Unilateral Multicystic Dysplastic Kidney Research Articles

Deep learning prediction of renal anomalies for prenatal ultrasound diagnosis

Deep learning algorithms have demonstrated remarkable potential in clinical diagnostics, particularly in the field of medical imaging. In this study, we investigated the application of deep learning models in early detection of fetal kidney anomalies. To provide an enhanced interpretation of those models’ predictions, we proposed an adapted two-class representation and developed a multi-class model interpretation approach for problems with more than two labels and variable hierarchical grouping of labels. Additionally, we employed the explainable AI (XAI) visualization tools Grad-CAM and HiResCAM, to gain insights into model predictions and identify reasons for misclassifications. The study dataset consisted of 969 ultrasound images from unique patients; 646 control images and 323 cases of kidney anomalies, including 259 cases of unilateral urinary tract dilation and 64 cases of unilateral multicystic dysplastic kidney. The best performing model achieved a cross-validated area under the ROC curve of 91.28% ± 0.52%, with an overall accuracy of 84.03% ± 0.76%, sensitivity of 77.39% ± 1.99%, and specificity of 87.35% ± 1.28%. Our findings emphasize the potential of deep learning models in predicting kidney anomalies from limited prenatal ultrasound imagery. The proposed adaptations in model representation and interpretation represent a novel solution to multi-class prediction problems.

Early diagnosis of solitary functioning kidney: comparing the prognosis of kidney agenesis and multicystic dysplastic kidney.

Individuals with congenital solitary functioning kidney (SFK) are at an increased risk of kidney damage. According to some studies, the risk is higher in unilateral kidney agenesis (UKA) than in unilateral multicystic dysplastic kidney (UMCDK). We hypothesized that with early detection of children with UKA and UMCDK, there would be no difference in the presence of hypertension, proteinuria, and reduced glomerular filtration rate (GFR) between UKA and UMCDK. Based on a long-term follow-up protocol, we evaluated a cohort of 160 children followed from birth for SFK (84 with UKA and 76 with UMCDK) detected by prenatal or routine neonatal ultrasound screening. Hypertension, proteinuria, and reduced GFR were monitored as markers of kidney damage. We compared the characteristics and outcomes of the subgroups of children with UKA and UMCDK. GFR was reduced in 42 (26.2%) children, of whom 41 showed only mild reduction. Hypertension and proteinuria were found in 22 (13.8%) and 14 (8.8%) children, respectively. Combined kidney damage was present in 57 (35.6%) children. The UMCDK and UKA subgroups differed in GFR at final examination, with UMCDK patients being significantly more likely to have normal GFR compared to UKA patients (82% vs. 67%; p = 0.039). One third of the children showed signs of SFK damage, albeit mild. Patients with UKA had reduced GFR significantly more often than those with UMCDK, but did not differ in the rates of hyperfiltration injury or congenital anomalies of the kidneys and urinary tract (CAKUT) in SFK.

Unilateral Multicystic Dysplastic Kidney Management: A National Survey.

Risks of contralateral kidney abnormalities and chronic kidney disease necessitate follow-up for unilateral multicystic dysplastic kidneys (MCDK). A nationwide survey of senior UK pediatricians was conducted. Of the 60 responses obtained, 62% routinely perform a dimercaptosuccinic acid scan to confirm diagnosis. Eight percent routinely perform a cystogram to investigate contralateral vesicoureteric reflux. Sixty-two percent would routinely measure renal function (frequency ranging from once only to "every 2 years"). Twenty-five percent recalled MCDK nephrectomy being performed within the previous 5 years. Respondents voiced concerns that national guidance may result in an overcautious approach but could balance consensus and safe variation, and offer families choice and reassurance. The mean estimated cost of follow-up from birth to 18 years ranged from £258 to £3854. Results demonstrate significant variation in management, highlighting the need for a clear pathway to decrease unwanted variability and to ensure those at high risk of renal sequelae are recognized early, without undue investigatory burden.

