Renal Dysgenesis and Cystic Disease of the Kidney: A Report of the Committee on Terminology, Nomenclature and Classification, Section on Urology, American Academy of Pediatrics

Glassberg, Kenneth I.; Stephens, F. Douglas; Lebowitz, Robert L.; Braren, Victor; Duckett, John W.; Jacobs, Edward C.; King, Lowell R.; Perlmutter, Alan D.

doi:10.1016/s0022-5347(17)43510-5

Cited by 69 publications

(22 citation statements)

References 43 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…1 The increasingly widespread use of prenatal diagnostic techniques has revealed that MCRD is apparently even more prevalent than had been assumed. 2 The association of hypertension, malignancy, and incomplete involution in MCRD mandate long-term follow-up, and much controversy has surrounded the question of whether nephrectomy is indicated routinely on diagnosis.…”

mentioning

confidence: 99%

Involution Rate of Multicystic Renal Dysplasia

et al. 1998

View full text Add to dashboard Cite

ABSTRACT. Objective. To document the involution rate and long-term results of management of multicystic dysplastic kidney.Materials and Methods. Data were collected retrospectively for all 23 infants (16 boys) with multicystic dysplastic kidney who were treated at our center over the last 19 years (1977)(1978)(1979)(1980)(1981)(1982)(1983)(1984)(1985)(1986)(1987)(1988)(1989)(1990)(1991)(1992)(1993)(1994)(1995). The diagnosis was based on prenatal ultrasound in 18 patients and on palpable abdominal mass in 5, and confirmed in all patients by postnatal ultrasound and radioisotope scan. Voiding cystography was performed in 18 patients to exclude vesicoureteral reflux. Mean follow-up was 46 months (range, 3 months to 5 years) and included serum creatinine measurements and renal ultrasonography.Results. Two groups of patients were identified. Ten (43.6%) with other urologic abnormalities (group A) and 13 patients without other urologic abnormalities (group B). Vesicoureteral reflux was observed in 4 patients. Nephrectomy was performed in 4 patients, all from group B. The other 19 patients were treated conservatively. Complete involution was observed in 8 patients in group A and 6 in group B after a mean follow-up period of 9.2 and 10 months, respectively. Two patients, 1 from each group, later underwent nephrectomy not because of no involution but because of an increase in the size of the kidney involved.Conclusion. Patients with multicystic renal dysplasia have significant associated urologic malformations, and the natural history of the disease is unpredictable. All patients require appropriate investigation of the urinary tract and long-term follow-up.The most outstanding finding of the study is the much higher involution rate of multicystic renal dysplasia and the rate of associated urologic abnormalities than that reported in the literature. Surgery remains an option for the patients in the absence of no involution. Pediatrics 1998;102(6). URL: http://www.pediatrics.org/cgi/content/ full/102/6/e73; multicystic dysplastic kidney, surgery, conservative.ABBREVIATIONS. MCRD, multicystic renal dysplasia; UPJ, ureteropelvic junction; VUR, vesicoureteral reflux; UVJ, ureterovesical junction. M ulticystic renal dysplasia (MCRD) is the most common form of cystic disease of the kidney in childhood.1 The increasingly widespread use of prenatal diagnostic techniques has revealed that MCRD is apparently even more prevalent than had been assumed. 2 The association of hypertension, malignancy, and incomplete involution in MCRD mandate long-term follow-up, and much controversy has surrounded the question of whether nephrectomy is indicated routinely on diagnosis. 3,4 To assess the natural history of this condition and, for purposes of proper management, the associated urinary malformations, we conducted a 19-year retrospective study of all infants with MCRD who had been diagnosed and treated in our tertiary care medical center. MATERIALS AND METHODSWe reviewed the charts and radiologic records of the 25 patients with MCRD who w...

