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Potter's syndrome

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The total absence or malformation of infant kidneys.

Description

Potters Syndrome is the term used to describe the total absence or malformation of infant kidneys in combination with other congenital abnormalities, including Potter's facies, pulmonary hypoplasia, oligohydramnios, failure of foetal urinary production and skeletomuscular anomalies such as clubbing of the hands and feet and contractures. Oligohydramnios (deficiency of the amount of amniotic fluid) is the cause of failure of lung development and of limb compression in the foetus. Males are more commonly affected than females. The affected children are usually stillborn or die shortly after birth. Authors disagree whether inheritance is autosomal dominant recessive.

Bibliography

  • H. Madisson:
    Ueber das Fehlen beider Nieren (Aplasia renum bilateralis).
    Zentralblatt für allgemeine Pathologie und pathologische Anatomie, Jena, 1934, 60: 1-8.
  • Edith L. Potter:
    Bilateral renal agenesis.
    Journal of Pediatrics, St. Louis, 1946, 29: 68-76.
  • Edith L. Potter:
    Facial characteristics of infants with bilateral renal agenesis.
    American Journal of Obstetrics and Gynecology, St. Louis, 1946, 51: 885-888.
  • A. D. Bain, J. S. Scott:
    Renal agensis and severe urinary tract dysplasia. A review of 50 cases with particular reference to the associated anomalies.
    British Journal of Medicine, London, 1860, 1: 841.

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