Involution of unilateral multicystic dysplastic kidney: Long term data from a single centre

Unilateral multicystic dysplastic kidney (MCDK) is congenital anomaly which can be detected by ultrasound during antenatal period. The reported rate of MCDK ranges between 1 in 2,200 to 1 in 4,300 live births. This study was aimed to assess the determinants associated with the MCDK with follow-up until teens to evaluate the evolution of the disorder. This was an observational longitudinal study, involution degrees were observed following the baseline measurement of the size of the MCDK as documented on postnatal ultrasound, and long-term complications and renal function were also recorded. A total of 350 patients were identified, while 90 patients fulfilled the criteria of the study and completed the study duration of 10 years. Out of which 19 (21.1%) patients reported involution of MCDK size during follow-up as complete and partial, 7 (7.7%) within first year of age, 5 (5.5%) during first 2 years and 7 (7.7%) within 4 years of age. Large-sized MCDK at the time of diagnosis is less likely to involute through the first decade of life. However, conventional treatment is rational with the absence of complications.

Hypertension in children with unilateral multicystic dysplastic kidney: a common but rarely diagnosed condition

Hypertension in children with unilateral multicystic dysplastic kidney: a common but rarely diagnosed condition

Associated Anomalies and Complications of Multicystic Dysplastic Kidney.

Background: To assess multicystic dysplastic kidneys (MCDK) in children, their complications and associated congenital genitourinary anomalies. Methods: Children with unilateral MCDK, evaluated between 2012 and 2020, were analyzed. In this retrospective study, data were obtained from electronic and paper health care records. Results: There were 80 children included. Follow-up time was 8.0 +/− 5.2 years (mean +/− standard deviation). None of them had hypertension. In total, 43.8% of the children had associated congenital genitourinary anomalies, most commonly cryptorchidism and vesicoureteral reflux (VUR), and 6.3% of these children had chromosomopathy. All of them had normal kidney function except one child with dysplasia of the contralateral kidney. Urinalysis was normal in 90% of children. Extrarenal malformations occurred in 22.5% of them. We observed spontaneous involution of MCDK in 38.8% of children in the observed period. Nephrectomy was performed in 12.5% of children, at an average age of 2.0 years. Conclusions: Children with a unilateral MCDK have a very good prognosis if the contralateral kidney is normal. Associated congenital genitourinary anomalies are common. Cryptorchidism was found to be the most common associated anomaly among boys, which is unique for this study. Most of these children do not suffer from hypertension, kidney dysfunction or other complications.

Prenatal sonographic findings in a cohort of foetuses with a confirmed 22q11.2 microdeletion at a single Chinese Tertiary Centre

The aim of this study was to present prenatal ultrasound findings, molecular testing results and pregnancy outcomes of cases with 22q11.2 deletion (del22q11.2) diagnosed prenatally. A total of 76 foetuses were included. All cases were diagnosed by using chromosomal microarray analysis. Data on prenatal diagnosis, ultrasound findings, pregnancy outcomes and inheritance of del22q11.2 were reviewed. Congenital heart defects (CHDs) were the most common indications (47/76, 61.8%) for prenatal testing and were isolated in 52.6% (40/76). The constitution of CHDs comprised predominantly of conotruncal defects (61.7%; 29/47). Other cardiac anomalies were encountered in 38.3% (18/47) of cases. Extracardiac findings, including unilateral multicystic dysplastic kidney, clubfoot, increased nuchal translucency, intrauterine growth retardation and polyhydramnios, were found in 31.6% (24/76) of cases, and were combined with CHDs in 7 cases. Twelve cases had normal sonographic scans at the time of prenatal diagnosis. Foetal CHDs, especially conotruncal defects, are the most predictive association with del22q11.2. The information about del22q11.2 should also be part of the contents in comprehensive pre-test counselling even for those who are referred for diagnostic testing with foetal extracardiac findings. Impact statement What is already known on this subject? 22q11.2 deletion (del22q11.2) is the most common microdeletion syndrome in humans. At present, the main indications for prenatal testing for del22q11.2 are pregnancies of abnormal sonographic findings, especially foetal congenital heart defects. What do the results of this study add? Many extracardiac malformations, including some lethal or mildly non-specific ones, could be associated with foetal del22q11.2. There were also del22q11.2 foetuses had normal sonographic scans at the time of prenatal diagnosis. What are the implications of these findings for clinical practice and/or further research? The information about del22q11.2 should also be part of the contents in comprehensive pre-test counselling even for those who are referred for diagnostic testing with indications other than foetal cardiac anomalies.

Utility of renal scintigraphy in diagnosis of multicystic dysplastic kidney.

