Thiamine responsive acute life threatening metabolic acidosis in exclusively breast-fed infants.

Background: Acute life threatening metabolic acidosis in exclusively breast fed infants due to thiamine deficiency is not described. Kashmir valley, a north Indian state has a population that largely consumes polished rice.

Methods: A six months prospective descriptive study of infants who presented with acute life threatening metabolic acidosis (Blood pH ≤ 7.0) due to thiamine deficiency.

Results: Twenty three infants (Eleven male; Twelve female) in the age range of 32 days to 4 months had a pH of ≤7 at admission. Onset of moaning was immediate (2-24 hours). Blood lactate levels were more than 15mmol/L. Blood thiamine levels of six infants in whom it was done ranged from 11-69 nmol/L (control 78-185 nmol/L). All infants were exclusively breast fed. Maternal staple diet consisted of polished rice. All mothers consumed rice after washing it thrice. Twelve lactating mothers were on customary dietary restrictions. Practice of straining rice after cooking was observed in thirteen. The commonest symptoms were irritability (82%) and reflux (56%). Commonest signs were tachycardia (100%) and moaning (73%). At presentation 52% were in cardiogenic shock. Response to thiamine was dramatic with moaning and irritability subsiding in two hours and tachycardia in four hours. Adequate perfusion was achieved in one hour. Eighteen patients seen at six months follow up had normal neurodevelopment.

Conclusions: Thiamine deficiency in an infant can present as sudden onset metabolic acidosis. If treated early, metabolic acidosis due to thiamine deficiency is associated with good immediate and long term prognosis even if pH is less than 7 at presentation.