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What is Majewski Osteodysplastic Primordial Dwarfism?

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  • Written By: Meshell Powell
  • Edited By: Melissa Wiley
  • Last Modified Date: 04 November 2016
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Majewski osteodysplastic primordial dwarfism is a rare form of dwarfism that causes a child to have an exceptionally short stature. Slow growth begins while the mother is pregnant and continues throughout the person's life. Most children born with this type of dwarfism will not even reach the size of an average newborn until they are more than two years of age. Several physical differences and health concerns are common among those born with this form of dwarfism, including hip dislocation, seizures, and breathing difficulties.

Many patients with Majewski osteodysplastic primordial dwarfism will have sparse amounts of hair, not just on the head, but the eyebrows and rest of the body are typically affected as well. Microcephaly, a condition marked by the head being considerably smaller than that what is considered normal, is also typically present. Scoliosis, an abnormal curvature of the spine, is also a common affliction among those born with Majewski osteodysplastic primordial dwarfism.

Aside from physical differences, there are some potential health challenges for patients born with Majewski osteodysplastic primordial dwarfism. Failure to thrive syndrome is common among these newborns. Failure to thrive is a term given to infants who do not grow in height or weight at a rate that is considered normal. Feeding problems may be present as well, adding to the potential dangers of this condition. Breathing problems may also be a problem in babies born with this type of dwarfism, often requiring an extended stay in the hospital.

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Some of the more noticeable signs of Majewski osteodysplastic primordial dwarfism often include a high-pitched or squeaky voice, small teeth, or hyperopia, more commonly known as being far-sighted. Eyeglasses are commonly needed at an early age for these patients. Children with this form of dwarfism are often sick with various infections or other medical issues, especially during the first few years of life.

The prognosis for patients diagnosed with Majewski osteodysplastic primordial dwarfism is varied and depends on the severity of accompanying health issues. Stroke and kidney failure are relatively common among patients with this disorder, and life expectancy is typically quite low. Many children do not survive past infancy or early childhood, although there have been documented instances of this type of primordial dwarfism in which the patient survives more more than two decades. It is of vital importance that the person diagnosed with this disorder seeks regular medical attention so that any potential problems can be caught and treated as early as possible.

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