Thiamine deficiency in infancy: Long-term follow-up

Aviva Mimouni-Bloch, Hadassa Goldberg-Stern, Rachel Strausberg, Amichai Brezner, Eli Heyman, Dov Inbar, Sara Kivity, Alex Zvulunov, Ignacio Sztarkier, Rami Fogelman, Aviva Fattal-Valevski

Research output: Contribution to journalArticlepeer-review

34 Scopus citations

Abstract

Background In 2003, several hundred Israeli infants risked thiamine deficiency after being fed a soy-based formula deficient in thiamine. Approximately 20 patients were seriously affected, and three of them died. We report the clinical presentation of acute encephalopathy in 11 children and the long-term sequelae of eight children who initially survived. Patients In the acute phase, six had bulbar signs, five had ophthalmologic signs and two had phrenic neuropathy. Three of the five patients with cardiac involvement had cardiomyopathy and died in the acute phase. One patient presented with a complete atrioventricular block. Results In the long-term, one patient, who was in a chronic vegetative state, died after 6 years. Seven children exhibited mental retardation and motor abnormalities, six developed severe epilepsy, two early kyphoscoliosis, and one patient remained with a complete atrioventricular block. Conclusions Infants who survive severe infantile thiamine deficiency have serious residual motor and cognitive sequelae as well as epilepsy.

Original languageEnglish
Pages (from-to)311-316
Number of pages6
JournalPediatric Neurology
Volume51
Issue number3
DOIs
StatePublished - 1 Jan 2014

Keywords

  • Wernike encephalopathy
  • atrioventricular block
  • cardiomyopathy
  • thiamine
  • vitamin deficiency

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