Potter's Syndrome
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National Potter Syndrome Support Group
This is an information and parent match non-profit organization.
Contact Wright01@Camalott.com Subject: NPSSG
National Potter's Syndrome Support Group (NPSSG)
225 Louisiana Street
Dyess AFB, TX 79607
(915) 692-0831
Bilateral Renal Agenesis is the absence of both kidneys at birth. It is a genetic disorder characterized by a failure of the kidneys to develop in a fetus. This absence of kidneys causes a deficiency of amniotic fluid (Oligohydramnios) in a pregnant woman. Normally, the amniotic fluid acts as a cushion for the developing fetus. When there is an insufficient amount of this fluid, compression of the fetus may occur resulting in further malformations of the baby.
This disorder is more common in infants born of a parent who has a kidney malformation, particularly the absence of one kidney (unilateral renal agenesis). Studies have proven that unilateral renal agenesis and bilateral renal agenesis are genetically related.
OLIGOHYDRAMNIOS SEQUENCE (Potter Syndrome)
(ol““ī-go-hi-dram“ne-os) deficiency in the amount of amniotic fluid.
Renal agenesis, which must occur prior to 31 days of fetal development, will secondarily limit the amount of amniotic fluid and thereby result in further anomalies during prenatal life. The renal agenesis may be the only primary defect, or it may be one feature of a more extensive caudal axis anomaly. Other types of urinary tract defects such polycystic kidneys or obstruction may also be responsible for oligohydramnios and its consequences. Another cause is chronic leakage of amniotic fluid from the time of midgestation. regardless of the cause, the secondary effects of oligohydramnios are the same and would appear to be the result of compression of the fetus. The cause of death is respiratory insufficiency, with a lack of the late development of alveolar sacs. A similar lag in late development of the lung is observed with the diaphragmatic hernia or asphyxiating thoracic dystropy. In both of these latter situations, there is external compression of the developing lung; this is considered the most likely cause in oligohydramnios.
When the oligohydramnios is secondary to agenesis or dysgenesis of both kidneys or agensis of one kidney and dysgenesis of the other, renal ultrasonagraphic evaluation of both parents and siblings of affected infants should be performed, since 9 percent of first degree relatives had asymptomatic renal malformations in a study by Roodhooft et al.
References - Potter, E. L.: Bilateral renal agensis. J. Peditr., 29:68, 1946.
Bain, A.D., and Scott, J.S.: renal agensis and severe urinary tract dysplasia. A review of 50 cases with particular reference to the associated anomalies. Br. Med. J., 1:841, 1960.
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