Table 5.
Blood* | Urine* |
---|---|
• Complete blood count | • Organic acids |
• Glucose | • Creatine metabolites |
• Blood gas | • Purines, pyrimidines |
• Urea, creatinine | • Glycosaminoglycans |
• Electrolytes (to calculate anion gap) | |
• AST, ALT | |
• TSH | |
• Creatine kinase | |
• Ammonia | |
• Lactate | |
• Amino acids | |
• Acylcarnitine profile, carnitine (free and total) | |
• Homocysteine | |
• Copper, ceruloplasmin** | |
• Biotinidase*** | |
• Ferritin, vitamin B12 when dietary restriction or pica are present | |
• Lead level when risk factors for exposure are present |
ALT Alanine aminotransferase; AST Aspartate aminotransferase; GDD Global developmental delay; ID Intellectual disability; TSH Thyroid-stimulating hormone.
*Perform testing after 4 h to 8 h of fasting. **Recommended tier-1 test in the TIDE protocol, but not by AAP, AAN. Consider as a first-line investigation when hepatomegaly, dystonia, abnormal liver function findings are present. ***Clinical expert recommendation only. Consider biotinidase testing when severe hypotonia, seizures are present.