FAMILIAL HYPOGLYCEMIA PRECIPITATED BY AMINO ACIDS

Abstract

Mann and Magath (1) in 1922 first described the clinical symptoms associated with hypogly- cemia, and in 1924 Harris (2) reviewed its rela- tionship to hyperinsulinism.Since then, numer- ous articles have appeared in the literature reviewing the causes of hypoglycemia, (3-6) describing individual cases and suggesting various surgical and medical methods of management.Although spontaneous hypoglycemia is evidence of abnormal carbohydrate metabolism, this ab- normality may be due to a variety of diseases.The present communication describes three cases of hypoglycemia occurring in one family, and one unrelated case, in which convulsions and profound lowering of the blood glucose were in- duced by the administration of proteins or amino acids.We believe this abnormal relationship between amino acids and glucose metabolism has not been previously described, and will be of great interest, not only to the clinician, but also to the biochemist and physiologist investigating carbohydrate and protein metabolism.CASES Case 1.A female infant was admitted to The Hospital for Sick Children, Great Ormond Street on 29th September 1954, at the age of 4 months for investigation of convulsions.Her mother's pregnancy and labor were normal.Her birth weight was 7 lbs.The infant was breast fed for 10 weeks and then given half cream cow's milk feeds with added sugar.At 3 months she began to have generalized convulsions.She had two or three fits every day and these usually occurred about midway between feeds but not after waking in the mornings.The fits lasted from a few minutes to half-an-hour.Examination showed a plump 12 lb. 3oz.infant with no abnormal physical signs.Her mental development was normal for her age.Since her sister (case 2) had convulsions associated with hypoglycemia, fasting blood was taken and this showed blood sugar levels varying from 40 to 60 mg.per 100 ml.The levels of blood sugar before feeds showed a gradual decline throughout the