Conclusion: A complete ASPA gene workup needs to be done to elucidate the mutation profile in our population. We present a series of patients with organic acidaemia complicated by pancreatitis. Patients A and B had propionic and methylmalonic acidaemias respectively. They presented with vomiting and developed severe, persistent abdominal pain requiring opiates, ketamine and intensive care support. Patient A had a marginally raised amylase but radiological findings were consistent with pancreatitis. She died after a prolonged PICU admission. Patient B had a 48 hour rise in amylase. He had a prolonged PICU stay and was discharged home on opiates. Patient C had propionic acidaemia and presented with vomiting. This resolved but he had an unexpected cardiac arrest, which he did not survive. Post-mortem showed pneumonia and pancreatitis. Patient D had methylmalonic acidaemia and died after a short illness. Post-mortem revealed acute on chronic pancreatitis, despite a normal amylase. These cases highlight: (i) Pancreatitis may be more common than thought (ii) Amylase is an unreliable marker. None of the patients had a significantly raised amylase. Diagnosis was based on clinical and radiological grounds (iii) Severe pancreatitis poses management difficulties in terms of analgesia and nutrition. Current best practice is to continue with enteral feeds and aggressive pain control, if necessary with intubation and sedation for a prolonged period. Background: Due to its property to enhance CPS-1, NCG could be used to treat OA-associated hyperammonemia. Design: Retrospective study in OAs patients with hyperammonemia episodes receiving NCG between 1995 and 2009. End-points were plasma ammonia (NH3), symptoms (neurological, psychiatric, psychomotor, hepatic) and safety Results: Overall, 57 patients were included. 48 episodes of hyperammonemia (60.4% of neonatal onset) in 41 patients (4 Isovaleric, 21 Methylmalonic, 16 Propionic Acidemias) were analyzed for efficacy. Median (range) episode duration was 6 days . Median (range) starting dose and duration of NCG was 75.5 mg/kg (13.3-303.0) and 4 days (1-15), respectively. The maintenance dose was slowly reduced; 43.8% of hyperammonemia episodes were treated with concomitant scavengers. Median (range) NH3 decreased from baseline of 215 μmol/L (76-1633) to 52.0 μmol/L (15-158) (last value under NCG). Consistent NH3 decrease was observed in all OAs, irrespective of concomitant scavengers. Median time to achieve NH3<=60 μmol/L under NCG was 36.5 hours. The clinical symptoms improved following NCG initiation. Among 74 adverse events observed, 22 were serious, mostly not drugrelated, except for 1 death (neurological damage). Conclusions: Carglumic acid rapidly and effectively reduces NH3 in OAassociated hyperammonemia, leading to an overall clinical improvement, with an acceptable safety profile. Over a period of 3 years 2498 patients with suspected metabolic disorder were evaluated. The patients showed 2:1 male:female ratio. The study group included asymptomatic screen positive neonates(n=25), symptomatic neonates and young children with acidosis, hypoglycaemia, hyperammonemia etc. Inborn error of metabolism was detected in~19.2% patients, prevalent being MMA(11.4%) and GA1(9.1%). Disorders such as PA, IVA,MSUD, tyrosinemia type1, canavan disease, biotinidase deficiency and others ranged from 0.5-2.4% of the affected subjects. In addition a few cases of rare disorders such as SSADH, 2 hydroxy glutaric aciduria etc were also diagnosed. Lactic acidosis and/or ketosis have been a consistent finding in several samples. Around 3.3% showed dicarboxylic aciduria either due to a metabolic defect / MCT administration or sickness. Early diagnosis has helped to initiate management in 7 MMA, 4 GA1, 2 IVA and 3 PA patients. Prenatal diagnosis in subsequent pregnancies was facilitated in referral laboratories for 2 MMA, 1 GA1 and 1 SSADH family. Thus it is observed that offering OA analysis within the country has helped to reveal inherited metabolic disorder profile amongst Indians and to initiate management and offer prenatal diagnosis to reduce the burden of recurrence in affected families. Human exposure of the mycotoxin fumonisin B (FB) is greatest in regions where maize products are the dietary staple. The tricarballylic acid moieties of FB are structurally similar to sphingolipids which can cause neural tube defects due to altered sphingolipid metabolism by inhibition of ceramide synthase. The toxic effects of FB can also lead to acute mycotoxicosis, esophageal cancer in humans and cancer, pulmonary edema, leukoencephalomalacia in animals. Case. A 5 days old female patient was admitted with elevated propionylcarnitine (5.5 μmol/L; cut-off 4.9 μmol/L) in expanded newborn screening. During second-tier tests, urinary organic acid analysis by GC-MS revealed excretion of "tricarballylic acid" (58 μmol/mol creatinine). The mother had vitamin B12 deficiency. Breast-feeding was withheld as a potential source of the baby's fumonisin exposure because her family traditionally consumed high amounts of maize and maize products. Tricarballylic aciduria ceased within three weeks. Samples of ground corn taken from the family revealed a high content of FB (4164 ppb; N< 100 ppb). This is the youngest baby reported to be exposed to FB. Early diagnosis of this baby with urinary organic acid analysis led to initiation of the regional investigation of contaminated maize products by the Turkish Ministry of Health. 