Multicystic dysplastic kidney
|Multicystic dysplastic kidney|
|Classification and external resources|
|ICD-10||Q61.4 (EUROCAT Q61.40-Q61.41)|
Multicystic dysplastic kidney (MCDK) is a condition that results from the malformation of the kidney during fetal development. The kidney consists of irregular cysts of varying sizes. Multicystic dysplastic kidney is a common type of renal cystic disease, and it is a cause of an abdominal mass in infants.
When a diagnosis of multicystic kidney is made in utero by ultrasound, the disease is found to be bilateral in many cases. Those with bilateral disease often have other severe deformities or polysystemic malformation syndromes. In bilateral cases, the newborn has the classic characteristic of Potter's syndrome.
The bilateral condition is incompatible with survival,as the contralateral system frequently is abnormal as well.Contralateral ureteropelvic junction obstruction is found in 3% to 12% of infants with multicystic kidney and contralateral vesicoureteral reflux is seen even more often, in 18% to 43% of infants . Because the high incidence of reflux, voiding cystourethrography usually has been considered advisable in all newborns with a multicystic kidney.[medical citation needed]
The cause of multicystic dysplastic kidney can be attributed to genetics.Renal dysplasia can be a consequence of a genetic syndrome, which in turn may affect the digestive tract, nervous system, or other areas of the urinary tract. If the mother had been taking certain prescription drugs such as those for hypertension, this may be a precipitating factor as well.
The mechanism of multicystic dysplastic kidney is a result of an abnormal induction of metanephric mesenchyme. This could be a result of a formation difficulty of the mesonephric duct. Some mutations in genes associated with renal dysplasia (in syndromes)have been determined. The mutations in question occur at EYA1 or SIX1 genes ( branchio-oto-renal syndrome). The PAX2 gene is also thought to play a role in MCDK.
MCDK is usually diagnosed by ultrasound examination before birth. Mean age at the time of antenatal diagnosis is about 28 weeks A microscopic analysis of urine in individuals with probable multicystic dysplastic kidney should be done. One meta-analysis demonstrated that unilateral MCDK occurs more frequently in males and the greater percentage of MCKD occur on the left side of the body.
MCDK is not treatable. However, the patient is observed periodically for the first few years during which ultrasounds are generally taken to ensure the healthy kidney is functioning properly and that the unhealthy kidney is not causing adverse effects. In severe cases MCDK can lead to neonatal fatality (in bilateral cases), however in unilateral cases the prognosis might be better (it would be dependent on associated anomalies).
- Multicystic Dysplastic Kidney Imaging at eMedicine
- Becker, W.; Meller, J.; Zappel, H.; Leenen, Andreas; Seseke, F. (2012-12-06). Imaging in Paediatric Urology. Springer Science & Business Media. p. 8. ISBN 9783642557859.
- Potter Syndrome~treatment at eMedicine
- Lager, Donna J.; Abrahams, Neil (2012-11-07). Practical Renal Pathology, A Diagnostic Approach: A Volume in the Pattern Recognition Series. Elsevier Health Sciences. p. 27. ISBN 1455737860.
- "Kidney Dysplasia". www.niddk.nih.gov. Retrieved 2015-11-24.
- Multicystic Renal Dysplasia at eMedicine
- Chang, Ling-Wei; Chang, Fong-Ming; Chang, Chiung-Hsin; Yu, Chen-Hsiang; Cheng, Yueh-Chin; Chen, Hsi-Yao (2002-07-01). "Prenatal diagnosis of fetal multicystic dysplastic kidney with two-dimensional and three-dimensional ultrasound". Ultrasound in Medicine & Biology 28 (7): 853–858. ISSN 0301-5629. PMID 12208325. – via ScienceDirect (Subscription may be required or content may be available in libraries.)
- Multicystic Renal Dysplasia~workup at eMedicine
- Schrueder, Michiel (2008). "Unilateral multicystic dysplastic kidney: a meta-analysis of observational studies on the incidence, associated urinary tract malformations and the contralateral kidney". Nephrology Dialysis Transplantation. Retrieved 2015.
- Stevenson, Roger E.; Hall, Judith G.; Everman, David B.; Solomon, Benjamin D. (2015-10-16). Human Malformations and Related Anomalies. Oxford University Press. p. 800. ISBN 9780199386048.
- Schwartz, M. William (2012-01-01). The 5 Minute Pediatric Consult. Lippincott Williams & Wilkins. p. 439. ISBN 9781451116564.
- al-Khaldi, N; Watson, A R; Zuccollo, J; Twining, P; Rose, D H (1994-06-01). "Outcome of antenatally detected cystic dysplastic kidney disease.". Archives of Disease in Childhood 70 (6): 520–522. ISSN 0003-9888. PMC 1029874. PMID 8048824.
- van Eijk, L.; Cohen-Overbeek, T. E.; den Hollander, N. S.; Nijman, J. M.; Wladimiroff, J. W. (2002-02-01). "Unilateral multicystic dysplastic kidney: a combined pre- and postnatal assessment". Ultrasound in Obstetrics and Gynecology 19 (2): 180–183. doi:10.1046/j.0960-7692.2001.00497.x. ISSN 1469-0705.