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. 2018 Aug 16;23(6):403–410. doi: 10.1093/pch/pxy093

Table 5.

Tier-1 laboratory investigations for unexplained GDD/ID

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.