Technological challenges and strategies for developing low-protein/protein-free cereal foods for specific dietary management

Food Research International

Volumn 54, Issue 1, 2013, Pages 935-950

  Zannini, Emanuele ^a   Kingston, Wilma ^a   Arendt, Elke K ^a   Waters, Deborah M ^a  

^a UNIVERSITY COLLEGE CORK   (Ireland)

Author keywords

Cereal foods; Low protein protein free; Medical nutrition therapy

Indexed keywords

ARTIFICIAL FLAVOURS; BUDGETARY ALLOCATION; LOW-PROTEIN/PROTEIN-FREE; MEDICAL NUTRITION THERAPY; METABOLIC DISORDERS; TECHNOLOGICAL ASPECTS; TECHNOLOGICAL CHALLENGES; TECHNOLOGY STRATEGIES;

AMINO ACIDS; BLENDING; COMPLEX NETWORKS; COSTS; MOLECULAR BIOLOGY; NUTRITION; PATIENT MONITORING; PATIENT TREATMENT; PROTEINS;

FOOD SAFETY;

EID: 84886298618     PISSN: 09639969     EISSN: None     Source Type: Journal    
DOI: 10.1016/j.foodres.2013.03.001     Document Type: Article

References (225)

1. AB Enzymes, I. (2006). AB Enzymes launches VERON® GMS+, an enzymatic monoglyceride replacer. In. Darmstad, Germany.
2. AB Enzymes, I. (2007a). Veron® 292 Description and specification. In. Darmstadt, Germany.
3. AB Enzymes, I. (2007b). Veron® Amylofresh description and specification. In.
4. AB Enzymes, I. (2012a). AB Enzymes announce expansion of VERON® product range - AB Enzymes. In. Darmstadt, Germany.
5. AB Enzymes I. Baking 2012, Vol. 2012. AB Enzymes, Darmstadt, Germany. A. Moelker (Ed.).
6. AB Enzymes, I. (2012c). Machinability and volume. In. Darmstadt, Germany.
7. Abdel-Aal E.-S.M. Functionality of starches and hydrocolloids in gluten-free foods. Gluten-free food science and technology 2009, 200-224. Wiley-Blackwell.
8. Acs E., Kovacs Z., Matuz J. Bread from corn starch for dietetic purposes. 1. Structure formation. Cereal Research Communications 1996, 24(4):441-449.
9. ADA Position of the American Dietetic Association: Integration of medical nutrition therapy and pharmacotherapy. Journal of the American Dietetic Association 2003, 103(10):1363-1370.
10. Afenjar A., Moutard M.-L., Doummar D., Guet A., Rabier D., Vermersch A.-I., et al. Early neurological phenotype in 4 children with biallelic PRODH mutations. Brain & Development 2007, 29(9):547-552.
11. Ahlborn G.J., Pike O.A., Hendrix S.B., Hess W.M., Huber C.S. Sensory, mechanical, and microscopic evaluation of staling in low-protein and gluten-free breads. Cereal Chemistry 2005, 82(3):328-335.
12. Amano Enzyme, I. (2007). Product listing food processing use. In (Vol. 2012). Nagoya, Japan.
13. American Dietetic Association Position of the American Dietetic Association: Integration of medical nutrition therapy and pharmacotherapy. Journal of the American Dietetic Association 2003, 103(10):1363-1370.
14. Anton A.A., Artfield S.D. Hydrocolloids in gluten-free breads: A review. International Journal of Food Sciences and Nutrition 2008, 59(1):11-23.
15. Aparicio M., Chauveau P., De Precigout V., Bouchet J.L., Lasseur C., Combe C. Nutrition and outcome on renal replacement therapy of patients with chronic renal failure treated by a supplemented very low protein diet. Journal of the American Society of Nephrology 2000, 11(4):708-716.
16. Araki E., Ikeda T.M., Ashida K., Takata K., Yanaka M., Iida S. Effects of rice flour properties on specific loaf volume of one-loaf bread made from rice flour with wheat vital gluten. Food Science and Technology Research 2009, 15(4):439-448.
17. Arendt E., Moroni A., Zannini E. Medical nutrition therapy: Use of sourdough lactic acid bacteria as a cell factory for delivering functional biomolecules and food ingredients in gluten free bread. Microbial Cell Factories 2011, 10:1-9.
18. Ayodele O.E., Alebiosu C.O. Burden of chronic kidney disease: An international perspective. Advances in Chronic Kidney Disease 2010, 17(3):215-224.
19. Baboolal K., McEwan P., Sondhi S., Spiewanowski P., Wechowski J., Wilson K. The cost of renal dialysis in a UK setting-A multicentre study. Nephrology, Dialysis, Transplantation 2008, 23(6):1982-1989.
20. Bailey C.G., Ryan R.M., Thoeng A.D., Ng C., King K., Vanslambrouck J.M., et al. Loss-of-function mutations in the glutamate transporter SLC1A1 cause human dicarboxylic aminoaciduria. The Journal of Clinical Investigation 2011, 121(1):446-453.
21. Bilginsoy C., Waitzman N., Leonard C.O., Ernst S.L. Living with phenylketonuria: Perspectives of patients and their families. Journal of Inherited Metabolic Disease 2005, 28(5):639-649.
22. Biliaderis C.G., Maurice T.J., Vose J.R. Starch gelatinization phenomena studied by differential scanning calorimetry. Journal of Food Science 1980, 45(6):1669-1674.
23. Biyani C.S., Cartledge J.J. Cystinuria-Diagnosis and management. EAU-EBU Update Series 2006, 4(5):175-183.
24. Blau N., van Spronsen F.J., Levy H.L. Phenylketonuria. Lancet 2010, 376(9750):1417-1427.
25. Bloksma A.W. Dough structure, dough rheology, and baking quality. Cereal Foods World 1990, 35:237-243.
26. Bodamer O.A., Sahoo T., Beaudet A.L., O'Brien W.E., Bottiglieri T., Stöckler-Ipsiroglu S., et al. Creatine metabolism in combined methylmalonic aciduria and homocystinuria. Annals of Neurology 2005, 57(4):557-560.
