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Pulmonary Interstitial Glycogenosis (PIG)

What Is Pulmonary Interstitial Glycogenosis (PIG)?

(puhl-muh-ner-ee, in-ter-stish-uhl, glahy-kuh-jen-uh-sis)

Pulmonary interstitial glycogenosis (PIG) is:

  • A type of interstitial lung disease that causes babies to have trouble breathing
  • A very rare disorder
  • Only first described in 2002
  • Sometimes called P.I.G. or PIG

Cause of Pulmonary Glycogenosis

  • The cause is unknown. 
  • Many babies with PIG also have preterm birth and congenital heart disease.

Signs and Symptoms

Symptoms are only seen in young babies. Babies usually show symptoms at birth or soon after birth. Symptoms include:

  • Fast breathing (called tachypnea)
  • Sinking in of the areas below the ribs, between the ribs, and in the neck with breathing (called retractions)
  • Abnormally low amount of oxygen in the blood (called low oxygen saturations)
  • Some babies may have pulmonary hypertension

Diagnosis of Pulmonary Glycogenosis

  • Visits with a lung doctor (called a pulmonologist)
  • Lung biopsy is usually needed.

Treatment for Pulmonary Glycogenosis

  • Additional oxygen (usually needed)
  • Intravenous (IV) steroids if needed
  • Additional nutrition to help gain weight and grow if needed
  • Flu shot every year to lower the chance of getting infections

Contact Us

For questions or more information, contact:
The Rare Lung Diseases Program
Division of Pulmonary Medicine
Cincinnati Children’s Hospital Medical Center

Last Updated 08/2019

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