Prader-Willi syndrome

Description

Prader-Willi syndrome is a genetic disorder caused by partial deletion of chromosome 15 (paternal) or disomy (maternal). Children with Prader-Willi syndrome have mental retardation and abnormal food-related behaviors. Prader-Willi syndrome is characterized by feeding problems during infancy, and hyperphagia, often resulting in obesity, in childhood and adolescence.

Possible effects on nutrient needs

Inadequate intakes are very prevalent in infancy for individuals with Prader-Willi syndrome:

• difficulty feeding because of problems with sucking and hypotonia

Excessive energy intakes after infancy lead to problems with overweight and obesity:

• short stature contributing to decreased energy needs, although now with growth hormone as usual treatment, this is not as significant an issue
• hypotonia and problems with muscle coordination, leading to decreased physical activity levels and decreased energy needs
• hyperphagia and absent satiety cues, leading to excessive intakes
• energy needs (e.g., kilocalories per centimeter) that are lower than those of children without Prader-Willi syndrome