27. Bollaín C., Angioloni A., Collar C. Bread staling assessment of enzyme-supplemented pan breads by dynamic and static deformation measurements. European Food Research and Technology 2005, 220(1):83-89.
28. Borrajo G. Newborn screening in Latin America at the beginning of the 21st century. Journal of Inherited Metabolic Disease 2007, 30(4):466-481.
29. Brown W.W., Peters R.M., Ohmit S.E., Keane W.F., Collins A., Chen S.-C., et al. Early detection of kidney disease in community settings: The kidney early evaluation program (KEEP). American Journal of Kidney Diseases 2003, 42(1):22-35.
30. Brunori G., Viola B.F., Parrinello G., De Biase V., Como G., Franco V., et al. Efficacy and safety of a very-low-protein diet when postponing dialysis in the elderly: A prospective randomized multicenter controlled study. American Journal of Kidney Diseases 2007, 49(5):569-580.
31. Brusilow S.W., Maestri N.E. Urea cycle disorders: Diagnosis, pathophysiology, and therapy. Advances in Pediatrics 1996, 43:127-170.
32. Buist N.R.M., Huntington K. Scene from the USA: The illogic of mandating screening without also providing for treatment. Journal of Inherited Metabolic Disease 2007, 30(4):445-446.
33. Calabria A., Peres C., Van Langeraert J. Transferring fermentation process methods from R&D to the manufacturing scale. Recent advances in fermentation technology VIII 2009, Marriott Mission Valley, San Diego, CA. R. VIII (Ed.).
34. Cauvain, S.P., & Young, L.S. (2007). Bread - the product technology of breadmaking. In (pp. 1-19): Springer US.
35. Chaisawang M., Suphantharika M. Pasting and rheological properties of native and anionic tapioca starches as modified by guar gum and xanthan gum. Food Hydrocolloids 2006, 20(5):641-649.
36. Chase H.P., Goodman S.I., O'Brien D. Treatment of homocystinuria. Archives of Disease in Childhood 1967, 42(225):514-520.
37. Chuang D.T. Maple syrup urine disease: It has come a long way. Journal of Pediatrics 1998, 132(3, Supplement):S17-S23.
38. Cianciaruso B., Pota A., Pisani A., Torraca S., Annecchini R., Lombardi P., et al. Metabolic effects of two low protein diets in chronic kidney disease stage 4-5-A randomized controlled trial. Nephrology, Dialysis, Transplantation 2008, 23(2):636-644.
39. Claes D.J., Jackson E. Cystinuria: Mechanisms and management. Pediatric Nephrology 2012, 27(11):2031-2038.
40. Cleary M.A. Phenylketonuria. Paediatrics & Child Health 2011, 21(2):61-64.
41. Coyne T., Olson M., Bradham K., Garcon M., Gregory P., Scherch L. Dietary satisfaction correlated with adherence in the modification of diet in renal-disease study. Journal of the American Dietetic Association 1995, 95(11):1301-1306.
42. Cranford S., Buehler M.J. Materiomics: Biological protein materials, from nano to macro. Nanotechnology, Science and Applications 2010, 3:127-148.
43. Crockett R., Ie P., Vodovotz Y. How do xanthan and hydroxypropyl methylcellulose individually affect the physicochemical properties in a model gluten-free dough?. Journal of Food Science 2011, 76(3):E274-E282.
44. Crombez E.A., Cederbaum S.D. Hyperargininemia due to liver arginase deficiency. Molecular Genetics and Metabolism 2005, 84(3):243-251.
45. Culliney K. No need to mix xylanases anymore, says Novozymes. Bakery and Snacks 2012, William Reed Business Media SAS, Montpellier, France.
46. Cupisti A., Morelli E., Meola M., Barsotti M., Barsotti G. Vegetarian diet alternated with conventional low-protein diet for patients with chronic renal failure. Journal of Renal Nutrition 2002, 12(1):32-37.
47. Dancis J., Hutzler J., Ampola M.G., Shih V.E., van Gelderen H.H., Kirby L.T., et al. The prognosis of hyperlysinemia: An interim report. American Journal of Human Genetics 1983, 35(3):438-442.
48. Dancis J., Hutzler J., Woody N.C., Cox R.P. Multiple enzyme defects in familial hyperlysinemia. Pediatric Research 1976, 10(7):686-691.
49. Darmon P., Kaiser M.J., Bauer J.M., Sieber C.C., Pichard C. Restrictive diets in the elderly: Never say never again?. Clinical Nutrition 2010, 29(2):170-174.
50. de Haas V., Carbasius Weber E.C., de Klerk J.B., Bakker H.D., Smit G.P., Huijbers W.A., et al. The success of dietary protein restriction in alkaptonuria patients is age-dependent. Journal of Inherited Metabolic Disease 1998, 21(8):791-798.
51. de Souza P.M., Magalhães P.d.O.e. Application of microbial α-amylase in industry - A review. Brazilian Journal of Microbiology 2010, 41:850-861.
52. Delcour J., Hoseney C. Chapter 2: Starch. Principles of cereal science and technology 2010, 23-51. AACC International, Inc., Kansas, U.S.A. 3rd Edition. J. Delcour (Ed.).
53. Deodato F., Boenzi S., Santorelli F.M., Dionisi-Vici C. Methylmalonic and propionic aciduria. American Journal of Medical Genetics. Part C, Seminars in Medical Genetics 2006, 142C(2):104-112.
54. Dinopoulos A., Matsubara Y., Kure S. Atypical variants of nonketotic hyperglycinemia. Molecular Genetics and Metabolism 2005, 86(1-2):61-69.
55. Dionisi-Vici C., Deodato F., Röschinger W., Rhead W., Wilcken B. 'Classical' organic acidurias, propionic aciduria, methylmalonic aciduria and isovaleric aciduria: Long-term outcome and effects of expanded newborn screening using tandem mass spectrometry. Journal of Inherited Metabolic Disease 2006, 29(2):383-389.
56. Dixon M.A., Leonard J.V. Intercurrent illness in inborn errors of intermediary metabolism. Archives of Disease in Childhood 1992, 67(11):1387-1391.
