Acid Maltase Deficiency Myopathy Follow-up
- Author: Stephen Kishner, MD, MHA; Chief Editor: Stephen Kishner, MD, MHA more...
Acid maltase deficiency is an inherited, autosomal recessive disorder; therefore, there are no prevention measures for it.
Respiratory and heart complications are common in the infantile form of acid maltase deficiency (AMD). Severe muscle weakness, including weakness of the respiratory muscles, is a complication of all 3 types of AMD.
The infantile form of acid maltase deficiency has a very unfavorable prognosis. Death usually occurs between ages 6 months and 2 years. A less severe infantile form that exhibits a better prognosis and improved survival has been identified. Patients with the late infantile form may survive for several years.
Educating patients and family members thoroughly about this condition is important. Parents and caregivers need to be instructed in all aspects of taking care of an infant or child with acid maltase deficiency (AMD). Increasing public awareness of this disease also is important, as more research is needed to find a cure for AMD.
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