Outcome of gram negative infection in immunocompromised children.

Background: Gram negative infection is an important cause of mortality in patients receiving chemotherapy for malignant disease or as conditioning for stem cell transplantation. The risk of infection is thought to be greatest in those patients who are neutropenic and it is routine for these patients to receive broad spectrum antibiotic therapy when febrile. This study evaluates the outcome of gram negative infection in a mixed haematology/oncology population in a single institution.

Visual loss and idiopathic intracranial hypertension in children with Alagille syndrome.

Background: Alagille syndrome (AGS) is an autosomal dominant, multisystem disorder defined by developmental abnormalities of the liver, heart, eye and skeleton. Although visual problems are recognised, the severity of visual loss and its link with idiopathic intracranial hypertension (IIH) has not been reported.

Aim: To review the incidence of visual loss and IIH in children with AGS managed at a National Paediatric Liver Unit between 1989 and 2004.