Utility of renal scintigraphy in diagnosis of multicystic dysplastic kidney.

PURPOSE: 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. METHODS: 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. RESULTS: 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). CONCLUSION: 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.

Outcomes of solitary functioning kidneys-renal agenesis is different than multicystic dysplastic kidney disease.

BACKGROUND: Multicystic dysplastic kidney (MCDK) disease and unilateral renal agenesis (URA) are well-known causes of a solitary functioning kidney (SFK) and are associated with long-term kidney injury. The aims of this study were to characterize the natural history of SFK at our center, define the risk factors associated with chronic kidney injury, and identify distinguishing features between URA and MCDK that predict outcome. METHODS: This was a retrospective cohort study of 230 SFK patients. We compared MCDK (n=160) and URA (n=70) according to clinical features at diagnosis and kidney outcomes over follow-up. Univariate and multivariate binary regression analysis was used to determine independent risk factors for chronic kidney injury, defined as the composite outcome of hypertension, proteinuria, or chronic kidney disease (eGFR <60 mL/min/1.73m2). RESULTS: URA had a higher prevalence of comorbid genetic syndromes (15 vs. 6%, p=0.04), non-renal anomalies (39 vs. 11%, p<0.001), and congenital anomalies of the kidney and urinary tract (CAKUT) (51 vs. 26%, p<0.001) than MCDK. Over follow-up, URA experienced more hypertension (19 vs. 3%, p=0.002), proteinuria (12 vs. 3%, p=0.03), and the composite outcome (19 vs. 6%, p=0.003) than MCDK. Independent risk factors for chronic kidney injury included CAKUT (OR 5.01, p=0.002) and URA (OR 2.71, p=0.04). CONCLUSIONS: In our population, URA was more likely to have associated syndromes or anomalies, and to have worse outcomes over time than MCDK. URA diagnosis was an independent risk factor for chronic kidney injury. Our results will be used to develop a standardized clinical pathway for SFK management. A higher resolution version of the Graphical abstract is available as Supplementary information.

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

Background/aim: The 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 Diyarbakir Children's Hospital and Diyarbakir Gazi Yasargil 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.

Antenatally detected ureterocele: Associated anomalies and postnatal prognosis.

OBJECTIVE: We purposed to review prenatal diagnoses of ureterocele, to determine the sonographic findings and additional abnormalities, and to illustrate the pregnancy outcomes of these patients. MATERIAL AND METHODS: We reviewed the records of 24 patients with the diagnosis of ureterocele in our referral center between January 2010-March 2017. Prenatal sonographic findings, antenatal course, and postnatal follow-up were obtained. RESULTS: The mean gestational age at first US diagnosis was 24.5 ± 2.9 weeks. 13 (54.1%) of fetuses were female, and 11 (45.9%) were male. Ureterocele was associated with the duplex kidney in 17 (70.8%), MCDK in 5 (20.8%) and hydronephrosis with a single system in 1 (4.2%) and pelvic kidney in 1 (4.2%) fetuses. Postnatal follow-up was achieved in 22 of 24 (91.6%) cases, and mean follow-up interval was 56 ± 14.2. Months. The diagnosis of ureterocele was confirmed in 22 (91.6%) cases postnatally. 15 of 22 (68%) cases were classified as extravesical ureterocele, and 7 (32%) cases were intravesical ureterocele. Postnatal confirmation of duplex kidney achieved in 16 of 17 (94.1%) patients. 17 (77.2%) patients were required surgical intervention, and 5 (22.8%) cases were managed conservatively. 15 of 16 (93.7%) cases who were diagnosed duplex kidney underwent surgery however 2 of 5 (40%) cases which were confirmed MCDK required an operation. Cystoscopic ureterocele incision was the initial approach for the surgical management and performed all of the cases which required surgery. It was curative in 10 of 17 (58.8%) patients and 7 (41.2%) cases needed to further operations. Ureteroselectomy and common-sheath ureteroneocystostomy was performed in 5 (29.1%) cases and. 2 (%11.7%) cases underwent partial nephrectomy. CONCLUSION: Ureterocele can be accurately diagnosed by prenatal sonography, and it is a significant clue for the diagnosis of a duplex kidney. Postnatal prognosis depends on associated anomaly and presence of reflux and upper pole function.

