Renal agenesis causes
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Shakiba Hassanzadeh, MD[2]
Overview
Renal agenesis may be associated with CAKUT (congenital anomalies of the kidney and urinary tract) or extra-renal anomalies, genetic syndromes or chromosomal disorders, and sequences.
Causes
Some associated anomalies, genetic syndromes and teratogenic causes of renal agenesis include:
| Associated Anomalies in Unilateral Renal Agenesis
(Modified table from Unilateral renal agenesis: a systematic review on associated anomalies and renal injury)[1] | ||||
|---|---|---|---|---|
| Associated CAKUT
(congenital anomalies of the kidney and urinary tract) (VUR (vesicoureteral reflux), PUV (posterior urethral valves), PUJO (pelviureteric junction obstruction), duplex kidney, ureterocele, and megaureter) |
32% | |||
| Urinary tract infection | 30% | |||
| Associated extra-renal anomalies | 31% | |||
| Female tract anomalies | 11% | |||
| Extra-renal Anomalies Associated with URA
(Modified table from Congenital Unilateral Renal Agenesis: Prevalence, Prenatal Diagnosis, Associated Anomalies. Data from Two Birth-Defect Registries)[2] | ||||
|---|---|---|---|---|
| Type | Extra-Renal Anomalies | |||
| One effected system/organ |
| |||
| Associated Sequences |
| |||
| Associated Genetic Syndromes |
| |||
| Associated Chromosomal Anomalies | ||||
| Associated Syndromatic Polymalformations (non-identified) | ||||
| Macrocephaly, hypertelorism | ||||
| Anencephaly, caudal regression | ||||
| Occipital meningoencephalocele, unicornate uterus | ||||
| Heart defect, limb anomaly | ||||
| Tetralogy of Fallot, radial agenesia | ||||
| Patent ductus arteriosus, cerebellum hypoplasia, supernumerary hemivertebra, tracheolaryngomalacia, Meckel’s diverticulum, agenesis of eyelashes | ||||
| Common arterial trunk, ambiguous genitalia, imperforate anus, cystic hygroma | ||||
| Dandy-Walker syndrome, VSD | ||||
| Radial agenesia, clubhand, hypoplastic left heart syndrome, transposition of great vessels, agenesis of lung, diaphragmatic hernia, polysplenia | ||||
| Spina bifida, stenosis of anus | ||||
| Vertebral dysplasia, scrotal hypospadias | ||||
| Imperforate anus, ambiguous genitalia | ||||
| URA or BRA and Some Genetic Disorders
(Modified table from Ultrasound diagnosis of fetal renal abnormalities)[3] | ||||
|---|---|---|---|---|
| Syndrome | Gene | |||
| Acro–renal–ocular syndrome | SALL 4 | |||
| Branchio–oto–renal syndrome |
| |||
| Ectrodactyly–ectodermal dysplasia–cleft syndrome | P63 | |||
| Pallistere Hall syndrome | GLI3 | |||
| Renal–coloboma syndrome | PAX2 | |||
| Townes–Brocks syndrome | SALL1 | |||
| Antley–Bixler syndrome | FGFR2 | |||
| Fraser syndrome | FRAS1 | |||
| Smith–Lemli-Opitz syndrome | DHCR7 | |||
| Goltz–Gorlin syndrome | PORCN | |||
| Kallman syndrome | KAL1 | |||
| Lenz microphthalmia | BCOR | |||
Potter syndrome may include bilateral renal agenesis (BRA), pulmonary hypoplasia and oligohydramnios.[4] (For further information about Potter syndrome, click here.)
Teratogenic Causes Associated with Renal Agenesis:[5][6][7]
- Uncontrolled maternal diabetes mellitus
- Use of drugs during pregnancy such as those drugs that inhibit the renin-angiotensin system
- High doses of vitamin A derivates (in animals)
References
- ↑ Westland R, Schreuder MF, Ket JC, van Wijk JA (2013). "Unilateral renal agenesis: a systematic review on associated anomalies and renal injury". Nephrol Dial Transplant. 28 (7): 1844–55. doi:10.1093/ndt/gft012. PMID 23449343.
- ↑ Laurichesse Delmas H, Kohler M, Doray B, Lémery D, Francannet C, Quistrebert J; et al. (2017). "Congenital unilateral renal agenesis: Prevalence, prenatal diagnosis, associated anomalies. Data from two birth-defect registries". Birth Defects Res. 109 (15): 1204–1211. doi:10.1002/bdr2.1065. PMID 28722320.
- ↑ Dias T, Sairam S, Kumarasiri S (2014). "Ultrasound diagnosis of fetal renal abnormalities". Best Pract Res Clin Obstet Gynaecol. 28 (3): 403–15. doi:10.1016/j.bpobgyn.2014.01.009. PMID 24524801.
- ↑ Zaffanello M, Brugnara M, Zuffante M, Franchini M, Fanos V (2009). "Are children with congenital solitary kidney at risk for lifelong complications? A lack of prediction demands caution". Int Urol Nephrol. 41 (1): 127–35. doi:10.1007/s11255-008-9437-5. PMID 18690548.
- ↑ Boix E, Zapater P, Picó A, Moreno O (2005). "Teratogenicity with angiotensin II receptor antagonists in pregnancy". J Endocrinol Invest. 28 (11): 1029–31. doi:10.1007/BF03345344. PMID 16483184.
- ↑ Nielsen GL, Nørgard B, Puho E, Rothman KJ, Sørensen HT, Czeizel AE (2005). "Risk of specific congenital abnormalities in offspring of women with diabetes". Diabet Med. 22 (6): 693–6. doi:10.1111/j.1464-5491.2005.01477.x. PMID 15910618.
- ↑ Tse HK, Leung MB, Woolf AS, Menke AL, Hastie ND, Gosling JA; et al. (2005). "Implication of Wt1 in the pathogenesis of nephrogenic failure in a mouse model of retinoic acid-induced caudal regression syndrome". Am J Pathol. 166 (5): 1295–307. doi:10.1016/S0002-9440(10)62349-8. PMC 1606386. PMID 15855632.