were excluded. The patients were divided into two groups: patients with UTI (recurrent UTI and single UTI) and patients without UTI. A total of 599 patients were included in this study. UTI occurred in 181 (30.2%) patients. There was a positive association between the size of stone and UTI (odds ratio (OR): 1.355, P = 0.007). Receiver operating characteristic curve analysis showed that the cut-off value of the size of stone for UTI was 5.3 mm, with a sensitivity of 74.9% and a specificity of 66.1% (area under the curve: 0.572 ± 0.028, P = 0.013). The presence of a metabolic risk factor and age at diagnosis under 2 years were significantly associated with both UTI and recurrence of UTI (OR: 2.272, P = 0.021, OR: 1.809, P = 0.028, respectively, for metabolic risk factor; OR: 1.212, P = 0.041, OR: 1.122, P = 0.046, respectively, for age at diagnosis under 2 years). Hypercalciuria was significantly associated with the recurrence of UTI (OR: 1.854, P = 0.017). The age at diagnosis, the presence of a metabolic risk factor and size of stone are significant risk factors for UTI in children with nephrolithiasis. The patients with idiopathic hypercalciuria, metabolic risk factor and age at diagnosis under 2 years have increased risk of recurrence of UTI.

Human IFT52 mutations uncover a novel role for the protein in microtubule dynamics and centrosome cohesion.

Mutations in genes encoding components of the intraflagellar transport (IFT) complexes have previously been associated with a spectrum of diseases collectively termed ciliopathies. Ciliopathies relate to defects in the formation or function of the cilium, a sensory or motile organelle present on the surface of most cell types. IFT52 is a key component of the IFT-B complex and ensures the interaction of the two subcomplexes, IFT-B1 and IFT-B2. Here, we report novel IFT52 biallelic mutations in cases with a short-rib thoracic dysplasia (SRTD) or a congenital anomaly of kidney and urinary tract (CAKUT). Combining in vitro and in vivo studies in zebrafish, we showed that SRTD-associated missense mutation impairs IFT-B complex assembly and IFT-B2 ciliary localization, resulting in decreased cilia length. In comparison, CAKUT-associated missense mutation has a mild pathogenicity, thus explaining the lack of skeletal defects in CAKUT case. In parallel, we demonstrated that the previously reported homozygous nonsense IFT52 mutation associated with Sensenbrenner syndrome [Girisha et al. (2016) A homozygous nonsense variant in IFT52 is associated with a human skeletal ciliopathy. Clin. Genet., 90, 536-539] leads to exon skipping and results in a partially functional protein. Finally, our work uncovered a novel role for IFT52 in microtubule network regulation. We showed that IFT52 interacts and partially co-localized with centrin at the distal end of centrioles where it is involved in its recruitment and/or maintenance. Alteration of this function likely contributes to centriole splitting observed in Ift52-/- cells. Altogether, our findings allow a better comprehensive genotype-phenotype correlation among IFT52-related cases and revealed a novel, extra-ciliary role for IFT52, i.e. disruption may contribute to pathophysiological mechanisms.

Exploration of postnatal integrated management for prenatal renal and urinary tract anomalies in China

Objective: The aim of this prospective observational study was to establish a suitable model for the postnatal follow-up and management of prenatal renal and urinary tract anomalies in Shanghai, China.Methods: Minhang and Changning maternal child health care hospitals were selected to establish the integrated management model. Newborns with prenatal renal and urinary tract anomalies in these two centers were eligible to participate in the study from 2015 to 2017. All newborns were classified into three groups based on prenatal findings: (1) severe/complex urinary tract dilatation (UTD) with ureterectasia, (2) other renal and urinary tract abnormalities, and (3) isolated mild to moderate UTD. The newborns underwent their first postnatal ultrasound and follow-up according to the presumed management strategy. Demographic and clinical data were collected from all institutes.Results: A total of 129 newborns fulfilled the study criteria, and 121 completed the postnatal evaluation. Ten newborns in group 1 (n = 13) were diagnosed with obstructive uropathy, including 9 with ureteropelvic junction obstruction (UPJO) and one with megaureter. All 13 newborns in group 2 had consistent postnatal results and were followed under previously established procedures. Sixty-seven cases in group 3 (n = 95) had a UTD at their first scan at 42 postnatal days, and two were diagnosed with UPJO. A total of 2 infants with UPJO underwent surgery, and 71 (65.7%, 71/108) of the UTD cases were resolved.Conclusions: The majority of the patients had a favorable outcome. Close multidisciplinary collaboration among obstetricians, neonatologists, pediatricians, and pediatric nephrologists and urologists is mandatory.