Are there any predictors of pyonephrosis in patients with renal calculus disease?

The objective of the study is to identify factors predicting development of pyonephrosis in patients of renal calculus disease (RCD), as this knowledge is largely unknown. Patients of RCD without pyonephrosis (Group 1) or with pyonephrosis (Group 2) presenting between December 2013 and November 2015 were evaluated. All patients of RCD who had undergone either percutaneous nephrostomy (PCN) or surgical management (percutaneous nephrolithotomy/pyelolithotomy/nephrectomy) were included. Patients treated conservatively, by extracorporeal shock-wave lithotripsy and patients of bilateral RCD were excluded. Data regarding demography, co-morbidities, associated urologic disease, previous intervention, clinical presentation, urinary culture, renal function, grade of hydronephrosis, stone characteristics were collected. 501 patients were included (Group 1: 410; Group 2: 91). Mean age in years (35.02 versus 35.48), sex ratio (2.12:1 versus 2.25:1) and mean body mass index (kg/m2) (22.27 versus 22.15) were similar in both groups. Prevalence of diabetes mellitus (3.41% versus 3.29%, p = 1.000) was similar. Group 2 patients had longer duration of symptoms (5.77 versus 8.96 months, p < 0.0001), associated urological diseases such as ipsilateral PUJO and ureteric calculus (4.63% versus 12.08%, p = 0.0125), moderate/severe-grade hydronephrosis (49.75% versus 92.30%, p < 0.0001), presence of staghorn calculus (20.73% versus 62.63%, p < 0.0001), multiple calculi (48.29% versus 68.13% p = 0.0007) and nonfunctioning kidney (1.70% versus 71.42%, p < 0.0001) as predictors of pyonephrosis. In logistic multivariate analysis, additionally, past history of urological surgery (p = 0.044) was found associated with pyonephrosis. Our study identified some conditions associated with patients of pyonephrosis. To prove their role as risk factors we recommend further studies.

Update on Multicystic Dysplastic Kidney.

Multicystic dysplastic kidney (MCDK) is the most common cause of cystic disease in children. It is characterized by multiple non-communicating cysts of varying sizes with no identifiable normal renal parenchyma. The incidence ranges from 1 in 1000 to 4300 live births, and it is one of the most commonly detected anomalies on prenatal ultrasound. MCDK has been shown to follow a benign course with relatively few sequelae and therefore should be managed conservatively. Currently, the key clinical questions revolve around the detection of anomalies in the contralateral kidney and follow-up imaging. The recent literature suggests that very limited radiographic evaluation of the MCDK is needed. The use of voiding cystourethrogram or nuclear medicine renal scans should be directed by any abnormalities on renal ultrasound or the development of urinary tract infections.

Outcomes and management strategies in pregnancies with early onset oligohydramnios.

OBJECTIVE: To evaluate the outcomes and management options in pregnancies with early onset oligohydramnios. MATERIALS AND METHODS: The file datas of all pregnancies diagnosed as oligohydramnios or anhydramnios before 27 gestational weeks between January 2006 and September 2013 were evaluated retrospectively. The underlying pathology and associated anomalies, karyotype analysis, the outcome of the pregnancy (either termination or labour), and gestational week at the time of diagnosis were analyzed. RESULTS: A total of 54 pregnancies were evaluated; mean gestational week at the time of the diagnosis was 19.8 ± 4.6. Mean maternal age was 27.28 ± 6.03. Thirty-seven pregnancies were anhydramniotic, 13 fetuses had associated anomalies, five of them had multicyctic dysplastic kidney, five had bilateral renal agenesis, one had hypoplastic right heart syndrome, one had clubfoot, and one had ventricular septal defect and cleft palate. Karyotyping was normal regarding the fetuses with structural anomalies. Nineteen patients had premature preterm rupture of membranes and 39 patients had termination of pregnancy. CONCLUSION: The prognosis of early onset oligohydramnios is poor. Main determinant is gestational week at the time of the diagnosis.

