Primordial dwarfism

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Primordial dwarfism is a rare form of dwarfism that results in a smaller body size in all stages of life beginning from before birth. The five subtypes of Primordial Dwarfism (listed below) are the most severe forms of the 200 types of dwarfism, and it is estimated that there are only 100 individuals in the world history with the disorder,[1] 40 of them in the US. Other sources list the number of persons currently afflicted as high as 200 in North America. It is unlikely that sufferers of primordial dwarfism will live past the age of 30.[2]

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[edit] Diagnosis

Since primordial dwarfism disorders are extremely rare, misdiagnosis is common. Because children with PD do not grow like other children, poor nutrition, a metabolic disorder, or a digestive disorder may be diagnosed initially. The correct diagnosis of PD may not be made until the child is 5 years old and it becomes apparent that the child has severe dwarfism.

[edit] Treatment

What causes primordial dwarfism is not yet known, and there are as yet no effective treatments for the disorder. The lack of normal growth in the disorder is not due to a deficiency of growth hormone, as in hypopituitary dwarfism. Administering growth hormone, therefore, has little or no effect on the growth of the individual with PD.

[edit] Types

There are many subtypes of Primordial Dwarfism. Some of these subtypes include:

  1. Seckel syndrome
  2. Majewski osteodysplastic primordial dwarfism (MOPD) type I
  3. Majewski osteodysplastic primordial dwarfism (MOPD) type II
  4. Majewski osteodysplastic primordial dwarfism (MOPD) type III
  5. Meier-Gorlin Syndrome
  6. Russell-Silver syndrome

[edit] See also

[edit] External links

[edit] References

  1. ^ http://tlc.discovery.com/tvlistings/episode.jsp?episode=0&cpi=55082&gid=0&channel=TLC
  2. ^ As seen on the 2006 TLC/Channel Four program on primordial dwarfism, The Smallest People in the World,
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