Characteristics and Findings of Childhood Urinary Tract Infection in the Last Decade

Background: The strong association between kidney and urinary tract anomalies and childhood urinary tract infection (UTI) often leads to imaging tests being performed. ­Objective: To describe the epidemiology, characteristics, and imaging findings in Thai children with UTI and compare results between boys and girls. Methods: We retrospectively reviewed the medical records of children with UTI aged < 15 years. Demographic characteristics and findings of investigations are presented. Results: One hundred seventy-eight boys and 170 girls with 432 UTI episodes were identified. The median (interquartile range) age at presentation was 1.4 (0.6–3.4) years, 1.0 for boys and 2.1 for girls (p < 0.001). Renal ultrasound, voiding cystourethrogram and ^99mTc dimercaptosuccinic acid (DMSA) renal scans were performed in 273, 223 and 113 children, respectively. Overall, 283 children (81.3%) had at least one imaging study done and anomalies of the kidney and urinary tract were detected in 158 (45.4%). Primary vesicoureteral reflux was detected in 73 (32.7%) children. The remaining abnormalities were hydronephrosis (n = 54). DMSA scans detected 54 children with dysplastic or scarred kidneys. Conclusions: First UTI in a group of Thai children occurred in approximately equal proportion in boys and girls but boys were younger at diagnosis. Kidney and urinary tract anomalies were detected in half of the children.

OR18-6 ADCY2 CNVs are Associated With Congenital Genitourinary Anomalies

Genitourinary (GU) anomalies are among the most common categories of birth defects and consist of disorders of sexual differentiation and congenital anomalies of kidney and urinary tract (CAKUT). Disorders of sexual differentiation include hypospadias (1 in 125 male births), cryptorchidism (3-6% of full term male births) and ambiguous genitalia (1:2000-1:4000 births). Though the prevalence of GU anomalies is high, their causes are poorly understood. A genetic change contributing to disease phenotypes is a copy number variant (CNV; a micro-deletion or micro-duplication of a small chromosomal region). When CNVs occur in dosage-sensitive genes, a disease phenotype can arise. Using array comparative genomic hybridization (aCGH), adenylyl cyclase 2 (ADCY2) CNVs were identified in a patient with hypospadias and a patient with ambiguous genitalia. ADCY2 is a member of the adenylyl cyclase class III family of genes that are responsible for converting ATP into cAMP. cAMP, is involved in several essential processes required for GU development and function including testicular descent and androgen production. Through a retrospective literature and database (DECIPHER, ICSA, ClinVar), nine patients with GU abnormalities and ADCY2 CNVs were identified. These GU anomalies consisted of upper and lower tract defects. The incidence of ADCY2 CNVs was determined by qPCR in a cohort of 378 GU abnormal patients and 45 control patients. ADCY2 CNVs (duplications) were present in 1.59% of patients with GU anomalies and 0% of control patients. The incidence of ADCY2 CNVs in the general population (Database of genomic variants) is 0.65%. Expression of ADCY2 in the GU tract was localized to renal tubules, bladder muscle, urethra, and interstitial cells of the testis with the highest expression in the testes. This expression pattern suggests a role for ADCY2 in steroid biosynthesis. When Adcy2 was overexpressed in the murine Leydig cell line MLTC-,1 cAMP compared to control MLTC-1 cells (4342.20 pmol/µl vs 2231.59 pmol/µl, two-way ANOVA <0.05), steroidogenic acute regulatory factor (StAR) expression and testosterone (52.33 ng/dL vs. undetectable, two-way ANOVA <0.05) were increased. Notably, the increase in testosterone production occurred independent of human chorionic gonadotropin (hCG) stimulation suggesting a ligand-independent, constitutively active system. In contrast to control cells transfected with an empty vector, hCG administration to ADCY2 overexpressing cells induced a slight additional increase in testosterone production. The lack of a robust response to hCG led to examination of the luteinizing hormone receptor (LHR). LHR was downregulated and internalized in Adcy2 overexpressing cells upon hCG stimulus. These findings suggest that microduplications in humans encompassing ADCY2 may contributed to lower tract genitourinary birth defects.

