Moebius-Poland syndrome and hypogonadotropic hypogonadism
نویسندگان
چکیده
منابع مشابه
Moebius syndrome in association with hypogonadotropic hypogonadism.
The association between hypogonadotropic hypogonadism and multiple CNS lesions in a variety of disorders suggests a possible causative link between these clinical findings. Neural afferent input into the hypothalamus from higher CNS centers modulates GnRH secretion and derangements of these neural pathways could potentially result in diminished gonadotropin secretion and hypogonadism. This repo...
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Clinical case A sixteen year old boy presented, three years before, gastrointestinal (abdominal pain, vomiting, diarrhea) and neurologic symptoms (headache, diplopia, ataxia, VI nerve paralysis), with fever, oral and genital aphtosis. Laboratory tests showed increased ESR, CRP). He carried B51HLA. Physical examination showed svere obesity, pseudomicropenis, rare pubic hair, pubertal stage G2P2....
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Hypogonadotropic hypogonadism (HH) is an uncommon cause of male infertility and a congenital or secondary disorder characterized by delayed or absent sexual maturation. Congenital abnormalities leading to HH are usually the consequence of deficient GnRH secretion occurring either in isolation (idiopathic hypogonadotropic hypogonadism (IHH)), or in association with anosmia (Kallmann syndrome; KS...
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The proper development and coordination of the hypothalamic-pituitary-gonadal (HPG) axis are essential for normal reproductive competence. The key factor that regulates the function of the HPG axis is gonadotrophin-releasing hormone (GnRH). Timely release of GnRH is critical for the onset of puberty and subsequent sexual maturation. Misregulation in this system can result in delayed or absent p...
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Clinical findings and endocrine studies are reported concerning six subjects (from two pedigrees) suffering from isolated hypogonadotropic hypogonadism. Their complete lack of any gonadotropin response to clomiphene stimulation, together with positive responses of follicle-stimulating hormone (FSH) and luteinizing hormone (LH) to LH-releasing-hormone stimulation (LH-RH) provide evidence for a h...
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ژورنال
عنوان ژورنال: European Journal of Pediatrics
سال: 2007
ISSN: 0340-6199,1432-1076
DOI: 10.1007/s00431-007-0473-4