Download Morquio Syndrome is a disease when your insides like your organs

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Transcript
Morquio Syndrome is a disease when your insides like your organs grow but your body does not. So you die at an early age. You
cannot catch this disease, you can only be born with it. One example would be if the body does not grow but the heart grows
too big for the body to hold, causing death.
Symptoms
The following symptoms are associated with Morquio's syndrome:
Abnormal Heart
Development
Knock-knees
Abnormal skeletal
development
Widely spaced teeth
Enlarged heart
Dwarfism
Hypermobile joints
Large fingers
Bell-shaped chest (flared
ribs)
Heart murmur
Compression of spinal cord
Below average height for
certain age
Patients with Morquio's syndrome appear healthy at birth. They are often present with spinal deformity, and there is growth
retardation and possibly genu valgum in the second or third year of life. A patient with Morquio's syndrome is likely to die at an
early age. Life span: some can die as early as 2 or 3 and some can live up to 60 or 70 years old.
Respiratory
By late teens and adulthood, the ribs are nearly horizontal and the sagittal diameter of the chest is greater than average. As a result,
respiratory expansion becomes considerably impaired. Moreover, frequent upper respiratory tract infections may occur due to the
malformation of the rib cage. The trachea is narrow and may collapse during head flexion.
Cardiac
Cardiac complications may occur, including cardiomyopathy, valvular disease, or a late onset of aortic regurgitation. Cardiac
anomalies are predominately left sided. Severe cases have resulted in death before the age of 20.
Ear
Hearing loss, inguinal hernia, and hepatomegaly are all problems associated with the ear. Hearing aids and tubes are often times
required.
Eye
Corneal opacity is typical once patients reach age 5; glaucoma of the eyes and pigmentary retinal degeneration may occur in older
patients. Ophthalmologic examination is needed at frequent intervals.
Skin
Cutaneous abnormalities may also be present, including loose, thickened, tough, and inelastic skin, particularly of the extremities.
Generalized telangiectasia of the face and limbs has also been reported.
Teeth
Appropriate dental care is required due to the hypoplasia of tooth enamel. Teeth often brown and discolor easily. The permanent
posterior teeth have pointed cusps; there is often times pitting of the bucal surfaces. The teeth are also widely spaced.
Mental Capacity
Intelligence and mentality is typically not impaired in Morquio type A. However, progressive mental deficiency does occur in
Morquio Type B.