Bilateral renal agenesis

Overview

What is bilateral renal agenesis?

Bilateral renal agenesis is a rare and almost always fatal health problem in which both kidneys are missing at birth. Kidneys normally develop during the second and third months (5th-12th weeks) of pregnancy. If neither kidney forms it means the baby is unable to make urine before or after birth. Fetal urine is the main component of amniotic fluid, the important fluid around the baby in the uterus which acts as a cushion during development. A baby with too little amniotic fluid around it (oligohydramnios) is "squashed" or compressed. A compressed baby doesn't have room to grow and develop which leads to multiple birth defects including lungs that can't function. About 40% of babies with bilateral renal agenesis are stillborn. The rest most often die within a few hours of birth due to underdeveloped lungs, with the longest reported survival at 39 days of life.

Having said this, there are a few case reports of infants undergoing numerous experimental treatments early in pregnancy involving weekly injections of artificial fluid around the baby to help their lungs develop. If healthy enough and given dialysis and a kidney transplant from birth, a very few number of babies with bilateral renal agenesis have survived into childhood. Maternal fetal medicine doctors (also called perinatologists) are high-risk pregnancy OB/gyns who have the most experience caring for moms and babies with bilateral renal agenesis. To find a Maternal fetal medicine doctor in a specific area, use the Society for Maternal Fetal Medicine doctor finder.

SOURCE: Emory University - Department of Human Genetics in collaboration with ThinkGenetic • https://www.thinkgenetic.com/diseases/bilateral-renal-agenesis/overview/2699 • DATE UPDATED: 2019-07-02

References

http://www.health.state.mn.us/divs/cfh/topic/diseasesconds/renalagenesis.cfm

http://www.uptodate.com/contents/prenatal-diagnosis-of-renal-agenesis

http://rarediseases.org/rare-diseases/renal-agenesis-bilateral

Pediatr Nephrol. 2007 Oct; 22(10): 1675?1684.

Published online 2007 Apr 17. doi: 10.1007/s00467-007-0479-1

Bienstock JL, Birsner ML, Coleman F, Hueppchen NA (2014) Successful in utero intervention for bilateral renal agenesis. Obstet Gynecol 124(2Pt 2 Suppl 1): 413-415.

http://www.ncbi.nlm.nih.gov/pubmed/25004316

Simone Sanna-Cherchi et al., Genetic approaches to human renal agenesis/hypoplasia and dysplasia. Pediatr Nephrol. 2007 Oct; 22(10): 1675?1684.

This content comes from a hidden element on this page.

The inline option preserves bound JavaScript events and changes, and it puts the content back where it came from when it is closed.

Remember Me