ABSTRACT
Congenital hyperinsulinism, or Persistent hyperinsulinaemic hypoglycaemia of infancy (PHHI), is a rare disorder of glucose metabolism. Manifestations include measurable serum insulin levels despite severe hypoglycaemia and maintenance glucose requirements greater than maximal hepatic production. Cystic fibrosis is the most common major life-shortening heritable disease in North America. Cystic fibrosis results from a defect in the c-AMP-induced transmembrane chloride channel and is inherited in an autosomal recessive fashion. Pulmonary and gastrointestinal (GI) complications secondary to abnormally viscous secretions are common. Solid pseudopapillary tumours most commonly affect females of reproductive age and have low malignant potential. Their imaging features reflect the mixed cystic and solid nature of the lesion with encapsulation and frequent intratumoural haemorrhage. Pancreaticoblastoma is the most common pancreatic tumour of small children with a mean age of presentation at four and a half years. Gastrinoma is the second most common islet cell tumour behind insulinoma.
