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Diseases

Genetic and Rare Diseases Information Center (GARD)

Pierson syndrome


Other Names for this Disease
  • Microcoria - congenital nephrotic syndrome
  • Microcoria - congenital nephrosis
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Your Question

My 5-month-old son has Pierson syndrome. He has renal failure and pin-point pupils and now is treated by peritoneal dialysis. I would like to ask about the prognosis and the serious complications associated with this disease.

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What are the signs and symptoms of Pierson syndrome?

The features and severity of Pierson syndrome can vary among affected people. Affected infants are usually born with serious and progressive kidney disease due to congenital nephrotic syndrome, although some do not have kidney failure until adulthood. Most require a renal transplant for end-stage kidney disease within the first decade of life.

Ocular (eye) abnormalities are another common feature of Pierson syndrome. Most affected infants are born with abnormally small pupils (microcoria). Other ocular abnormalities may include cataractsglaucoma, retinal detachments, and blindness.

Those that survive past infancy typically have neurological disabilities and developmental delays. Many children with Pierson syndrome don't achieve normal milestones such as sitting, standing, and talking.[1]
Last updated: 3/23/2016

What is the long-term outlook for people with Pierson syndrome?

Pierson syndrome carries a serious prognosis because of the eventual failure of the kidneys.[1] Most affected infants progress towards renal failure within the first days or months of life.[2] A kidney transplant may restore kidney function.[1]

Glaucoma, cataracts and retinal detachments require their usual treatment. Lifelong monitoring is required, but affected children may not live beyond the first decade of life.[1]
Last updated: 3/23/2016

References
Other Names for this Disease
  • Microcoria - congenital nephrotic syndrome
  • Microcoria - congenital nephrosis
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.