Article Text
Abstract
Traditional management of congenital nephrotic syndrome has been early bilateral nephrectomy with renal replacement therapy and subsequent transplantation. Management in the United Kingdom has moved towards unilateral nephrectomy and pharmacotherapy with ACE inhibition and non-steroidal anti-inflammatories to limit renal function and hence protein leak. The frequent administration of intravenous albumin is a mandatory requirement in almost all patients in the early stages of the disease. We report a series of seven patients in whom parents were trained in the administration of albumin via a central line in the home setting, with reduction in the need for prolonged hospital admission or attendance. We demonstrate that albumin can be safely administered in the home setting, over a short period of time per infusion, and is well tolerated. No mortality was directly attributable to the home albumin program. Complication rates were favourable compared to other home parenteral therapies. Administration of intravenous albumin can be safely performed in the home setting, reducing the length of in-patient stay and potentially delaying the need for nephrectomy and renal replacement therapy.