To compare the prevalence of vesicoureteral reflux (VUR), febrile urinary tract infection (fUTI), and chronic kidney disease (CKD) among patients with multicystic dysplastic kidney (MCDK) diagnosed by renal scintigraphy (RS) versus follow-up renal ultrasound (RUS) alone. This was a retrospective review of patients seen at a tertiary care center from 2010 to 2020 with MCDK diagnosed by RS or follow-up RUS. Differences in the prevalence of VUR, fUTI, and CKD by cohort were assessed using logistic regression analysis, Pearson X2 , and Fisher's Exact tests. Temporal trends in diagnostic methods used (RUS versus RUS + RS) were evaluated using the Cochran-Armitage trend test. One-hundred seventy-two patients were included: 50% (n=86) underwent RUS + RS and 50% (n=86) underwent RUS alone to diagnose MCDK. Prevalence of VUR, fUTI, and CKD did not significantly vary between groups. Among patients who had a VCUG, 4.4% had contralateral VUR (1.7% RUS + RS group; 7.4% RUS group; p=0.19) and 14.5% had at least one fUTI (16.3% RUS + RS group; 12.8% RUS group; p=0.52). Females were significantly more likely to have at least one fUTI (p=0.04). Four patients (2.3%) developed CKD, all in the RUS + RS cohort (p=0.12). Diagnosis of MCDK by RUS versus RUS + RS did not significantly vary over time (p=0.17). Patients with unilateral MCDK confirmed by RS versus RUS alone do not significantly vary in the prevalence of VUR, fUTI, or CKD. Renal scintigraphy studies may not be necessary in unilateral MCDK diagnosis but continue to be used.

Presence of Cervical Vertebral Anomalies with Concomitant Non-Communicating Hydrocephalus and Multicystic Kidney in a Female Fetus: Where VACTERL-H Meets MURCS

Background Congenital multisystemic lesions with co-occurrence of non-random malformations, such as VACTERL-H or MURCS association, often pose serious threads to the newborn and still constitute an antenatal diagnostic dilemma. Case report A malformed fetus with VACTERL-H association at 20 gestational weeks had a skin-covered neural tube defect (NTD) of the lower cervical spine, concomitant hydrocephalus, as well as unilateral multicystic dysplastic kidney and the suspicion of mullerian duct anomaly as potentially assigned to MURCS association. Discussion/Conclusion We were able to demonstrate how well-defined, standardized volumetric reconstruction of diagnostic views displaying fetal pathology in utero might aid early and precise diagnosis of multi-organ malformations. Application of modern diagnostic imaging tools is helpful in delineation of the most likely diagnoses (VACTERL-H vs. MURCS) as further specified during detailed pathologic work-up and might consequently facilitate individually tailored interdisciplinary counseling, as in the case presented here.

Ovarian cysts

Ovarian cysts

Reducing Unnecessary Imaging in Children With Multicystic Dysplastic Kidney or Solitary Kidney.

Children with isolated unilateral multicystic dysplastic kidney (MCDK) or congenital solitary kidney (CSK) undergo serial renal ultrasonography with variable frequency until they are transitioned to adult care. A growing body of literature suggests the value of frequent ultrasonography in this population is limited, providing no benefit to overall outcomes. Despite emerging evidence, ultrasound remains overused, resulting in avoidable health care expenditures and unnecessary use of resources. With our initiative, we aimed to improve quality of care by reducing avoidable ultrasounds in these children. This was a single-center, prospective, interrupted time series of children <18 years with ultrasound-confirmed isolated unilateral MCDK or CSK in the outpatient nephrology clinic to evaluate the effect of a decision-making algorithm on the proportion of children receiving an avoidable ultrasound. An algorithm depicting a consensus, evidence-based protocol for managing pediatric MCDK or CSK was refined through content expert feedback and usability testing to standardize frequency of ultrasonography. Ultrasounds were deemed necessary after birth, at 6 months, and at 2, 5, 10, and 15 years. Differences pre- and postintervention were determined by using a U chart and t and F tests for significance. The algorithm resulted in a 47% reduction (P < .001) in the proportion of avoidable ultrasounds ordered in children with MCDK and CSK. This reduction was sustainable over a 6-month period and would result in at least $46 000 annual savings. Introduction of a clinical decision-making algorithm was associated with a reduction in avoidable ultrasound testing. Improving adherence across providers may allow for an even more pronounced reduction.

