Examination of the importance of nutritional management in the growth process of 2 patients with organic acid metabolism disorders

Ayako Yoshida 1) , Minatsu Ishige (Wada) Minatsu 1) , Junichi Suzuki 1) , Hiroshi Saito 1) , Tatsuhiko Urakami 1) , Masari Takahashi 1) , Hideo Mugishima 1)

1) Nihon University School of Medicine Pediatrics Department of Pediatrics

J-STAGE release date 20131104

Keywords: methylmalonic acidemia , propionic acidemia , organic acid metabolism disorder , failure to thrive, nutritional management

We report the course of 2 cases of growth failure in organic acid metabolism disorder in our hospital. Methylmalonic acidemia patients who developed at 7 days of age were delayed in diagnosis and initiation of appropriate treatment, and left severe mental retardation and failure to thrive due to sequelae and poor control due to hyperammonemia. Although nutritional management is performed by tube feeding, vomiting is strong and the height is changing to -2.6 SD. On the other hand, in the case of propionic acidemia, which also developed at 7 days of age, early detection and appropriate early treatment were started. Sufficient protein and calorie nutrition was taken based on the treatment criteria, and the height remained at -0.3 SD. Proper nutritional management can be expected to provide a good growth prognosis even for organic acid metabolism disorders.