57. Dodge T. Production of industrial enzymes. Enzymes in food technology 2009, 44-58. Wiley-Blackwell, Singapore. 2nd ed. R.J. Whitehurst, M. van Oort (Eds.).
58. Dykes L., Rooney L. Phenolic compounds in cereal grains and their health benefits. Cereal Foods World 2007, 52(3):105-111.
59. Edelmann L., Wasserstein M.P., Kornreich R., Sansaricq C., Snyderman S.E., Diaz G.A. Maple syrup urine disease: Identification and carrier-frequency determination of a novel founder mutation in the Ashkenazi Jewish population. The American Journal of Human Genetics 2001, 69(4):863-868.
60. Eggermann T., Zerres K., Nunes V., Font-Llitjos M., Bisceglia L., Chatzikyriakidou A., et al. Clinical utility gene card for: Cystinuria. European Journal of Human Genetics 2012, 20(2).
61. El Nahas A.M., Bello A.K. Chronic kidney disease: The global challenge. Lancet 2005, 365(9456):331-340.
62. Elias E.M. Durum wheat products. Seminar on durum wheat quality in the Mediterranean region 1993, Vol. 22. CIHEAM-IAMZ, Zaragoza, Spain.
63. El-Said M.F., Badii R., Bessisso M.S., Shahbek N., El-Ali M.G., El-Marikhie M., et al. A common mutation in the CBS gene explains a high incidence of homocystinuria in the Qatari population. Human Mutation 2006, 27(7):719-723.
64. Erez A., Nagamani S.C.S., Lee B. Argininosuccinate lyase deficiency-Argininosuccinic aciduria and beyond. American Journal of Medical Genetics. Part C, Seminars in Medical Genetics 2011, 157(1):45-53.
65. Esteller M.S., Amaral R.L., Lannes S.C.D.S. Effect of sugar and fat replacers on the texture of baked goods. Journal of Texture Studies 2004, 35(4):383-393.
66. European Commission The demographic future of Europe - From challenge to opportunity 2006, brussels: EUR-Lex. E. Community (Ed.).
67. ExPASy Enzyme entry: EC 3.2.1.133 SIB bioinformatics resource portal http://enzyme.expasy.org/EC/3.2.1.133.
68. Flynn M.P., Martin M.C., Moore P.T., Stafford J.A., Fleming G.A., Phang J.M. Type II hyperprolinaemia in a pedigree of Irish travellers (nomads). Archives of Disease in Childhood 1989, 64(12):1699-1707.
69. Fouque D., Wang P., Laville M., Boissel J.P. Low protein diets delay end-stage renal disease in non-diabetic adults with chronic renal failure. Nephrology Dialysis Transplantation 2000, 15(12):1986-1992.
70. Francis D., Humphrey M., Boneh A. Newborn screening for glutaric acidaemia type !: The dietary challenge. Journal of Inherited Metabolic Disease 2005, 28(Suppl. 1).
71. Gallagher E., Gormley T.R., Arendt E.K. Recent advances in the formulation of gluten-free cereal-based products. Trends in Food Science & Technology 2004, 15(3-4):143-152.
72. Gámbaro A., Giménez A.N.A., Ares G., Gilardi V. Influence of enzymes on the texture of brown pan bread. Journal of Texture Studies 2006, 37(3):300-314.
73. Gan-Schreier H., Kebbewar M., Fang-Hoffmann J., Wilrich J., Abdoh G., Ben-Omran T., et al. Newborn population screening for classic homocystinuria by determination of total homocysteine from guthrie cards. The Journal of Pediatrics 2010, 156(3):427-432.
74. Geboes K., Geboes K.P. Diagnosis and treatment of coeliac disease. F1000 Medicine Reports 2009, 1:32.
75. Georgiou N.A., Garssen J., Witkamp R.F. Pharma-nutrition interface: The gap is narrowing. European Journal of Pharmacology 2011, 651(1-3):1-8.
76. Gibbs T.C., Payan J., Brett E.M., Lindstedt S., Holme E., Clayton P.T. Peripheral neuropathy as the presenting feature of tyrosinaemia type I and effectively treated with an inhibitor of 4-hydroxyphenylpyruvate dioxygenase. Journal of Neurology, Neurosurgery, and Psychiatry 1993, 56(10):1129-1132.
77. Giménez A., Varela P., Salvador A., Ares G., Fiszman S., Garitta L. Shelf life estimation of brown pan bread: A consumer approach. Food Quality and Preference 2007, 18(2):196-204.
78. Giugliani R. Newborn screening for lysosomal diseases: Current status and potential interface with population medical genetics in Latin America. Journal of Inherited Metabolic Disease 2012, 35(5):871-877.
79. Goesaert H., Brijs K., Veraverbeke W.S., Courtin C.M., Gebruers K., Delcour J.A. Wheat flour constituents: How they impact bread quality, and how to impact their functionality. Trends in Food Science & Technology 2005, 16(1-3):12-30.
80. Goodman S.I., Markey S.P., Moe P.G., Miles B.S., Teng C.C. Glutaric aciduria; a "new" disorder of amino acid metabolism. Biochemical Medicine 1975, 12(1):12-21.
81. Gray J.A., Bemiller J.N. Bread staling: Molecular basis and control. Comprehensive Reviews in Food Science and Food Safety 2003, 2(1):1-21.
82. Griva K., Ng H.J., Loei J., Mooppil N., McBain H., Newman S.P. Managing treatment for end-stage renal disease - A qualitative study exploring cultural perspectives on facilitators and barriers to treatment adherence. Psychology & Health 2012, 1-17.
83. Guarda A., Rosell C.M., Benedito C., Galotto M.J. Different hydrocolloids as bread improvers and antistaling agents. Food Hydrocolloids 2004, 18(2):241-247.
84. Guerzoni M.E., Gianotti A., Vernocchi P. Chapter 34 - Effect of starch addition to fluid dough during the bread making process. Flour and breads and their fortification in health and disease prevention 2011, 375-384. Academic Press, San Diego. P. Victor, W. Ronald, P. Vinood (Eds.).
