Recent reports of pancreatitis in patients with inborn errors of metabolism have appeared in the literature. Pancreatitis has been reported to be a complication primarily of the branched chain amino acid disorders.1Kahler SG Sherwood WG Woolf D et al.Pancreatitis in patients with organic acidemias.J Pediatr. 1994; 124: 239-243Abstract Full Text Full Text PDF PubMed Scopus (97) Google Scholar However, it also has been reported in several organic acid and fatty acid disorders such as carnitine palmitoyl-transferase II deficiency.2Tein I Christodoulou J Donner E McInnes RR Carnitine palmitoyl-transferase II deficiency: a new cause of recurrent pancreatitis.J Pediatr. 1994; 124: 938-940Abstract Full Text PDF PubMed Scopus (23) Google Scholar To our knowledge there have been no reports of pancreatitis in patients with glutaric acidemia type I. We describe one such patient, a 22-year-old severely disabled woman known to have glutaric acidemia type I. This patient, who resides in a chronic care facility, has severe dystonia, generalized joint contractures, severe scoliosis, and is unable to speak or communicate effectively. She was receiving a 1700 calorie, low-protein diet (15% protein, 40% carbohydrate, and 45% lipid) on our recommendation. The high-lipid diet was instituted to maintain a low recommended nutrient intake (RNI) for protein (1.7 gm/kg per day). Our patient came to the hospital in moderate distress with recurrent retching, intolerance of gastrostomy feedings, and copious bilious drainage from the gastrostomy tube. Her abdomen was soft and not distended; tenderness was difficult to elicit. Bowel sounds were absent. The serum amylase concentration was substantially elevated at 744 U/L (12.40 μkat/L) (normal, 23 to 120 U/L [0.38 to 2.00 μkat/L]). Serum amylase and lipase levels peaked at 1022 U/L (17.04 μkat/L) and 1280 U/L (12.34 μkat/L) (normal, 23 to 300 U/L [0.38 to 5.00 μkat/L]), respectively. The calcium level was normal at 8.8 mg/dl (2.2 mmol/L) (normal, 8.4 to 10.4 mg/dl [2.1 to 2.6 mmol/L]). The patient was kept in a fasting state and suction was applied to the gastrostomy tube. Antibiotic therapy was started because of the possibility of ascending cholangitis and intravenous fluids were administered. Her condition steadily improved and when the patient was discharged she was tolerating full feedings (total 1800 calories/day, 32% lipids). No viral or bacterial pathogens have been isolated and there has been no recurrence of the pancreatitis. The precise pathogenic mechanism for pancreatitis in our patient remains an enigma. A contributing factor may be the high lipid intake by patients receiving protein-restricted diets, as reported in the literature. A 17-year-old boy with Crohn disease had acute pancreatitis while receiving total parenteral nutrition with a 20% fat emulsion. A rechallenge after recovery resulted in the quick return of the signs and symptoms of acute pancreatitis. The authors concluded that the acute pancreatitis was caused by intolerance to the high concentration of lipid.3Lashner BA Kirsner JB Hanauer SB Acute pancreatitis associated with high-concentration lipid emulsion during total parenteral nutrition therapy for Crohn's disease.Gastroenterology. 1986; 90: 1039-1041PubMed Google Scholar The authors suggested that a high-lipid diet is inappropriate for patients wtih Crohn disease and that their caloric needs should not be met by increasing the proportion of lipids in their diets. We suggest that low-protein, high-lipid diets may also be associated with an increased risk for pancreatitis in patients with glutaric acidemia type I and in patients with other organic acidemias. 9/35/71613