El tratamiento en varones sintomáticos con hiperplasia suprarrenal no clásica (HSCNC) podría mejorar la talla adulta

PASQUALINI T; ALONSO G; FERNÁNDEZ C; BUZZALINO N.; DAIN L

ARCHIVOS ARGENTINOS DE PEDIATRIA

SOC ARGENTINA PEDIATRIA

Lugar: Buenos Aires; Año: 2013 vol. 11 p. 35 - 38

0325-0075

Although, corticoid replacement is recommended for those LOCAH with clinical manifestations, asymptomatic patients do not need treatment. We describe clinical features at diagnosis, treatment, and growth till adult height, in 4 boys. At diagnosis, age ranged ftom 9.2-11.9 years old and they presented pubarche (n=2), accelerated bone age (n=1) and precocious puberty (n=1). All of them were compound heterozygotes carrying p.V281L. Since bone age was significantly advanced for chronological age at diagnosis (13.3 ±0.6 vs. 10.2±1.1, p=0.008), hydrocortisone therapy was began. Although, adult mean height was not different from target height (-0.38±0.7 vs. -0.04±0.5, p= 0.062), it was lower than height at pubertal onset (p=0.028), with a mean height Z score decrement of 1.16±0.6 SDS. In conclusion, in symptomatic patients, accurate treatment of LOCAH may enhance growth. Advanced bone age at diagnosis and the loss of height during pubertal development suggest the need of therapy.