ABSTRACT 674

Abstract

Background and aims: Congenital metabolic disorders are a rare but important cause of critical illness in children. Practical challenges like obtaining vascular access in low birth weight children are common. Certain children need liver transplantation and intra-operative CVVH. Aims: To understand their demographic and treatment profile for outcome prediction and service provision in these patients. Methods: 31-bedded tertiary PICU in the UK with 1500 admissions/year. Retrospective analysis of the CVVH electronic database over the past 3 years. Results: 21 patients with congenital metabolic disorders underwent CVVH. The median age was 7 years (2 days-11 years) and weight 16Kg (1.8–29.3). Propionic acidaemia and primary hyperoxaluria were two of the common diagnoses. 3 patients were significantly coagulopathic. All patients were invasively ventilated and 4 patients needed inotropic support. 2 patients also underwent total plasma exchange on a total of 4 occasions. Maximum serum ammonia was 1200. The median total duration of CVVH was 70 hrs (29 - 552). The median PICU length of stay was 4 days (1 - 29). 2 children died, both within the first 48 hrs with multiorgan dysfunction, one had undergone liver transplantation in the preceding 24 hrs. 4 patients who survived had ongoing requirement for haemodialysis noted at PICU discharge. Conclusions: Patients with congenital metabolic disorders require prolonged CVVH therapy and PICU stay. Though survival good, many require multiple admissions and some undergo organ transplant. This data would be helpful when identifying their levels of intensity of intensive care and service provision. There is a need for a national / international database of children undergoing CVVH.