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- W2064278323 abstract "The course of the female infant herein presented indicates that glucagon (G) deficiency was the cause of severe hypoglycemia; only two such causes (from Europe) have previously been described. She presented acutely at age 5 mos. with an abnormal cry, inspiratory stridor, a blood sugar (BS,mg/dl) of 30, and serum insulin (I,μU/ml) < 5. Subsequently, BS was persistently 15-30 and simultaneous I = < 5-10. Growth hormone, cortisol, catecholamine, and thyroxine were normal and there was no ketoacidosis or hepatomegaly. Intravenous (IV) (G) induced a BS rise of 45 in 10 mins. and an I.V. push of alanine (250 mg/kg) did not cause a rise in the BS over a two hour period. After frequent feedings and diazoxide (to 30mg/kg/d) failed to control the BS, a 90% pancreatectomy was performed for suspected hyperinsulinism. The gross and microscopic appearance of the pancreas was normal except that immunofluorescence revealed reduced numbers of ∞ and β cells. Serum G (by RIA after extraction) was undetectable in the only preoperative (pre-OP) value obtained, when BS = 18 and I < 5. Hypoglycemia presisted post-OP, and on two occasions no G was detectable when I was <5 and BS 30. Also, no G was detectable in q15 min specimens x 4 after an IV push of 250 mg/kg of arginine. IV G at 8 ng/kg/min stabilized the BS both pre-and post OP. After failure to respond to diazoxide post OP, nomogly-cemia has been maintained with sub Q protamine zinc G (0.5 mg/kg/d in two divided doses) and supplemental carbohydrate feedings. G deficiency as a cause of infantile hypoglycemia should be ruled out prior to pancreatectomy for suspected hyperinsulinism." @default.
- W2064278323 created "2016-06-24" @default.
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- W2064278323 date "1981-04-01" @default.
- W2064278323 modified "2023-10-18" @default.
- W2064278323 title "429 INFANTILE HYPOGLYCEMIA DUE TO GLUCAGON DEFICIENCY" @default.
- W2064278323 doi "https://doi.org/10.1203/00006450-198104001-00440" @default.
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