85. Guldberg P., Henriksen K.F., Sipila I., Guttler F., de la Chapelle A. Phenylketonuria in a low incidence population: molecular characterisation of mutations in Finland. Journal of Medical Genetics 1995, 32(12):976-978.
86. Gupta R., Gigras P., Mohapatra H., Goswami V.K., Chauhan B. Microbial α-amylases: A biotechnological perspective. Process Biochemistry 2003, 38(11):1599-1616.
87. Hager A.S., Axel C., Arendt E.K. Status of carbohydrates and dietary fiber in gluten-free diets. Cereal Foods World 2011, 56(3):109-114.
88. Hager A.-S., Wolter A., Czerny M., Bez J., Zannini E., Arendt E., et al. Investigation of product quality, sensory profile and ultrastructure of breads made from a range of commercial gluten-free flours compared to their wheat counterparts. European Food Research and Technology 2012, 235(2):333-344.
89. Hallan S.I., Coresh J., Astor B.C., Åsberg A., Powe N.R., Romundstad S., et al. International comparison of the relationship of chronic kidney disease prevalence and ESRD risk. Journal of the American Society of Nephrology 2006, 17(8):2275-2284.
90. Hansen A., Schieberle P. Generation of aroma compounds during sourdough fermentation: Applied and fundamental aspects. Trends in Food Science & Technology 2005, 16(1-3):85-94.
91. Haros M., Rosell C., Benedito C. Effect of different carbohydrases on fresh bread texture and bread staling. European Food Research and Technology 2002, 215(5):425-430.
92. Harries J.T., Piesowicz A.T., Seakins J.W., Francis D.E., Wolff O.H. Low proline diet in type I hyperprolinaemia. Archives of Disease in Childhood 1971, 46(245):72-81.
93. Hedlund G.L., Longo N., Pasquali M. Glutaric acidemia type 1. American Journal of Medical Genetics. Part C, Seminars in Medical Genetics 2006, 142C(2):86-94.
94. Heiniö R.-L., Nordlund E., Poutanen K., Buchert J. Use of enzymes to elucidate the factors contributing to bitterness in rye flavour. Food Research International 2012, 45(1):31-38.
95. Hillege H.L., Janssen W.M.T., Bak A.A.A., Diercks G.F.H., Grobbee D.E., Crijns H.J.G.M., et al. Microalbuminuria is common, also in a nondiabetic, nonhypertensive population, and an independent indicator of cardiovascular risk factors and cardiovascular morbidity. Journal of Internal Medicine 2001, 249(6):519-526.
96. Ihle B.U., Becker G.J., Whitworth J.A., Charlwood R.A., Kincaid-Smith P.S. The effect of protein restriction on the progression of renal insufficiency. The New England Journal of Medicine 1989, 321(26):1773-1777.
97. Jaeken J. Disorders of proline and serine metabolism. Inborn metabolic diseases: Diagnosis and treatment 2006, 253-319. Springer, Heidelberg, Germany. 4th ed. J. Fernandes, J.M. Saudubray, G. van den Berghe, J.H. Walter (Eds.).
98. Jameson E., Morris A.A.M. Nutrition in metabolic disease. Journal of Paediatrics and Child Health 2011, 21(9):401-405.
99. Järvinen O., Hietala M., Aalto A.M., Arvio M., Uutela A., Aula P., et al. A retrospective study of long-term psychosocial consequences and satisfaction after carrier testing in childhood in an autosomal recessive disease: Aspartylglucosaminuria. Clinical Genetics 2000, 58(6):447-454.
100. Kabra M. Dietary management of inborn errors of metabolism. Indian Journal of Pediatrics 2002, 69(5):421-426.
101. Kalorama (2011). The world market for clinical nutrition products (infant, parenteral and enteral foods). In (4th ed., pp. 1-220): http://www.kaloramainformation.com/Clinical-Nutrition-Products-6126530/.
102. Kanekar S., Verbrugge J. Metabolic disorders of the brain: Part II. Seminars in Ultrasound, CT 2011, 32(6):615-636.
103. Kaur M. Medical foods from natural sources 2009, Springer, 233 Spring Street, New York, Ny 10013, United States.
104. Keetels C.J.A.M., van Vliet T., Walstra P. Relationship between the sponge structure of starch bread and its mechanical properties. Journal of Cereal Science 1996, 24(1):27-31.
105. Kilibwa M. Polydextrose as anti-staling agent 2004, Vol. 6830770. United States Patent and Trademark Office, United States. D. U. Inc. (Ed.).
106. Kitts D.D., Weiler K. Bioactive proteins and peptides from food sources. Applications of bioprocesses used in isolation and recovery. Current Pharmaceutical Design 2003, 9(16):1309-1323.
107. Kobayashi K., Sinasac D.S., Iijima M., Boright A.P., Begum L., Lee J.R., et al. The gene mutated in adult-onset type II citrullinaemia encodes a putative mitochondrial carrier protein. Nature Genetics 1999, 22(2):159-163.
108. Kolker S., Christensen E., Leonard J.V., Greenberg C.R., Boneh A., Burlina A.B., et al. Diagnosis and management of glutaric aciduria type I-Revised recommendations. Journal of Inherited Metabolic Disease 2011, 34(3):677-694.
109. Komrower G.M., Lambert A.M., Cusworth D.C., Westall R.G. Dietary treatment of homocystinuria. Archives of Disease in Childhood 1966, 41(220):666-671.
110. Kornbrust B.A., Forman T., Matveeva I. Applications of enzymes in breadmaking. Breadmaking: Improving quality 2012, 470-498. Woodhead Publishing Limited, Cambridge, UK. 2nd ed. S.P. Cauvain (Ed.).
111. Kusunose C., Fujii T., Matsumoto H. Role of starch granules in controlling expansion of dough during baking. Cereal Chemistry Journal 1999, 76(6):920-924.
112. La Du B.N., Zannoni V.G., Laster L., Seegmiller J.E. The nature of the defect in tyrosine metabolism in alcaptonuria. Journal of Biological Chemistry 1958, 230(1):251-260.
