Pediatr Gastroenterol Hepatol Nutr.  2019 Sep;22(5):493-499. 10.5223/pghn.2019.22.5.493.

Thiamine Deficiency in a Child with Short Bowel Syndrome and Review

• Roilides I
• Vasilaki K
• Xinias I
• Iosifidis
• Antachopoulos C
• Roilides E

• KMID: 2457752
• DOI: http://doi.org/10.5223/pghn.2019.22.5.493

Abstract

Thiamine (vitamin B₁) is a water-soluble vitamin that is not endogenously synthesized in humans. It is absorbed by the small intestine, where it is activated. Its active form acts as a coenzyme in many energy pathways. We report a rare case of thiamine deficiency in a 3.5-year old boy with short bowel syndrome secondary to extensive bowel resection due to necrotizing enterocolitis during his neonatal age. The patient was parenteral nutrition-dependent since birth and had suffered from recurrent central catheter-related bloodstream infections. He developed confusion with disorientation and unsteady gait as well as profound strabismus due to bilateral paresis of the abductor muscle. Based on these and a very low thiamine level he was diagnosed and treated for Wernicke encephalopathy due to incomplete thiamine acquisition despite adequate administration. He fully recovered after thiamine administration. After 1999 eight more cases have been reported in the PubMed mostly of iatrogenic origin.

Keyword

Wernicke encephalopathy; Total parenteral nutrition; Thiamine deficiency; Short bowel syndrome

MeSH Terms

Child*
Enterocolitis, Necrotizing
Gait Disorders, Neurologic
Humans
Intestine, Small
Male
Parenteral Nutrition, Total
Paresis
Parturition
Short Bowel Syndrome*
Strabismus
Thiamine Deficiency*
Thiamine*
Vitamins
Wernicke Encephalopathy
Thiamine
Vitamins

Figure

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  Fig. 1 (A) Brain magnetic resonance imaging (MRI) findings prior to intramuscular thiamine supplementation therapy: bilateral symmetric hyper-intense signals in the periventricular grey matter, in the periaqueductal area, and both medial thalami (arrows). (B) Brain MRI findings reversed 10 days after therapy.

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