Hypermanganesemia in Children With Liver and Portosystemic Circulation Disorders: A Systematic Review

Area of Science:

• Pediatric Gastroenterology and Hepatology
• Neuroscience
• Toxicology

Background:

• Children with chronic liver disease or portosystemic shunts may experience long-term neurocognitive issues.
• Hypermanganesemia (elevated manganese levels) is a potential contributing factor in these children.

Purpose of the Study:

• To systematically review liver and portosystemic circulation disorders associated with hypermanganesemia in children.
• To characterize reported neurocognitive outcomes in affected children.

Main Methods:

• Systematic literature search of MEDLINE, PubMed, and Embase databases up to February 2023.
• Assessment of study quality using Joanna Briggs Institute (JBI) critical appraisal tools.
• Evaluation of risk of bias using the Navigation Guide framework.

Main Results:

• Eighteen studies including 103 pediatric patients were analyzed.
• Seventy patients showed evidence of hypermanganesemia, with elevated manganese levels and/or characteristic MRI changes.
• Manganese levels and MRI abnormalities often normalized after treatment for the underlying liver or shunt disorder.

Conclusions:

• Portosystemic shunting is a likely significant risk factor for hypermanganesemia in pediatric liver and circulation disorders.
• Gaps in research limit definitive conclusions on the independent contribution of hypermanganesemia to neurocognitive outcomes.
• A structured approach is needed, incorporating shunt status, manganese levels, and longitudinal neurocognitive assessment.