What are the Different Types of Pompe Disease?
The infantile form is most severe, and is often fatal. It usually presents by the age of 6 months.
Types of Pompe Disease
Pompe disease exists in three forms-infantile, juvenile, and adult-onset. The infantile form is most severe, and is often fatal. Symptoms usually appear by the age of 6 months. The heart, skeletal, and respiratory muscles are involved. Heart failure and respiratory failure are common causes of death in the affected patient.
The heart muscles are less likely to be involved when symptoms appear at an older age. Infants and children older than 6 months with the juvenile (intermediate) form generally have no cardiac disease. Symptoms in patients with adult-onset Pompe disease usually appear between the second and sixth decades of life. Muscle weakness leading to respiratory failure ultimately causes death. Involvement of the heart is not observed in the adult-onset form.
