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Hijová Emília, Bertková Izabela, Štofilová Jana, Strojný Ladislav, Chmelárová Anna and Bomba Alojz

gastrointestinal disorders: what to recommend? Therapeutic Advances in Gastroenterology 2010, 3: 307-319 16. Mack DR: Probiotics in inflammatory bowel diseases and associated conditions. Nutrients 2011, 3: 245-264 17. Kuno T, Tsukamoto T, Hara A, Tanaka T: Cancer chemoprevention through the induction of apoptosis by natural compounds. JBC 2012, 3: 156-173. 18. Martin KR: Targeting apoptosis with dietary bioactive agents. Experimental Biology and Medicine (Maywood) 2006, 231: 117-129. 19. Pulli B, Ali M, Forghani R, Schob S, Hsieh KLC, Wojtkiewicz G

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Cirila Hlastan Ribič, Jožica Maučec Zakotnik, Barbara Koroušić Seljak, Rok Poličnik, Urška Blaznik, Nataša Fidler Mis, Ivan Eržen, Chen Ji and Francesco P. Cappucio

insulin resistance. Diabetologia 2004; 47: 300-3. 4. Cumming RG, Mitchell P, Smith W. Dietary sodium intake and cataract: the blue mountains eye study. Am J Epidemiol 2000; 151: 624-6. 5. D’Elia L, Rossi G, Ippolito R, Cappuccio FP, Strazzullo P. Habitual salt intake and risk of gastric cancer: a metaanalysis of prospective studies. Clin Nutr 2012. doi: 10.1016/j. clnu.2012.01.003. 6. Cappuccio FP, Kalaitzidis RG, Duneclift S, Eastwood JB.Unravelling the links between calcium excretion, salt intake, hypertension, kidney

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Mohamed Abdelhaq Abbes and Karima Bereksi-Reguig

from Australia and the United States. Health & Place 38: 96-102, 2016. 8. Bray GA, Popkin BM. Dietary fat intake does affect obesity. Am J Clin Nutr 68(6-1): 1157-73, 1998. 9. Wyckoff EP, Evans BC, Manasse SM, Butryn ML, Forman EM. Executive functioning and dietary intake: Neurocognitive correlates of fruit, vegetable, and saturated fat intake in adults with obesity. Appetite 111: 79-85, 2017. 10. Casas-Agustench P, Arnett DK, Smith CE et al. Saturated Fat Intake Modulates the Association between an Obesity Genetic Risk

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Jadwiga Charzewska, Zofia Chwojnowska, Bożena Wajszczyk and Elżbieta Chabros

References Alexy U, Sichert-Hellert W, Kersting M. 2002. Fifteen- year time trends in energy and macronutrient intake in German children and adolescents: results of the DONALD study, British J Nutr 87:595–604. Bray GA and Popkin BM. 1998. Dietary fat intake does affect obesity! Am J Cl Nutr 68:1157–73. Charzewska J, Chabros E, Wajszczyk B, Chwojnowska Z, Kraszewska E. 2009. Underweight, overweight, and obesity trends in Warsaw adolescents during the 35 years period, 1971–2006. Eur J Epidem 24(suppl 1):31. Charzewska J, Chabros E. 2010

Open access

Alexandra Sima

-2062, 2017. 10. American Diabetes Association . Standards of Medical Care in Diabetes. Approaches to glycemic treatment. Diabetes Care 41 (Suppl. 1): 1-159, 2018. 11. Seidelmann SB, Claggett B, Cheng S et al . Dietary carbohydrate intake and mortality: a prospective cohort study and meta-analysis. Lancet Public Health 3(9): 419-28, 2018.

Open access

Jana Kazandjieva, Dimitrina Guleva, Assia Nikolova and Sonya Márina


Leucinosis (maple syrup urine disease - MSUD) is an inherited aminoacidopathy and organic aciduria caused by severe enzyme defect in the metabolic pathway of amino acids: leucine, isoleucine, and valine. The classical variant of the disease is characterized by accumulation of both amino and α-keto acids, particulary the most toxic rapid elevation of circulating leucine and its ketoacid, α-ketoisocaproate, which cause encephalopathy and life-threatening brain swelling. However, patients with the most severe form, classical maple syrup urine disease, may appear normal at birth, but develop acute metabolic decompensation within the first weeks of life with typical symptoms: poor feeding, vomiting, poor weight gain, somnolence and burnt sugar-smelling urine, reminiscent of maple syrup. Early diagnosis and dietary intervention improve the patient’s condition, prevent severe complications, and may allow normal intellectual development.

