S. M. M. Musabbir Uddin

ACTH deficiency arises as a result of decreased or absent production of adrenocorticotropic hormone (ACTH) by the pituitary gland.

A decline concentration of ACTH in the blood leads to a reduction in the secretion of adrenal hormones, resulting in adrenal insufficiency. It leads to weight loss, lack of appetite, weakness, nausea, vomiting and hypotension.

Sign & Symptoms:

ACTH deficiency can either be congenital or acquired, and it is manifestations are clinically indistinguishable from those of glucocorticoid deficiency. Symptoms included weakness, hypotension, nausea, vomiting and lack of appetite. Low blood level of sugar and dilution of hyponatremia.

May occur, however potassium are typically normal as affected patient are deficient in glucocorticoids and not mineralocorticoids due to their renin-angiotensin-aldosterone system. The pituitary gland ACTH hormone maybe undetectable in blood test but the level of adrenal hormone cortisol is abnormally low. Concentration of 17-hydroxycorticosteroid and 17-ketoacids produced from the adrenal cortex. Some adrenal hormones that are decreased are precursors of male sex hormone and also known as “pre-androgens”.

Although males with this disorder usually have a normal hair pattern but in female may have little pubic and axilla hair.

Causes:

The exact causes of ACTH deficiency are remaining unknown. A defect in the brain’s hypothalamus or in the pituitary gland may be cause of ACTH deficiency. Also there is a congenital form of ACTH deficiency that has been tracked to mutation of the T-Box 19 gene on the long arm of chromosome one and the corticotrophin releasing hormone gene on the long arm of chromosome eight. The heritance pattern is thought to be autosomal recessive.

Genetic diseases are determined by the combination of genes for particular traits are on the chromosomes received from father and mother. The risk for two carrier parents to both pass the defective gene and, therefore have an affected child is 25% with each pregnancy. The risk to have a child who is a carrier like parents is 50% with each pregnancy.

Secondary adrenal insufficiency results from insufficient production or release of pituitary hormone ACTH. It may be caused by prolonged corticosteroid therapy. ACTH production doesn’t return to normal for several months after completion of therapy. Central nervous system tumors sometimes are affecting the pituitary glands. Sometimes granuloma disease may affect the pituitary glands. Sometimes death of pituitary gland after the pregnancy may affect the ACTH insufficiency.

Diagnosis:

When ACTH deficiency is suspected, blood samples are taken for analysis, especially of the level of cortisol in the blood. If the concentration of the cortisol is low, then it is typically indicate that the level of ACTH is very low. Sometimes AXTH stimulation test may be administrated.

Treatment:

Hormone replacement therapy with cortisol is the treatment of choice for the disorder, with such therapy; the patient can lead normal life.

The writer is a Students Universal Medical College