Densidad minera osea (DMO) corporal total y L2-L4 antes y despues de la pubertad en niños con fenilcetonuria ( PKU). .

FRAGA C.; KESELMAN A.; CASSINELLI H.; GRUÑEIRO PAPENDIECK L,; CHIESA A

Lima Perú.

Congreso; XX Reunion Anual de SLEP; 2008

SLEP

PKU treatment consists in a phenylalanine (Phe) restricted diet and a free phe protein substitute. Poor bone mineralization, influenced by nutritional status was described in these patients,  In order to asses the bone mineralization achieved with our nutritional approach 16 prepubertal (PP)(9.4±1.4years) and 11 late pubertal(P) (13.8±1.2years), early detected PKU children were evaluated with total (T) and  Lumbar(L2-4)BMD  measured by DEXA, expressed as Z-score compared to normal argentine children. Protein, calcium and Vit D recommendations in all children were completely fulfilled  with  the substitute (80% of protein recommendation) and in >8 years of age  calcium supplementation. Longitudinal follow up was performed in 8 and outcome compared with Wilcoxon test for paired samples. Correlation of T and L2-4BMD with HeightSDS, BMISDS, dietary phe tolerance, previous mean serum phe, adequacy of metabolic control, and pubertal height gain were estimated for each period. TBMD in PP was 0.02±0.7 and L2-4:-0.34±0.6 while in P TBMD: -0.38±0.9 and L2-4:-0.46±1.2.  No differences were found (n:8) in TBMD (PP:0.09±0.8 vs P:-0.08±0.8) nor in  L2-4 (PP-0.29±0.7 vs P-0.10±1.2) Significant correlations were found between TBMD and L2-4 with height in either period PP r0.75 and P r0.83. (both p<0.01) and between L2-4 with height gained in puberty r.0.70 p<0.01. Conclusion: T and L2-4BMD before and after puberty were normal in our studied patients suggesting adequacy of the therapeutic approach. An appropriate growth during childhood and puberty was positively related to  bone status.