We present a 4-month old infant with leucinosis dignosed 3 months earlier, due to elevated levels of amino acids: leucine, isoleucine and valine. The patient was full-term neonate with an uncomplecated delivery, without any family history of metabolic disorder or consanguinity. The infant was referred to a dermatologist, because of maculopapular exanthema on the scalp, trunk, upper and lower extremities, and exfoliative dermatitis of the perioral, particularly anogenital regions, associated with diarrhea. Skin involvement was associated with poor general condition of the infant exhibiting severe hypotension, anemic syndrome, dyspepsia and neurological symptoms. Exanthema developed a few days after the initiation of nutritional therapy for MSUD: isoleucine-, leucine-, and valine-free powdered medical food (MSUD-2) supplemented with iron. Zink levels were within normal ranges. Rapid skin improvement occurred after adequate branched-chain amino acids supplementation was commenced under regular laboratory control (normal zinc serum level with deficiencies of leucine and valine), skin hygiene with antiseptics, emollients and low potent topical corticosteroids.

Treatment of acute metabolic decompensation and dietary restriction of branched-chain amino acids are the main aspects in the management of maple syrup urine disease. Common findings in patients with MSUD include: plasma amino acid imbalance, particularly of essential amino acids, failure to thrive attributed to restriction of particular precursor amino acids and natural proteins, micronutrient deficiencies or higher energy requirement due to chronic illness or inflammation. Due to low intake of branched-chain amino acids, some patients develop skin lesions known as acrodermatitis enteropathica-like syndrome.

Here we report a case of an infant who developed acrodermatitis enteropathica-like skin eruptions due to branched-chain amino acid deficiency during treatment of maple syrup urine disease. According to available world literature, this is the first report of acrodermatitis enteropathica-like syndrome in an infant with maple syrup urine disease (leucinosis) in the Republic of Bulgaria.

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Andrzej Rutkowski, Sebastian A. Kaczmarek, Marcin Hejdysz and Dorota Jamroz

Poultry Feedstuffs (1989). WPSA, Beekbergen, The Netherlands. Gdala J. (1998). Composition, properties and nutritive value of dietary fibre of legume seeds. Areview. J. Anim. Feed Sci., 7: 131-149. Guillamon E., Pedrosa M.M., Burbano C., Cuadrado C., De Cortes Sanchez M., Muzquiz M. (2008). The trypsin inhibitors present in seed of different grain legume species and cultivar. Food Chem., 107: 68-74. Haug W., Lantzsch H.J. (1983). Sensitive method for the rapid determination of phytate in cereals and cereal products. J. Sci

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Laura Mihalache, Otilia Niță, Andreea Gherasim, Irina Ringhilescu, Alina Delia Popa, Mariana Graur and Lidia Iuliana Arhire

, Ebbeling CB, Cifuentes M, Lera L, Bustos N, Ludwig DS. Effects of replacing the habitual consumption of sugar-sweetened beverages with milk in Chilean children. Am J Clin Nutr 88: 605-611, 2008. 5. Bowman SA, Gortmaker SL, Ebbeling CB, Pereira MA, Ludwig DS. Effects of fast-food consumption on energy intake and diet quality among children in a national household survey. Pediatrics 113: 112-118, 2004. 6. Ranjit N, Evans MH, Byrd-Williams C, Evans AE, Hoelscher DM. Dietary and activity correlates of sugar-sweetened beverage consumption among

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Nădăşan Valentin, Sîmpetrean Andreea, Tarcea Monica and Abram Zoltan

.1016/j.atherosclerosis.2014.03.013. 7. Guasch-Ferré M, Babio N, Martínez-González MA, et al. Dietary fat intake and risk of cardiovascular disease and all-cause mortality in a population at high risk of cardiovascular disease. Am J Clin Nutr. 2015;102(6):1563-1573. doi: 10.3945/ajcn.115.116046. 8. Kris-Etherton PM, Fleming JA. Emerging nutrition science on fatty acids and cardiovascular disease: nutritionists’ perspectives. Adv Nutr. 2015;6:326S-337S. doi: 10.3945/an.114.006981. 9. Hooper L, Summerbell CD, Thompson R, et al. Reduced or modified

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Jan Niemiec, Julia Riedel, Tadeusz Szulc and Małgorzata Stępińska

Feeding Wheat Distillers Dried Grains with Solubles (DDGS) to Laying Hens and its Effect on Performance and Egg Quality

The purpose of this experiment was to determine the effect of wheat DDGS as a feed ingredient on the performance of laying hens and their egg quality. ISA Brown laying hens were administered a feed mixture containing 15% (E1) or 20% (E2) wheat DDGS for 12 weeks. The hens from the control group (C) received a standard diet based on soybean meal as the main protein source only. Laying performance (laying %), average egg weight (g), average daily feed intake (g/hen), and feed conversion ratio (kg/kg eggs) were recorded over the study period. Egg quality traits (egg weight, thick albumen quality, yolk colour, yolk percentage, shell percentage and shell thickness) were evaluated twice: before the start and at the end of the experiment. There was no effect of dietary DDGS on laying performance or on feed intake. The average egg weight was significantly lower in both experimental groups and the feed conversion ratio was lower compared to the control group. Dietary wheat DDGS did not affect the main egg quality parameters except for thick albumen quality. Eggs from hens fed the diet with DDGS had higher values of Haugh unit than those from the control hens. These results suggest that wheat DDGS can be used in amounts of up to 20% as a component of feed mixtures for flocks of laying hens.