Multicystic dysplastic kidney: a retrospective study.

OBJECTIVE: To report the renal structural and functional anomalies in children with multicystic dysplastic kidneys. METHODS: Retrospective descriptive analysis of 47 children with multicystic dysplastic kidney seen in a pediatric nephrology unit over a period of 6 years. RESULTS: Antenatal diagnosis of multicystic dysplastic kidney was made in 34 (72.3%) patients. On follow up of 31 children for more than 12 months, 21 (68%) had involution, 4 [13%] had non-regression, and 4 (13%) were nephrectomized. Vesico-ureteric reflux (n=13; 28%) was the commonest renal abnormality. The serum creatinine values were higher (P=0.006) in children with contralateral reflux. Sub-nephrotic proteinuria was noted in 9 (29%) and was significantly associated with complete involution (P=<0.023). None of the patients developed hypertension and 2 (6.4%) had renal failure. CONCLUSIONS: Close nephrological follow-up is needed in children with multicystic dysplasia of kidneys.

Prenatal diagnosis and epidemiology of multicystic kidney dysplasia in Europe.

OBJECTIVES: The aim of this study is to describe the prenatal diagnosis and epidemiology of multicystic kidney dysplasia (MCKD). METHODS: The study is based on routinely collected data from a European database of major congenital anomalies including 13 registries with cases born in 1997-2006 and covering 1 458 552 births. RESULTS: There were 601 MCKD cases giving an overall prevalence of 4.12 per 10 000 births with regional variation. In live births, 87% of cases had an isolated renal anomaly and 13% had associated major nonrenal anomalies (chromosomal, syndrome or other major anomalies). For the cases with isolated renal anomalies, 51/386 (11%) and 7/386 (2%) choose to terminate the pregnancy or resulted in an intrauterine fetal death, respectively. The prenatal detection rate was 88% in both unilateral and bilateral cases. Birth outcome differed with 92% of unilateral MCKD cases being liveborn compared with 33% of bilateral MCKD cases. For unilateral MCKD cases, 84% had an isolated renal anomaly compared with 51% of bilateral MCKD cases (p < 0.001). CONCLUSIONS: Cases with unilateral MCKD are mainly liveborn, and only 16% have associated major malformations or a syndrome. Cases with bilateral MCKD are often associated with nonrenal major congenital anomalies or part of a syndrome, and only one third of bilateral MCKD cases in this study were liveborn. Prenatal detection rate of MCKD was high for both unilateral and bilateral cases. © 2014 John Wiley & Sons, Ltd.

Bilateral pelvi-ureteric junction obstruction: our experience in a developing country.

BACKGROUND: Bilateral pelvi-ureteric junction (PUJ) obstruction is rare and causes high morbidity and mortality. Recent advances have led to its diagnosis and management in the perinatal period. However, open surgery is still the mainstay of treatment in less endowed nations where late presentation is the norm. MATERIALS AND METHODS: All patients with PUJ obstruction diagnosed and managed at the University of Maiduguri Teaching Hospital between January 2006 and December 2011 were retrospectively reviewed. All had open surgery and all repairs were stented with double J stents or appropriate size feeding tube. RESULTS: A total of 18 patients were analyzed, 11 males and 7 females with a ratio of 1.57:1 and mean age of 27.5 years (age range 2-38). The main clinical features were loin pain (72.22%) and fever (72.22%); while the main complications at presentation were hydronephrosis (55.56%), impaired renal function (61.11%) and pyelonephritis (50%). The causes of obstruction were mainly congenital (50.0%) and due to schistosomal fibrosis/stricture (22.2%). Preliminary double J stents, tube nephrostomies and hemodialysis were used to recover renal function before surgery. Anderson-Hynes (41.7%) and Heineke-Mirhulicz (19.4%) pyeloplasty were the main procedures performed. The main post-operative complications were urinary tract infections (67.67%) and leakage (11.11%). The mean duration of symptoms was 23.72 months and the mean hospital stay was 13 days. The mortality rate was 5.56%. CONCLUSION: Bilateral PUJ obstruction is uncommon in Maiduguri, with congenital causes and schistosomal fibrosis as the most common etiologies. Aggressive treatment aimed at recovering renal function is necessary before open pyeloplasty if morbidity and mortality is to be reduced. Open pyeloplasty remained the best treatment option with favorable outcome.

