Involution Of Multicystic Dysplastic Kidney Research Articles

OP32.08: Involution of multicystic dysplastic kidney: does initial size matter?

<scp>OP</scp>32.08: Involution of multicystic dysplastic kidney: does initial size matter?

Involution of multicystic dysplastic kidney: Is it predictable?

ObjectiveTo evaluate the clinical course of multicystic dysplastic kidney (MCDK) and to reveal any criteria indicating spontaneous involution. Material methodsHospital records of patients with MCDK followed in two different institutions in 1994–2009 were reviewed and data were analyzed regarding involution. ResultsRecords of 96 patients were reviewed, of whom 46 were diagnosed antenatally and followed for more than 1 year. Fourteen patients had undergone nephrectomy. There was one case of hypertension which resided with nephrectomy. There was no malignancy. Involution rate was 53.6% (15/28) for right-sided and only 16.7% (3/18) for left-sided kidneys. The initial size of the kidney was found to be another predictive parameter for involution. Initial sizes of 43 (15 involuted and 28 non-involuted) kidneys were documented. Mean standard deviation score for involuting and non-involuting kidneys was −3.19 and 3.12, respectively. The chance of involution for a large kidney on the left was zero; however, involution risk for a small right-sided kidney was 67%. ConclusionReviewing a 15-year period of our patient records conveyed data supporting current literature mainly encouraging non-operative management of MCDK. Further studies are required; however, our two objective indicators, the initial size and side of dysplastic kidney, may contribute to the management.

Renal size and sonographic involution of multicystic dysplastic kidney

Renal size and sonographic involution of multicystic dysplastic kidney

'Congenital solitary functioning kidneys: which ones warrant follow-up into adult life?'

Congenital anomalies of the kidney and urinary tract (CAKUT) are the leading cause of paediatric end-stage renal failure (ESRF) [1, 3]. The clinical spectrum of CAKUT includes a variety of malformations of the urinary tract. Understanding the rate of progression for the different CAKUT categories and predicting long-term outcome is critical for a correct clinical management of these patients and for the transition of care from paediatric to adult nephrology. Among CAKUT categories, congenital solitary functioning kidney (SFK) has been the object of debate whether it constitutes a benign condition or presents a significant risk of progression to ESRF [2–14]. In the present issue of Nephrology Dialysis and Transplantation, Westland et al. report on the results of the KIMONO-study (REF ID pending). The authors analysed a large cohort of 206 children with SFK of congenital origin to identify indicators of renal injury. Among them, 116 had a primary congenital solitary functioning kidney (pSFK) and 90 had a secondary solitary functioning kidney (sSFK) after unilateral nephrectomy due to congenital anomalies of the kidney or urinary tract. Ipsilateral CAKUT was present in ~30% of children from both groups. Among the patients with pSFK (which encompasses unilateral renal agenesis and multicystic dysplastic kidney), ipsilateral anomalies were significantly more frequent in cases with renal agenesis. Similarly, high blood pressure was present in comparable proportions between pSFK and sSFK, and it was significantly higher in the subgroup with renal agenesis. About 20% of children were using renoprotective agents and ~12% had albuminuria. Overall, >30% of patients with SFK showed evidence of renal injury. These data indicate that a significant fraction of children with congenital solitary kidney show evidence of renal parenchyma damage early in life and can potentially progress to ESRF in adulthood. These numbers are consistent with our previous report on long-term outcome in CAKUT patients, in which 50% of children with solitary kidney reached ESRF by the age of 30 [11]. Westland et al. explored the rate of progression by generalized estimated equation (GEE) analysis (REF ID pending). Microalbuminuric-range proteinuria appeared at the age of 18 years in both pSFK and sSFK, and children with CAKUT showed a more pronounced increment. GEE analyses (to be taken cautiously) point to a slow progression of renal injury during adolescence, which suggests that a significant fraction of patients will progress to renal failure during adulthood. Again, these data seem to be supported by our outcome data, in which the majority of patients with SFK reached ESRF after the age of 18 [11]. Therefore, a large number of children who are diagnosed with congenital solitary kidney in early life will progress to chronic renal failure requiring replacement therapy in adulthood, without a smooth transition from paediatric care. The difficulties of understanding the natural history of congenital solitary kidney stand on several observations that have not been elucidated so far and remain unresolved by the present report. First, a clear definition of the phenotype of the patients investigated is not available. Westland et al. cluster unilateral renal agenesis and the involution of multicystic dysplastic kidney together into one category and all solitary functioning kidneys that result from other congenital anomalies of the urinary tract that require nephrectomy or lead to a complete loss of function of one kidney in another category. This stratification, although potentially useful, is arbitrary in consideration of the possibly different molecular origin and clinical course of the different conditions. Therefore, until molecular genetics can improve the diagnosis, any classification and grouping for statistical purposes is arbitrary and invariably results in

Multicystic dysplastic kidney in children — A need for conservative and long term approach

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. 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 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. 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.

Renal agenesis: report of an interesting case

Renal agenesis is generally thought to result from a lack of induction of the metanephric blastema by the ureteral bud, which may be secondary to ureteral bud maldevelopment and/or to a problem with the formation of the mesonephric duct. Uncommonly, post-natal involution of multicystic dysplastic kidneys results in solitary kidney. Unilateral renal agenesis may be associated with ipsilateral genitourinary anomalies and VATER abnormalities. The interest of this case lies in the association of unilateral renal agenesis with the presence of an ipsilateral pelvic dilated ureter, ipsilateral seminal vesicle hypoplasia and absence of the vas deferens.

