From Big Medical Encyclopedia

FRUKTOZURYYa (fructose + wetting Greek uron; a synonym Leva I climb a riya) — availability of fructose (levulose) in urine.

At adults normal fructose in urine is absent. T. often arises at children of early age, especially at premature; in the first 10 days of life of the child concentration of fructose (see) in urine averages 70 mg / 100 ml. The organism of the baby in comparison with an organism of the adult acquires bigger amount of carbohydrates on

1 kg of weight, however regulation of digestion of carbohydrates at the baby is easily broken that leads to the increased allocation with urine not only fructose, but also other sugars (see the Melituria).

Allocate the fruktozuriya caused by hereditary deficit of enzyme of fruktozo-1,6-diphosphatase; so-called high-quality F., caused by insufficiency of enzyme of a ketohexokinase; a fruktozuriya as a symptom at diseases of a liver or disturbances of carbohydrate metabolism at a diabetes mellitus (see a diabetes mellitus).

The disturbance of exchange of fructose caused by hereditary deficit of enzyme of fruktozo-1,6-diphosphatase and which is followed by its intolerance is for the first time described in

1970 by Baker and Uayngrad (L. Baker, A. I. Winegrad). The disease is inherited on autosomal recessively type. Frequency of this pathology in population is not determined yet. Fruktozo-1,6-difosfataza catalyzes formation of frukto-zo-6-phosphate from fruktozo-1,6-diphosphate. At this fermental defect fructose cannot turn into glucose. Lack of enzyme causes also blocking of a gluconeogenesis (see. Carbohydrate metabolism), and the level of glucose in blood in this case is supported due to disintegration of a glycogen. At long starvation reserves of a glycogen are exhausted, there comes the heavy hypoglycemia and a lactacidemia (see). Reception of fructose or sorbite provokes like starvation a heavy hypoglycemia (see), acidosis (see) and acetonuria (see). However it is necessary to consider that at babies at an elevated temperature or failure from food stocks of a glycogen can be also easily exhausted and within several hours the life-threatening crisis caused by acidosis and a hypoglycemia can develop. The disease is clinically shown after reception of fructose, sucrose or sorbite. The main symptoms — apathy, a hypomyotonia, increase in a liver, a spasm and a lump. In the subsequent at children obesity develops (see), increase in a liver is noted. At a lab. a research find a hypoglycemia, a lactacidemia, acetonuria and an alaninuriya. On EEG come to light patol. changes. To enter to the child with the diagnostic purpose fructose, glitserol or dihydroxy acetone dangerously in connection with development of a lactacidemia and hypoglycemia. The final diagnosis is made on the basis of the data obtained at a biopsy of a liver.

Purpose of a diet with an exception of fructose yields good results. Non-compliance with a diet can lead to a lethal outcome.

At high-quality F. removal of fructose with urine depends on amount of the carbohydrates accepted with food (sugar, fruit, honey). With age F. at children usually disappears since in connection with expansion of a diet consumption of sugars relatively decreases. The smaller amount of sugars contains also in a diet of adults.

At a diabetes mellitus F. arises along with a glucosuria (see the Glycosuria). At diseases of a liver, in particular at Konovalov's disease — Wilson (see Gepato-tserebraljnaya dystrophy), T. depends on the use of fructose; points emergence to a functional hepatic unsufficiency F. after loading fructose in number of 50 g and less (see Bath test).

For diagnosis F. apply Selivanov's test with resorcin (see Selivanov test). In the presence in urine of fructose mix is painted in cerise color.

Bibliography: Badalyan L. O., Thabo l both N V. A. and Veltishchev Yu. E. Hereditary diseases at children, page 108, M., 1'971; In and to e of H. D. and. lake of Fructose-1,6-diphosphatase deficiency, another enzyme defect which can present itself with clinical features of tyrosinosis, Clin. chim. Acta, v. 55, p. 41, 1974; In i with-k e 1 H. Die nicht-diabetischen Melliturien des Kindes, in book: IN'od. Probi, d. Padiat., hrsg. v. A. Hottinger n. H. Hauser,

Bd 6, S. 313, Basel — N. Y., 1960.

V. P. Lebedev, V. A. Tabolin.