Abstract
Peritoneal dialysis (PD) or hemodialysis (HD) are still recommended for the treatment of neonatal maple syrup urine disease (MSUD), but present many risks and disadvantages.
We treated 4 MSUD decompensated neonates with nasogastric continuous feeding programmed on the basis of daily plasma aminoacids. Before starting treatment their neurologic status was greatly deteriorated and leucine values were between 3.0 and 3.3 mmol/l. A normo - hypercaloric (100-130/kg/day) diet, with fluid intake ranging between 100 and 150 ml/kg/day and BCAA-free mixture (2g/kg/day) as the only source of aminoacids was then immediately administered by continuous nasogastric feeding. Valine and isoleucine were reintroduced 2-3 days later to avoid deficiencies.
This kind of feeding was well tolerated and dialysis was never necessary. Leucine values normalized (<0.5 mmol/l) in 7-12 days. All the patients, now between 17 months and 4 years of age have normal height and weight velocities and psychomotor development. This data show that also neonatal MSUD may be treated by diet alone provided it is carefully prepared on the basis of daily aminoacids plasma values.
Article PDF
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Parini, R., Sereni, L. & Bagozzi, D. 153 NASOGASTRIC CONTINUOUS FEEDING AS THE ONLY TREATMENT IN NEONATAL MAPLE SYRUP URINE DISEASE. Pediatr Res 30, 653 (1991). https://doi.org/10.1203/00006450-199112000-00183
Issue Date:
DOI: https://doi.org/10.1203/00006450-199112000-00183