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Galactosemia, Diet and Dairy Products

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Galactosemia, Diet and Dairy Products Galactosemia, diet and dairy products: a review İLYAS ERDEM TONGUÇ*, CEM KARAGÖZLÜ *Corresponding author Ege University Faculty of Agriculture, Department of Dairy Technology İlyas Erdem Bornova, Izmir, Turkey Tonguç KEYWORDS: Food allergies, food intolerance, galactose, galactosemia, dairy products. ABSTRACT: Galactosemia is a rare, autosomal recessive condition in which resulting the inability to convert galactose to glucose. Individuals with galactosemia are intolerant of dietary lactose and galactose, primarily found in milk and milk products. If untreated, the disorder can cause liver failure, kidney dysfunction, sepsis, and death. If it is diagnosed soon after birth and treated by removal of lactose and galactose from the diet, the symptoms will resolve and many of the long-term complications, including cataracts and mental retardation, can be prevented. In this review brief information on galactosemia has been given and dietary treatments, especially dairy products consumption for galactosemia patients were discussed. INTRODUCTION GALACTOSEMIA Adverse reactions to food have been seen frequently Galactose is a monosaccharide and have been noted for two millenniums. At least 30% with less sweetness and solubility of individuals experience one or more episodes of food in water compared to glucose. intolerance in their lifetime (1). Increasing public and Chemical structures of galactose Intolerances medical interest have also popularized claims that a isomers are given in Figure 1. Figure 1. Chemical variety of physical and psychological symptoms are the When combined with glucose structure of galactose (8) result of food hypersensitivity. Increase in the number through a dehydration reaction, of patients are therefore deserving investigations on lactose is formed. Galactose is found in dairy products, sugar this subject (2). Such reactions may be mediated beets, and other gums and mucilages. Recognizable amount by immunologic, pharmacologic, toxic, infectious, of galactose in blood and urea can be the signs of some idiosyncratic, metabolic, and neuropsychologic pathological situations (7). mechanisms. Of all adverse reactions to foods and Galactosemia is an autosomal recessive inborn error of food additives, < 20% are due to true allergy or carbohydrate metabolism that results in the inability to hypersensitivity, defined as an event in which a definite metabolize galactose to glucose. It occurs in approximately immunopathologic process can be shown and a cause- 1 in 60,000 newborns (9). Galactosemia is a relatively rare and-effectrelation can be proven (3). inherited enzyme deficiencywith variable worldwide incidence Various terms have been used to describe the adverse reported between 1:30-40,000 in Europe (10) and 1:1,000,000 reactions to food. In 1995 the European Academy of in Japan (11). The incidence in the United States, with Allergology and Clinical Immunology (EAACI) Position mostcases ascertained by newborn screening, is currently vol 24(6) - November/December 2013 - Paper on Food Allergy established a classification of estimated at 1:53,000 (National Newborn Screening and food allergy based on pathogenetic mechanisms. Genetics Resource Center; 2002 Newborn Screening and According to this classification, adverse reactions to Genetic Testing Symposium) (12). Classical galactosemia was food comprise two main groups, toxic and non-toxic first described by von Reuss 1908 (12, 13). Mason and Turner reactions, the latter being subdivided into immune- originally described the condition in 1935, when galactose was Industry Hi Tech mediated (food allergy) and non-immune-mediated found in the urine of an affected patient (9). In the mid 1950s, based on the pathogenic mechanisms involved. The Komrower et al. (1956), demonstrated the accumulation of non-immunological reactions may depend on enzymatic, galactose-1-phosphate in red blood cells, and the condition FOOD pharmacological and, although still unclear, include was enzymatically defined as a deficiency of the galactose- Agro causes such as irritants and psychosomatic responses 1-phosphate uridyltransferase (GALT) enzyme by Kalckar in (4, 5). The term “hypersensitivity” have been proposed 1956 (12, 14). Individuals with galactosemia are intolerant of and is now used as the umbrella term to cover all kinds dietary lactose and galactose, primarily found in milk and milk of adverse reactions to food such as reactions to food products. If untreated, the disorder can cause liver failure, additives, side effects to drugs, psychological reactions kidney dysfunction, sepsis, and death. If it is diagnosed soon blamed on environmental factors, behavioral disorders, after birth and treated by removal of lactose and galactose and others (2, 6). Food hypersensitivity can be subdivided from the diet, the symptoms will resolve and many of the into two groups (2). The term “food allergy” is often long-term complications, including cataracts and mental used non-specifically to include intolerances or psycho- retardation, can be prevented (12). emotional reactions, but should instead be restricted to Dietary lactose is hydrolyzed to glucose and galactose by reactions mediated by classical immune mechanisms lactase in the intestine. Galactose is then further metabolized and non-allergic food hypersensitivity, previously called to uridyl diphosphate (UDP)-glucose by a series of reactions food intolerance, is a non-immune mediated food involving three enzymes, galactokinase (GALK),galactose-1- hypersensitivity (2, 6). phosphate (PO4) uridyl transferase (GALT), and UDP galactose- 60 4-epimerase (GALE). Although galactosemia and galactosuria of galactosemia with a known pathophysiology. Galactitol, can be caused by a deficiency of any one of these enzymes, produced by reduction of free galactose by aldose reduction, classic galactosemia, the condition of greatest clinical produces swelling of lens fibers. Adolescents and adults importance, is caused by a defect in GALT (9). who include higher amounts of galactose in their diet may In individuals with GALT deficiency, galactose and galactose- rarely develop cataracts that interfere with ability to function 1-PO4 accumulate in tissues, as well as the reduction product, and may benefit from lensectomy and intraocular lens galactitol, and the oxidation product, galactonate, which are placement. A less well known, but visually more devastating derived from alternate metabolic pathways for galactose (9). ophthalmologic complication is vitreous hemorrhage during Galactokinase catalyzes the conversion of galactose to the acute neonatal syndrome (17). galactose-1-PO4. Galactose-1-PO4 is then converted to glucose- 1-PO4 by a two-step reaction involving GALT and UDP galactose- Ovarian failure 4-epimerase. GALT catalyzes the transfer of UDP to galactose- Hypergonadotropic hypogonadism is common in females 1-PO4 to form UDP galactose. UDP-galactose is then converted with galactosemia but has not been reported in males (12, to UDP-glucose by UDP galactose-4-epimerase. Galactose 16, 18)The spectrum varies from severe primary ovarian failure is incorporated into glycoproteins and glycolipids, using UDP- requiring hormone support in order to achieve secondary galactose as the substrate for galactosylation reactions. sexual characteristics to premature menopause. Pregnancies without hormonal intervention have been reported (12, 16, 19)The mechanism of ovarian dysfunction is unknown, but it has been hypothesized that chronic exposure to galactose- 1-phosphate and galactitol may directly damage the ovaries (20). Females with classical galactosemia require monitoring of gonadotropin levels and initiation of estrogen and progestin support as needed (12, 21). Intolerances Neurologic sequelae Cerebral oedemas Cerebral oedema is one of the common sequela of galactosemia in newborn infants. Animal models have suggested that the mechanism of cerebral oedema may be due to elevations in brain galactitol concentrations and alterations in glucose, adenosine triphosphate, and phosphocreatine levels increase osmolality (22, 12). Fıgure 2. Lactose is metabolized by lactase in the intestine to galactose and glucose. Galactose is metabolized in the liver via Cognitive impairment the pathway shown in the figure. The enzymes required include (1) galactokinase, (2) galactose-1-PO4 uridyltransferase (GALT),and Many studies have reported that mean intelligence quotient Agro (3)UDP galactose-4-epimerase. The defective enzyme in classic (IQ) scores are reduced in galactosemic children and adults galactosemia is GALT (2). Galactose-1-PO4 and galactose, as (12, 16, 23, 24, 25). Differences between studies have resulted FOOD well as galactitol and galactonate, are elevated in patients with classic galactosemia (9). from a variety of factors. For example, differences in screening and diagnostic practices mean that the initiation of dietary Industry Hi Tech treatment may vary systematically among countries (12). The gold standard for diagnosis is the demonstration of near total absence of GALT activity in red cells. Transfusion of red Speech difficulty cells from a normal donor can interfere with this determination. Speech difficulty is common in galactosemia. One study Measurement of accumulated galactose-1-phosphate in red reported that 56% of patients with galactosemia over 3 years - cells has diagnostic utility even if dietary galactose has been of age have difficulties with speech, with 92 % of these patients vol 24(6) - November/December 2013 withdrawn, although benign variants
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