Question of the Week

Question :

Posted On : 21 Feb 2007

3 months male child wt-3.3 kg, repeatedly admitted after every 7days from the age of 2 months of life with complaints of vomiting, fits or respiratory distress. He is on exclusive mother feed. He has been found to have persistent hyponatremia, hyperkalemia and metabolic acidosis and was HYPOTENSIVE. His serum ACTH, 17 OH-progesterone and plasma renin were sent after 12 hours of commencement of normal saline. Results are following: Serum ACTH - 135 mg-dl {normal-46 mg,dl}
Plasma renin-0.29 mg,dl
17 OH-Progesterone -12 mg,dl {age adjusted Less Than 2mg,dl}
SERUN ANION GAP-25.
TREATMENT GIVEN: I,V FLUID Normal Saline FOR INITIAL two DAYS, TREATMENT FOR HYPERKALEMIA, HYROCORTISONE in stress dose {40mg,m2,day in three divided doses continued for 7days ,then shift to 20 mg,m2,day in divided doses}, FLORINEF }0.2 MG,DAY IN TWO DIVIDED DOSES}
Serum CORTISOL, ALDOSTERONE TESTOSTERONE could not be sent initially due to financial constraint. Later when patient remained hyponatremic {plasma Na ranges between 129-131mmol,l} on 0.2mg Florinef, diagnosis was reevaluated and following investigation were sent. {Patient is continously taking medication when investigation was sent}
-TESTOSTERONE LEVEL- LOW NORMAL
-SERUM CORTISOL - LOW NORMAL
-ALDOSTERONE -REPORT AWAITED.
MY QUESTIONS ARE:
WHAT ARE THE DIFFERENTIAL DIAGNOSIS? WHAT FURTHER MANAGEMENT IS REQUIRED?

Expert Answer :

No expert answer available.

Answer Discussion :

Dr.Sudha Singh