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enda-pediatric-fludrocortisone-infants-nacl

Recommends fludrocortisone 100 µg daily for children with primary adrenal insufficiency and confirmed aldosterone deficiency; for infants under 12 months, adds sodium chloride supplementation. Triggered by PAI with aldosterone deficiency, evidenced by salt craving, hyponatremia, hyperkalemia, or elevated renin with low aldosterone.

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Prescribe Fludrocortisone for Children with Aldosterone Deficiency; Provide Sodium Chloride Supplements for Infants

STEP 1 — Gather Information

Confirm primary adrenal insufficiency (PAI) diagnosis; assess aldosterone deficiency via plasma renin (elevated) and aldosterone (low), plus clinical signs: salt craving, hyponatremia, hyperkalemia. Determine patient age (infant <12 months vs child ≥1 year). Record weight and blood pressure.

STEP 2 — Rule In / Rule Out

Is aldosterone deficiency confirmed (low aldosterone with high renin or clinical salt-wasting)?

  • Yes → proceed to STEP 3.
  • No → do not initiate fludrocortisone or sodium chloride supplement; reassess for other causes.

STEP 3 — Classify or Stratify

Stratify by age:

  • Infant (<12 months)
  • Child (≥1 year)

STEP 4 — Decide

  • Infant: prescribe sodium chloride supplement 1–2 g/day divided into multiple feeds; monitor weight, serum electrolytes, and blood pressure.
  • Child: prescribe fludrocortisone 100 µg once daily (preferably morning); monitor for hypertension, edema, and electrolyte balance.

Clinical Guardrails / Mimics / Pitfalls

Avoid salt restriction in infants; excessive fludrocortisone may cause hypertension, edema, and suppressed renin. Do not use fludrocortisone alone in infants without sodium chloride supplementation due to mineralocorticoid resistance. Monitor for signs of over-replacement (hypertension, hypokalemia) and under-replacement (persistent salt craving, poor growth, hyponatremia). Do not rely solely on fludrocortisone dose adjustment for blood pressure control; consider antihypertensives if needed.

Clinical Clinical Example

A 7-month-old infant with known PAI presents with poor weight gain, frequent salt craving, serum sodium 128 mmol/L, potassium 5.8 mmol/L, renin markedly elevated, aldosterone low. Diagnosed with aldosterone deficiency. Prescribed sodium chloride supplement 1.5 g/day divided into breastfeeds. After one week, weight improved, sodium normalized to 135 mmol/L, potassium 4.2 mmol/L. Continued supplement with monthly electrolyte checks.

Source: Diagnosis and Treatment of Primary Adrenal Insufficiency: An Endocrine Society Clinical Practice Guideline, Endocrine Society, 2016, DOI:10.1210/jc.2015-1710

TODO: consider adding scripts/calc.py for the enda-pediatric-fludrocortisone-infants-nacl calculator

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