Potter syndrome


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Pot·ter syn·drome

(pot'ĕr),
renal agenesis with hypoplastic lungs and associated neonatal respiratory distress, hemodynamic instability, acidosis, cyanosis, edema, and characteristic (Potter) facies; death usually occurs from respiratory insufficiency, which develops before uremia.

Potter syndrome

Potter phenotype Neonatology A complex of findings caused by prenatal renal failure and oligohydramnios; without the cushioning amniotic fluid, produced by the kidneys, the uterus presses directly on infant's face–see Potter's face–and limbs, which are held in abnormal positions or contractures; oligohydramnios also cause hypoplasia of lungs–which don't function properly at birth Pathogenesis Intrauterine renal defects–eg, bilateral renal agenesis–and ↓ amniotic fluid production; no kidneys, no amniotic fluid.

Pot·ter syn·drome

(pot'ĕr sin'drōm)
Renal agenesis with hypoplastic lungs and associated neonatal respiratory distress, hemodynamic instability, acidosis, cyanosis, edema, and characteristic (Potter) facies; death usually occurs from respiratory insufficiency, which develops before uremia.

Potter,

Edith L., U.S. perinatal pathologist, 1901–.
Potter classification of polycystic kidney
Potter disease - Synonym(s): Potter facies
Potter facies - characteristic facies seen in severe renal malformations. Synonym(s): Potter disease
Potter syndrome - renal agenesis, with hypoplastic lungs and associated neonatal respiratory distress.
References in periodicals archive ?
Her medical history was notable because of a prior pregnancy complicated by a stillbirth with Potter syndrome, delivered 2 months before conception of the current pregnancy.
Classic Potter Syndrome occurs when the developing fetus has bilateral renal agenesis, where as type-i due to autosomal recessive polycystic kidney disease (ARPKD).
DISCUSSION: Potter Syndrome Type I is due to Autosomal Recessive Polycystic Kidney Disease (ARPKD), which occurs at a frequency of approximately one in 40,000 infants and is linked to a mutation in the gene PKHD1.
CONCLUSION: Potter syndrome due to different causes present with same characteristic facies associated with other congenital anomalies.
Guptha S et al; Potter syndrome, eMedicine, Jun 2010.
1) Adult GCTs can be associated with Peutz-Jeghers and Potter syndromes.