113. Lazaridou A., Duta D., Papageorgiou M., Belc N., Biliaderis C.G. Effects of hydrocolloids on dough rheology and bread quality parameters in gluten-free formulations. Journal of Food Engineering 2007, 79(3):1033-1047.
114. Leveking (2012). Food enzymes. In.
115. Levey A.S., Atkins R., Coresh J., Cohen E.P., Collins A.J., Eckardt K.U., et al. Chronic kidney disease as a global public health problem: Approaches and initiatives - A position statement from Kidney Disease Improving Global Outcomes. Kidney International 2007, 72(3):247-259.
116. Levy H.L., Vargas J.E., Waisbren S.E., Kurczynski T.W., Roeder E.R., Schwartz R.S., et al. Reproductive fitness in maternal homocystinuria due to cystathionine β-synthase deficiency. Journal of Inherited Metabolic Disease 2002, 25(4):299-314.
117. Liu X. GMO and protein engineering. Enzymes in food technology 2009, 18-43. Wiley-Blackwell, Singapore. 2nd ed. R.J. Whitehurst, M. van Oort (Eds.).
118. Locatelli F., Vecchio L.D., Pozzoni P. The importance of early detection of chronic kidney disease. Nephrology, Dialysis, Transplantation 2002, 17(Suppl. 11):2-7.
119. Lock E.A., Gaskin P., Ellis M.K., Robinson M., Provan W.M., Smith L.L. The effect of a low-protein diet and dietary supplementation of threonine on tyrosine and 2-(2-nitro-4-trifluoromethylbenzoyl) cyclohexane-1,3-dione-induced corneal lesions, the extent of tyrosinemia, and the activity of enzymes involved in tyrosine catabolism in the rat. Toxicology and Applied Pharmacology 1998, 150(1):125-132.
120. Loeber J. Neonatal screening in Europe; the situation in 2004. Journal of Inherited Metabolic Disease 2007, 30(4):430-438.
121. Longo M.A., Sanromán M.A. Production of food aroma compounds: Microbial and enzymatic methodologies. Food Technology and Biotechnology 2006, 44(3):335-353.
122. MacDonald A., Asplin D. Phenylketonuria: Practical dietary management. The Journal of Family Health Care 2006, 16(3):83-85.
123. Mackenzie H.S., Brenner B.M. Current strategies for retarding progression of renal disease. American Journal of Kidney Diseases 1998, 31(1):161-170.
124. Macleod E.L., Ney D.M. Nutritional management of phenylketonuria. Annales Nestlé (English ed.) 2010, 68(2):58-69.
125. Macsai M.S., Schwartz T.L., Hinkle D., Hummel M.B., Mulhern M.G., Rootman D. Tyrosinemia type II: Nine cases of ocular signs and symptoms. American Journal of Ophthalmology 2001, 132(4):522-527.
126. Magan N., Aldred D., Blackburn C.d.W. Managing microbial spoilage in cereal and baking products. Food Spoilage Microorganisms 2006, 194-212.
127. Mandayam S., Mitch W.E. Dietary protein restriction benefits patients with chronic kidney disease. Nephrology 2006, 11(1):53-57.
128. Marklova E. Good outcome od hydroxymethylglutaric aciduria in an adult. Journal of Inherited Metabolic Disease 2005, 28(Suppl. 1).
129. Marks A., Black C., Fluck N., Smith W.C.S., Prescott G.J., Clark L.E., et al. Translating chronic kidney disease epidemiology into patient care-The individual/public health risk paradox. Nephrology, Dialysis, Transplantation 2012, 0:1-8.
130. Maruyama H., Ogawa M., Nishio T., Kobayashi K., Saheki T., Sunohara N. Citrullinemia type II in a 64-year-old man with fluctuating serum citrulline levels. Journal of Neurological Sciences 2001, 182(2):167-170.
131. Mayatepek E., Kallas K., Anninos A., Müller E. Effects of ascorbic acid and low-protein diet in alkaptonuria. European Journal of Pediatrics 1998, 157(10):867-868.
132. McCabe-Sellers B.J. Position of the American Dietetic Association: Integration of medical nutrition therapy and pharmacotherapy. Journal of the American Dietetic Association 2010, 110(6):950-956.
133. McClellan W., Warnock D.G., McClure L., Campbell R.C., Newsome B.B., Howard V., et al. Racial differences in the prevalence of chronic kidney disease among participants in the Reasons for Geographic and Racial Differences in Stroke (REGARDS) cohort study. Journal of the American Society of Nephrology 2006, 17(6):1710-1715.
134. Melancon S.B., Dallaire L., Lemieux B., Robitaille P., Potier M. Dicarboxylic aminoaciduria: An inborn error of amino acid conservation. Journal of Pediatrics 1977, 91(3):422-427.
135. Milas N.C., Nowalk M.P., Akpele L., Castaldo L., Coyne T., Doroshenko L., et al. Factors associated with adherence to the dietary protein intervention in the modification of diet in renal disease study. Journal of the American Dietetic Association 1995, 95(11):1295-1300.
136. Mishra S., Rai T. Morphology and functional properties of corn, potato and tapioca starches. Food Hydrocolloids 2006, 20(5):557-566.
137. Mitch W.E. Beneficial responses to modified diets in treating patients with chronic kidney disease. Kidney International 2005, 67(S94):S133-S135.
138. Mitchell S.C., Smith R.L. Trimethylaminuria: The fish malodor syndrome. Drug Metabolism and Disposition 2001, 29(4):517-521.
139. Miyazaki M., Maeda T., Morita N. Starch retrogradation and firming of bread containing hydroxypropylated, acetylated, and phosphorylated cross-linked tapioca starches for wheat flour. Cereal Chemistry Journal 2005, 82(6):639-644.
140. Miyazaki M., Maeda T., Morita N. Bread quality of frozen dough substituted with modified tapioca starches. European Food Research and Technology 2008, 227(2):503-509.
141. Miyazaki M., Morita N. Effect of heat-moisture treated maize starch on the properties of dough and bread. Food Research International 2005, 38(4):369-376.