Conservative management of multicystic dysplastic kidney: clinical course and ultrasound outcome

The aim of this study was to describe the clinical course and ultrasound outcome of prenatally detected multicystic dysplastic kidney. Fifty-three children with unilateral multicystic dysplastic kidney detected by prenatal ultrasound between 1989 and 2004 were included in the analysis. All children were submitted to conservative management with follow-up visits every six months. Follow-up ultrasound examinations were performed at six-month intervals during the first two years of life and yearly thereafter. The following clinical parameters were evaluated: blood pressure, urinary tract infection, renal function, and growth. The following ultrasound parameters were evaluated: involution of multicystic dysplastic kidney and contralateral renal growth. The mean follow-up time was 68 months. Two children presented hypertension during follow-up and five had urinary tract infection (only one with recurrent episodes). There was no malignant degeneration of multicystic dysplastic kidney. A total of 334 ultrasound scans were analyzed. US scan demonstrated involution of the multicystic dysplastic kidney in 48 (90%) cases, including complete involution in nine (17%). The involution rate was faster in the first 30 months of life. There was progressive compensatory renal hypertrophy of the contralateral renal unit; the rate of growth was greater in the first 24 months of life. The results of prolonged follow-up of children with conservatively managed multicystic dysplastic kidney suggest that clinical approach is safe, the incidence of complications is small, and that there is a clear tendency for multicystic dysplastic kidney to decrease in size. Our data also suggest that the involution rate of multicystic dysplastic kidney as well as the growth of the contralateral kidney is greater in the first 24 months of life.

Tratamento conservador do rim displásico multicístico: curso clínico e ultra-sonográfico

OBJETIVO: Descrever o curso clínico e a evolução ultra-sonográfica de pacientes com rim displásico multicístico tratados conservadoramente. MÉTODOS: Foram incluídas no estudo 53 crianças com rim displásico multicístico unilateral diagnosticado pela ultra-sonografia fetal entre 1989 e 2004. Todos os pacientes foram submetidos a protocolo sistemático, incluindo tratamento conservador e exames clínicos, laboratoriais e ultra-sonográficos periódicos. Os exames foram realizados com periodicidade de 6 meses, nos 2 primeiros anos, e anualmente, após esse período. No curso clínico, foram avaliados: pressão arterial, infecção do trato urinário, função renal e crescimento dos pacientes. Na evolução ecográfica, foram avaliados a involução do rim displásico multicístico e o crescimento do rim contralateral. RESULTADOS: O tempo médio de seguimento foi de 68 meses. Dois pacientes apresentaram hipertensão arterial no seguimento. Cinco tiveram infecção urinária (apenas um com episódios repetidos). Não houve degeneração maligna do rim displásico multicístico. Foram realizados 334 exames ultra-sonográficos seriados. Houve involução do rim displásico multicístico em 90% dos casos, sendo que nove (17%) desapareceram. O ritmo de involução foi maior nos 30 primeiros meses de vida. Houve progressiva hipertrofia compensatória do rim contralateral, sendo o ritmo mais intenso nos 2 primeiros anos de vida. CONCLUSÃO: Os resultados do acompanhamento prospectivo de crianças portadoras de rim displásico multicístico tratadas conservadoramente sugerem que a conduta clínica é segura, a incidência de complicações é mínima e há uma nítida tendência de involução ultra-sonográfica da unidade renal afetada. Os dados sugerem, ainda, que o ritmo de involução do rim displásico multicístico, assim como da hipertrofia compensatória do rim contralateral, sejam maiores nos 2 primeiros anos de vida.

A FAMILY STUDY AND THE NATURAL HISTORY OF PRENATALLY DETECTED UNILATERAL MULTICYSTIC DYSPLASTIC KIDNEY

We document the inheritance pattern of multicystic dysplastic kidney in 3 affected families and screen first-degree relatives of a cohort of children with prenatally detected multicystic dysplastic kidney for renal anomalies. The study also afforded an opportunity to document the natural history of prenatally detected multicystic dysplastic kidney. We identified 3 families during clinical treatment of children with prenatally detected multicystic dysplastic kidneys. Other members of these families were evaluated with renal ultrasonography. For the family screening study index cases were identified from a fetal uropathy database. A total of 94 first-degree relatives (52 parents, 35 full siblings and 7 half siblings) of 29 children with prenatally detected multicystic dysplastic kidneys were studied with urinary tract ultrasonography, blood pressure measurement, urinalysis and plasma biochemistry. Two families had affected sibling pairs, 1 of which also had a half sibling with vesicoureteral reflux. The third family included 3 individuals with multicystic dysplastic kidney and 1 with renal agenesis thought to have resulted from involution of multicystic dysplastic kidney. This family is consistent with autosomal dominant inheritance with variable expressivity and reduced penetrance. In the screening study ultrasonography did not demonstrate significant renal anomalies in any of the 94 first-degree relatives of the multicystic dysplastic kidney index cases. Followup assessment of prenatally detected multicystic dysplastic kidneys in index cases demonstrated total involution in 52% at a median age of 6.5 years with no multicystic dysplastic kidney related morbidity. Multicystic dysplastic kidney can be familial but is most commonly a sporadic anomaly. Formal screening of relatives is not recommended. Followup data on a cohort of children with prenatally detected multicystic dysplastic kidney add further support to conservative management.

Renal agenesis: An acquired condition?

Renal agenesis is a relatively common congenital anomaly, although its etiology is unknown. It is clear that some solitary kidneys are the result of postnatal involution of multicystic dysplastic kidneys. The authors present a series of nine neonates with abnormal prenatal renal ultrasound findings; subsequent postnatal investigations showed an absent kidney. Five children had a prenatal diagnosis of multicystic dysplastic kidney (MDK) and two of hydronephrosis. In five cases (56%) there was a contralateral renal unit anomaly. It appears that MDK, and occasionally hydronephrosis, can involute prenatally, producing the postnatal appearance of renal agenesis.