A Predictive Model of Postnatal Surgical Intervention in Children With Prenatally Detected Congenital Anomalies of the Kidney and Urinary Tract.

The aim of this study was to identify predictive factors and develop a model to assess individualized risk of postnatal surgical intervention in patients with antenatal hydronephrosis. This is a retrospective cohort study of 694 infants with prenatally detected congenital anomalies of kidney and urinary tract with a median follow-up time of 37 months. The main event of interest was postnatal surgical intervention. A predictive model was developed using Cox model with internal validation by bootstrap technique. Of 694 patients, 164 (24%) infants underwent surgical intervention in a median age of 7.8 months. Predictors of the surgical intervention in the model were: baseline glomerular filtration rate, associated hydronephrosis, presence of renal damage and the severity of renal pelvic dilatation. The optimism corrected c statistic for the model was 0.84 (95%CI, 0.82–0.87). The predictive model may contribute to identify infants at high risk for surgical intervention. Further studies are necessary to validate the model in patients from other settings.

Congenital Anomalies of Kidney and Urinary Tract (CAKUT): Single Center Experience for 14 Years

GIRIS ve AMAC: Bobrek ve idrar yollarinin konjenital anomalileri (DBUSA) genellikle asemptomatiktir ve cesitli toplumlarda son donem bobrek yetmezliginin% 30-60' inin sebebidir. CalismamizdA DBUSA'li hastalarin uzun sureli epidemiyolojik, demografik ve klinik ozelliklerini paylasmayi amacladik. YONTEM ve GERECLER: Calismaya, Nisan 2002-Ocak 2016 tarihleri arasinda Kocaeli Universitesi Tip Fakultesi Pediatrik Nefroloji Anabilim Dali tarafindan takip edilen, 0-18 yas arasi 11725 hastadan toplam 4625 DBUSA hastasi dahil edildi. Toplamda DBUSA olan 4625 hasta teshis edildi. Gecici hidronefroz tespit edilen 4164 hasta calismadan cikarildi. Calisilan 461 hastanin kayitlari retrospektif olarak degerlendirilmis ve SPSS 20.0 programi ile analiz edilmistir. BULGULAR: DBUSA sikligi% 3,9 idi. Olgularin erkek / kadin orani % 48,8 (225) /% 51,2 (236) idi. VUR % 31,5 (145) hastada tespit edildi, bunlarin % 71,7 (104) kadin ve% 28,3 (41) erkekti. Hidronefroz, 75 (% 162) hastada mevcuttu. Bu hastalarin antenatal tani orani % 30,7 idi (23). UPJ obstruksiyonu 26 (% 70,2) ve VUR 21 (% 14,4) hidronefroz ile iliskili en sik konjenital bobrek ve uriner sistem anomalileri idi. Olgularin % 50,5 (233) 'unde idrar yolu enfeksiyonu oykusu vardi. VUR tanisi olan hastalarda bu siklik % 90,3 olarak saptandi. Kronik bobrek hastaligi 8 hastada mevcuttu. Bu hastalarin 4'unde (% 50) altta yatan neden VUR idi. TARTISMA ve SONUC: Son yillarda, gecici hidronefroz prevalansi antenatal USG'nin yaygin kullanimi ile artmistir. Bu nedenle uzun donemde kronik bobrek hastaligi riski yuksek olan DBUSA'li hastalar gecici hidronefrozdan ayrilmali ve ucuncu basamak referans merkezlerinde pediatrik nefroloji ve pediatrik uroloji ile takip edilmelidir.

Congenital Anomalies of Kidney and Urinary Tract (CAKUT): Single Center Experience for 14 Years

Congenital Anomalies of Kidney and Urinary Tract (CAKUT): Single Center Experience for 14 Years

Glomerular disease patients have higher odds not to reach quality targets in chronic dialysis compared with CAKUT patients: analyses from a nationwide German paediatric dialysis registry.

