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Prader-Willi Syndrome after age 15 years
Archives of Disease in Childhood
|March 1, 1981
Summary
Prader-Willi syndrome patients over 15 experience severe obesity and insatiable appetite. Common issues include educational subnormality, hypogonadism, and scoliosis, with cor pulmonale being a frequent cause of mortality.
Area of Science:
- Medical Science
- Genetics
- Endocrinology
Background:
- Prader-Willi syndrome is a complex genetic disorder.
- Key features include hyperphagia, obesity, and developmental delays.
Purpose of the Study:
- To describe the clinical characteristics and outcomes of 24 Prader-Willi syndrome patients over 15 years old.
- To highlight the primary challenges and mortality causes in this cohort.
Main Methods:
- Retrospective case series description.
- Analysis of clinical data from 24 patients with Prader-Willi syndrome.
Main Results:
- Extreme obesity and insatiable appetite were principal handicaps.
- Comorbidities included diabetes (3 patients), short stature, scoliosis, and severe intellectual disability.
- Nine patients died between ages 3 and 23, most commonly from cor pulmonale.
Conclusions:
- Prader-Willi syndrome presents significant challenges including severe obesity and associated health complications.
- Cor pulmonale is a critical factor in mortality for this population.