Open AccessHipogonadismo hipogonadotropo en paciente con síndrome de Klinefelter y tumor hipotálamo- hipofisario
Author(s) -
Anunciación Beisti Ortego,
Antonio de Arriba Muñoz,
Marta Ferrer Lozano,
José Miguel Martínez de Zabarte Fernández,
Carlota Calvo Escribano,
José Ignacio Labarta Aizpún
Publication year - 2015
Publication title -
archivos argentinos de pediatría
Language(s) - Spanish
Resource type - Journals
SCImago Journal Rank - 0.236
H-Index - 19
eISSN - 1668-3501
pISSN - 0325-0075
DOI - 10.5546/aap.2015.e6
Subject(s) - luteinizing hormone , hypergonadotropic hypogonadism , medicine , klinefelter syndrome , hormone , endocrinology , follicle stimulating hormone
Klinefelter Syndrome is the most frequent cause of hypergonadotropic hypogonadism in men. A flat response at luteinizing hormone releasing hormone stimulation test could be the first sign of hypothalamic tumor in these patients. We report the case of a patient diagnosed by neonatal screening with Klinefelter Syndrome, 47 XXY, that at 17 years follow-up presents analytical modification of the response to luteinizing hormone releasing hormone stimulation test with suppressed luteinizing hormone and follicle-stimulating hormone values; lately he presents with headache and loss of left eye vision. A magnetic resonance imaging of the brain showed a mixed germ cell hypothalamus tumor, requiring surgery, chemotherapy and radiotherapy with optimal response.