JPH04126051A - Nutritional preparation for patient suffering from phenylketonuria - Google Patents
Nutritional preparation for patient suffering from phenylketonuriaInfo
- Publication number
- JPH04126051A JPH04126051A JP2248170A JP24817090A JPH04126051A JP H04126051 A JPH04126051 A JP H04126051A JP 2248170 A JP2248170 A JP 2248170A JP 24817090 A JP24817090 A JP 24817090A JP H04126051 A JPH04126051 A JP H04126051A
- Authority
- JP
- Japan
- Prior art keywords
- phenylketonuria
- patients
- powder
- preparation
- nutritional preparation
- Prior art date
- Legal status (The legal status is an assumption and is not a legal conclusion. Google has not performed a legal analysis and makes no representation as to the accuracy of the status listed.)
- Pending
Links
- 201000011252 Phenylketonuria Diseases 0.000 title claims abstract description 26
- 238000002360 preparation method Methods 0.000 title claims abstract description 21
- 235000016709 nutrition Nutrition 0.000 title claims abstract description 13
- 239000000843 powder Substances 0.000 claims abstract description 27
- 150000001413 amino acids Chemical class 0.000 claims abstract description 16
- 229910052500 inorganic mineral Inorganic materials 0.000 claims abstract description 11
- 239000011707 mineral Substances 0.000 claims abstract description 11
- 235000013343 vitamin Nutrition 0.000 claims abstract description 11
- 229940088594 vitamin Drugs 0.000 claims abstract description 11
- 229930003231 vitamin Natural products 0.000 claims abstract description 11
- 239000011782 vitamin Substances 0.000 claims abstract description 11
- 108010076119 Caseins Proteins 0.000 claims abstract description 8
- 102000011632 Caseins Human genes 0.000 claims abstract description 8
- 235000021246 κ-casein Nutrition 0.000 claims abstract 4
- 239000004615 ingredient Substances 0.000 claims abstract 3
- COLNVLDHVKWLRT-QMMMGPOBSA-N L-phenylalanine Chemical compound OC(=O)[C@@H](N)CC1=CC=CC=C1 COLNVLDHVKWLRT-QMMMGPOBSA-N 0.000 claims description 22
- 235000001014 amino acid Nutrition 0.000 claims description 15
- 150000001720 carbohydrates Chemical class 0.000 claims description 10
- 235000014633 carbohydrates Nutrition 0.000 claims description 10
- 235000010755 mineral Nutrition 0.000 claims description 10
- 235000008939 whole milk Nutrition 0.000 claims description 6
- 235000005911 diet Nutrition 0.000 claims description 5
- 230000037213 diet Effects 0.000 claims description 5
- 108090000765 processed proteins & peptides Proteins 0.000 claims description 5
- 235000004252 protein component Nutrition 0.000 claims description 5
- 108010028463 kappa-casein glycomacropeptide Proteins 0.000 claims description 4
- COLNVLDHVKWLRT-UHFFFAOYSA-N phenylalanine Natural products OC(=O)C(N)CC1=CC=CC=C1 COLNVLDHVKWLRT-UHFFFAOYSA-N 0.000 claims description 3
- 229910000831 Steel Inorganic materials 0.000 claims description 2
- 239000010959 steel Substances 0.000 claims description 2
- 206010023388 Ketonuria Diseases 0.000 claims 1
- 208000007976 Ketosis Diseases 0.000 claims 1
- 230000035764 nutrition Effects 0.000 claims 1
