Hunter syndrome history and symptoms: Difference between revisions

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The symptoms of Hunter syndrome (MPS II) are generally not apparent at birth, but usually start to become noticeable after the first year of life. Often, the first symptoms of Hunter syndrome may include [[inguinal hernias]], [[ear infection]]s, runny noses, and [[common cold|colds]]. Since these symptoms are quite common among all infants, they are not likely to lead a doctor to make a diagnosis of Hunter syndrome right away. As the buildup of GAG continues throughout the cells of the body, signs of Hunter syndrome become more visible.
The symptoms of Hunter syndrome (MPS II) are generally not apparent at birth, but usually start to become noticeable after the first year of life. Often, the first symptoms of Hunter syndrome may include [[inguinal hernias]], [[ear infection]]s, runny noses, and [[common cold|colds]]. Since these symptoms are quite common among all infants, they are not likely to lead a doctor to make a diagnosis of Hunter syndrome right away. As the buildup of GAG continues throughout the cells of the body, signs of Hunter syndrome become more visible.
==History and Symptoms==
==History and Symptoms==
Juvenile form (early-onset, severe form):
===Juvenile Form====
It is the early-onset and severe form of the disease.


*Aggressive behavior
*Aggressive behavior
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*Spasticity
*Spasticity


Late (mild) form:
===Late Form===


*Mild to no mental deficiency
*Mild to no mental deficiency


Both forms:
===Common Features===


*[[Carpal tunnel syndrome]]
*[[Carpal tunnel syndrome]]
*Coarse features of the face
*Coarse features of the face
*Deafness (gets worse over time)
*Deafness
*Increased hair ([[hypertrichosis]])
*[[Hypertrichosis]]
*Joint stiffness
*Joint stiffness
*Large head ([[macrocephaly]])
*[[Macrocephaly]]
 
The continued storage of GAG in cells can lead to organs being affected in important ways. The thickening of the heart valves along with the walls of the heart can result in progressive decline in cardiac function. The walls of the airway may become thickened as well, leading to breathing problems while sleeping (obstructive airway disease). People with Hunter syndrome may also have limited lung capacity due to pulmonary involvement. As the liver and spleen grow larger with time, the belly may become distended, making hernias more noticeable. All major joints (including the wrists, elbows, shoulders, hips, and knees) may be affected by Hunter syndrome, leading to joint stiffness and limited motion. Progressive involvement of the finger and thumb joints results in decreased ability to pick up small objects. The effects on other joints, such as hips and knees, can make it increasingly difficult to walk normally. If carpal tunnel syndrome develops, a further decrease in hand function can occur. The bones themselves may be affected, resulting in short stature.  Finally, the storage of GAG in the brain can lead to delayed development with subsequent mental retardation. The rate and degree of progression may be different for each person with Hunter syndrome.
There is a broad range of severity in the symptoms of Hunter syndrome. It is important to note that though the term "mild" is used by physicians in comparing people with Hunter syndrome, the effects of even mild disease are quite serious. Two of the most significant areas of variability concern the degree of mental retardation and expected lifespan.


==References==
==References==

Revision as of 18:39, 26 February 2013

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

The symptoms of Hunter syndrome (MPS II) are generally not apparent at birth, but usually start to become noticeable after the first year of life. Often, the first symptoms of Hunter syndrome may include inguinal hernias, ear infections, runny noses, and colds. Since these symptoms are quite common among all infants, they are not likely to lead a doctor to make a diagnosis of Hunter syndrome right away. As the buildup of GAG continues throughout the cells of the body, signs of Hunter syndrome become more visible.

History and Symptoms

Juvenile Form=

It is the early-onset and severe form of the disease.

  • Aggressive behavior
  • Hyperactivity
  • Decreased mental function
  • Severe mental retardation
  • Spasticity

Late Form

  • Mild to no mental deficiency

Common Features

References

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