Rapid genetic analysis, imaging with 18F-DOPA-PET/CT scan and laparoscopic surgery in congenital hyperinsulinism.

Congenital hyperinsulinism (CHI) is responsible for profound hypoglycaemia which needs aggressive treatment in order to prevent neurological damage. Mutations in seven different genes have been held responsible for the inappropriate insulin secretion, typical of this condition. The most common cause of CHI is autosomal recessive mutations in the ABCC8 and KCNJ11 genes which encode for two subunits (SUR 1 and Kir6.

Localized but unresectable neuroblastoma: treatment and outcome of 145 cases. Italian Cooperative Group for Neuroblastoma.

Purpose: To define factors that influence outcome in children with localized but unresectable neuroblastoma by retrospective investigation of response to therapy and outcome in 21 Italian institutions.

Patients And Methods: Of 145 assessable children diagnosed between 1979 and 1990, 77 were treated between 1979 and 1984 with three consecutive standard-dose (SD) protocols, and 68 between 1985 and 1990 with a high-dose (HD) protocol. All protocols included chemotherapy, followed by resection of primary tumor if feasible.