142. Mohsen S.M., Yaseen A.A., Ammar A.M., Mohammad A.A. Quality characteristics improvement of low-phenylalanine toast bread. International Journal of Food Science & Technology 2010, 45(10):2042-2051.
143. Morgan K.R., Hutt L., Gerrard J., Every D., Ross M., Gilpin M. Staling in starch breads: The effect of antistaling α-amylase. Starch-Starke 1997, 49(2):54-59.
144. Morton D.H., Strauss K.A., Robinson D.L., Puffenberger E.G., Kelley R.I. Diagnosis and treatment of maple syrup disease: A study of 36 patients. Pediatrics 2002, 109(6):999-1008.
145. Novozymes (2010). Enzymes produced by genetically modified microorganisms. In (Vol. 2012).
146. Nugent R.A., Fathima S.F., Feigl A.B., Chyung D. The burden of chronic kidney disease on developing nations: A 21st century challenge in global health. Nephron. Clinical Practice 2011, 118(3):C269-C276.
147. O'Brien W.M., La Du B.N., Bunim J.J. Biochemical, pathologic and clinical aspects of alcaptonuria, ochronosis and ochronotic arthropathy: Review of world literature (1584-1962). American Journal of Medicine 1963, 34(6):813-838.
148. Ojijo N.K.O., Shimoni E. Influence of xanthan gum and tapioca starch on the retrogradation and gelation of finger millet (Eleusine coracana L. Gaertner) starch pastes. Journal of Texture Studies 2007, 38(1):100-115.
149. Ono H., Suto T., Kinoshita Y., Sakano T., Furue T., Ohta T. A case of carbamoyl phosphate synthetase 1 deficiency presenting symptoms at one month of age. Brain & Development 2009, 31(10):779-781.
150. Otero A., Gayoso P., Garcia F., de Francisco A.L. Epidemiology of chronic renal disease in the Galician population: Results of the pilot Spanish EPIRCE study. Kidney International 2005, 68(S99):S16-S19.
151. Özboy Ö. Development of corn starch-gum bread for phenylketonuria patients. Nahrung/Food 2002, 46(2):87-91.
152. Park S.H., Chung O.K., Seib P.A. Effects of varying weight ratios of large and small wheat starch granules on experimental straight-dough bread. Cereal Chemistry 2005, 82(2):166-172.
153. Park S.H., Wilson J.D., Chung O.K., Seib P.A. Size distribution and properties of wheat starch granules in relation to crumb grain score of pup-loaf bread. Cereal Chemistry 2004, 81(6):699-704.
154. Park S.-H., Wilson J.D., Seabourn B.W. Starch granule size distribution of hard red winter and hard red spring wheat: Its effects on mixing and breadmaking quality. Journal of Cereal Science 2009, 49(1):98-105.
155. Patton S.R. Adherence to diet in youth with type 1 diabetes. Journal of the American Dietetic Association 2011, 111(4):550-555.
156. Pavone L., Mollica F., Levy H.L. Asymptomatic type II hyperprolinaemia associated with hyperglycinaemia in three sibs. Archives of Disease in Childhood 1975, 50(8):637-641.
157. Pawlowska A.M., Zannini E., Coffey A., Arendt E.K. "Green preservatives": Combating fungi in the food and feed industry by applying antifungal lactic acid bacteria. Advances in Food and Nutrition Research 2012, 217.
158. Pena L., Burton B.K. Survey of health status and complications among propionic acidemia patients. American Journal of Medical Genetics. Part A 2012, 158A(7):1641-1646.
159. Peng J.H., Lapitan N.V. Characterization of EST-derived microsatellites in the wheat genome and development of eSSR markers. Functional & Integrative Genomics 2005, 5(2):80-96.
160. Phornphutkul C., Introne W.J., Perry M.B., Bernardini I., Murphey M.D., Fitzpatrick D.L., et al. Natural history of alkaptonuria. The New England Journal of Medicine 2002, 347(26):2111-2121.
161. Pitkanen S.T., Salo M.K., Heikinheimo M. Hereditary tyrosinaemia type I: From basics to progress in treatment. Annals of Medicine 2000, 32(8):530-538.
162. Plauth M., Cabré E., Riggio O., Assis-Camilo M., Pirlich M., Kondrup J., et al. ESPEN guidelines on enteral nutrition: Liver disease. Clinical Nutrition 2006, 25(2):285-294.
163. Prost C., Poinot C.P., Rannou C., Arvisenet G. Bread aroma. Breadmaking - Improving quality 2012, 523-596. Woodhead Publishing Ltd., Cambridge, UK. S.P. Cauvain (Ed.).
164. Quental S., Vilarinho L., Martins E., Teles E.L., Rodrigues E., Diogo L., et al. Incidence of maple syrup urine disease in Portugal. Molecular Genetics and Metabolism 2010, 100(4):385-387.
165. Raninen K., Lappi J., Mykkänen H., Poutanen K. Dietary fiber type reflects physiological functionality: Comparison of grain fiber, inulin, and polydextrose. Nutrition Reviews 2011, 69(1):9-21.
166. Rewers M.J. Epidemiology of celiac disease: What are the prevalence, incidence, and progression of celiac disease?. NIH consensus development conference on celiac disease 2004, NIH, Bethesda, Maryland. C.O. Elson (Ed.).
167. Robards K. Strategies for the determination of bioactive phenols in plants, fruit and vegetables. Journal of Chromatography. A 2003, 1000(1-2):657-691.
168. Rosell C.M. Enzymatic manipulation of gluten-free breads. Gluten-free food science and technology 2009, 83-98. Wiley-Blackwell, Oxford, UK. E. Gallagher (Ed.).
169. Rudi H., Uhlen A.K., Harstad O.M., Munck L. Genetic variability in cereal carbohydrate compositions and potentials for improving nutritional value. Animal Feed Science and Technology 2006, 130(1-2):55-65.
170. Ryan L.A.M., Dal Bello F., Arendt E.K. The use of sourdough fermented by antifungal LAB to reduce the amount of calcium propionate in bread. International Journal of Food Microbiology 2008, 125(3):274-278.
