Impaired Sertoli cell function in males diagnosed with Noonan syndrome.
SourceJournal of Pediatric Endocrinology & Metabolism, 21, 11, (2008), pp. 1079-1084
Article / Letter to editor
Display more detailsDisplay less details
Journal of Pediatric Endocrinology & Metabolism
SubjectIGMD 1: Functional imaging; IGMD 3: Genomic disorders and inherited multi-system disorders; IGMD 5: Health aging / healthy living; IGMD 6: Hormonal regulation; IGMD 8: Mitochondrial medicine; ONCOL 3: Translational research; ONCOL 5: Aetiology, screening and detection; UMCN 5.2: Endocrinology and reproduction
In order to study male gonadal function in Noonan syndrome, clinical and laboratory data, including inhibin B, were gathered in nine pubertal males diagnosed with Noonan syndrome. Bilateral testicular maldescent was observed in four, and unilateral cryptorchidism occurred in two. Puberty was delayed in three patients. Luteinising hormone (LH) levels were normal in all patients in our series, while follicle stimulating hormone (FSH) levels were raised in seven. Inhibin B was low in six males and just above the lower limit of normal in two. Importantly, all three men with normal testicular descent displayed signs of Sertoli cell dysfunction, indicating, in contrast to earlier reports, that bilateral cryptorchidism does not seem to be the main contributing factor to impairment of testicular function in Noonan syndrome. These findings suggest different mechanisms of disturbance in male gonadal function, which is frequently associated with Sertoli dysfunction.
Upload full text
Use your RU credentials (u/z-number and password) tolog in with SURFconextto upload a file for processing by the repository team.