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. 2019 Apr 11;42(1 Suppl 1):178–185. doi: 10.1590/1678-4685-GMB-2018-0105

Table 1. Common features of patients with aminoacidopathies detected in high-risk Brazilians.

Age at diagnosis Percentage
< 1 month 12
1 - 12 months 52
1 - 3 years 11
3 - 10 years 14
> 10 years 11
Gender Percentage
Female 45
Male 55
Clinical features Percentage
Neurological abnormalities 90
Feeding difficulties 30
Hepatomegaly 22
Vomiting 15
Dysmorphies 7
Failure to thrive 3
Neurological signs Percentage
Psychomotor delay/mental retardation 61
Seizures 60
Hypotonia/hypertonia/dystonia 36
Cerebral atrophy 27
Macrocephaly 8
Laboratory findings Percentage
Metabolic acidosis 41
Hypoglycemia 41
Lactic acidemia 14