Citation: | LU Chenhui, HE Yongxi, WEI Haodong, et al. Development and Application Progress of Food for Special Medical Purpose for Phenylketonuria[J]. Science and Technology of Food Industry, 2022, 43(16): 413−420. (in Chinese with English abstract). doi: 10.13386/j.issn1002-0306.2021070262. |
[1] |
邓伟, 丰琳, 陈小凤. 235361例新生儿疾病筛查情况分析[J]. 中国继续医学教育,2021,13(12):129−132. [DENG W, FENG L, CHEN X F. Analysis of 235361 cases of neonatal disease screening[J]. China Continuing Medical Education,2021,13(12):129−132. doi: 10.3969/j.issn.1674-9308.2021.12.036
DENG W, FENG L, CHEN X F. Analysis of 235361 cases of neonatal disease screening[J]. China Continuing Medical Education, 2021, 13(12): 129-132. doi: 10.3969/j.issn.1674-9308.2021.12.036
|
[2] |
毛新梅, 刘媛, 何江, 等. 宁夏苯丙酮尿症患儿苯丙氨酸羟化酶基因突变特点[J]. 中华围产医学杂志,2015,18(3):204−209. [MAO X M, LIU Y, HE J, et al. Mutation characteristics of phenylalanine hydroxylase gene in children with phenylketonuria in Ningxia[J]. Chinese Journal of Perinatal Medicine,2015,18(3):204−209. doi: 10.3760/cma.j.issn.1007-9408.2015.03.008
MAO X M, LIU Y, HE J, et al. Mutation characteristics of phenylalanine hydroxylase gene in children with phenylketonuria in Ningxia[J]. Chinese Journal of Perinatal Medicine, 2015, 18(3): 204-209. doi: 10.3760/cma.j.issn.1007-9408.2015.03.008
|
[3] |
王亚男, 刘永, 赵柏丽. 洛阳地区79829例新生儿先天性甲状腺功能低下症和苯丙酮尿症筛查结果分析[J]. 中国优生与遗传杂志,2017,25(1):96−97,119. [WANG Y N, LIU Y, ZHAO B L. Analysis of screening results of 79829 neonates with congenital hypothyroidism and phenylketonuria in Luoyang area[J]. Chinese Journal of Birth Health & Heredity,2017,25(1):96−97,119.
WANG Y N, LIU Y, ZHAO B L. Analysis of screening results of 79829 neonates with congenital hypothyroidism and phenylketonuria in Luoyang area[J]. Chinese Journal of Birth Health & Heredity, 2017, 25(1): 96-97, 119.
|
[4] |
赵振东, 黄慈丹, 许海珠, 等. 海南省380996名新生儿苯丙酮尿症筛查及苯丙氨酸羟化酶基因分析[J]. 中华医学杂志,2020,100(26):2054−2058. [ZHAO Z D, HUANG C D, XU H Z, et al. Screening for phenylketonuria and phenylalanine hydroxylase gene analysis of 380996 newborns in Hainan Province[J]. National Medical Journal of China,2020,100(26):2054−2058. doi: 10.3760/cma.j.cn112137-20200120-00134
ZHAO Z D, HUANG C D, XU H Z, et al. Screening for phenylketonuria and phenylalanine hydroxylase gene analysis of 380996 newborns in Hainan Province[J]. National Medical Journal of China, 2020, 100(26): 2054-2058. doi: 10.3760/cma.j.cn112137-20200120-00134
|
[5] |
邹团标, 李智, 姚莉琴, 等. 云南新生儿苯丙酮尿症和先天性甲状腺功能低下症的筛查研究[J]. 中国优生与遗传杂志,2020,28(2):198−242. [ZOU T B, LI Z, YAO L Q, et al. Screening study of phenylketonuria and congenital hypothyroidism in Yunnan newborns[J]. Chinese Journal of Birth Health & Heredity,2020,28(2):198−242.
