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[Phosphoethanolamine in the blood and urine in sick children]
1Centrum dĕdicných metabolických poruch fakultní nemocnice 2 s fakultní poliklinikou, Praha.
Insights
Elevated urinary phosphoethanolamine (PEA) levels are common in infants and can indicate impaired phospholipid metabolism in conditions beyond hypophosphatasia.
Area of Science:
- Biochemistry
- Clinical Chemistry
- Metabolic Disorders
Context:
- Assessed urinary phosphoethanolamine (PEA) in 866 patients with suspected amino acid metabolism disorders.
- Serum PEA elevation (>10 mumol/l) found in 2.1% of patients.
- Urinary PEA elevation (>10 mmol/mol creatinine) found in 42% of patients.
Purpose:
- To investigate the significance of phosphoethanolamine (PEA) concentrations in urine and serum.
- To determine the relationship between PEA levels, age, and various health conditions.
- To differentiate PEA elevation as a primary metabolic disease marker versus a secondary finding.
Summary:
- Urinary PEA concentration shows a significant age-dependent trend, being highest in infancy and declining with age.
- Significantly higher mean urinary PEA was observed in infants with CNS dysfunction, skeletal affections, and hepatopathies compared to healthy controls.
- In older children, elevated urinary PEA was primarily associated with skeletal affections, even excluding hypophosphatasia.
Impact:
- Identifies elevated urinary PEA as a potential biomarker for impaired phospholipid metabolism in CNS, liver, and skeletal tissues.
- Highlights that while PEA elevation is specific to hypophosphatasia in some cases, it can be a secondary indicator in other conditions.
- Provides insights into PEA's role in pediatric metabolic and systemic diseases, aiding in differential diagnosis.
Abstract:
The phosphoethanolamine (PEA) concentration in morning urine was assessed by liquid chromatography in 866 patients examined because of suspected impaired aminoacid metabolism. In 763 patients the blood was also examined. A serum concentration of PEA above 10 mumol/l was recorded in 2.1%. The concentration of PEA in urine above 10 mmol/mol creatinine was recorded in 42%. The authors revealed a significant relationship between urinary PEA excretion and the patient's age. The urinary PEA concentrations are higher during the first weeks and months of life, in older children and during adolescence its excretion declines and this trend was recorded also in the author's group of patients. In a group of 111 infants with impaired function of the CNS, in 66 infants with systemic skeletal affections and in 73 infants with hepatopathies a significantly higher mean urinary PEA concentration was found that in the control group of healthy infants. In children aged 3-14 years and in the group of older children the mean PEA concentration was elevated only in patients with systemic skeletal affections, even after elimination of patients with hypophosphatasia. Long-term or intermittently increased PEA excretion is a manifestation of specific metabolic disease only in hypophosphatasia. In other diseases it can be interpreted as a secondary finding conditioned by an impaired phospholipid metabolism at the level of cellular membranes in the CNS, liver or skeleton.