The most likely underlying cause of the findings in this patient is a defect in which of the following? a. angiotensin-converting enzyme b. aquaporin c. 11a-Hydroxylase d. renin e. vasopressin receptors
A 3-month-old boy is brought to the emergency department because of a 20day history of lethargy. Physical examination shows no other abnormalities. The results of laboratory studies are shown:
serum:
Na+ 165 mEa/L (N=139-146)
Cl- 130 mEq/L (N=95-105)
Osmolality 334 mOsmol/kg H2O (N=282-295)
urine:
specific gravity 1.001
osmolality 117 mOsmol/kg H2O (N>200)
He is admitted to the hospital. His urine output is increased. His serum ADH (vasopressin) concentraion is 24 pg/mL (N=1-5); aldosterone and renin concentrations are within the reference ranges. The urine osmolality remains unchanges after administration of 1-deamino-B-arginine vasopressin. An MRI of the brain and pituitary gland shows no abnormalities. Ultrasonography shows normal kidneys. The most likely underlying cause of the findings in this patient is a defect in which of the following?
a. angiotensin-converting enzyme
b. aquaporin
c. 11a-Hydroxylase
d. renin
e. vasopressin receptors
The renal urine output is increased along with increased sodium and chloride concentration.
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