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.
(10) defined various syndromes (VATER association, CHARGE association, Potter syndrome, SCHISIS syndrome, trisomy 18) in 14.2 % of patients with EA/TEF.
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.
(3.) Guptha S et al; Potter syndrome, eMedicine, Jun 2010.
(1) Adult GCTs can be associated with Peutz-Jeghers and Potter syndromes. Juvenile GCTs are exceedingly rare but can also be associated with mesodermal dysplastic syndromes characterized by the presence of enchondromatosis and hemangioma formation, such as Ollier disease or Maffucci syndrome.