Retrospective evaluation of the pediatric multicystic dysplastic kidney patients: experience of two centers from southeastern Turkey

Background/aimThe objective of this study is to determine the clinical features of unilateral multicystic dysplastic kidney (MCDK) patients.Materials and methods The demographic, clinical, laboratory, and radiologic features of MCDK patients at Diyarbakır Children’s Hospital and Diyarbakır Gazi Yaşargil Training and Research Hospital between January 2008-June 2019 were retrospectively evaluated. Results A total of 111 [59 (53.2%) male and 52(46.8%) female] patients with MCDK were followed for a mean period of 41.89 ± 32.03 months. MCDK was located on the left and right sides in 46 (41.4%) and 65 (58.6%) of the children, respectively (p > 0.05). A total of 87 (78.4%) patients had antenatal diagnosis. The mean age at diagnosis was 13.7 ± 34.2 months. Of the 49 voiding cystourethrogram (VCUG)-performed patients, vesicoureteral reflux was detected in 11 patients (22.4%). Other associated urological anomalies in the patients were detected in 12 (10.8%) patients. On Tc-99m dimercaptosuccinic acid (DMSA) scintigraphy which was performed in all patients showed scarring in four children. Eight patients had history of UTI (7.2%). Renal failure, hypertension, and proteinuria were diagnosed in three children (2.7%). Sixty-nine (62%) patients developed compensatory hypertrophy. Conclusion All cases should be followed up closely and VCUG should be reserved for patients with recurrent UTI and other urological problems indicated by ultrasonography and abnormal DMSA scan results.

Predictors of poor neonatal outcomes in fetuses diagnosed with congenital urinary tract anomalie.

Urinary tract anomalies account for approximately one-quarter of all antenatally detected anomalies. The aim of this study was to identify factors associated with severe adverse neonatal outcomes of a prenatally diagnosed urinary tract anomaly. A retrospective-prospective study included 101 pregnant women with prenatally diagnosed fetal urinary tract anomalies presented to the Council for Fetal Anomalies. Prenatal diagnoses were compared with autopsy findings in cases of terminated pregnancy or with clinical and operative findings of the infants. The mortality rate in the group of patients with fetal obstructive uropathy (60 patients) was 10% and in the group of patients with fetal multicystic dysplastic kidney (38 patients) 15.7%. Surgery was performed on 53.4% of the children, whereas more than half of the operations involved resolving associated urinary tract anomalies. Postoperative renal function deterioration occurred in 19% of the children. The prognosis of renal function in obstructive uropathies is excellent if oligoamnios does not develop prenatally and in case of timely provided surgical care is provided postnatally. The finding of the bilateral multicystic dysplastic kidney is associated with poor prognosis. The prognosis in fetal unilateral multicystic dysplastic kidney depends primarily on the condition of the contralateral kidney and the existence of associated anomalies.

Prenatal diagnosis and outcome of unilateral multicystic kidney

We reviewed the records of 144 patients. The mean gestational age at first US diagnosis was 27.5 ± 4.3 weeks. An anomaly of the contralateral kidney was detected in 25% of cases. An extrarenal anomaly was detected in 13.8%. Karyotype analysis was performed in 16.6% of cases and revealed trisomy 18 in 2 cases with extrarenal defects. Karyotype analysis was normal in all the patients who had isolated multicystic dysplastic kidney (MCDK). The diagnostic accuracy of prenatal ultrasound was 92.2%. Contralateral kidney anomaly was detected 33.9% of patients, and half of these were vesicoureteral reflux. Antihypertensive therapy was required in 2.6% of cases. Nephrectomy was performed in 8%, and partial or total involution of MCDK was achieved in 33.9% of patients. MCDK can be accurately diagnosed by prenatal sonography, and prognosis depends on extrarenal and contralateral renal abnormalities. In isolated cases, require of surgery is rare, and serial follow-up is suggested to determine involution. Impact statement What is already known on this subject? Multicystic dysplastic kidney (MCDK) is one of the most renal anomalies and is associated with numerous renal and extrarenal abnormalities. It can lead to severe consequences in the neonatal period. What do the results of this study add? The accuracy of prenatal ultrasonography is excellent for detecting MCDK. In isolated unilateral cases, chromosomal aberrations are low, and the majority of them involute spontaneously. A periodic follow-up of the contralateral kidney is mandatory due to an increased risk of an anomaly. Genital anomaly risk is increased in males. What are the implications of these findings for clinical practice and/or further research? Detailed evaluation and follow-up of the contralateral kidney are crucial for counselling in isolated cases. Karyotype analysis in isolated unilateral MCDK is debateable. Postnatal prognosis is encountering, and the majority of patients have no requirement of surgery.

