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. PROCEDURE: All episodes of laboratory proven gram negative infection in patients receiving chemotherapy between January 2004 and March 2006 were included. A retrospective case-note based analysis was carried out to identify diagnosis, treatment, neutrophil count at time of infection, antibiotic therapy and clinical outcome. RESULTS: One hundred six episodes of gram negative infection in 72 patients were identified. Sixty-seven percent of these were in patients receiving chemotherapy for malignant disease, 33% were in patients undergoing stem cell transplantation. Overall ICU admission rate was 13% and mortality was 6%. Coliforms, Pseudomonas and Klebsiella were the commonest isolates, and ICU admission and mortality rates were higher with Pseudomonas and Klebsiella infection than coliforms. After stem cell transplantation most episodes of gram negative infection occurred in non-neutropenic patients, while the reverse was true after chemotherapy. CONCLUSION: Gram negative infection remains a significant cause of both ICU admission and mortality. Gram negative infection is largely confined to neutropenic periods after chemotherapy but not after stem cell transplantation. The isolation of Pseudomonas or Klebsiella confers a higher chance of both ICU admission and death.

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. SUBJECTS AND METHODS: Retrospective case note review of children who fulfilled criteria for diagnosis of AGS and had an ophthalmic examination by a paediatric ophthalmologist. RESULTS: Fifty-five children with AGS were evaluated. Of these, 41 children fulfilled diagnostic criteria and had a documented ophthalmic examination. Six children had undergone liver transplantation. Three children had a definite diagnosis of IIH, 2 of whom developed postliver transplant. All 3 were treated medically, but 1 child with IIH required lumboperitoneal shunting. All 3 children with definite IIH have normal vision after treatment. Another child with probable undiagnosed IIH has bilateral optic atrophy and is registered blind. Two children with AGS are registered partially sighted, one with rod cone dystrophy and the other with pigmentary retinopathy and right disc atrophy. SUMMARY: Although visual abnormalities are well described in children with AGS, a minority of children have significant progressive visual loss. Idiopathic intracranial hypertension has been identified as a potentially treatable precipitating factor. CONCLUSIONS: We recommend annual fundoscopy in the follow-up of children with AGS to facilitate early detection and appropriate management of IIH to prevent visual loss.