ea0045p59 | Miscellaneous/other | BSPED2016
Estebanez Maria Salomon
, Douzgou Sofia
, Fedee Joanne
, Semple Robert
, Skae Mars
Introduction: Generalised lipodystrophy is clinically characterised by lipoatrophy, hepatomegaly, hypertriglyceridemia, insulin resistance and acromegaloid features. It is recognised that diencephalic syndrome is a rare presentation of hypothalamic tumours in infants and young children. Children with this disorder have profound emaciation and generalised loss of subcutaneous fat, growth acceleration, hyperkinesia and euphoria. Hypothalamic tumours, particularly pilocytic astro...