What is Pulmonary Interstitial Glycogenosis (PIG)?
(PUHL-muh-ner-ee, in-ter-STISH-uhl, glahy-kuh-JEN-uh-sis)
Pulmonary interstitial glycogenosis:
- Is a type of interstitial lung disease that causes babies to have trouble breathing
- Is a very rare disorder
- Was only first described in 2002
- Is sometimes called P.I.G. or PIG
What Causes Pulmonary Interstitial Glycogenosis?
- The cause is unknown.
- Many babies with PIG also have preterm birth and congenital heart disease.
What are Signs and Symptoms of Pulmonary Interstitial Glycogenesis?
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 or hypoxemia)
- Pulmonary hypertension (a type of high blood pressure) in some babies
How is Pulmonary Interstitial Glycogenosis Diagnosed?
- Visits with a lung doctor (called a pulmonologist)
- Chest CT scan
- Lung biopsy is sometimes needed
How is Pulmonary Interstitial Glycogenosis Treated?
- Additional oxygen (usually needed)
- Intravenous (IV) steroids if needed
- Additional nutrition to help gain weight and growth if needed
- Flu shot every year to lower the chance of getting infections