171. Ryan L.A.M., Zannini E., Dal Bello F., Pawlowska A., Koehler P., Arendt E.K. Lactobacillus amylovorus DSM 19280 as a novel food-grade antifungal agent for bakery products. International Journal of Food Microbiology 2011, 146(3):276-283.
172. Sabanis D., Tzia C. Effect of hydrocolloids on selected properties of gluten-free dough and bread. Food Science and Technology International 2011, 17(4):279-291.
173. Sanjurjo P., Rodriguez-Soriano J., Vallo A., Arranz A., Rubio V. Neonatal citrullinaemia with satisfactory mental development. European Journal of Pediatrics 1991, 150(10):730-731.
174. Saudubray J.-M., van den Berghe G., Walter J.H. Transport defects of amino acids at the cell membrane. Inborn metabolic diases - Diagnosis and treatment 2012, Springer, Heidelberg, Germany. 5th ed. J.-M. Saudubray, G. van den Berghe, J.H. Walter (Eds.).
175. Scalone L., Borghetti F., Brunori G., Viola B.F., Brancati B., Sottini L., et al. Cost-benefit analysis of supplemented very low-protein diet versus dialysis in elderly CKD5 patients. Nephrology, Dialysis, Transplantation 2010, 25(3):907-913.
176. Schober T.J. Manufacture of gluten-free specialty breads and confectionery products. Gluten-free food science and technology 2009, 130-180. Wiley-Blackwell.
177. Schwarzlaff S.S., Johnson J.M., Barbeau W.E., Duncan S. Guar and locust bean gums as partial replacers of all-purpose flour in bread: An objective and sensory evaluation. Journal of Food Quality 1996, 19(3):217-229.
178. Sebastio G., Nunes V. Lysinuric protein intolerance. GeneReviews™ 2006, University of Washington, Seattle: NCBI. R.A. Pagon, T.D. Bird, C.R. Dolan, K. Stephens, M.P. Adam (Eds.).
179. Selinheimo E., Autio K., Kruus K., Buchert J. Elucidating the mechanism of laccase and tyrosinase in wheat bread making. Journal of Agricultural and Food Chemistry 2007, 55(15):6357-6365.
180. Sestili F., Botticella E., Bedo Z., Phillips A., Lafiandra D. Production of novel allelic variation for genes involved in starch biosynthesis through mutagenesis. Molecular Breeding 2010, 25(1):145-154.
181. Shahani K.M., Arnold R.G., Kilara A., Dwivedi B.K. Role of microbial enzymes in flavor development in foods. Biotechnology and Bioengineering 1976, 18(7):891-907.
182. Shephard E.A., Treacy E.P., Phillips I.R. Clinical utility gene card for: Trimethylaminuria. European Journal of Human Genetics 2012, 20(3).
183. Silano M., De Vincenzi M. Bioactive antinutritional peptides derived from cereal prolamins: A review. Food / Nahrung 1999, 43(3):175-184.
184. Silano V., Vittozzi L. The status of health in the European union: Towards a healthier Europe. EUGLOREH 2009, European Commission, Italy.
185. Simon E., Fingerhut R., Baumkötter J., Konstantopoulou V., Ratschmann R., Wendel U. Maple syrup urine disease: Favourable effect of early diagnosis by newborn screening on the neonatal course of the disease. Journal of Inherited Metabolic Disease 2006, 29(4):532-537.
186. Singh N., Kaur L. Morphological, thermal, rheological and retrogradation properties of potato starch fractions varying in granule size. Journal of the Science of Food and Agriculture 2004, 84(10):1241-1252.
187. Skovby F., Gaustadnes M., Mudd S.H. A revisit to the natural history of homocystinuria due to cystathionine beta-synthase deficiency. Molecular Genetics and Metabolism 2010, 99(1):1-3.
188. Strauss K.A., Mazariegos G.V., Sindhi R., Squires R., Finegold D.N., Vockley G., et al. Elective liver transplantation for the treatment of classical maple syrup urine disease. American Journal of Transplantation 2006, 6(3):557-564.
189. Summar M.L., Dobbelaere D., Brusilow S., Lee B. Diagnosis, symptoms, frequency and mortality of 260 patients with urea cycle disorders from a 21-year, multicentre study of acute hyperammonaemic episodes. Acta Paediatrica 2008, 97(10):1420-1425.
190. Sutton V.R., Chapman K.A., Gropman A.L., MacLeod E., Stagni K., Summar M.L., et al. Chronic management and health supervision of individuals with propionic acidemia. Molecular Genetics and Metabolism 2012, 105(1):26-33.
191. Swarna M., Rao D.N., Reddy P.P. Dicarboxylic aminoaciduria associated with mental retardation. Human Genetics 1989, 82(3):299-300.
192. Syvänen A.-C., Ikonen E., Manninen T., Bengtström M., Söderlund H., Aula P., et al. Convenient and quantitative determination of the frequency of a mutant allele using solid-phase minisequencing: Application to aspartylglucosaminuria in Finland. Genomics 1992, 12(3):590-595.
193. Tada K., Kure S. Non-ketotic hyperglycinaemia: Molecular lesion, diagnosis and pathophysiology. Journal of Inherited Metabolic Disease 1993, 16(4):691-703.
194. Tanner L., Näntö-Salonen K., Venetoklis J., Kotilainen S., Niinikoski H., Huoponen K., et al. Nutrient intake in lysinuric protein intolerance. Journal of Inherited Metabolic Disease 2007, 30(5):716-721.
195. Teijema H.L., van Gelderen H.H., Giesberts M.A., Laurent de Angulo M.S. Dicarboxylic aminoaciduria: An inborn error of glutamate and aspartate transport with metabolic implications, in combination with a hyperprolinemia. Metabolism 1974, 23(2):115-123.
196. Theofilou P. Medication adherence in Greek hemodialysis patients: The contribution of depression and health cognitions. International Journal of Behavioral Medicine 2012, 1-8.
197. UN World population prospects: The 2008 revision comprehensive tables. United Nations publication 2008, Vol. 1. United Nations. UN (Ed.).
198. UN Population ageing and the non-communicable diseases. Population facts 2012, UN. D. o. E. a. S. Affairs (Ed.).
199. Vallons K.J.R., Arendt E.K. Effects of high pressure and temperature on the structural and rheological properties of sorghum starch. Innovative Food Science & Emerging Technologies 2009, 10(4):449-456.
200. Van de Bor M., Mooy P., van Zoeren D., Berger R., van Gelderen H.H., Teijema H.L. Successful treatment of severe carbamyl phosphate synthetase I deficiency. Archives of Disease in Childhood 1984, 59(12):1183-1185.
201. van de Luijtgaarden M.W.M., Noordzij M., Wanner C., Jager K.J. Renal replacement therapy in Europe-A summary of the 2009 ERA-EDTA Registry annual report. Clinical Kidney Journal 2012, 5(2):109-119.
202. van der Maarel M.J.E.C., van der Veen B., Uitdehaag J.C.M., Leemhuis H., Dijkhuizen L. Properties and applications of starch-converting enzymes of the α-amylase family. Journal of Biotechnology 2002, 94(2):137-155.
203. van der Meer S.B., Poggi F., Spada M., Bonnefont J.P., Ogier H., Hubert P., et al. Clinical outcome and long-term management of 17 patients with propionic acidaemia. European Journal of Pediatrics 1996, 155(3):205-210.
204. van Gelderen H.H., Teijema H.L. Hyperlysinaemia. Harmless inborn error of metabolism?. Archives of Disease in Childhood 1973, 48(11):892-895.
205. Van Gosen L. Organic acidemias: A methylmalonic and propionic focus. Journal of Pediatric Nursing 2008, 23(3):225-233.
206. van Karnebeek C.D.M., Stockler S. Treatable inborn errors of metabolism causing intellectual disability: A systematic literature review. Molecular Genetics and Metabolism 2012, 105(3):368-381.
207. Varshney R.K., Hoisington D.A., Tyagi A.K. Advances in cereal genomics and applications in crop breeding. Trends in Biotechnology 2006, 24(11):490-499.
208. Veríssimo C., Garcia P., Simões M., Robalo C., Henriques R., Diogo L., et al. Nonketotic hyperglycinemia: A cause of encephalopathy in children. Journal of Child Neurology 2012, 28(2):251-254.
209. Vitaglione P., Napolitano A., Fogliano V. Cereal dietary fibre: A natural functional ingredient to deliver phenolic compounds into the gut. Trends in Food Science & Technology 2008, 19(9):451-463.
210. Volkert D., Berner Y.N., Berry E., Cederholm T., Coti Bertrand P., Milne A., et al. ESPEN guidelines on enteral nutrition: Geriatrics. Clinical Nutrition 2006, 25(2):330-360.
211. Waters D.M., Ryan L.A., Murray P.G., Arendt E.K., Tuohy M.G. Characterisation of a Talaromyces emersonii thermostable enzyme cocktail with applications in wheat dough rheology. Enzyme and Microbial Technology 2011, 49(2):229-236.
212. Waterval W.A.H., Scheijen J.L.J.M., Ortmans-Ploemen M.M.J.C., Habets-van der Poel C.D., Bierau J. Quantitative UPLC-MS/MS analysis of underivatised amino acids in body fluids is a reliable tool for the diagnosis and follow-up of patients with inborn errors of metabolism. Clinica Chimica Acta 2009, 407(1-2):36-42.
213. Weir M.R., Fink J.C. Salt intake and progression of chronic kidney disease: An overlooked modifiable exposure? A commentary. American Journal of Kidney Diseases 2005, 45(1):176-188.
214. Whistler R.L., BeMiller J.N. Carbohydrate chemistry for food scientists 1997, American Association of Cereal Chemists, Inc., Minnesota, US. 1st ed.
215. WHO Demographic and socioeconomic statistics. World Health Statistics 2012 2012, 166. WHO Press, Geneva, Switzerland. W.H. Organization (Ed.).
216. Wijburg F.A., Nassogne M.C. Disorders of the urea cycle and related enzymes. Inborn metabolic diseases: Diagnosis and treatment 2012, 297-310. Springer, Heidelberg, Germany. 5th ed. J.M. Saudubray, G. van den Berghe, J.H. Walter (Eds.).
217. Williams P.A. An overview of the structure-function relationships of hydrocolloids. Gums and stablisers for the food industry 2006, 13:15-29. The Royal Society of Chemistry, Cambridge, UK. P.A. Williams, G.O. Phillips (Eds.).
218. Wolff J.A., Barshop B., Nyhan W.L., Leslie J., Seegmiller J.E., Gruber H., et al. Effects of ascorbic acid in alkaptonuria: Alterations in benzoquinone acetic acid and an ontogenic effect in infancy. Pediatric Research 1989, 26(2):140-144.
219. Woody N.C., Hutzler J., Dancis J. Further studies of hyperlysinemia. American Journal of Diseases of Children 1966, 112(6):577-580.
220. Xu R., Zhang L., Zhang P., Wang F., Zuo L., Wang H. Comparison of the prevalence of chronic kidney disease among different ethnicities: Beijing CKD survey and American NHANES. Nephrology, Dialysis, Transplantation 2009, 24(4):1220-1226.
221. Yoshikawa T.T. Epidemiology and unique aspects of aging and infectious diseases. Clinical Infectious Diseases 2000, 30(6):931-933.
222. Zannini E., Jones J.M., Renzetti S., Arendt E.K. Functional replacements for gluten. Annu Rev Food Sci Technol 2012, 3:227-245.
223. Zatková A., de Bernabé D.B.V., Poláková H., Zvarík M., Feráková E., Bošák V., et al. High frequency of alkaptonuria in Slovakia: Evidence for the appearance of multiple mutations in HGO involving different mutational hot spots. The American Journal of Human Genetics 2000, 67(5):1333-1339.
224. Zhang Q.-L., Rothenbacher D. Prevalence of chronic kidney disease in population-based studies: Systematic review. BMC Public Health 2008, 8:117.
225. Zoccali C., Kramer A., Jager K.J. Epidemiology of CKD in Europe: An uncertain scenario. Nephrology, Dialysis, Transplantation 2010, 25(6):1731-1733.