Jove
Visualize
Contact Us
JoVE
x logofacebook logolinkedin logoyoutube logo
ABOUT JoVE
OverviewLeadershipBlogJoVE Help Center
AUTHORS
Publishing ProcessEditorial BoardScope & PoliciesPeer ReviewFAQSubmit
LIBRARIANS
TestimonialsSubscriptionsAccessResourcesLibrary Advisory BoardFAQ
RESEARCH
JoVE JournalMethods CollectionsJoVE Encyclopedia of ExperimentsArchive
EDUCATION
JoVE CoreJoVE BusinessJoVE Science EducationJoVE Lab ManualFaculty Resource CenterFaculty Site
Terms & Conditions of Use
Privacy Policy
Policies

Related Experiment Videos

Pubertal development in the Prader-Labhart-Willi syndrome.

R Kauli, R Prager-Lewin, Z Laron

    Acta Paediatrica Scandinavica
    |November 1, 1978
    PubMed
    Summary

    Sexual maturation varies significantly in Prader-Labhart-Willi (PLW) syndrome, with delayed, normal, or precocious puberty observed. Hypothalamic lesion variability likely causes these diverse pubertal patterns.

    Related Concept Videos

    You might also read

    Related Articles

    Articles linked to this work by shared authors, journal, and citation graph.

    Sort by
    Same author

    Gonadotropin-suppressive therapy in girls with early and fast puberty affects the pace of puberty but not total pubertal growth or final height.

    The Journal of clinical endocrinology and metabolism·2002
    Same author

    Sexual precocity in boys: accelerated versus slowly progressive puberty gonadotropin-suppressive therapy and final height.

    The Journal of clinical endocrinology and metabolism·2001
    Same author

    Seasonality of birth in children with diabetes. Maternally transmitted infection might increase risk of diabetes.

    BMJ (Clinical research ed.)·2001
    Same author

    Longitudinal analysis of growth over the first 3 years of life in Turner's syndrome.

    The Journal of pediatrics·2000
    Same author

    Experience with cyproterone acetate in the treatment of precocious puberty.

    Journal of pediatric endocrinology & metabolism : JPEM·2000
    Same author

    Echocardiographic dimensions and function in adults with primary growth hormone resistance (Laron syndrome).

    The American journal of cardiology·2000

    Area of Science:

    • Endocrinology
    • Pediatric Endocrinology
    • Neuroendocrinology

    Background:

    • Prader-Labhart-Willi (PLW) syndrome is a complex genetic disorder.
    • Sexual maturation abnormalities are frequently reported in PLW syndrome.
    • The underlying mechanisms of these pubertal disturbances remain incompletely understood.

    Purpose of the Study:

    • To investigate the patterns of sexual maturation in patients with PLW syndrome.
    • To assess the hypothalamic-pituitary-gonadal axis function using LH-RH stimulation tests.
    • To correlate pubertal development with potential hypothalamic involvement.

    Main Methods:

    • Studied 14 patients (10 female, 4 male) with PLW syndrome.
    • Documented patterns of pubertal development: delayed, normal, and precocious.
    • Performed gonadotropin-releasing hormone (LH-RH) stimulation tests in 11 patients.

    Main Results:

    • Observed wide variability in pubertal development: 5 delayed, 4 normal, 5 precocious (1 true, 4 incomplete).
    • LH-RH tests showed normal LH/FSH responses in those with normal puberty.
    • Blunted LH/FSH responses to LH-RH were noted in patients with delayed or arrested puberty; one patient showed augmented LH response after priming.

    Conclusions:

    • Sexual maturation patterns in PLW syndrome are highly variable.
    • Variability is likely due to differences in the location and extent of hypothalamic lesions.
    • Hypothalamic dysfunction may represent an active process extending beyond the perinatal period.

    Related Experiment Videos