ZOU T B, LI Z, YAO L Q, et al. Screening study of phenylketonuria and congenital hypothyroidism in Yunnan newborns[J]. Chinese Journal of Birth Health & Heredity, 2020, 28(2): 198-242.
|
[6] |
LICHTER-KONECKI U, VOCKLEY J. Phenylketonuria: Current treatments and future developments[J]. Drugs,2019,79(5):495−500. doi: 10.1007/s40265-019-01079-z
|
[7] |
VAN SPRONSEN F J, BLAU N, HARDING C, et al. Phenylketonuria[J]. Nature Reviews Disease Primers,2021,7(1):1−19. doi: 10.1038/s41572-020-00234-1
|
[8] |
WIEDEMANN A, OUSSALAH A, JEANNESSON E, et al. Phenylketonuria, from diet to gene therapy[J]. M S-Medecine Sciences,2020,36(8-9):725−734.
|
[9] |
GRISCH-CHAN H M, SCHWANK G, HARDING C O, et al. State-of-the-art 2019 on gene therapy for phenylketonuria[J]. Human Gene Therapy,2019,30(10):1274−1283. doi: 10.1089/hum.2019.111
|
[10] |
盛晓静, 王强, 石爱民, 等. 制备低苯丙氨酸特膳食品的研究进展[J]. 食品科学,2016,37(21):285−290. [SHENG X J, WANG Q, SHI A M, et al. Research progress on the preparation of low-phenylalanine special dietary products[J]. Food Science,2016,37(21):285−290. doi: 10.7506/spkx1002-6630-201621048
SHENG X J, WANG Q, SHI A M, et al. Research progress on the preparation of low-phenylalanine special dietary products[J]. Food Science, 2016, 37(21): 285-290. doi: 10.7506/spkx1002-6630-201621048
|
[11] |
陆颖, 蒙萌. 在第七届全国中西医结合营养学术会议的讲话[C]// 舟山: 第七届全国中西医结合营养学术会议资料汇编, 中国中西医结合学会, 2016: 87-88
LU Y, MENG M. Speech at the 7th National Conference on Integrated Traditional Chinese and Western Medicine Nutrition[C]// Zhoushan: Collection of materials from the 7th National Conference on Integrated Traditional Chinese and Western Medicine Nutrition, China Association of Integrative Medicine, 2016: 87-88.
|
[12] |
MANTA-VOGLI P D, DOTSIKAS Y, LOUKAS Y L, et al. The phenylketonuria patient: A recent dietetic therapeutic approach[J]. Nutritional Neuroscience,2020,23(8):628−639. doi: 10.1080/1028415X.2018.1538196
|
[13] |
GILANI G S, COCKELL K A, SEPEHR E. Effects of antinutritional factors on protein digestibility and amino acid availability in foods[J]. Journal of Aoac International,2005,88(3):967−987. doi: 10.1093/jaoac/88.3.967
|
[14] |
EVANS S, ADAM S, ADAMS S, et al. Uniformity of food protein interpretation amongst dietitians for patients with phenylketonuria (PKU): 2020 UK National Consensus Statements[J]. Nutrients,2020,12(8):1−11.
|
[15] |
AYDAS S B, SIRIN S, ASLIM B. Biochemical analysis of centaurea depressa phenylalanine ammonia lyase (PAL) for biotechnological applications in phenylketonuria (PKU)[J]. Pharmaceutical Biology,2016,54(12):2838−2844. doi: 10.1080/13880209.2016.1185634
|
[16] |
CLIFF M A, LAW J R, LUCKER J, et al. Descriptive and hedonic analyses of low-phe food formulations containing corn (zea mays) seedling roots: Toward development of a dietary supplement for individuals with phenylketonuria[J]. Journal of the Science of Food and Agriculture,2016,96(1):140−149. doi: 10.1002/jsfa.7074
|
[17] |
EISSA H A, ABDALLAH Z Y, KHALIL W K B, et al. Effect of natural pal-enzyme on the quality of egg white and mushroom flour and study its impact on the expression of PKU related genes and phenylalanine reduction in mice fed on[J]. Journal, Genetic Engineering & Biotechnology,2017,15(2):443−451.
|
[18] |
仇志荣. 果蔬营养与药用[M]. 北京: 中国旅游出版社, 1992: 243-253
QIU Z R. Nutrition and medicinal use of fruits and vegetables[M]. Beijing: China Tourism Press, 1992: 243-253.
|
[19] |
VAN WEGBERG A M J, MACDONALD A, AHRING K, et al. The complete european guidelines on phenylketonuria: Diagnosis and treatment[J]. Orphanet Journal of Rare Diseases,2017,12(162):1−56.
