complications

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Hyperinsulinaemia, resulting from islet cell hyperplasia, is a common early complication. The associated hypoglycaemia may be controlled with steroids or glucagon. Rarely, subtotal pancreatectomy may be necessary.

In the long term, this syndrome is associated with an increased incidence of solid tumours such as nephroblastoma and hepatoblastoma. The incidence of these are increased if there is hemihypertrophy.

Last reviewed 01/2018

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