Nierenzysten und zystische Nierenerkrankungen bei Kindern (AWMF S2k-Leitlinie)

This consensus-based guideline was developed by all relevant German pediatric medical societies. Ultrasound is the standard imaging modality for pre- and postnatal kidney cysts and should also exclude extrarenal manifestations in the abdomen and internal genital organs. MRI has selected indications. Suspicion of a cystic kidney disease should prompt consultation of a pediatric nephrologist. Prenatal management must be tailored to very different degrees of disease severity. After renal oligohydramnios, we recommend delivery in a perinatal center. Neonates should not be denied renal replacement therapy solely because of their age. Children with unilateral multicystic dysplastic kidney do not require routine further imaging or nephrectomy, but long-term nephrology follow-up (as do children with uni- or bilateral kidney hypo-/dysplasia with cysts). ARPKD (autosomal recessive polycystic kidney disease), nephronophthisis, Bardet-Biedl syndrome and HNF1B mutations cause relevant extrarenal disease and genetic testing is advisable. Children with tuberous sclerosis complex, tumor predisposition (e. g. von Hippel Lindau syndrome) or high risk of acquired kidney cysts should have regular ultrasounds. Even asymptomatic children of parents with ADPKD (autosomal dominant PKD) should be monitored for hypertension and proteinuria. Presymptomatic diagnostic ultrasound or genetic examination for ADPKD in minors should only be done after thorough counselling. Simple cysts are very rare in children and ADPKD in a parent should be excluded. Complex renal cysts require further investigation.

P1813CLINICAL CHARACTERIZATION OF CONGENITAL SOLITARY FUNCTIONING KIDNEY IN CHILDREN

Abstract Background and Aims Congenital anomalies of the kidney and urinary tract (CAKUT) are the major cause of chronic kidney disease and end-stage kidney disease in childhood. Solitary functioning kidney (SFK) is part of the spectrum of CAKUT. Congenital SFK is mainly due to unilateral renal agenesis (URA) and multi-cystic dysplastic kidney (MCDK). With the implementation of routine fetal ultrasound screening, SFK is increasingly recognized before birth, significantly raising the number of patients referred to paediatric nephrology units for clinical monitoring during childhood. Understanding the pathophysiology of SFK is pivotal for guiding the clinical management and informing long-term outcome. However, observational studies performed in children with SFK are controversial, especially about the need, methodology and timing of functional assessment. This may be at least in part due to the fact that different models of SFK, including congenital and acquired after unilateral nephrectomy, are often grouped together. The aim of this study was to assess the clinical, laboratory and functional features of congenital SFK caused by URA and MCDK in children, with a particular focus on the role of renal scintigraphy in estimating kidney function during childhood and adolescence. Method We retrospectively collected clinical, laboratory and instrumental records of all consecutive pediatric patients (aged 0-18 years) affected by congenital SFK caused by URA or MCDK referred to the Nephrology and Dialysis Unit of Meyer Children’s Hospital of Florence (Italy) from 1992 to 2019. Patients with unilateral kidney hypodysplasia were excluded. In particular, we reviewed data from ultrasound scanning and sequential renal scintigraphy over time. URA and MCDK were compared for clinical features, long-term course and outcome. Results A total of 155 patients with congenital SFK were included in the study and divided in two groups according to the cause of SFK (URA, n=100; MCDK, n=55). The median length of follow-up was 47 and 45 months, respectively. Male sex and ethnicity were equally distributed in the two groups. Prenatal diagnosis was more frequent in MCDK group. We did not observe either preterm birth or low birth weight in patients enrolled. Overall, the clinical features were not statistically different between the two groups. In particular, SFK associated CAKUT, including vesicoureteral reflux, occurred at a comparable frequency. Also, measurement of kidney length by ultrasound scanning, which is often considered suggestive of compensatory hypertrophy, did not differ between groups. Although renal clearance from sequential renal scintigraphy appeared not statistically different between URA and MCDK, the latter seems to reach complete functional adaptation more rapidly and earlier in the first two years of life. Conclusion The clinical course and long-term outcome of SFK has been a topic of extensive debate. Due to poor-quality of data (unclear inclusion/exclusion criteria, lack of uniformity in data collection and outcome definition), generalization of findings from observational studies to all patients with SFK could be inappropriate. Congenital SFK could represent the most unbiased group to analyze and this study provides a thorough clinical characterization of a large and strictly selected cohort. Insights from sequential renal scintigraphy suggest a different trend in reaching single kidney complete functional adaptation in URA and MCDK. These results could potentially reveal significant differences in the pathophysiologic mechanisms of reaching compensatory hypertrophy and functional adaptation by the solitary kidney in the two models. Whether confirmed in larger cohorts, these findings could provide important implications for follow-up planning, informing the need, methodology and timing for function assessment, tailoring the clinical management and understanding long-term prognosis.