|
[20] |
PENA M J, ALMEIDA M F, VAN DAM E, et al. Special low protein foods for phenylketonuria: Availability in europe and an examination of their nutritional profile[J]. Orphanet Journal of Rare Diseases,2015,10(162):1−6.
|
[21] |
SCORTEGAGNA M L, DE OLIVEIRA V R, PASINI I, et al. Low phenylalanine breads as an alternative for patients with phenylketonuria[J]. British Food Journal,2019,122(1):26−35. doi: 10.1108/BFJ-03-2019-0213
|
[22] |
ALIREZA A, HAJAR A. Effect of type and amount of modified corn starches on qualitative properties of low-protein biscuits for phenylketonuria[J]. Food Science & Nutrition,2020,8(1):281−290.
|
[23] |
PARLAK O, DUNDAR A N. Production of low protein and gluten-free cookies for phenylketonuria PKU and/or celiac patients[J]. Czech Journal of Food Sciences,2021,39(1):29−34. doi: 10.17221/145/2020-CJFS
|
[24] |
SHRUTI P, ALEENA S, KARADKA G, et al. Development of low phenylalanine flour for phenylketonuric patient[J]. Journal of Food Processing and Preservation,2020,44(11):e14894−e14894.
|
[25] |
WOORE M S, FLINT-GARCIA S A, HOLLAND J B. The potential to breed a low-protein maize for protein-restricted diets[J]. Crop Science,2021,61(6):4202−4217. doi: 10.1002/csc2.20600
|
[26] |
WOOD G, EVANS S, POINTON-BELL K, et al. Special low protein foods in the UK: An examination of their macronutrient composition in comparison to regular foods[J]. Nutrients,2020,12(6):1893−1893. doi: 10.3390/nu12061893
|
[27] |
周志伟, 张嘉芷, 许永红, 等. 低苯丙肽配方食品的研制[J]. 食品工业科技,1999(S1):104−108. [ZHOU Z W, ZHANG J Z, XU Y H, et al. Development of low-phenylpropeptide formula food[J]. Science and Technology of Food Industry,1999(S1):104−108.
ZHOU Z W, ZHANG J Z, XU Y H, et al. Development of low-phenylpropeptide formula food[J]. Science and Technology of Food Industry, 1999(S1): 104-108.
|
[28] |
刘国信. 特殊配方奶粉: 标准规定不完善, 市场需求难满足[J]. 食品安全导刊,2015(28):68−69. [LIU G X. Special formula milk powder: The standard regulations are not perfect, and the market demand is difficult to meet[J]. Food Safety Guide,2015(28):68−69.
LIU G X. Special formula milk powder: The standard regulations are not perfect, and the market demand is difficult to meet[J]. Food Safety Guide, 2015, (28): 68-69.
|
[29] |
杨国民. 一种用于苯丙酮尿症患者的配方粉及其制备方法: 中国, 201810750645.5[P]. 2020-01-17
YANG G M. A formula powder for patients with phenylketonuria and its preparation method: China, 201810750645.5[P]. 2020-01-17.
|
[30] |
曾珍, 赵一楠, 唐雅男, 等. 一种用于苯丙酮尿症患者的食用配方粉: 中国, 201710910616.6[P]. 2018-03-06
ZENG Z, ZHAO Y N, TANG Y N, et al. An edible formula powder for patients with phenylketonuria: China, 201710910616.6[P]. 2018-03-06.
|
[31] |
魏晴霞, 杨林西. 低苯丙氨酸酪蛋白粉的研制[J]. 兰州医学院学报,1991,17(3):127−129. [WEI Q X, YANG L X. Preparation of low phenylalanine casein powder[J]. Journal of Lanzhou Medical College,1991,17(3):127−129.
WEI Q X, YANG L X. Preparation of low phenylalanine casein powder[J]. Journal of Lanzhou Medical College, 1991, 17(3): 127-129.
|
[32] |
CABRERA-PADILLA R Y, PINTO G A, GIORDANO R L C, et al. A new conception of enzymatic membrane reactor for the production of whey hydrolysates with low contents of phenylalanine[J]. Process Biochemistry,2009,44(3):269−276. doi: 10.1016/j.procbio.2008.10.019
|
[33] |
VILLAFUERTE F, PEREZ E, MAHFOUD A, et al. Obtaining protein products low in phenylalanine from milk whey and chachafruto (Erythrina edulis triana)[J]. Archivos Latinoamericanos De Nutricion,2019,69(1):25−33. doi: 10.37527/2019.69.1.004
|
[34] |
OZER I, OZER K. Production of low protein products used in treatment of congenital metabolic diseases: A pilot study in the stage of experimental animals[J]. Kafkas Universitesi Veteriner Fakultesi Dergisi,2011,17(2):263−267.
|
[35] |
BU T T, ZHOU M J, ZHENG J X, et al. Preparation and characterization of a low-phenylalanine whey hydrolysate using two-step enzymatic hydrolysis and macroporous resin adsorption[J]. Lwt-Food Science and Technology,2020:132.
|
[36] |
SU Y J, WANG Y Z, LU C, et al. Removal of phenylalanine from egg white powder: Two-step enzymatic method combined with activated carbon adsorption[J]. Process Biochemistry,2021,104(2021):101−109.
|
[37] |
盛晓静. 大米蛋白中苯丙氨酸脱除技术研究与产品开发[D]. 北京: 中国农业科学院, 2017
SHENG X J. Research and product development of phenylalanine removal technology from rice protein[D]. Beijing: Chinese Academy of Agricultural Sciences, 2017.
|
[38] |
TONG X H, LIAN Z T, MIAO L M, et al. An innovative two-step enzyme-assisted aqueous extraction for the production of reduced bitterness soybean protein hydrolysates with high nutritional value[J]. Lwt-Food Science and Technology,2020,134(2020):110151.
|
[39] |
李志刚, 光翠娥, 干建平. 低苯丙氨酸花生肽混合物的制备[J]. 食品与机械,2017,33(11):184−189. [LI Z G, GUANG C E, GAN J P. Preparation of low phenylalanine peanut peptide mixture[J]. Food and Machinery,2017,33(11):184−189.
LI Z G, GUANG C E, GAN J P. Preparation of low phenylalanine peanut peptide mixture[J]. Food and Machinery, 2017, 33(11): 184-189.
|
[40] |
李志刚, 张宇靖, 张晨, 等. 低苯丙氨酸山杏仁肽的制备[J]. 食品与生物技术学报,2019,38(9):92−96. [LI Z G, ZHANG Y J, ZHANG C, et al. Preparation of low-phenylalanine amygdalin[J]. Journal of Food and Biotechnology,2019,38(9):92−96. doi: 10.3969/j.issn.1673-1689.2019.09.013
LI Z G, ZHANG Y J, ZHANG C, et al. Preparation of Low-Phenylalanine Amygdalin[J]. Journal of Food and Biotechnology, 2019, 38(9): 92-96. doi: 10.3969/j.issn.1673-1689.2019.09.013
|
[41] |
GALVAO C M A, PINTO G A, JESUS C D F, et al. Producing a phenylalanine-free pool of peptides after tailored enzymatic hydrolyses of cheese whey[J]. Journal of Food Engineering,2009,91(1):109−117. doi: 10.1016/j.jfoodeng.2008.08.009
|
[42] |
SILVESTRE M P C, VIEIRA C R, SILVA M R, et al. Use of an enzymatic process for extracting and hydrolyzing rice proteins aiming at phenylalanine removal[J]. International Journal of Food Engineering,2009,5(1):1−11.
|
[43] |
中华人民共和国国家卫生和计划生育委员会. GB 29922-2013 文后参考文献著录规则[S]. 北京: 中国标准出版社, 2013
National Health and Family Planning Commission of the People’s Republic of China. GB 29922-2013 Rules for the description of references after the text[S]. Beijing: China Standard Press, 2013.
|
[44] |
SOLTANIZADEH N, MIRMOGHTADAIE L. Strategies used in production of phenylalanine-free foods for pku management[J]. Comprehensive Reviews in Food Science and Food Safety,2014,13(3):287−299. doi: 10.1111/1541-4337.12057
|
[45] |
LOPES D C F, DELVIVO F M, SILVESTRE M P C. Use of activated carbon for removing phenylalanine from reconstituted skim milk powder hydrolysates[J]. Lwt-Food Science and Technology,2005,38(5):447−453. doi: 10.1016/j.lwt.2004.07.021
|
[46] |
SU Y J, WANG Y Z, MCCLEMENTS D J, et al. Selective adsorption of egg white hydrolysates onto activated carbon: Establishment of physicochemical mechanisms for removing phenylalanine[J]. Food Chemistry,2021,364(2021):130285.
