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A Reference Range for Plasma Levels of Inorganic Pyrophosphate in Children Using the ATP Sulfurylase Method
Eva Bernhard1, Yvonne Nitschke1, Gus Khursigara2
1Department of General Pediatrics, Muenster University Children's Hospital, Muenster, Germany.
Insights
This study establishes a normal reference range for plasma inorganic pyrophosphate (PPi) in children, crucial for diagnosing mineralization disorders. The findings support using plasma PPi as a pediatric biomarker.
Area of Science:
- Biochemistry
- Pediatric Medicine
- Genetics
Background:
- Rare inherited disorders like hypophosphatasia involve altered inorganic pyrophosphate (PPi) levels.
- Plasma PPi is a potential biomarker for mineralization disorders.
Purpose of the Study:
- Establish a reference range for plasma PPi in the pediatric population.
- Validate plasma PPi as a biomarker for childhood mineralization disorders.
Main Methods:
- Collected plasma samples from 200 children (1 day to 18 years).
- Measured PPi using a validated adenosine triphosphate (ATP) sulfurylase method.
Main Results:
- Established a pediatric plasma PPi reference range of 2.36–4.44 µM (median 3.17 µM).
- No significant differences observed between sexes or pediatric age groups.
- Assay demonstrated analytical sensitivity from 0.15 to 10 µM PPi with <10% variability.
Conclusions:
- The established pediatric PPi range aligns with adult levels.
- The ATP sulfurylase method is proposed as a diagnostic tool for pediatric plasma PPi measurement.
Purpose:
Generalized arterial calcification of infancy, pseudoxanthoma elasticum, autosomal recessive hypophosphatemic rickets type 2, and hypophosphatasia are rare inherited disorders associated with altered plasma levels of inorganic pyrophosphate (PPi). In this study, we aimed to establish a reference range for plasma PPi in the pediatric population, which would be essential to support its use as a biomarker in children with mineralization disorders.
Methods:
Plasma samples were collected from 200 children aged 1 day to 18 years who underwent blood testing for medical conditions not affecting plasma PPi levels. PPi was measured in proband plasma utilizing a validated adenosine triphosphate (ATP) sulfurylase method.
Results:
The analytical sensitivity of the ATP sulfurylase assay consisted of 0.15 to 10 µM PPi. Inter- and intra-assay coefficients of variability on identical samples were below 10%. The standard range of PPi in the blood plasma of children and adolescents aged 0 to 18 years was calculated as 2.36 to 4.44 µM, with a median of 3.17 µM, with no difference between male and female probands. PPi plasma levels did not differ significantly in different pediatric age groups.
Main Conclusions:
Our results yielded no noteworthy discrepancy to the reported standard range of plasma PPi in adults (2-5 µM). We propose the described ATP sulfurylase method as a diagnostic tool to measure PPi levels in plasma as a biomarker in the pediatric population.
