Explain Adrenal Hypoplasia?

March 02 22:41 2019 Print This Article

What is an adrenal hypoplasia?
It is defined as a disorder in which there is an involvement of the endocrine tissues mainly adrenal glands. It is a x linked and genetic disorder. It mainly involves the males.

What are the signs and symptoms of adreno hypoplasia?
It includes the vomiting, difficulty in eating, dehydration or a low blood pressure which is referred as the hypo glycemia and also involves the shock. The adult onset cases have also been described. The males which are affected do not have a male sex hormones which may lead to the under development of reproductive tissues, un descended testes which is referred as the cryptor chidism, puberty in the later life and the inability to give birth to a child which is referred as the infertility. These features are referred as the hypo gonadotropic hypo gonadism. It rarely affects the females and a few cases of this dis order have seen in which the female sex hormones are absent. It may lead to the under development of reproductive tissues, absence of the menstruation and puberty in the later life.

What is the presentation of adrenal hypo plasia?
It includes the reduction in the function of adrenal gland. It occurs due to the incomplete development of outer layer of gland which is referred as the adrenal cortex. This disorder starts in the infancy or child hood. It may lead to the vomiting, difficulty in eating, dehydration or a low blood pressure which is referred as the hypo glycemia and also involves the shock. The adult onset cases have also been described. The males which are affected do not have a male sex hormones which may lead to the under development of reproductive tissues, un descended testes which is referred as the cryptor chidism, puberty in the later life and the inability to give birth to a child which is referred as the infertility. These features are referred as the hypo gonadotropic hypo gonadism. It rarely affects the females and a few cases of this disorder have seen in which the female sex hormones are absent. It may lead to the under development of reproductive tissues, absence of the menstruation and puberty in the later life.

What is the genetics of adrenal hypo plasia?
It includes the mutations in gene NROB1 which is present on the X chromosome. It leads to the X linked disorder. This gene guides the making of transcription factor protein known as the DAX 1 which controls the activity of few genes. In case of mutation or deletion of NROB 1 gene there is a problem which leads to the formation of adrenal glands in the brain or reproductive tissue involving the hypo thalamus and pituitary gland. It may involve the ovaries or testes. They are very important for the production of hormones that control other activities. There is a requirement of these hormones in a certain amount otherwise one sees a signs and symptoms of this disorder. It is inherited and x linked.

How useful was this post?

Click on a star to rate it!

  Article "tagged" as: