Fructose-1,6-diphosphatase deficiency in Israel

S. W. Moses, N. Bashan, B. F. Flasterstein, A. Rachmel, A. Gutman

Research output: Contribution to journalArticlepeer-review

8 Scopus citations

Abstract

The clinical and biochemical data on nine patients belonging to six families with fructose-l,6-diphosphatase deficiency are reported. Two of the six families were Jewish, three were Moslem Arabs and one was of Druze origin. All patients had had neonatal hypoglycemia, lactic acidosis and an abnormal fructose or glycerol loading test. At a later age, instances of hypoglycemia occurred in patients both with and without preceding illness. Hypoglycemic attacks were associated with severe hyperuricemia and metabolic acidosis. Therapeutic measures included a restriction in fructose intake and avoidance of prolonged fasting, particularly during febrile episodes.

Original languageEnglish
Pages (from-to)1-4
Number of pages4
JournalIsrael Journal of Medical Sciences
Volume27
Issue number1
StatePublished - 1 Jan 1991
Externally publishedYes

Keywords

  • enzyme deficiency
  • fructose-1-6-diphosphatase
  • hypoglycemia

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