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Infantile encephalopathy AND lactic acidosis — Clinical Guidelines

Overview

Infantile encephalopathy associated with lactic acidosis is a severe metabolic condition characterized by elevated serum lactate levels, metabolic acidosis, and neurological dysfunction, often linked to underlying etiologies such as thiamine deficiency, metabolic disorders, or medication-induced toxicity 3 5 10 11.

Diagnosis

Elevated serum lactate levels

Metabolic acidosis (decreased blood pH)

Neurological symptoms indicative of encephalopathy

Consideration of underlying causes (thiamine deficiency, liver disease, malignancy, medication toxicity) 3 4 5 10 11

Laboratory tests: lactate levels, blood gas analysis, thiamine levels 3 5

Imaging and neurological assessments to evaluate encephalopathy severity 3

Management

Supportive care: Fluid resuscitation, hemodynamic stabilization 1

Thiamine supplementation: Intravenous thiamine for suspected deficiency 5 10 11

Metformin discontinuation: If metformin-associated lactic acidosis is suspected 1 6 7

Extracorporeal treatments: Consideration for severe cases to correct acidemia and remove lactate/metformin 1

Management of underlying cause: Address specific etiologies (e.g., malignancy, liver disease) 3 4

Special Populations

Pediatrics: High index of suspicion for thiamine deficiency during parenteral nutrition 5 11

Comorbidities: Consider thiamine deficiency in patients with liver disease or malignancy 3 4 5

Key Recommendations

Initiate intravenous thiamine supplementation promptly in cases of suspected thiamine deficiency, especially in pediatric patients receiving parenteral nutrition 5 10 11 (Evidence: Strong)

Discontinue metformin and provide supportive care including fluid resuscitation in suspected metformin-associated lactic acidosis 1 6 7 (Evidence: Moderate)

Consider extracorporeal treatments for severe cases to manage metabolic acidemia effectively 1 (Evidence: Weak)

Investigate and address underlying causes such as malignancy, liver disease, or other metabolic disorders contributing to lactic acidosis 3 4 (Evidence: Moderate)

References

1 Roberts DM, Ghannoum M. Expanding the evidence for managing metformin poisoning to support decision-making. Clinical toxicology (Philadelphia, Pa.) 2023. link 2 Dai Y, Wang Y, Zeng Y, Zhang C, Zhou Z, Shi D. Linezolid and the risk of lactic acidosis: Data mining and analysis of the FDA Adverse Event Reporting System. Journal of clinical pharmacy and therapeutics 2020. link 3 Dejman A, Riveros J. Vitamin B1 for type B metabolic acidosis: An underrecognized approach. Saudi journal of kidney diseases and transplantation : an official publication of the Saudi Center for Organ Transplantation, Saudi Arabia 2018. link 4 Dean RK, Subedi R, Gill D, Nat A. Consideration of alternative causes of lactic acidosis: Thiamine deficiency in malignancy. The American journal of emergency medicine 2017. link 5 Ramsi M, Mowbray C, Hartman G, Pageler N. Severe lactic acidosis and multiorgan failure due to thiamine deficiency during total parenteral nutrition. BMJ case reports 2014. link 6 Margiani C, Zorcolo L, Mura P, Saba M, Restivo A, Scintu F. Metformin-associated lactic acidosis and temporary ileostomy: a case report. Journal of medical case reports 2014. link 7 Bolleku E, Idrizi A, Barbullushi M, Bajrami V, Likaj E, Thereska N et al.. Metformin-induced lactic acidosis associated with multiorganic failure. Medical archives (Sarajevo, Bosnia and Herzegovina) 2013. link 8 Wills BK, Bryant SM, Buckley P, Seo B. Can acute overdose of metformin lead to lactic acidosis?. The American journal of emergency medicine 2010. link 9 Lalau JD, Race JM. Lactic acidosis in metformin therapy: searching for a link with metformin in reports of 'metformin-associated lactic acidosis'. Diabetes, obesity & metabolism 2001. link 10 . Lactic acidosis traced to thiamine deficiency related to nationwide shortage of multivitamins for total parenteral nutrition -- United States, 1997. MMWR. Morbidity and mortality weekly report 1997. link 11 Lange R, Erhard J, Eigler FW, Roll C. Lactic acidosis from thiamine deficiency during parenteral nutrition in a two-year-old boy. European journal of pediatric surgery : official journal of Austrian Association of Pediatric Surgery ... [et al] = Zeitschrift fur Kinderchirurgie 1992. link 12 Matuda S, Kitano A, Sakaguchi Y, Yoshino M, Saheki T. Pyruvate dehydrogenase subcomplex with lipoamide dehydrogenase deficiency in a patient with lactic acidosis and branched chain ketoaciduria. Clinica chimica acta; international journal of clinical chemistry 1984. link90151-7)

Infantile encephalopathy AND lactic acidosis