Paediatric dialysis patients still suffer from high morbidity rates. To improve this, quality assurance programs like the German QiNKid (Quality in Nephrology for Children)-Registry have been developed. In our study, the significance of underlying renal disease on a range of clinical and laboratory parameters impacting morbidity and mortality was analysed. Our aim was to evaluate whether or not disease-specific dialysis strategies should be considered in planning dialysis for a patient. Inclusion criteria were defined as follows: (1) CAKUT (congenital anomalies of the kidney and urinary tract) or glomerular disease patient, (2) < 18years of age, (3) haemodialysis or peritoneal dialysis patient. Only measurements obtained from day 90 to 365 after the date of the first dialysis in the registry were analysed. Laboratory (serum albumin, haemoglobin, ferritin, calcium, phosphate, parathyroid hormone) and clinical parameters (height, blood pressure) were analysed using mixed effects models accounting for the correlation of repeated measures in individual patients. The study cohort comprised n = 167 CAKUT and n = 55 glomerular disease patients. Glomerular disease patients had significantly higher odds of hypoalbuminemia (OR 13.90, 95% CI 1.35-159.99; p = 0.0274), anaemia (OR 3.31, 95% CI 1.22-9.13; p = 0.0197), hyperphosphatemia (OR 9.69, 95% CI 2.65-37.26; p = 0.0006) and diastolic hypertension (OR 3.38, 95% CI 1.20-9.79; p = 0.0212). Glomerular disease patients might require more intensive dialysis regimens. The evaluation of hydration status should be given more attention, since conditions differing between the cohorts can be linked to overhydration. The QiNKid-Registry allows monitoring of the quality of paediatric dialysis in a nationwide cohort.

Value of renal pelvic diameter and urinary tract dilation classification in the prediction of urinary tract anomaly.

The aim of this study was to identify the cut-offs of postnatal anteroposterior renal pelvic diameter (APRPD), according to the urinary tract dilation (UTD) classification system, to identify the predictors of final diagnosis of UTD and the need for surgery. A total of 260 infants (336 renal units) with prenatally detected UTD were prospectively evaluated on serial ultrasonography by the same radiologist. Additional voiding cystourethrography and scintigraphy was done according to the clinical algorithm. Prenatal and postnatal APRPD in patients with transient dilation were significantly lower than in those with urinary tract anomalies (UTA). On follow up, the slope of decrease in APRPD was significantly higher in transient dilation compared with UTA. APRPD 10 mm at first-month ultrasonography, predicted UTA with a sensitivity of 83.1%, and specificity of 71.1%. On multivariate analysis the likelihood of surgical intervention and final diagnosis were predicted independently by the UTD system risk group. Careful ultrasonography evaluation can avoid unnecessary testing in patients with transient or clinically insignificant dilation. The UTD classification system is valid for evaluation of postnatal hydronephrosis and is reliable in predicting the need for surgical intervention.

Outcome of posterior urethral valve in 64 children: a single center's 22-year experience.

Posterior urethral valve (PUV) is the most serious form of congenital anomalies of kidney and urinary tract (CAKUT) in boys with significant risk of progression to chronic kidney disease (CKD). We present our long-term results in children with PUV. Retrospective chart review of 113 children with PUV followed within the years of 1996-2018 was performed. Clinical, laboratory and epidemiologic parameters were analyzed for their impact on renal outcome. The median age of diagnosis was 1.00 month (1.00-132.00) and the median follow-up period was 70 months (60.00-216.00). Antenatal diagnosis was present in 33 patients (51.5%) mainly with bilateral hydronephrosis and oligohydramnios. The most common postnatal presentation was recurrent urinary tract infection (UTI) in 14 cases (21.9%) and incontinence in three cases (4.7%). Vesicoureteral-reflux (VUR) was present in 31 cases (48.4%). All patients had surgery and urinary diversion was needed in 18 (28.2%). Varying stages of chronic kidney disease (CKD) developed in 23 cases (35.9%) and rise in serum creatinine was especially prominent after the 4th year of follow-up. Of 23 CKD patients, seven (10.9%) were in ESRD and on dialysis. Mortality occurred in one (1.5%) patient. Hypertension, proteinuria and high initial serum creatinine (>1.28 mg/dL) were statistically significant risk factors for CKD, as expected. Surprisingly VUR and UTI did not show such a significant impact on CKD development. Antenatal detection was with significantly less risk for CKD. Our results confirm that PUV has a considerable risk for CKD development. Antenatal diagnosis, management of proteinuria and hypertension may modify this progression. But already injured kidneys still have a potential risk. The need for further research to evaluate the impact of any intervention on long term renal outcome is obvious.

