Thiamine Deficiency Leading to Refractory Lactic Acidosis in a Pediatric Patient

Abstract

Thiamine plays a critical role in energy metabolism. Critically ill children and adults may develop thiamine deficiency with ultimately increased mortality due to potentially irreversible consequences of severe type B lactic acidosis. We report a case of an unvaccinated term neonate with malignant pertussis requiring extracorporeal membrane oxygenation and continuous renal replacement therapy, who developed profound lactic acidosis of unknown etiology. After countless evaluations for likely causes, the patient was ultimately determined to have thiamine deficiency and her acidosis resolved rapidly with vitamin supplementation.

Micronutrient status during paediatric critical illness: A scoping review

“…Studies included in the scoping review investigated serum/plasma levels, reporting micronutrient status was determined on admission day in majority of patients. There were a number of studies describing low serum/plasma micronutrient levels during critical illness including thiamine, riboflavin, folate, vitamin B6, vitamin B12, vitamin A, β-carotene, zinc, selenium, iron and chromium [ [31] , [32] , [33] , [34] , [35] , [36] , [37] , [38] , [39] , [40] , [41] ], as well as studies describing serum/plasma levels unchanged or high during critical illness vitamin E, vitamin B6, copper and manganese [ 38 , [42] , [43] , [44] ]. A number of these studies reported factors associated with serum/plasma levels of micronutrients levels including the use of continuous renal replacement therapy, cardiac surgery and systemic inflammatory response [ 21 , 22 , 30 , 31 , 37 , 38 , 42 , [44] , [45] , [46] , [47] , [48] , [49] , [50] , [51] , [52] , [53] , [54] , [55] ].…”

“… Micronutrient Role Age Reference range (US) Reference range (SI) Levels reported Median (interquartile range) Direction of serum change Low No change ≈ High Studies describing changes in serum levels of micronutrients during critical illness Thiamine/Vitamin B1 (p) Thiamine is involved in a number of intermediate metabolism associated with energy production including converting pyruvate from glucose into acetyl co-enzyme A for entry into the Krebs cycle, during thiamine deficiency alters intermediate metabolism resulting in lactic acidosis [ 31 ]. 5.3–7.9 μg/mL 5.5 μg/mL (5.1, 6.5) Low Low serum thiamine levels [ [32] , [33] , [34] , 36 , 65 ] have been reported in 12.5–32% patients [ 31 , 42 , 47 ] Pyridoxine/Vitamin B6 (p) Vitamin B6 is required for 150 enzyme reactions, including inflammatory pathways including the kynurenine pathway, sphingosine 1-phosphate metabolism, the transsulfuration pathway, and serine and glycine metabolism [ 95 ]. 5–50 μg/l Levels not reported Low No change ≈ A single case report of vitamin B6 (and thiamine deficiency) [ 36 ].…”

“… Medication Low thiamine levels was not associated with diuretic use [ 47 ] CCRT Low levels of selenium, zinc and chromium [ 38 ] Dilution effects of dialysate in renal replacement therapy result in losses of selenium, thiamine, folate, pyridoxine and vitamin C and accumulation of manganese [ 80 ]. Cardiac surgery In case study reports thiamine deficiency was associated with lactic acidosis in septic shock [ 32 ], extracorporeal membrane oxygenation [ 33 ], haematological malignancies [ 65 ] and following cardiac surgery [ 34 ]. Low selenium levels have been described in children requiring cardiopulmonary bypass [ 54 ] Multi-organ failure Low plasma selenium levels are associated with multi-organ failure [ 53 ] Lymphopenia Low zinc levels were also found in patients with lymphopenia [ 51 ].…”

“…Low selenium levels have been described in children requiring cardiopulmonary bypass [ 54 ] Multi-organ failure Low plasma selenium levels are associated with multi-organ failure [ 53 ] Lymphopenia Low zinc levels were also found in patients with lymphopenia [ 51 ]. In case study reports thiamine deficiency was associated with lactic acidosis in septic shock [ 32 ], extracorporeal membrane oxygenation [ 33 ], haematological malignancies [ 65 ] and following cardiac surgery [ 34 ]. Severity of illness score Low serum iron levels were associated with severity of illness score critically ill children [ 52 ] but not in neonates [ 43 ].…”

“… Low plasma selenium levels are associated with severity of risk scores [ 48 , 50 , 52 ]. Lactic acidosis In case study reports thiamine deficiency was associated with lactic acidosis in septic shock [ 32 ], extracorporeal membrane oxygenation [ 33 ], haematological malignancies [ 65 ] and following cardiac surgery [ 34 ]. Studies describing supplementation of micronutrients during critical illness Zinc Two studies considered micronutrient supplementation in critically ill children.…”