Voiding Cystourethrogram in Children With Unilateral Multicystic Dysplastic Kidney: Is It Still necessary?

To evaluate the value of the voiding cystourethrogram (VCUG) in children with multicystic dysplastic kidney (MCDK) who have a normal versus abnormal contralateral kidney and bladder ultrasound (US), and assess the risk of having vesicoureteral reflux (VUR) or urinary tract infection (UTI) based on the US results. A retrospective chart review including children with unilateral MCDK with postnatal US and VCUG available at our institution between January 2008 and September 2017 was performed. Analysis was done to find association between abnormal contralateral US and contralateral VUR and UTI. One hundred and fifty-six children were analyzed; 118(75.6%) patients had a normal contralateral kidney US, while 38(24.4%) had abnormal US. The rate of severe contralateral VUR (grade IV and V) was 2 (1.7%) and 5 (13.2%) in children with normal and abnormal contralateral US, respectively. The risk analysis demonstrated a significant association between severe VUR on the contralateral kidney and an abnormal contralateral US (odds ratio=7.73; 95%CI: 1.43-41.81; P=0.018) and no significant association with UTI (odds ratio=1.58; 95%CI: 0.50-4.94; P=0.435). Our data suggests, the rate of severe contralateral VUR in children with unilateral MCDK and normal contralateral kidney is low. VCUG should be considered for infants with proven MCKD and alterations on the contralateral kidney on US. Following patients with MCDK and normal contralateral kidney without the use of VCUG is a reasonable approach, unless there is development of signs and symptoms of recurrent UTI or deterioration of the renal function. We found that abnormal contralateral kidney US was associated with severe VUR.

Multicystic dysplastic kidney – treat each case on its merits

ObjectivesTo assess outcomes of unilateral multicystic dysplastic kidney (MCDK) managed at an Australasian centre over a 15 year period. To assess if MCDK involution could be predicted based on change noted between first two postnatal ultrasound scans 6 months apart. Subjects and MethodsA retrospective study was performed. ResultsOne-hundred-and-six cases of unilateral MCDK were studied. Eighty-four of these presented antenatally. Twenty-two MCDK cases presented postnatally.Urological anomalies associated with MCDK included vesicoureteric reflux (VUR), ureterocele and contralateral pelviureteric junction obstruction (PUJO). Children undergoing surgical intervention for these anomalies were offered concurrent MCDK nephrectomy. Morbidity associated with MCDK under surveillance included febrile culture-positive urinary tract infection in 20 cases (20.7%), hypertension in four (3.7%) and Wilms' tumor in one (0.9%). Thirty-six cases (34%) underwent complete involution, 32 (30.2%) were in the process of involuting and 38 cases (35.8%) underwent nephrectomy because of failure of involution or associated morbidity.If the MCDK reduced in cranio-caudal interpolar length by 20% or more between the first postnatal USS and the next one 6 months later, then it was very likely to involute spontaneously. If the MCDK did not reduce in cranio-caudal interpolar length by 20% between the first postnatal scan and the next one 6 months later, then it was highly likely to fail to involute, and in our study, correlated with the outcome of nephrectomy. ConclusionAlthough MCDK is a benign condition, it should be carefully investigated and followed-up, as involution may not occur in over a third. In some cases, morbidity may occur. Each case of MCDK should be managed on its own merits. Level of EvidenceLevel II – Prognosis study, Retrospective.

