Webster's New World Medical Dictionary



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Webster s New World Medical Dictionary (1)

Maroteaux-Lamy syndrome
A form of
mucopolysaccharidosis with onset before age 3 that
is characterized by an inability to metabolize der-
matan 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 col-
lection 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 sur-
gery. Due to the heart damage caused by the syn-
drome, death usually occurs before the patient
reaches age 40. Also known as mucopolysaccharido-
sis type VI. See also 
mucopolysaccharidosis.
marriage, cousin
See 
consanguinity.
marrow
See 
bone marrow.
marsh fever
See 
malaria.
Martin-Bell syndrome
See 
fragile X syndrome.
MASA syndrome
A syndrome named for its
characteristics: mental retardation, aphasia, shuf-
fling gait, and adducted thumbs. Features of the syn-
drome include mental retardation and aphasia
(lack of speech); adducted (clasped) thumbs,
absent extensor pollicis longus and/or brevis mus-
cles to the thumb, shuffling gait, and leg spasticity;
small body size; and lumbar lordosis (swayback).
MASA is inherited as an X-linked trait, and so it
affects mainly boys. Also known as clasped thumbs
and mental retardation, congenital clasped thumbs
with mental retardation, adducted thumbs with
mental retardation, and Gareis-Mason syndrome.
See also 
adducted thumbs.

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