Peripheral and central hypomyelination with hypogonadotropic hypogonadism and hypodontia
نویسندگان
چکیده
منابع مشابه
I-3: Hypogonadotropic Hypogonadism
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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Olf/Ebf transcription factors have been implicated in numerous developmental processes, ranging from B-cell development to neuronal differentiation. We describe mice that carry a targeted deletion within the Ebf2 (O/E3) gene. In Ebf2-null mutants, because of defective migration of gonadotropin releasing hormone-synthesizing neurons, formation of the neuroendocrine axis (which is essential for p...
متن کاملGonadotropins in Infertile Men with Idiopathic Hypogonadotropic Hypogonadism
Background Stimulatory therapy with gonadotrpins is an effective treatment to induce spermatogenesis in men with idiopathic hypogonadotroptic hypogonadism (IHH). The aim of this study was to assess the effectiveness of human chorionic gonadotropin / human menopausal gonadotropin on hypogonadotropic infertile men. MaterialsAndMethods This study included fifty-six azoospermic infertile men with I...
متن کاملFamilial hypothalamic hypogonadotropic hypogonadism.
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...
متن کاملCentral Hypogonadotropic Hypogonadism: Genetic Complexity of a Complex Disease
Central hypogonadotropic hypogonadism (CHH) is an emerging pathological condition frequently associated with overweight, metabolic syndrome, diabetes, and midline defects. The genetic mechanisms involve mutations in at least twenty-four genes regulating GnRH neuronal migration, secretion, and activity. So far, the mechanisms underlying CHH, both in prepubertal and in adulthood onset forms, rema...
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ژورنال
عنوان ژورنال: Neurology
سال: 2006
ISSN: 0028-3878,1526-632X
DOI: 10.1212/01.wnl.0000247666.28904.35