Urine hepcidin, netrin-1, neutrophil gelatinase-associated lipocalin and C-C motif chemokine ligand 2 levels in multicystic dysplastic kidney.

ABSTRACTIntroduction:Glomerular hyperfiltration may lead to proteinuria and chronic kidney disease in unilateral multicystic dysplastic kidney (MCDK). We aimed to investigate the urine neutrophil-gelatinase-associated lipocalin (NGAL), netrin-1, hepcidin, and C-C motif chemokine ligand-2 (MCP-1/CCL-2) levels in patients with MCDK.Methods:Thirty-two patients and 25 controls were included. The urine hepcidin, netrin-1, NGAL, and MCP-1/CCL-2 levels were determined by ELISA.Results:The patients had higher serum creatinine (Cr) levels, urine albumin, and netrin-1/ Cr ratio with lower GFR. There were positive correlations between urine protein/Cr, MCP-1/CCL-2/Cr, and netrin-1 with NGAL (r = 0.397, p = 0.031; r = 0.437, p = 0.041, r = 0.323, p = 0.042, respectively). Urine netrin-1/Cr was positively correlated with MCP-1/ CCL-2/Cr (r = 0.356, p = 0.045). There were positive associations between the presence of proteinuria and netrin-1/ Cr, MCP-1/CCL-2/Cr, and NGAL/Cr [Odds ratio (OR): 1.423, p = 0.037, OR: 1.553, p = 0.033, OR: 2.112, p = 0.027, respectively)]. ROC curve analysis showed that netrin-1/Cr, MCP-1/CCL-2/Cr, and NGAL/Cr had high predictive values for determining proteinuria p = 0.027, p = 0.041, p = 0.035, respectively). Urine hepcidin/ Cr was negatively correlated with tubular phosphorus reabsorption and was positively correlated with urine NGAL/Cr (r = -0.418, p = 0.019; r = 0.682, p = 0.000; respectively).Conclusions:MCP-1/CCL-2 may play a role in the development of proteinuria in MCDK. Netrin-1 may be a protective factor against proteinuria-induced renal injury. Urine hepcidin/Cr may reflect proximal tubule damage in MCDK. Urine NGAL/Cr may be a predictor of tubule damage by proteinuria.

Diagnosis, management, and outcome of obstructed hemivagina and ipsilateral renal agenesis (OHVIRA syndrome): Is there a correlation between MRI findings and outcome?

AimTo review the experience of a single tertiary center with obstructed hemivagina and ipsilateral renal agenesis (OHVIRA syndrome) and evaluate if MRI findings correlate with outcome. MethodsPatients (n = 32) diagnosed with OHVIRA syndrome between 2001 and 2019 were analyzed. Presenting symptoms, age of menarche, age at operation, MRI findings, management, and outcome were reviewed. In sagittal MRI planes, distance from hematocolpos to perineum was measured. Measurements were compared among patients who underwent single-stage vaginoplasty and hemihysterectomy. ResultsMean age at diagnosis and mean age of menarche was 16.8 ± 6.4 (10−33) and 12.8 ± 1.0 (10–15). Main presenting complaints were abdominal pain/dysmenorrhea. Eighteen anomalies (56.3%) were on right side. Twenty-eight had ipsilateral renal agenesis, 3 patients had normal renal anatomy and one had unilateral multicystic dysplastic kidney. In MRI, 21 patients had hematocolpos, 11 patients had both hematocolpos and hematometra. 28 patients underwent single-stage vaginoplasty and vaginal septum resection. One had hemihysterectomy due to sepsis at presentation. Three patients had hemihysterectomy due to proximal vaginal septum and impossibility of vaginoplasty. During follow-up, 7 cases (21.9%) had married and 5 of these (71.4%) were pregnant or had delivered. Five patients had reoperation during follow-up. MRI images of 19 patients indicated mean distances from hematocolpos to perineum whom underwent vaginoplasty or hemihysterectomy were 33.9 ± 18.1 mm (10–79 mm) and 87.3 ± 11.0 mm (80–100), respectively (p = .009). ConclusionsGold standard treatment of OHVIRA syndrome is single-stage vaginoplasty. Distance from hematocolpos to perineum in MRI may correlate with surgical outcome. Hemihysterectomy may be an alternative for extreme proximal vaginal septum or infectious complications.