[n-3 and n-6 fatty acids in plasma at birth and one year of age and relationship with feeding]. / Acidos grasos n-3 y n-6 en plasma al nacer y al año de edad y relación con el tipo de alimentación.

AIM: Compare the variations of long-chain polyunsaturated fatty acids (LCPUFA) levels at birth and at the first year of age in children on extended breast-feeding, medium term breast-feeding and formula feeding. PATIENTS: 77 healthy term infants divided in three groups: A (N=25): extended breast-feeding (more than 6 months), B (N=26): medium term breast-feeding (more than 3 and less than 5 months) and C (N=26): exclusive formula feeding. Fatty acids in plasma were measured at birth and at the first year of age. RESULTS: There were no differences in the levels at birth. However, there is a significant decrease in the proportion of the main LCPUFA, docosahexaenoic acid (DHA) and arachidonic acid (AA), between birth and the first year of age. At one year of age, the percentage of DHA in Group A differs significantly between the other two: 2.46+/-0.84 vs. 1.80+/-0.48 and 1.89+/-0.75 (p<0.01). CONCLUSIONS: 1. At birth, there are no differences in LCPUFA. 2. A significant decrease in the main LCPUFA is observed with age. 3. The extended breast-feeding group shows higher DHA levels than the other two. Therefore, breast-feeding for more than 6 months is required to achieve higher plasma DHA values.

[Infantile spasms as the first manifestation of propionic acidemia]. / Espasmos infantiles como primera manifestación de la acidemia propiónica.

Propionic acidemia is a disorder of branch-chain amino acids, the side chain of cholesterol and odd-chain fatty acid metabolism that leads to the accumulation of toxic acid metabolites. The clinical features typically begin shortly after birth, although they can also appear in young adulthood. We report the case of a 3-year-old boy with atypical onset, who at 6 months presented bursts of infantile spasms and a hypsarrhythmic electroencephalogram. He was treated with vigabatrin. At 8 months magnetic resonance imaging showed a wider than normal subarachnoidal space, and hyperintense thalamus, globus pallidus and subthalamic nucleus. Biochemical and genetic analysis confirmed propionic acidemia. Specific therapy was started and the patient is not currently under anti-epileptic treatment and his electroencephalogram is normal. This onset of propionic acidemia is unusual, and we believe that treatment with vigabatrin protected the basal ganglia from irreversible excitotoxic damage.