- 150000003722 vitamin derivatives Chemical class 0.000 claims 1
- 239000003925 fat Substances 0.000 abstract description 14
- 235000018102 proteins Nutrition 0.000 abstract description 11
- 102000004169 proteins and genes Human genes 0.000 abstract description 11
- 108090000623 proteins and genes Proteins 0.000 abstract description 11
- 239000003921 oil Substances 0.000 abstract description 9
- 238000002156 mixing Methods 0.000 abstract description 6
- -1 glucide Substances 0.000 abstract description 5
- 230000009965 odorless effect Effects 0.000 abstract description 3
- 230000009967 tasteless effect Effects 0.000 abstract description 3
- 239000000203 mixture Substances 0.000 description 16
- 235000019197 fats Nutrition 0.000 description 13
- 239000000047 product Substances 0.000 description 12
- 235000019640 taste Nutrition 0.000 description 9
- 235000019198 oils Nutrition 0.000 description 8
- 108010046377 Whey Proteins Proteins 0.000 description 7
- 102000007544 Whey Proteins Human genes 0.000 description 7
- 239000005862 Whey Substances 0.000 description 6
- 229920002472 Starch Polymers 0.000 description 4
- 239000005018 casein Substances 0.000 description 4
- BECPQYXYKAMYBN-UHFFFAOYSA-N casein, tech. Chemical compound NCCCCC(C(O)=O)N=C(O)C(CC(O)=O)N=C(O)C(CCC(O)=N)N=C(O)C(CC(C)C)N=C(O)C(CCC(O)=O)N=C(O)C(CC(O)=O)N=C(O)C(CCC(O)=O)N=C(O)C(C(C)O)N=C(O)C(CCC(O)=N)N=C(O)C(CCC(O)=N)N=C(O)C(CCC(O)=N)N=C(O)C(CCC(O)=O)N=C(O)C(CCC(O)=O)N=C(O)C(COP(O)(O)=O)N=C(O)C(CCC(O)=N)N=C(O)C(N)CC1=CC=CC=C1 BECPQYXYKAMYBN-UHFFFAOYSA-N 0.000 description 4
- 235000021240 caseins Nutrition 0.000 description 4
- 238000000034 method Methods 0.000 description 4
- 239000008107 starch Substances 0.000 description 4
- GUBGYTABKSRVRQ-QKKXKWKRSA-N Lactose Natural products OC[C@H]1O[C@@H](O[C@H]2[C@H](O)[C@@H](O)C(O)O[C@@H]2CO)[C@H](O)[C@@H](O)[C@H]1O GUBGYTABKSRVRQ-QKKXKWKRSA-N 0.000 description 3
- 235000013305 food Nutrition 0.000 description 3
- 239000008101 lactose Substances 0.000 description 3
- 235000013336 milk Nutrition 0.000 description 3
- 239000008267 milk Substances 0.000 description 3
- 210000004080 milk Anatomy 0.000 description 3
- 230000001953 sensory effect Effects 0.000 description 3
- 235000019698 starch Nutrition 0.000 description 3
- 230000001225 therapeutic effect Effects 0.000 description 3
- 108090000746 Chymosin Proteins 0.000 description 2
- 206010012735 Diarrhoea Diseases 0.000 description 2
- 208000000481 Maternal phenylketonuria Diseases 0.000 description 2
- 239000008280 blood Substances 0.000 description 2
- 210000004369 blood Anatomy 0.000 description 2
- 235000013351 cheese Nutrition 0.000 description 2
- 229940080701 chymosin Drugs 0.000 description 2
- 239000003240 coconut oil Substances 0.000 description 2
- 235000019864 coconut oil Nutrition 0.000 description 2
- 210000001035 gastrointestinal tract Anatomy 0.000 description 2
- 239000000463 material Substances 0.000 description 2
- GNOLWGAJQVLBSM-UHFFFAOYSA-N n,n,5,7-tetramethyl-1,2,3,4-tetrahydronaphthalen-1-amine Chemical compound C1=C(C)C=C2C(N(C)C)CCCC2=C1C GNOLWGAJQVLBSM-UHFFFAOYSA-N 0.000 description 2
- 230000003204 osmotic effect Effects 0.000 description 2
- 102000004196 processed proteins & peptides Human genes 0.000 description 2
- 108010058314 rennet Proteins 0.000 description 2
- 229940108461 rennet Drugs 0.000 description 2
- DQJCDTNMLBYVAY-ZXXIYAEKSA-N (2S,5R,10R,13R)-16-{[(2R,3S,4R,5R)-3-{[(2S,3R,4R,5S,6R)-3-acetamido-4,5-dihydroxy-6-(hydroxymethyl)oxan-2-yl]oxy}-5-(ethylamino)-6-hydroxy-2-(hydroxymethyl)oxan-4-yl]oxy}-5-(4-aminobutyl)-10-carbamoyl-2,13-dimethyl-4,7,12,15-tetraoxo-3,6,11,14-tetraazaheptadecan-1-oic acid Chemical compound NCCCC[C@H](C(=O)N[C@@H](C)C(O)=O)NC(=O)CC[C@H](C(N)=O)NC(=O)[C@@H](C)NC(=O)C(C)O[C@@H]1[C@@H](NCC)C(O)O[C@H](CO)[C@H]1O[C@H]1[C@H](NC(C)=O)[C@@H](O)[C@H](O)[C@@H](CO)O1 DQJCDTNMLBYVAY-ZXXIYAEKSA-N 0.000 description 1
- GUBGYTABKSRVRQ-XLOQQCSPSA-N Alpha-Lactose Chemical compound O[C@@H]1[C@@H](O)[C@@H](O)[C@@H](CO)O[C@H]1O[C@@H]1[C@@H](CO)O[C@H](O)[C@H](O)[C@H]1O GUBGYTABKSRVRQ-XLOQQCSPSA-N 0.000 description 1
- 206010010356 Congenital anomaly Diseases 0.000 description 1
- 208000012239 Developmental disease Diseases 0.000 description 1
- WQZGKKKJIJFFOK-GASJEMHNSA-N Glucose Natural products OC[C@H]1OC(O)[C@H](O)[C@@H](O)[C@@H]1O WQZGKKKJIJFFOK-GASJEMHNSA-N 0.000 description 1
- 108010015899 Glycopeptides Proteins 0.000 description 1
- 102000002068 Glycopeptides Human genes 0.000 description 1
- UQABYHGXWYXDTK-UUOKFMHZSA-N GppNP Chemical compound C1=2NC(N)=NC(=O)C=2N=CN1[C@@H]1O[C@H](COP(O)(=O)OP(O)(=O)NP(O)(O)=O)[C@@H](O)[C@H]1O UQABYHGXWYXDTK-UUOKFMHZSA-N 0.000 description 1
- FFEARJCKVFRZRR-BYPYZUCNSA-N L-methionine Chemical compound CSCC[C@H](N)C(O)=O FFEARJCKVFRZRR-BYPYZUCNSA-N 0.000 description 1
- OUYCCCASQSFEME-QMMMGPOBSA-N L-tyrosine Chemical compound OC(=O)[C@@H](N)CC1=CC=C(O)C=C1 OUYCCCASQSFEME-QMMMGPOBSA-N 0.000 description 1
- 208000012902 Nervous system disease Diseases 0.000 description 1
- 208000025966 Neurological disease Diseases 0.000 description 1
- 240000007594 Oryza sativa Species 0.000 description 1
- 235000007164 Oryza sativa Nutrition 0.000 description 1
- 108091005804 Peptidases Proteins 0.000 description 1
- 108010069013 Phenylalanine Hydroxylase Proteins 0.000 description 1
- 102100038223 Phenylalanine-4-hydroxylase Human genes 0.000 description 1
- 239000004365 Protease Substances 0.000 description 1
- 102100037486 Reverse transcriptase/ribonuclease H Human genes 0.000 description 1
- 235000019485 Safflower oil Nutrition 0.000 description 1
- 240000004668 Valerianella locusta Species 0.000 description 1
- 235000003560 Valerianella locusta Nutrition 0.000 description 1
- 230000002378 acidificating effect Effects 0.000 description 1
- WQZGKKKJIJFFOK-VFUOTHLCSA-N beta-D-glucose Chemical compound OC[C@H]1O[C@@H](O)[C@H](O)[C@@H](O)[C@@H]1O WQZGKKKJIJFFOK-VFUOTHLCSA-N 0.000 description 1
- 235000019658 bitter taste Nutrition 0.000 description 1
- 239000006227 byproduct Substances 0.000 description 1
- 239000002131 composite material Substances 0.000 description 1
- 235000005687 corn oil Nutrition 0.000 description 1
- 239000002285 corn oil Substances 0.000 description 1
- 238000011033 desalting Methods 0.000 description 1
- 239000008121 dextrose Substances 0.000 description 1
- 235000020805 dietary restrictions Nutrition 0.000 description 1
- 201000010099 disease Diseases 0.000 description 1
- 208000037265 diseases, disorders, signs and symptoms Diseases 0.000 description 1
- 230000035622 drinking Effects 0.000 description 1
- 239000003995 emulsifying agent Substances 0.000 description 1
- 239000000796 flavoring agent Substances 0.000 description 1
- 235000019634 flavors Nutrition 0.000 description 1
- 235000013312 flour Nutrition 0.000 description 1
- 238000009472 formulation Methods 0.000 description 1
- 150000002632 lipids Chemical class 0.000 description 1
- 239000007788 liquid Substances 0.000 description 1
- 238000004519 manufacturing process Methods 0.000 description 1
- 230000008774 maternal effect Effects 0.000 description 1
- 208000030159 metabolic disease Diseases 0.000 description 1
- 229930182817 methionine Natural products 0.000 description 1
- 230000000926 neurological effect Effects 0.000 description 1
- 235000015097 nutrients Nutrition 0.000 description 1
- 239000002244 precipitate Substances 0.000 description 1
- 125000002924 primary amino group Chemical group [H]N([H])* 0.000 description 1
- 235000009566 rice Nutrition 0.000 description 1
- 230000000630 rising effect Effects 0.000 description 1
- 239000003813 safflower oil Substances 0.000 description 1
- 235000005713 safflower oil Nutrition 0.000 description 1
- 239000003549 soybean oil Substances 0.000 description 1
- 235000012424 soybean oil Nutrition 0.000 description 1
- 239000000126 substance Substances 0.000 description 1
- 239000006228 supernatant Substances 0.000 description 1
- 239000013589 supplement Substances 0.000 description 1
- 208000024891 symptom Diseases 0.000 description 1
- 239000006188 syrup Substances 0.000 description 1
- 235000020357 syrup Nutrition 0.000 description 1
- OUYCCCASQSFEME-UHFFFAOYSA-N tyrosine Natural products OC(=O)C(N)CC1=CC=C(O)C=C1 OUYCCCASQSFEME-UHFFFAOYSA-N 0.000 description 1
- 238000000108 ultra-filtration Methods 0.000 description 1
- 235000015112 vegetable and seed oil Nutrition 0.000 description 1
- 239000008158 vegetable oil Substances 0.000 description 1
- 239000002699 waste material Substances 0.000 description 1
- 235000021119 whey protein Nutrition 0.000 description 1
Landscapes
- Coloring Foods And Improving Nutritive Qualities (AREA)
Abstract
Description
【発明の詳細な説明】
皮栗上例打朋光夏
本発明はフェニルケトン尿症（以下ＰＫυ）患者および
母性Ｐ Ｋ Ｕ 患者が日常摂取するフェニルケトン尿
症患者用栄養鋼製物に関する。DETAILED DESCRIPTION OF THE INVENTION The present invention relates to a nutritional steel product for phenylketonuria patients (hereinafter referred to as PKυ) and maternal P KU patients that are ingested daily.
ｋ米肢歪
ＰＫＵとはフェニルアラニン（Ｐｈｅ）をチロシンに変
換するフェニルアラニン水酸化酵素を先天的に欠落して
いるため、Ｐｈｅが代謝されず血中に蓄積され、神経障
害および発育障害をもたらす先天性代謝異常症である。K rice limb distortion PKU is a congenital condition in which the phenylalanine hydroxylase that converts phenylalanine (Phe) to tyrosine is congenitally lacking, so Phe is not metabolized and accumulates in the blood, causing neurological and developmental disorders. It is a metabolic disorder.
したがって、ＰＫＵ患者は医師の厳重な監督下のもとで
Ｐｈｅ摂取量を厳密に管理し、体内にＰｈｅが蓄積しな
いような食事制限が行われる。このような目的のために
、蛋白質成分の全て、あるいは大部分を、Ｐｈｅ以外の
アミノ酸混合物で置換した特殊調製粉乳（以下特配とい
う）が市販されている。しかしながら、本島はその主た
る蛋白質成分がアミノ酸混合物であるために、腸管内で
の浸透圧が高くなり、下痢の症状を生しせしめたり、あ
るいは不快な風味を呈するという欠点があった。そこで
、 ｐｒｖｇ者やその親から、より飲み易い特配に対
する要望が強かった。このような背景にあって、 Ｙａ
ｍａｓｈｉｔａ らは蛋白質をプロテアーゼで分解し、
Ｐｈｅ含量の少ない画分を得ることに成功しくＪ、Ｆｏ
ｏｄ Ｓｃｉ、、 ４１：１０２９−１０３２１９７６
） 、このようなペプチドを低フェニルアラニンペプチ
ド（ＬＰＰ） と呼ぶようになった。この原理に基づ
き、ＬＰＰを工業的に製造する技術が開発され、すでに
特開昭６１−６８４２６に開示されている。このような
方法で製造されたＬＰＰを特徴とする特配は、従来のも
のと比べると淡白な味であり、治療成績も良好で、ｐＫ
ｕ患者や担当の医師からは好評である。しかし、ＬＰＰ
は蛋白質を酵素分解して調製されるため、歩留が低く、
コストが高いという欠点がある。さらに、味が淡白だと
云っても、アミノ酸混合物に比べればかなり改善されて
いるものの、通常の食品の概念からすればまだまだ受は
入れ難い味がする。Therefore, PKU patients strictly control their Phe intake under the strict supervision of a doctor, and are subject to dietary restrictions to prevent Phe from accumulating in their bodies. For this purpose, specially formulated milk powder (hereinafter referred to as "special formula") in which all or most of the protein components are replaced with a mixture of amino acids other than Phe is commercially available. However, since the main protein component of Honjima is a mixture of amino acids, it has the disadvantage that the osmotic pressure in the intestinal tract becomes high, causing symptoms of diarrhea or an unpleasant flavor. Therefore, there was a strong demand from prvg drinkers and their parents for a special drink that was easier to drink. Against this background, Ya
mashita et al. decompose proteins with protease,
J, Fo successfully obtained a fraction with low Phe content.
od Sci, 41:1029-10321976
), such peptides have come to be called low-phenylalanine peptides (LPPs). Based on this principle, a technology for industrially producing LPP has been developed and has already been disclosed in Japanese Patent Application Laid-Open No. 68426/1983. The special product featuring LPP produced by this method has a bland taste compared to conventional products, has good therapeutic results, and has a low pK
It has been well received by patients and their doctors. However, L.P.P.
is prepared by enzymatically decomposing proteins, so the yield is low;
It has the disadvantage of high cost. Furthermore, although the taste is said to be bland, although it is considerably improved compared to amino acid mixtures, it still tastes unacceptable from the perspective of a normal food.
日 く ” し ゛ と る−本発明者らは、
従来のフェニルケトン尿症患者用組成物のこのような欠
点を改善することを目的として安価に大量入手が可能で
あり、Ｐｈｅを殆ど含まず、かつ無味無臭な素材を探索
し、本発明を成すに到った。The inventors of the present invention
In order to improve these drawbacks of conventional compositions for patients with phenylketonuria, we searched for a material that can be obtained in large quantities at low cost, contains almost no Phe, and is tasteless and odorless, and the present invention was achieved. reached.
