ESPE Abstracts

Background: Small amount of methanol is produced in the intestine by hydrolysis of pectin from foods specially fruits and vegetables by intestinal microbiome and is absorbed and oxidized in the liver first to formaldehyde then formic acid and is excreted through feces.

Case Report: A 4.3-year-old boy from first cousin consanguine parents was brought due to history of periodic sleepiness, seizure, diarrhea and vomiting and the lab tests showed blood sugar of 11, 20, 22 and 34 mg/dl. His physical examination was all right with normal growth parameters. After work up with the diagnosis of hyperinsulinemic hypoglycemia corn starch and diazoxide were started for him. He was lost from follow up and his medicines were discontinued by family. He came again when he was 11.5 years old with the signs of polyuria, polydipsia and weight loss. Oral glucose tolerance test was done with peak of 253 mg/dl of glucose and 188 μIU/ml of insulin and Homeostatic model assessment (Insulin resistance) HOMA-IR was 18 (normal < 2.6) that was in accordance with type 2 diabetes. Meglitinide was started for him. Blood sugar became normal and his mother herself discontinued the medicine after some days. Three days later he became sleepy and then went to coma. He was brought to our hospital again. He was in coma and after 3 – 4-day fluid therapy he became awake for 2-3 days but with imbalance in walking and speech problems in 9 months admission. All of the laboratory tests for organ system damage and inborn errors of metabolism were done and were normal. A toxicology investigation in a special laboratory showed high methanol = 12.2 mg/dL (acceptable level: 0.2 – 3). His eyes, kidney and liver were normal. Formaldehyde and formic acid were low. With the suspicion of alcohol dehydrogenase deficiency oral daily Zinc that not only is in the structure of enzyme also is its cofactor was administered and he became completely normal. Genetic study was done that confirmed the diagnosis. Now his height is 175 cm (midparental height: 171.5) and he is studying in the last year of law faculty.

Conclusion: High methanol due to alcohol dehydrogenase deficiency may be the reason of hypo and hyperglycemia and coma.