Study of the association between the BMP4 gene and congenital anomalies of the kidney and urinary tract / Estudo da associação entre o gene BMP-4 e anomalias congênitas do rim e trato urinário

OBJECTIVE: To determine the frequency of different phenotypes for congenital anomalies of the kidney and urinary tract (CAKUT) in a Brazilian sample, and to evaluate the association between the CAKUT phenotypes and the BMP4 gene. METHODS: In this study, 457 Brazilian individuals were analyzed in an attempt to establish the association between the BMP4 gene and the CAKUT diagnosis. A case-control sample was genotyped for three BMP4 gene polymorphisms. RESULTS: Association data was established with CAKUT sample as a whole and with the three most important CAKUT phenotypes: multicystic dysplastic kidney disease (MDK), ureteropelvic junction obstruction (UPJO) and vesicoureteral reflux (VUR). When the sample was segregated in these three phenotypes, associations between the BMP4 gene were observed with UPJO and with MDK. Conversely, VUR was not associated to the polymorphisms of the BMP4 gene. CONCLUSIONS: The present data suggest that Brazilian individuals with polymorphisms of the BMP4 gene have a higher risk to develop CAKUT, especially the malformations related to nephrogenesis and initial branching such as MDK and UPJO. Conversely, VUR appeared not to be related to BMP4 gene. .

OBJETIVO: Determinar a frequência de diferentes fenótipos de anomalias congênitas do rim e trato urinário (CAKUT) em uma amostra brasileira e avaliar a associação entre os CAKUT e o gene BMP-4. MÉTODOS: Neste estudo, analisamos 457 indivíduos brasileiros em uma tentativa de estabelecera associação entre o gene BMP-4 e o diagnóstico de CAKUT. As amostras de caso e de controle foram genotipadas em busca de três polimorfismos do gene BMP-4. RESULTADOS: Os dados de associação foram estabelecidos com a amostra de CAKUT como um todo e com os três fenótipos de CAKUT mais importantes: rim displásico multicístico (RDM), obstrução da junção ureteropélvica (UPJO) e refluxo vesico-ureteral (VUR). Quando a amostra foi separada nesses três fenótipos, encontramos associações entre o gene BMP-4 com UPJO e com RDM. Por outro lado, o VUR não foi associado aos polimorfismos do gene BMP-4. CONCLUSÕES: Esses dados sugerem que os indivíduos brasileiros com polimorfismos do gene BMP-4 apresentam maior risco de desenvolver CAKUT, principalmente as malformações relacionadas a nefrogênese e ramificação inicial, como RDM e UPJO. Por outro lado, o VUR parece não estar relacionado ao gene BMP-4. .

Study of the association between the BMP4 gene and congenital anomalies of the kidney and urinary tract.

