How should adrenal white matter dysplasia do

summary

After the occurrence of adrenoleukodystrophy, there will be psychomotor disorders, decreased vision and other manifestations, which need targeted treatment. Let's take a look at the adrenal white matter dysplasia how to do it.

How should adrenal white matter dysplasia do

First of all: at present, some people use diet therapy to limit the intake of VLCFA in their diet and take Lorenzo oil at the same time, so as to reduce the synthesis of VLCFA and make C26: C22 return to normal. After the above treatment, the patient's peripheral nerve conduction velocity was improved, fat accumulation was reduced, and the condition was relieved.

Second: adrenocortical hormone replacement therapy: most children have adrenocortical insufficiency, so corticosteroid replacement therapy is often necessary, but this treatment has no effect on nervous system lesions, can not improve symptoms, and can not prevent the deterioration of lesions. It is mainly suitable for Addison patients, but has no obvious improvement for brain type patients.

Finally, bone marrow transplantation can correct the metabolic disorder of VLCFA, reconstruct the enzyme activity, improve the clinical symptoms, prevent dementia, and the effect of early treatment is better. With the improvement of bone marrow transplantation technology and the early detection of asymptomatic ALD, bone marrow transplantation has a certain therapeutic prospect. However, bone marrow transplantation itself has a certain mortality, and the cost of treatment is relatively much higher, which is suitable for early childhood patients.

matters needing attention

The prognosis of this disease is poor, generally 1-3 years after neurological symptoms. In the active treatment at the same time, can eat some rich in unsaturated fatty acids diet, help to stabilize the disease.