A 10 day old male infant is presented to the ER with severe electrolyte imbalance and life-threatening arrhythmia. The parents reported a 3-day history of poor feeding and lethargy. On examination, he was bradycardic with signs of dehydration. Blood labs showed hyponatremia and hyperkalemia. A diagnosis of congenital adrenal hyperplasia with salt-wasting crisis secondary to 21-hydroxylase deficiency was confirmed by genetic studies.
What is most likely underlying mechanism of this salt wasting?

Question

A 10 day old male infant is presented to the ER with severe electrolyte imbalance and life-threatening arrhythmia. The parents reported a 3-day history of poor feeding and lethargy. On examination, he was bradycardic with signs of dehydration. Blood labs showed hyponatremia and hyperkalemia. A diagnosis of congenital adrenal hyperplasia with salt-wasting crisis secondary to 21-hydroxylase deficiency was confirmed by genetic studies.  
 What is most likely underlying mechanism of this salt wasting?

Accepted Answer

lack of synthesis of cortisol and aldosterone

Answer

normal feedback mechanisms do not control doc production

Answer

lack of cortisol raises ACTH drive thus more DOC is produced

Answer

decreased cortisol and adrenal androgens production

Answer

deoxycorticosterone (DOC) is produced in excess.

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A 10 day old male infant is presented to the ER with severe electrolyte imbalanc...

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