Hello and welcome to the Zero to Finals podcast.

My name is Tom and in this episode I'm going to be talking to you about Prada Willie Syndrome.

If you want to follow along with written notes on this topic,

you can follow along at zero tofinals.com slash Prada Willie

or in the genetic condition section of the zero to finals pediatrics book.

So let's get straight into it.

Prada Willie syndrome is a genetic condition caused by the loss of functional genes on the

proximal arm of the chromosome 15 inherited from the father.

This can be due to a deletion of this portion of the chromosome,

or when both copies of chromosome 15 are inherited from the mother. There's a few key features to look

out for in your exams. Constant insatiable hunger that leads to obesity. Poor muscle tone as an infant, which we call hypotonia,

mild to moderate learning disability, hypogonadism, fair soft skin that's prone to bruising,

mental health problems, particularly anxiety, dysmorphic features, narrow forehead,

almond-shaped eyes, strabismus which is a squint, a thin upper lip and a downturned mouth.

A quick Tom tip for you. The key feature everyone remembers for

Prada Willie syndrome is the insatiable hunger. Feeding can often be a challenge initially due to

hypotonia and it's only later on that food seeking and excessive eating occurs. It's worth remembering some other key

facts about the condition, such as the treatment with growth hormone and the poor muscle tone,

so that you know more than just the link with appetite. There's no cure for Pardo-Willi

syndrome. Carefully limiting access to food under the guidance of a dietitian is required to control weight.

This usually requires locking food in cupboards, putting a lock on the fridge and even controlling

access to rubbish bins. Under dietitian guidance, they require lower than normal calorie intake,

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