Rickets during extended total parenteral nutrition in a boy with a VIP-producing ganglioneuroma and the WDHA syndrome

Abstract

A four-month-old boy was operated on for a retroperitoneal neuroblastoma, stage IV-S. Six months later he developed the WDHA syndrome and was treated with total parenteral nutrition (TPN) for the following 18 months. The tumour had transformed into a ganglioneuroma which produced vasoactive intestinal peptide. After nine months of TPN he developed rickets, despite the fact that a recommended daily dose of vitamin D had been given. The rickets healed after the addition of therapeutic doses of vitamin D and an oral calcium phosphate supplement. The vitamin D status of children receiving long-term TPN should be monitored. Higher doses of vitamin D than are generally recommended may be necessary during long-term TPN in infants and young children. The calcium and phosphorus contents of standard TPN regimens in such cases may also be insufficient.