Disease of Morquio

The disease of Morquio, or Mucopolysaccharidose of the type IV, is a Maladie lysosomale, of the family of the mucopolysaccharidoses and clinically dominated by the osseous attack. Its incidence is variable according to the countries: 1/76 000 in Northern Ireland, 1/300 000 in British Columbia in Canada and 1/640 000 in Australia where it is 6 times less frequent than the mucopolysaccharidose of the type I. It thus represents only one small proportion of the whole of the mucopolysaccharidoses whose total incidence is of 1/29 000 in Australia.

Form clinical

There exist several forms of the disease of Morquio:

the type has, severe form or moderate form
the type B

Treatment

There does not exist any specific treatment of the disease of Morquio of the type A. medullary transplantation is not indicated, because it does not have or little effect on the osseous attack. A murine model was generated to test the effectiveness of possible therapeutic. The treatment is thus primarily symptomatic and rests in particular on a very specialized orthopedic assumption of responsibility. In the event of intervention, the anesthetic assumption of responsibility is also very specific because of the risks to intubation because of instability of the cervical column.

Mode of transmission

recessive autosomic Transmission

Sources

Disease of Morquio Association To overcome the Diseases Lysosomales

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