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HEREDITARY FRUCTOSE INTOLERANCE IN FOUR SWEDISH FAMILIES
Author(s) -
Köhlin Per,
Melin Kerstin
Publication year - 1968
Publication title -
acta pædiatrica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.772
H-Index - 115
eISSN - 1651-2227
pISSN - 0803-5253
DOI - 10.1111/j.1651-2227.1968.tb07281.x
Subject(s) - retching , fructose , medicine , weaning , vomiting , endocrinology , saccharin , physiology , lipolysis , adipose tissue , food science , biology
Summary A brief review is given of hereditary fructose intolerance. Seven adult cases of the disease from four families, two of which are distantly related, are reported. Among their relations at least two more persons have shown symptoms of fructose intolerance. The first symptoms appeared in infancy on weaning, when sweetened formulas or fruit juice supplements were given, and consisted of retching, vomiting, and sweating. All the subjects quickly developed strong aversion to foods containing fructose. None had dental caries. All were physically and mentally normal. The characteristic hypoglycaemia and hypophosphataemia were observed in all cases in intravenous fructose tolerance tests. The plasma FFA showed an initial fall after injection of fructose, followed by a sharp rise. The initial fall probably supports the view that fructose is readily metabolized in adipose tissue in subjects with HFI. The subsequent rise may be due to lipolysis. The urinary excretion of adrenaline was examined in one case, and raised values were recorded during the fructose tolerance tests; the significance of this is discussed.