Electrolyte Imbalance in pediatric oncall

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Rakesh Kumar, Puja Bhagat Department of Pediatrics, Katihar Medical College, Katihar, Bihar, India ...

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Question
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 hyponateramia, hyperkalemia and metabolic acidosis AND was HYPOTENSIVE. his serum ACTH, 17 OH-progesterone and plasma renin was 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 < 2mg/dl) ---SERUN ANION GAP ------25 TREATMENT GIVEN: 1-I/V FLUID NORMAL SALINE FOR INITIAL two DAYS 2-TREATMENT FOR HYPERKALEMIA 3-HYROCORTISONE on stress dose(40mg/m2/day in three divided doses continued for 7days ,then shift to 20 mg/m2/day in divided doses) 4- FLORINEF 0.2 MG/DAY IN TWO DIVIDED DOSES serum CORTISOL, ALDOSTERONE TESTOSTERONE couldnot be sent initially due to financial constrained. Later when patient remain hyponateremic ( plasma Na ranges between 129----131mmol/l) on 0.2mg Florinef , diagnosis was reevaluated and following investigation was sent , note patient is continuously taking medication when investigation was sent ---TESTOSTERONE LEVEL---- LOW NORMAL -SERUM CORTISOL ------- LOW NORMAL -ALDOSTERONE ---REPORT AWAITED MY QUESTIONS ARE: 1- WHAT ARE THE DIFFERENTIAL DIAGNOSIS 2- WHAT FURTHER MANAGEMENT REQUIRED
Answer
Rule out the following: PSEUDOHYPOALDOSTERONISM 17-Hydroxylase Deficiency Syndrome Adrenal Hypoplasia KETOACIDOSIS DIABETIC KETOACIDOSIS RENAL FAILURE Back to All Questions

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