| Multicystic dysplastic kidney |
| Classification and external resources |
| Specialty |
medical genetics |
| ICD-10 |
Q61.4 (EUROCAT Q61.40-Q61.41) |
| eMedicine |
radio/458 |
This article is about kidney dysplasia in humans. For kidney dysplasia in dogs and cats, see Familial renal disease in animals.
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.[1]
Contents
- 1 Symptoms
- 2 Cause
- 3 Pathophysiology
- 4 Diagnosis
- 5 Treatment
- 6 References
- 7 Further reading
Symptoms
Ultrasound Scan -Vesicoureteral reflux
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.[2] In bilateral cases, the newborn has the classic characteristic of Potter's syndrome.[3][4]
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]
Cause
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.[5]
Pathophysiology
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). It is though PAX2 gene, may also play a role in MCDK.[6]
Diagnosis
MCDK is usually diagnosed by ultrasound examination before birth. Mean age at the time of antenatal diagnosis is about 28 weeks[7] A microscopic analysis of urine in individuals with probable multicystic dysplastic kidney should be done.[8] 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.[9]
Treatment
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 neo-natal fatality (in bilateral cases), however in unilateral cases the prognosis might be better(it would be dependent on associated anomalies).[10][11]
Epidemiology
In regards to the epidemiology of multicystic dysplasia kidney, the incidence of MCDK is estimated to be 1 in every 4,000 live births, making it rare in terms of the general population.[5]
References
- ^ "Multicystic Dysplastic Kidney Imaging: Overview, Radiography, Computed Tomography".
- ^ 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 & Management: Medical Care, Surgical Care, Consultations".
- ^ 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.
- ^ a b "Kidney Dysplasia". www.niddk.nih.gov. Retrieved 2015-11-24.
- ^ "Multicystic Renal Dysplasia: Background, Pathophysiology, Epidemiology".
- ^ 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: Laboratory Studies, Imaging Studies, Other Tests". emedicine.medscape.com. Retrieved 2015-11-24.
- ^ 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.
Further reading
- 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.
|
Congenital malformations and deformations of urinary system (Q60–Q64, 753)
|
|
| Abdominal |
| Kidney |
- Renal agenesis/Potter sequence, Papillorenal syndrome
- cystic
- Polycystic kidney disease
- Meckel syndrome
- Multicystic dysplastic kidney
- Medullary sponge kidney
- Horseshoe kidney
- Renal ectopia
- Nephronophthisis
- Dent's disease
- Alport syndrome
|
|
| Ureter |
- Ectopic ureter
- Megaureter
- Duplicated ureter
|
|
|
| Pelvic |
| Bladder |
|
|
| Urethra |
- Epispadias
- Hypospadias
- Posterior urethral valves
|
|
|
| Vestigial |
|
|
|
Index of the urinary system
|
|
| Description |
- Anatomy
- Physiology
- Development
- Cells
|
|
| Disease |
- Electrolyte and acid-base
- Congenital
- Neoplasms and cancer
- Other
- Symptoms and signs
- Urine tests
- Blood tests
|
|
| Treatment |
- Procedures
- Drugs
- Intravenous fluids
|
|
|
|
Medicine
|
|
Specialties
and
subspecialties |
|
Surgery
|
|
- Cardiac surgery
- Cardiothoracic surgery
- Colorectal surgery
- Eye surgery
- General surgery
- Neurosurgery
- Oral and maxillofacial surgery
- Orthopedic surgery
- Hand surgery
- Otolaryngology (ENT)
- Pediatric surgery
- Plastic surgery
- Reproductive surgery
- Surgical oncology
- Thoracic surgery
- Transplant surgery
- Trauma surgery
- Urology
- Vascular surgery
|
|
Internal medicine
|
|
- Allergy / Immunology
- Angiology
- Cardiology
- Endocrinology
- Gastroenterology
- Geriatrics
- Hematology
- Hospital medicine
- Infectious disease
- Nephrology
- Oncology
- Pulmonology
- Rheumatology
|
|
Obstetrics and gynaecology
|
|
- Gynaecology
- Gynecologic oncology
- Maternal-fetal medicine
- Obstetrics
- Reproductive endocrinology and infertility
- Urogynecology
|
|
Diagnostic
|
|
- Radiology
- Interventional radiology, Nuclear medicine
- Pathology
- Anatomical pathology, Clinical pathology, Clinical chemistry, Clinical immunology, Cytopathology, Medical microbiology, Transfusion medicine
|
|
Specialties
|
|
- Addiction medicine
- Adolescent medicine
- Anesthesiology
- Dermatology
- Disaster medicine
- Diving medicine
- Emergency medicine
- Family medicine
- General practice
- Hospital medicine
- Intensive-care medicine
- Medical genetics
- Neurology
- Occupational medicine
- Ophthalmology
- Oral medicine
- Pain management
- Palliative care
- Pediatrics
- Physical medicine and rehabilitation
- Preventive medicine
- Psychiatry
- Radiation oncology
- Reproductive medicine
- Sexual medicine
- Sleep medicine
- Sports medicine
- Transplantation medicine
- Tropical medicine
|
|
|
| Medical education |
- Medical school
- Bachelor of Medicine, Bachelor of Surgery
- Bachelor of Medical Sciences
- Master of Medicine
- Master of Surgery
- Doctor of Medicine
- Doctor of Osteopathic Medicine
- MD-PhD
|
|
| Related topics |
- Allied health
- Nanomedicine
- Molecular oncology
- Personalized medicine
- Veterinary medicine
- Physician
- History of medicine
|
|
|
|
|
