From: Pancreatic involvement in patients with inborn errors of metabolism
 | Case 1 | Case 2 | Case 3 | Case 4 | Case 5 | Case 6 |
---|---|---|---|---|---|---|
Underlying disease | MMA | MMA | PPA | PPA | FAO | Hyperornithinemia |
Age/Sex | 20 y/M | 15 y/M | 14 y/M | 10 y/M | 15 y/M | 35 y/M |
Birth history | 37 wks, 2.7Â kg, C/sec | GA 37 wks, 2.47Â kg, C/sec | GA 36 wks, 3.2Â kg, C/sec | Full term, 3.7Â kg, NSVD | Full term, NSVD | Full term, 3.73Â kg, NSVD |
Age of diagnosis | Perinatal period | Perinatal period | Perinatal period | Perinatal period | 40Â m | 7 y |
NH3 (μg/dL) | 500 |  > 1000 | 400 | 1200 | 300 | Normal |
Management | Special formula (Propimex) Low-protein diet L-carnitine supplement | Low-protein diet Special formula (MPA) | Freq. feeding Raw corn starch | Insulin Low-protein diet |