show abstract

mentioning

confidence: 99%

Involution Rate of Multicystic Renal Dysplasia

et al. 1998

View full text Add to dashboard Cite

show abstract

“…Renal concentration is seldom seen on renal nuclear scan. When the cysts are small and stroma predominates, the condition is referred to as solid cystic dysplasia [6]. When an identifiable renal pelvis is associated with what appears to be a multicystic dysplastic kidney, the condition is referred to as the hydronephrotic form of multicystic kidney [4].…”

Section: Discussionmentioning

confidence: 99%

“…The bilateral condition is incompatible with life though rarely survival for few months has been reported [7]. Multicystic refers to a dysplastic entity, and polycystic refers to an inherited entity, all without dysplasia and presence of nephrons throughout the kidney [6]. In polycystic disease, oligohydramnios is common because of the lack of normal urine production by the foetus.…”

Section: Discussionmentioning

confidence: 99%

Are therapeutic stem cells justified in bilateral multicystic kidney disease? A review of literature with insights into the embryology

et al. 2007

View full text Add to dashboard Cite

Aim was to describe the challenges faced in the management of bilateral multicystic kidney disease (MCKD). A case of antenatally detected bilateral polycystic disease was referred at 28 weeks of gestation. The patient was advised to continue pregnancy till term and be in regular follow-up. Postnatally, the male baby passed urine in normal stream and was diagnosed as bilateral multicystic kidney disease on ultrasonography. He developed symptoms of renal failure. The baby was operated with right pyeloplasty and left pyelostomy, as the left ureter was atretic. The histopathology was consistent with bilateral multicystic kidney disease. Postoperatively, the baby was stable with intermittent episodes of metabolic acidosis that were managed medically and with peritoneal dialysis. Autologous stem cells were injected at the age of 1 year into the aorta at the level of the renal arteries clamping the aorta below. Repeat biopsy at time of stem cell injection showed 5/10 glomeruli showing global sclerosis on right side and 5/15 glomeruli showing global sclerosis on left side. The only improvement seen was in decreased doses of medicines to keep the child metabolically stable. The baby kept struggling but succumbed at the age of 17 months and 15 days. Post mortem bilateral renal biopsies demonstrated presence of primitive renal tubules and blastemal cells that were not demonstrated earlier. Survival for few months in bilateral multicystic kidney disease is thus possible with adequate treatment, the novel use of stem cells in these cases may prove beneficial in future though it is too early to comment further.

show abstract

“…In the latter case, dysplas tic structures may have been completely obscured by inflammatory changes [7], Although the pathological evaluation of kidneys with reflux nephropathy may give some clues to its pathogen esis, clear correlations between pathologic lesions and pathogenic mechanisms are difficult to establish. There fore, the term 'reflux nephropathy' should be used in a broad sense, encompassing congenital and acquired re nal lesions associated with VUR [27],…”

Section: Discussionmentioning

confidence: 99%

Reflux Nephropathy: A Clinico-Pathological Study of 16 Cases

Gil-Salom¹,

Sánchez

Chuan³

et al. 1991

Urol Int

View full text Add to dashboard Cite

The pathologic features of 16 nephrectomy specimens obtained from patients with a radiological diagnosis of reflux nephropathy were analyzed. Chronic pyelonephritis was diagnosed in 7 cases, renal dysplasia in 5 and segmental atrophy in 4. Clear correlations between pathologic pictures and pathogenic mechanisms involved in the development of renal lesions in kidneys with reflux are difficult to establish. These results show, however, that ‘reflux nephropathy’ is a broad term, which encompasses both congenital and acquired renal lesions.

show abstract

Renal Dysgenesis and Cystic Disease of the Kidney: A Report of the Committee on Terminology, Nomenclature and Classification, Section on Urology, American Academy of Pediatrics

Cited by 69 publications

References 43 publications

Involution Rate of Multicystic Renal Dysplasia

Involution Rate of Multicystic Renal Dysplasia

Are therapeutic stem cells justified in bilateral multicystic kidney disease? A review of literature with insights into the embryology

Reflux Nephropathy: A Clinico-Pathological Study of 16 Cases

Contact Info

Product

Resources

About