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Posted On : 08 Oct 2020

15 days baby with congenital adrenal hyperplasia on Florinef, hydrocortisone, salt 5 mg. Suddenly feverish, dehydrated, ill, progressed to shock. His Na is 188. If we have no facilities for dialysis. How to manage, is it preferable to give Dexamethasone at that time, do we stop Florinef? What is the suitable fluid rate and concentration in case of good urine output, and what to do if anuric?

Expert Answer :

Answer Discussion :

E

Ebtihal Khalid

0

no not suitable to give dexamethason at that time and to stop florine and better to give half solution dextrose

4 years ago

H

HR Hasib

0

Cyproheptadine hydrochloride 5mg Dexamethasone 0.5 mg

How many time taking in a day
Or pricsription please
Iam drug addicted

4 years ago

C

Chandra Bhagat

0

Dextrose in baby sline increase by 25% to improve dehydration.
I hope no need to stop florinda and to give dexa.

4 years ago

Answer 1

This obvious case of hypernatremic dehydration is most probably iatrogenic due to oral salt intake {coupled with a possible reduced fluid, milk intake which is common in early neonatal period}. For routine cases of CAH on Florinef and hydrocortisone, there is no indication for exogenous salt administration and I cannot understand the rationale of oral salt in this baby. The baby should be managed as a case of moderate to severe degree hypernatremic dehydration with isotonic saline initially and then when the sodium level has reduced, change to 0.45 percent saline when the mental function has improved. Care should be taken to AVOID rapid reduction in sodium level {max reduction upto 10-12 mEq, L per day} to prevent the risk of brain edema which can be fatal if not detected and treated promptly.
Management of shock and dehydration will correct the oliguriapernuria if not progressed to intrinsic renal failure. Dialysis is not required for hypernatremia as it is due to dehydration, exogenous salt intake. Dialysis is required only in case of severe and symptomatic hyperkalemia and edema which is not evident in this case. Florinef can be withheld temporarily till the sodium returns to normal if the potassium level is NORMAL. Dexamethasone can be given during the shock state to tide over the crisis and then shifted back to Hydrocortisone {initially in higher doses and then reduced to usual doses}.
Fluid management of hypernatremic dehydration depends upon the severity which is usually underestimated. It is available in standard pediatric textbooks.
Anuria in such a case indicates renal parenchymal involvement usually secondary to prolonged pre-renal failure due to dehydration and may require dialysis if the baby has symptomatic hyperkalemia and edema. A trial of Furosemide challenge may be tried to open up the kidneys if the dehydration has been corrected adequately.
It would be wise to transfer the case to a higher center if the baby is not responding to standard therapy and before he may acutely need dialysis {as mentioned above}.

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