Prader-Willi Syndrome

Definition

Prader-Willi syndrome is a genetic disease characterized by hypotonic infantile period, obesity due to hyperphagia, short stature, hypogonadism and learning difficulties.

Symptoms

  • Severe hypotonia and feeding difficulties in infancy
  • Excessive appetite and obesity in childhood
  • Short stature, small hands and feet
  • Behavioral problems

Reasons

Deletion of paternal genes in the q11-q13 region of chromosome 15, or uniparental disomy.

Diagnosis

Clinical criteria, methylation test.

Treatment

Growth hormone therapy, behavioral therapy, strict dietary control.

Which Polyclinic Should You Apply To?

Pediatric Endocrinology Polyclinic or Genetics Polyclinic

What to Do in an Emergency?

In case of respiratory distress due to obesity or diabetic ketoacidosis, the emergency room should be consulted.

This page is for general information purposes only.