OBJECTIVE: To determine the frequency of different phenotypes for congenital anomalies of the kidney and urinary tract (CAKUT) in a Brazilian sample, and to evaluate the association between the CAKUT phenotypes and the BMP4 gene. METHODS: In this study, 457 Brazilian individuals were analyzed in an attempt to establish the association between the BMP4 gene and the CAKUT diagnosis. A case-control sample was genotyped for three BMP4 gene polymorphisms. RESULTS: Association data was established with CAKUT sample as a whole and with the three most important CAKUT phenotypes: multicystic dysplastic kidney disease (MDK), ureteropelvic junction obstruction (UPJO) and vesicoureteral reflux (VUR). When the sample was segregated in these three phenotypes, associations between the BMP4 gene were observed with UPJO and with MDK. Conversely, VUR was not associated to the polymorphisms of the BMP4 gene. CONCLUSIONS: The present data suggest that Brazilian individuals with polymorphisms of the BMP4 gene have a higher risk to develop CAKUT, especially the malformations related to nephrogenesis and initial branching such as MDK and UPJO. Conversely, VUR appeared not to be related to BMP4 gene.

Prenatal diagnosis of multicystic dysplastic kidney disease in the second trimester screening.

OBJECTIVE: Multicystic dysplastic kidney (MCDK) is the most common antenatally diagnosed cystic renal pathology. It refers to the presence of multiple kidney cysts surrounded by dysplastic parenchymal tissue. The typical MCDK ultrasound finding shows multiple nonconnecting thin-walled cysts within an irregularly outlined kidney. We want to present our own cases as an addition to the relatively scarce literature about MCDK. METHOD: We retrospectively review 54 cases (21 bilateral and 33 unilateral) with antenatally diagnosed MCDK between the years 1998 and 2012. We compare our data with the published literature and discuss different parameters. RESULTS: Our findings show a male predominance of 1.8 : 1 and a left side/right side ratio of 0.73 : 1. Most of the cases were isolated findings, but 20.3% were associated with anomalies. One case was a Goldenhar syndrome. Of the bilateral MCDK cases, 14.3% had chromosomal defects. The mean gestational age at time of diagnosis was 20-21 weeks. Renal length was above the 90th normal percentile in 96.2% of the cases. CONCLUSION: Isolated unilateral MCDK has good prognosis. Bad prognosis is linked to association with other anomalies as well as to bilateral MCDK.

Outcome of sonographically suspected fetal ovarian cysts.

OBJECTIVE: Fetal ovarian cysts (FOCs) are the most common intra-abdominal cystic structures formed during antenatal period. The aim of this study was to evaluate prenatal characteristics and postnatal outcome of sonographically suspected FOCs. METHODS: We reviewed prenatal sonographic data and postnatal medical records of 29 fetuses that were suspected to have ovarian cysts in our hospital, between January 2001 and September 2012. Perinatal outcomes were obtained for all cases. RESULTS: In a total of 29 cases, we confirmed initial antenatal diagnosis of ovarian cyst in 20 cases (68.9%) at postnatal period. Diagnosis of remaining nine cases revealed urogenital sinus anomaly in four cases, multicystic dysplastic kidney in two cases, mesenteric cyst in one case, intestinal duplication cyst in one case and lymphangioma in one case postnatally. In seven of the 20 ovarian cysts (35%), resolution of the cyst occurred prenatally. Intrauterine death of a fetus occurred at second trimester. In the postnatal period, most often cases (11/20) were spontaneously resolved and surgery was performed on two babies due to ovarian torsion (2/20). CONCLUSION: Differential diagnosis of FOCs should always include genitourinary tract disorders, gastrointestinal tract disorders and other intra-abdominal cystic structures.

Simultaneous multicystic kidney and Anderson-Fabry disease: 2 separate entities or same side of the coin.

We present the case of a 54-year-old man with multicystic kidney and concomitant Anderson-Fabry disease. He was referred to our hospital with multiple renal and hepatic cysts, without apparent family history of autosomal dominant polycystic kidney disease. His clinical history suggested Anderson-Fabry disease, so an extensive work-up for Anderson-Fabry disease was subsequently undertaken. The alpha-galactosidase activity in his serum was low, and a final diagnosis of Anderson-Fabry disease with concomitant multicystic kidney was confirmed by genetic analysis.

Prevalence and pattern of cystic kidney diseases in Ilorin, Nigeria.

