caa a22 4500 469045035 CHVBK 20180323132822.0 cr unu---uuuuu 170328e19920901xx s 000 0 eng 10.1007/BF01800012 doi (NATIONALLICENCE)springer-10.1007/BF01800012 Fructose and glucagon loading in siblings with fructose-1,6-diphosphatase deficiency in fed state [Elektronische Daten] [T. Nagai, T. Yokoyama, T. Hasegawa, Y. Tsuchiya, N. Matsuo] Summary: Hypoglycaemia induced by fructose administration is one of the diagnostic clues to fructose-1,6-diphosphatase (FDPase) deficiency (McKusick 229700). However, the pathological mechanism of this reactive hypoglycaemia is not fully known. This paper describes two siblings with FDPase deficiency, diagnosed enzymatically in leukocytes, who failed to correct reactive hypoglycaemia after glucagon administration even in the fed state, supporting a possibility that disturbed hepatic phosphorylase activity may be a main cause of reactive hypoglycaemia. SSIEM and Kluwer Academic Publishers, 1992 Nagai T. Division of Neurology and Endocrinology, Tokyo Metropolitan Kiyose Children's Hospital, 1-3-1 Umezono Kiyose, 204, Tokyo, Japan aut Yokoyama T. Division of Neurology and Endocrinology, Tokyo Metropolitan Kiyose Children's Hospital, 1-3-1 Umezono Kiyose, 204, Tokyo, Japan aut Hasegawa T. Division of Neurology and Endocrinology, Tokyo Metropolitan Kiyose Children's Hospital, 1-3-1 Umezono Kiyose, 204, Tokyo, Japan aut Tsuchiya Y. Division of Neurology and Endocrinology, Tokyo Metropolitan Kiyose Children's Hospital, 1-3-1 Umezono Kiyose, 204, Tokyo, Japan aut Matsuo N. Department of Pediatrics, Keio University School of Medicine, Tokyo, Japan aut Journal of Inherited Metabolic Disease Kluwer Academic Publishers 15/5(1992-09-01), 720-722 0141-8955 15:5<720 1992 15 10545 https://doi.org/10.1007/BF01800012 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/BF01800012 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Nagai T. Division of Neurology and Endocrinology, Tokyo Metropolitan Kiyose Children's Hospital, 1-3-1 Umezono Kiyose, 204, Tokyo, Japan aut NATIONALLICENCE 700 1- Yokoyama T. Division of Neurology and Endocrinology, Tokyo Metropolitan Kiyose Children's Hospital, 1-3-1 Umezono Kiyose, 204, Tokyo, Japan aut NATIONALLICENCE 700 1- Hasegawa T. Division of Neurology and Endocrinology, Tokyo Metropolitan Kiyose Children's Hospital, 1-3-1 Umezono Kiyose, 204, Tokyo, Japan aut NATIONALLICENCE 700 1- Tsuchiya Y. Division of Neurology and Endocrinology, Tokyo Metropolitan Kiyose Children's Hospital, 1-3-1 Umezono Kiyose, 204, Tokyo, Japan aut NATIONALLICENCE 700 1- Matsuo N. Department of Pediatrics, Keio University School of Medicine, Tokyo, Japan aut NATIONALLICENCE 773 0- Journal of Inherited Metabolic Disease Kluwer Academic Publishers 15/5(1992-09-01), 720-722 0141-8955 15:5<720 1992 15 10545 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer