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Related Experiment Videos

[Prader-Labhart-Willi syndrome].

W H Fesseler, J R Bierich

    Monatsschrift Kinderheilkunde : Organ Der Deutschen Gesellschaft Fur Kinderheilkunde
    |December 1, 1983
    PubMed
    Summary

    Prader-Labhart-Willi-Syndrome (PLWS) patients exhibit growth hormone deficiency and metabolic issues like diabetes. Surgical intervention for obesity may be beneficial when diet fails.

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    [Growth hormone profile in constitutional and acquired developmental delay].

    Monatsschrift Kinderheilkunde : Organ der Deutschen Gesellschaft fur Kinderheilkunde·1990

    Area of Science:

    • Pediatric Endocrinology
    • Metabolic Disorders
    • Genetics

    Context:

    • Prader-Labhart-Willi-Syndrome (PLWS) is a complex genetic disorder affecting multiple endocrine functions.
    • Patients often present with characteristic features including short stature, obesity, and developmental delays.

    Purpose:

    • To evaluate growth hormone (GH) secretion, metabolic status, and treatment outcomes in pediatric PLWS patients.
    • To assess the efficacy of GH therapy and surgical interventions for obesity management in PLWS.

    Summary:

    • 12 PLWS patients (9 male, 3 female) were assessed for height, GH levels, and metabolic parameters.
    • Growth hormone deficiency was noted in several patients, with one showing positive response to GH therapy.
    • Metabolic abnormalities included prediabetes and type-2 diabetes, linked to insulin resistance and obesity.
    • Hypogonadotropic hypogonadism was prevalent in pubertal patients.
    • Gastric partitioning was effective for weight loss in two patients with obesity.
    • Chromosomal abnormalities were identified in two patients.

    Impact:

    • Highlights the endocrine and metabolic challenges in PLWS, emphasizing the need for comprehensive management.
    • Suggests GH therapy as a viable option for growth promotion without adverse metabolic effects.
    • Underscores the critical role of obesity management, recommending surgical options when conservative measures fail.
    • Identifies potential genetic factors contributing to PLWS phenotypes.

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