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Severe hypoglycemic encephalopathy due to hypoallergenic formula in an infant

Erika Ogawa1, Mika Ishige1, Yuno Takahashi1

  • 1Department of Pediatrics and Child Health, Nihon University School of Medicine, Tokyo, Japan.

Pediatrics International : Official Journal of the Japan Pediatric Society
|June 22, 2016
PubMed

Insights

Prolonged exclusive use of hypoallergenic formula (HF) can lead to severe biotin and carnitine deficiencies in infants, causing metabolic crises and irreversible brain damage. Healthcare providers must caution parents about the risks associated with long-term HF feeding.

Area of Science:

  • Pediatric Nutrition
  • Metabolic Disorders
  • Infant Health

Background:

  • Hypoallergenic formulas (HF) are often used for infants with suspected or confirmed allergies.
  • Nutritional adequacy of long-term exclusive HF feeding requires careful consideration.

Observation:

  • A 7-month-old infant presented with severe hypoglycemia, metabolic acidosis, and encephalopathy.
  • The infant had been exclusively fed a hypoallergenic formula (HF) since birth.
  • Initial investigations ruled out inborn errors of metabolism.

Findings:

  • Treatment with glucose, vitamins, biotin, and L-carnitine resolved the acute metabolic crisis.
  • The infant developed irreversible brain damage despite prompt treatment.
  • Biotin and L-carnitine deficiencies were diagnosed, attributed to prolonged exclusive HF feeding.

Implications:

  • Exclusive, prolonged use of hypoallergenic formulas may lead to specific nutrient deficiencies (biotin, carnitine).
  • Healthcare professionals should educate parents on the potential risks and nutritional limitations of long-term exclusive HF use.
  • Early recognition and management of metabolic decompensation in infants are crucial, even when the cause is nutritional.

Keywords:
acute encephalopathybiotin deficiencycarnitine deficiencyhypoallergenic formulahypoglycemia

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