Applicability of Magnetic Resonance Imaging in the Assessment of Fetal Urinary Tract Malformations

ObjectiveTo assess the applicability of magnetic resonance imaging (MRI) to complement ultrasound in the diagnosis of fetal urinary tract anomalies. MethodsThis was a retrospective cohort study that included 41 women between 19 weeks and 37 weeks and 6 days of gestation carrying fetuses with malformations of the urinary tract which were initially diagnosed by ultrasound and then referred for MRI. In all cases, the diagnosis was confirmed after birth either through imaging or autopsy. A surface coil was positioned over the abdomen and T2-weighted sequences were obtained in the axial, coronal, and sagittal planes; T1 in at least one plane; and three-dimensional (3-D) TRUFI in fetuses with dilatation of the urinary tract. ResultsMean gestational age at the time of MRI examination was 28.21 weeks. The rapid T2 sequences allowed all the anomalies of the fetal urinary tract to be assessed, whereas 3-D TRUFI sequencing proved very useful in evaluating anomalies involving dilatation of the urinary tract. The signs of pulmonary hypoplasia characterized by hypointense signal in the T2-weighted sequences were identified in 13 of the 41 fetuses. ConclusionMRI confirmed and added information to the ultrasound regarding fetal urinary tract anomalies, as well as information related to the other associated malformations, their progress in the prenatal period, and possible postnatal prognosis.

One-year follow-up results of infants with antenatal hydronephrosis

Objection: Our purpose is to present the results of the one year- follow up of infants who were diagnosed with antenatal hydronephrosis. Methods: Infants (0-24 months) with diagnosis of antenatal hydronephrosis who were brought into pediatric nephrology outpatient clinics of our hospital were followed up according to the Turkish Pediatric Nephrology Society CAKUT (Congenital Anomalies of Kidney and Urinary Tract) Guidelines. Results: Ninety-three infants (64 males, 0.83 years, range 0.1-2.1 years) previously diagnosed as antenatal hydronephrosis received diagnosis of healthy infant (n=5; 5,4 %) mild (n=70; 75,3%), moderate (n=14; 15,1%), and severe (n=4; 4,3%) hydronephrosis. Voiding cystourethrogram (VCUG) was performed in 10 (10,7%) patients, 2 of them had unilateral grade 4 vesicoureteral reflux (VUR). MAG3 scan was performed in 8 (8,6%) patients, two of them had ureteropelvic junction obstruction, one of these patients had undergone pyeloplasty. After one year follow-up of 88 infants, 47 (53,4%) patients had normal, 23 (26.1%) patients had mild, 11 (12,5%) patients had moderate and 7 (8%) patients had severe hydronephrosis. Three cases (3,4%) progressed from moderate to severe disease. Conclusion: The most frequent cause of antenatal hydronephrosis among our patients was transient antenatal hydronephrosis. However follow-up of cases with a tendency to progress is important.

Outcomes of kidney transplants in pediatric patients with the vertebral defects, anal atresia, cardiac defects, tracheoesophageal fistula, renal anomalies, limb abnormalities association.

In this single-center retrospective study, we analyzed kidney transplant outcomes in nine pediatric patients with VACTERL [vertebral defects, anal atresia, cardiac defects, tracheoesophageal fistula, renal anomalies, limb abnormalities] association-making this the largest study of its kind. Of 743 pediatric kidney transplant recipients at our center (1980-2017), nine had documented diagnoses of VACTERL association. All nine had congenital anorectal malformations and renal anomalies, five had vertebral defects, and one had a bifid thumb and tracheoesophageal fistula. Renal anomalies included dysplasia (n=6), aplasia (n=3), and horseshoe kidney (n=2). Congenital lower urinary tract anomalies included neurogenic bladder (n=6), obstructive uropathy (n=4), anovesicular fistula (n=1), rectourethral fistula (n=1), and posterior urethral valves (n=1). Age at transplant ranged from 1.2 to 15years (mean, 7.3; standard deviation [SD], 5.5); 6 (67%) were male, and 3 (33%) were female; 6 (67%) had a living related donor, and 3 (33%) had a deceased donor. The overall graft survival rate was 78% (range, 1.5 to 25.2years; mean, 10.5; SD, 8.9). One month post-transplant, one recipient died with a functioning graft. At 3.7years post-transplant, one graft failed because of recurrent pyelonephritis. Post-transplant urologic complications included pyelonephritis (n=6), vesicoureteral reflux (n=5), and graft hydronephrosis (n=4). We conclude that pediatric patients with VACTERL association can be safely transplanted-careful patient selection with vigilance and intervention for pre- and post-transplant urologic complications is essential.