RESUMO
We describe 3 patients with short bowel syndrome who had persistently elevated serum methylmalonic acid (MMA) levels while being treated for vitamin B12 deficiency. Following treatment for presumed small bowel bacterial overgrowth, MMA levels normalized. Among patients with short bowel syndrome, MMA levels may have limited specificity for vitamin B12 deficiency.
Assuntos
Síndrome da Alça Cega/diagnóstico , Ácido Metilmalônico/sangue , Síndrome do Intestino Curto/complicações , Deficiência de Vitamina B 12/diagnóstico , Biomarcadores/sangue , Síndrome da Alça Cega/sangue , Síndrome da Alça Cega/etiologia , Criança , Pré-Escolar , Diagnóstico Diferencial , Feminino , Humanos , Masculino , Síndrome do Intestino Curto/microbiologia , Vitamina B 12/uso terapêutico , Deficiência de Vitamina B 12/sangue , Deficiência de Vitamina B 12/tratamento farmacológico , Deficiência de Vitamina B 12/etiologia , Complexo Vitamínico B/uso terapêuticoRESUMO
Fish oil-based lipid emulsions (FOLEs) have been used to treat cholestasis in children with intestinal failure-associated liver disease (IFALD). When FOLEs are dosed at 1 g/kg/d, essential fatty acid (EFA) deficiency typically does not occur. We describe the clinical course of a severely malnourished parenteral nutrition-dependent infant with IFALD. Baseline EFA panels were normal upon starting FOLE at 1 g/kg/d. Despite biochemical improvement in IFALD, weight velocity was below target and biochemical EFA status worsened, even after correction for other factors affecting weight. The FOLE dose was increased to 1.5 g/kg/d, resulting in improvement of weight velocity and EFA status. This suggests that in severely malnourished infants being treated for IFALD, higher doses of FOLE may be required for adequate growth and to prevent EFA deficiency.