Cystic kidney disease is an important cause of chronic renal failure. Since the utilization of imaging techniques in the diagnosis of diseases has become widespread, cystic kidney disease is now being increasingly diagnosed. This study is designed to determine the prevalence and pattern of cystic kidney disease at the Nephrology Unit of University of Ilorin Teaching Hospital (UITH), Ilorin. All consecutive adult patients seen in the Nephrology Unit of UITH during a ten-year period (January 1999-December 2008) were studied for the presence of cystic kidney disease. The results were analyzed with specific reference to age, gender, annual incidence, type of cystic disease, location of cyst, mode of presentation, complications and prognosis. A total of 67 out of 436 renal patients (15.4%) studied had cystic kidney disease. A progressive annual increase in the number of cases was noticed. The age-range was 20-83 years with a mean of 47.4 +/- 16.2 years and the peak incidence was in the third and sixth decades with male to female ratio of 1.3:1. The types of cystic kidney disease identified in the study were: 26 simple cysts (38.8%), 35 polycystic kidney disease (53.3%) and six multicystic kidney disease (8.9%). The most common mode of presentation was abdominal pain followed by hypertension, urinary tract infection, chronic renal failure and palpable abdominal mass, in decreasing order. Our study indicates that cystic kidney disease is not an uncommon problem among our renal patients and the incidence is on the increase. Although, routine screening of family members with cystic kidney disease still remains a contentious issue because the knowledge may evoke anxiety in terms of employment and insurance, screening of symptomatic cases or those that develop hypertension, hematuria and proteinuria is strongly recommended.

Multicystic dysplastic kidney in children--a need for conservative and long term approach.

OBJECTIVE: We retrospectively studied our cases of Multicystic Dysplastic Kidney (MCDK). The review was aimed at identifying the pattern of the disease in Indian Scenario and the required management thereof. METHODS: We studied the clinical, radiological and nuclear scan findings of 22 patients with unilateral MCDK. They were diagnosed and/or treated in our unit from 1999 to 2007. The diagnosis was achieved by Ultrasound and further confirmed by DMSA scans. Other ancillary investigations like Micturating cystourethrogram were done if indicated. These patients were followed and followup investigations consisted of renal ultrasound, blood pressure measurement, and urinalysis and blood biochemistry RESULTS: A total of 22 patients (18 boys and 4 girls) with unilateral MCDK were investigated and followed for a mean period of 41 months. MCDK was detected on antenatal ultrasound only in 12(55%) and postnatally in 10(45%) babies. Mean age for postnatal diagnosis was 20 months. Follow up ultrasound revealed complete involution of MCDK in 3 patients and partial regression in 11 patients. The size of dysplastic kidney was unchanged in 4 patients and a further 4 patients underwent nephrectomy. Indications of nephrectomy were parental anxiety in 2, hypertension in 1 and palpable mass in 1. CONCLUSION: Large proportion (45%) of patients in presented series are diagnosed post natally contrary to western world where more than 80% are diagnosed antenatally. Uncomplicated isolated MCDK carry good prognosis with nephrectomy required in only a few patients. Association with other urological anomalies in ipsilateral/contralateral genitourinary tract is important to identify as they have worse outcome in terms of ultimate renal function. All patients with simple/complex unilateral MCDK should be advised long term follow up for the possible development of hypertension and/or hyper infiltration injury.

Unilateral multicystic dysplastic kidney: a meta-analysis of observational studies on the incidence, associated urinary tract malformations and the contralateral kidney.

