Thiamine-responsive, life-threatening, pulmonary hypertensive crisis with encephalopathy in young infants: A case series.

Thiamine (Vitamin B1) deficiency (TD), although reduced in incidence, is still seen in infants. We describe a rarely reported form of infantile TD with life-threatening pulmonary hypertensive crisis and severe encephalopathy, with dramatic response to thiamine supplementation. Study design: Descriptive case series. Six young infants with mean age 76 days (range 1-3 months), manifesting rapidly progressive encephalopathy and cardio-pulmonary arrest were included. All infants underwent cardiac, neuroimaging and metabolic evaluations. All six infants had similar presentation with severe pulmonary arterial hypertension (PAH), hypotensive shock, metabolic acidosis and severe encephalopathy. All infants were exclusively breast-fed. Thiamine treatmwnt resulted in dramatic improvement in haemodynamic and neurological function in all the infants. There were no major neurological deficits on follow up. A high index of suspicion is warranted for this rarely described form of TD, as early identification helps in preventing mortality and morbidity.

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Déclaration de conflit d'intérêts

Declaration of competing interest All authors of the manuscript titled “Thiamine-responsive, life-threatening, pulmonary hypertensive crisis with encephalopathy in young infants: A case series” hereby declare that none of us have any conflict of interest in relation to this publication. And, we also declare that, there is no financial and personal relationships with other people or organizations that could inappropriately influence our work.