Maroteaux-Lamy syndrome

Maroteaux-Lamy syndrome A form of mucopolysaccharidosis with onset before age 3 that is characterized by an inability to metabolize dermatan sulfate. This leads to abnormal accumulation of dermatan sulfate, mostly in the peripheral tissues. The result is mild to severe changes in muscle, bone, skin, and other tissues, particularly the heart. Diagnosis is made through examination of leukocytes and cultured skin fibroblasts, or 24-hour urine collection to search for high levels of dermatan sulfate. There is no current treatment for Maroteaux-Lamy syndrome, but individual symptoms and problems may respond to physical therapy, medication, or surgery. Due to the heart damage caused by the syndrome, death usually occurs before the patient reaches age 40. Also known as mucopolysaccharidosis type VI. See also mucopolysaccharidosis.