BACKGROUND: Many papers are published on cohorts with unilateral multicystic dysplastic kidney (MCDK) patients, but show variable results as to the incidence of associated urinary tract abnormalities. The objective of this study was to describe the status of the urinary tract, including contralateral hypertrophy and malformations, in patients with unilateral MCDK based on a meta-analysis of the literature, taking into account the timing of diagnosis (pre- versus postnatal) as a possible source of bias. METHODS: A systematic review of the scientific literature in English was conducted using PubMed and Embase. A meta-analysis was performed with the studies that were identified using our reproducible search. RESULTS: Based on analysis of the data in 19 populations, the overall incidence of unilateral MCDK is 1 in 4300 with an increasing trend over the years. A total of 67 cohorts with over 3500 patients with unilateral MCDK were included in the meta-analysis. Fifty-nine percent of patients were male and the MCDKs were significantly more often found on the left side (53.1%). Associated anomalies in the solitary functioning kidney were found in 1 in 3 patients, mainly vesicoureteric reflux (VUR, in 19.7%). In patients with VUR, 40% have severe contralateral VUR, defined as grade III-V. Contralateral hypertrophy, present in 77% of patients after a follow-up of at least 10 years, showed a trend to be less pronounced in patients with VUR. Timing of the diagnosis of MCDK did not essentially influence the results. CONCLUSIONS: These aggregate results provide insight into the incidence, demographic data and associated anomalies in patients with unilateral MCDK. One in three patients with unilateral MCDK show anomalies in the contralateral, solitary functioning kidney. However, studies into the long-term consequences of these anomalies are scarce.

Histomorphology of renal dysplasia--an autopsy study.

A retrospective analysis of pediatric autopsies in the past 18 years was done with the aim of studying the histomorphology of renal dysplasia. Renal dysplasia comprised 150 (3.66%) of the 4,099 pediatric autopsies from 20 weeks of gestation to 1 year of life. Primitive ducts with the fibromuscular collar, the sine qua non of renal dysplasia, was seen in all cases. Lobar disorganization and cysts were seen in all cases except for the 7 cases of hypodysplasia. Other elements were seen in varying proportions: cartilage in 33.7%, bone in 1.08%, thickening of basement membrane of the primitive ducts in 64.13%, extramedullary hematopoiesis in 98.9%, nerve twigs in 72.8%, and nodular renal blastema in 2.17% cases. In unilateral multicystic dysplasia/renal agenesis, the contralateral kidney showed abnormalities in 44.45% and 47.37% of cases, respectively.

Riñón multiquístico: ¿existe alguna nueva evidencia científica que recomienda la nefrectomía? / Multicystic kidney: is there any new scientific evidence to recommend nefrectomy?

Con el fin de tratar de crear evidencia científica sobre la conveniencia o no de la extirpación del riñón multiquístico (RM), se ha realizado una revisión sistemática de la bibliografía de las últimas 2 décadas, seleccionando los estudios con determinados criterios. Asimismo se analiza nuestra casuística en los últimos 20 años. Se ha obtenido una tabla de evidencia con 1.082 casos de RM, con un seguimiento entre 2 y 7 años. Ningún caso malignizó. Las complicaciones han sido infección urinaria (IU) en un 5% e hipertensión arterial (HTA) en un 0,7%. En nuestra serie (68 casos) se realizó nefrectomía en 10 casos. El 82% desaparecieron totalmente (66,6% antes de los 5 años y 15,6% entre los 5 y 15 años de seguimiento). El 18% involucionó parcialmente. No ha aparecido ningún caso de malignización, limitándose las complicaciones a IU (6) e HTA en un caso. El seguimiento ecográfico periódico en el RM continúa siendo la aproximación terapéutica más razonable según los resultados de nuestra serie y de la revisión sistemática de la literatura relacionada (AU)

With the aim to create a scientific evidence of the convenience or not of removing the Multicystic Kidney (MK), a systematic review has been done over the last 20 years, selecting those articles with determinant criterions. Our experience has been also evaluated. We have obtained an evidence table of 1082 MK, with a follow-up from 2 to 7 years. No case degenerated. The complications were: urinary tract infection(UTI) in 5% and hipertensión(HTA) in 0.7%. In our serie (68 cases): nephrectomy was done in 10 cases. 82% completely involved (66.6% before 5 years and 15.6% from 5 to 15 years of follow-up). 18% involved partially. No case degenerated. The complications were UTI (6 cases) and HTA in one. Periodical ultrasound follow-up is our recommendation for MK due to the results of our serie and from the systematic review of the literature (AU)