Información del artículo
El síndrome de Prader-Willi (SPW) en la edad adulta se caracteriza por alteración de la composición corporal, hiperfagia y obesidad, con elevada morbilidad y mortalidad cardiovascular. La evidencia científica demuestra que en el SPW existe una deficiencia parcial de hormona de crecimiento (GH), por lo que cabría esperar que estos pacientes tuvieran las mismas manifestaciones clínicas que se observan en el síndrome de deficiencia de GH en la edad adulta (DGHA), y que obtuvieran los mismos beneficios con el tratamiento sustitutivo hormonal. Así, recientemente, se ha demostrado en adultos con SPW que el tratamiento con GH durante 12 meses mejora la composición corporal y el estado psicológico, sin afectar de forma significativa el metabolismo hidrocarbonado y lipídico. Sin embargo, faltan estudios prospectivos con grupos de controles adecuados que confirmen estos resultados, antes de aceptar la indicación del tratamiento con GH en este síndrome.
Palabras clave:
Síndrome de Prader-Willi
Tratamiento con GH
Déficit de GH
Prader-Willi syndrome (PWS) is a genetic disorder. In adults it is characterized by abnormal body composition, excessive eating and progressive obesity with increased cardiovascular morbidity and mortality. Scientific evidence shows that there is partial growth hormone (GH) deficiency in PWS. Therefore, these patients could be expected to show the same clinical manifestations as those observed in adults with GH deficiency syndrome (GHDS) and to obtain the same benefit from hormone replacement therapy as GHDS patients. A recent study has demonstrated that GH treatment for 12 months in adults with PWS improved body composition and psychological status without producing pronounced adverse effects on glucose and lipid metabolism. However, larger, prospective studies with suitable control groups are required to confirm these results before GH replacement is used as therapy in adults with PWS.
Key words:
Prader-Willi syndrome
GH therapy
GH deficiency
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