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Adrenocortical insufficiency

  1. ACTH deficiency is usually secondary to pituitary hypoplasia, destructive lesions such as craniopharyngioma or congenital defects.
  2. Chronic idiopathic adrenocortical insufficiency caused by damage to the adrenal glands is rare in children
  3. Diag -  Low serum Na+, cl- with hyperkalemia.
    1. Increased urinary Na+ Cl- and decreased K+
    2. Eosinophilia
    3. Calcification on x-ray abdomen

Cushing syndrome

Cushing disease -bilateral adrenal hyperplasia secondary to excessive pituitary ACTH often from a basophilic pituitary adenoma
Cushing syndrome in children
---40-50%-cushing disease
---45% adrenal carcinoma
---10%adrenal adenoma
Diag:- Polycythemia. eosinopenia and lymphopenia.
-   Elevated Cortisol levels
-   Overnight dexamethasone suppression test

Familial Glucocorticoid Deficiency

  1. This form of chronic adrenal insufficiency is characterized by isolated deficiency of glucocorticoids, elevated levels of ACTH, and normal aldosterone production
  2. The salt-losing manifestations present in most other forms of adrenal insufficiency do not occur; instead, patients present primarily with hypoglycemia, seizures, and increased pigmentation during the 1st decade of life.
  3. The disorder affects both sexes equally and is inherited in an autosomal recessive manner.
  4. There is marked adrenocortical atrophy with relative sparing of the zona glomerulosa.
  5. A number of mutations in the gene for the ACTH receptor have been described in some (approximately 40%) but not all of these patients.
  6. These patients don’t present with features of mineralocorticoid deficiency (shock, dehydration, low sodium and high potassium); which are usually seen in CAH due to 21 alpha hydroxylase deficiency.

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