|
[47] |
GOLDAR P, GIVIANRAD M H, SHAMS A. Effect of ultrafiltered milk permeate and non-dairy creamer powder concentration on low phenylalanine yoghurt’s physicochemical properties during storage[J]. Journal of Food Science and Technology-Mysore,2016,53(7):3053−3059. doi: 10.1007/s13197-016-2278-9
|
[48] |
LOPEZBAJONERO L J, LARACALDERON P, GALVEZMARISCAL A, et al. Enzymatic production of a low-phenylalanine product from skim milk powder and caseinate[J]. Journal of Food Science,1991,56(4):938−942. doi: 10.1111/j.1365-2621.1991.tb14610.x
|
[49] |
郑惠娜, 周春霞, 陈志成, 等. 胰酶酶解珍珠贝分离蛋白制备低苯丙氨酸寡肽制品[J]. 食品工业科技,2016,37(8):215−224. [ZHENG H N, ZHOU C X, CHEN Z C, et al. Preparation of low phenylalanine oligopeptide products by trypsin hydrolysis of pearl oyster protein isolate[J]. Food Industry Science and Technology,2016,37(8):215−224.
ZHENG H N, ZHOU C X, CHEN Z C, et al. Preparation of low phenylalanine oligopeptide products by trypsin hydrolysis of pearl oyster protein isolate[J]. Food Industry Science and Technology, 2016, 37(8): 215-224.
|
[50] |
SILVESTRE M P C, DA SILVA M C, DE SOUZA M W S, et al. Hydrolysis degree, peptide profile and phenylalanine removal from whey protein concentrate hydrolysates obtained by various proteases[J]. International Journal of Food Science and Technology,2013,48(3):588−595. doi: 10.1111/ijfs.12003
|
[51] |
GIESLER L, LINKE D, RABE S, et al. Hydrolysis of wheat gluten by combining peptidases of flammulina velutipes and electrodialysis[J]. Journal of Agricultural and Food Chemistry,2013,61(36):8641−8649. doi: 10.1021/jf401716m
|
[52] |
CARREIRA R L, SILVA M R, STARLING A L P, et al. Association of two enzymes for obtaining low phenylalanine protein hydrolysates from wheat flour[J]. International Journal of Food Engineering,2008,4(7):13.
|
[53] |
CAPOBIANGO M, LOPES D C F, CARREIRA R L, et al. Optimization of enzyme assisted processes for extracting and hydrolysing corn proteins aiming phenylalanine removal[J]. International Journal of Food Engineering,2007,3(6):10.
|
[54] |
VAN CALCAR S C, NEY D M. Food products made with glycomacropeptide, a low-phenylalanine whey protein, provide a new alternative to amino acid-based medical foods for nutrition management of phenylketonuria[J]. Journal of the Academy of Nutrition and Dietetics,2012,112(8):1201−1210. doi: 10.1016/j.jand.2012.05.004
|
[55] |
LIM K, VAN CALCAR S C, NELSON K L, et al. Acceptable low-phenylalanine foods and beverages can be made with glycomacropeptide from cheese whey for individuals with pku[J]. Molecular Genetics and Metabolism,2007,92(1-2):176−178. doi: 10.1016/j.ymgme.2007.06.004
|
[56] |
SOLVERSON P, MURALI S G, BRINKMAN A S, et al. Glycomacropeptide, a low-phenylalanine protein isolated from cheese whey, supports growth and attenuates metabolic stress in the murine model of phenylketonuria[J]. American Journal of Physiology-Endocrinology and Metabolism,2012,302(7):E885−E895. doi: 10.1152/ajpendo.00647.2011
|
[57] |
SOLVERSON P, MURALI S G, LITSCHER S J, et al. Low bone strength is a manifestation of phenylketonuria in mice and is attenuated by a glycomacropeptide diet[J]. PLoS One,2012,7(9):e45165. doi: 10.1371/journal.pone.0045165
|
[58] |
STROUP B M, SAWIN E A, MURALI S G, et al. Amino acid medical foods provide a high dietary acid load and increase urinary excretion of renal net acid, calcium, and magnesium compared with glycomacropeptide medical foods in phenylketonuria[J]. Journal of Nutrition and Metabolism,2017,2017(1909101):1−12.
|
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