すなわち、本発明は、安価に大量人手が可能で、しかも
Ｐｈｅを殆ど含まず、かつ無味無臭な蛋白質素材を配合
したＰＫ［Ｉ治療用栄養調製物を提供することを目的と
する。That is, an object of the present invention is to provide a nutritional preparation for treating PK[I that can be produced in large quantities at low cost, contains almost no Phe, and contains a tasteless and odorless protein material.
−” るための
本発明は、ＰＫＬＩ患者または母性ＰＫＵ患者の食餌に
κ−カゼイングリコマクロペプチド（以下ＧＭＰ）を配
合することを特徴とするフェニルケトン犀症患者用栄養
調製物に関する。The present invention relates to a nutritional preparation for phenylketonosis patients characterized by incorporating κ-casein glycomacropeptide (hereinafter referred to as GMP) into the diet of PKLI patients or maternal PKU patients.
本発明におけるＧＭＰはこれを蛋白質成分として単独で
配合してもあるいはその一部として配合してもよい、
ＧＭＰはカゼインにキモシンを作用させた時、に−カ
ゼインから遊離してくる分子量約ｓ、ｏｏ。GMP in the present invention may be blended alone as a protein component or as a part thereof.
GMP has a molecular weight of approximately s,00 which is liberated from casein when chymosin acts on it.
の糖ペプチドである。キモシンはに一カゼインのＮ末端
から数えて１０５番目のＰｈｅと１０６番目のメチオニ
ンの間を切断し、１０６番目以鋒０ペプチドをＧＭＰと
いう、このＧＭＰのアミノ酸配列を見る限り、Ｐｈｅが
全く含まれていないことは古くから知られていた。しか
し、これまでＧＭＰを工業的に製造する技術がなく、誰
もＧＮＰをＰＫＵ治療用組成物に配合することに思い到
らなかった。is a glycopeptide. Chymosin cleaves between the 105th Phe and the 106th methionine counting from the N-terminus of casein, and the peptide after the 106th peptide is called GMP.As far as the amino acid sequence of GMP is concerned, it does not contain any Phe. It has been known for a long time that this is not the case. However, until now there has been no technology for industrially producing GMP, and no one has thought of incorporating GNP into a composition for treating PKU.
Ｇ？ＩＰは通常、チーズ製造時に排出される副産物であ
るホエー中に含有されている。このホエー中には、ＧＮ
Ｐの他にも多種な蛋白質が大量に含まれており、ホエー
蛋白質を濃縮したーｐｃをＰＫＵ患者患者用特配合する
ことは公知である（特開昭５９５１１１） 、 Ｌかし
、ｗｐｃ中のＰｈｅ含量は３〜４％にも達し、←ＰＣを
特徴とする特配を寞用に供することはできない。G? IP is typically contained in whey, a byproduct of cheese production. In this whey, GN
In addition to P, it contains a large amount of various other proteins, and it is known that a concentrated whey protein-PC is specially formulated for PKU patients (Japanese Patent Application Laid-Open No. 595111). The Phe content reaches as high as 3-4%, making it impossible to provide a special product featuring ←PC for commercial use.
ＧＭＰをホエーから分離する技術もすでに開示されてい
る（特公昭５Ｂ−２３４００）が、この方法で分離され
たＧＮＰの純度は低く、Ｐｈｅ含量は２〜３％もあり、
ＰＫＵ治療用組成物素材として使えない、現在市販され
ているＰＫＵ患者用特粉特配Ｐｈｅ含置装６７■／１０
０ｇ粉であり、このレヘルにＰｈｅ含量を調整するため
にはＧＮＰのＰｈｅ含量は１．０％以下、好ましくは０
．５％以下でなければならない、このようなＧＮＰは、
レンネットカゼインを調製するさいに得られる排液を酸
性のｐＨとして生成する沈澱を除去し、得られる上澄を
脱塩処理して得るか（特開昭６３−２８４１９９）ある
いは、チーズホエーのｐＨを酸性に調整した後に限外濾
過（特願平１−９７５８３）することによって初めて人
手できるようになった。A technique for separating GMP from whey has already been disclosed (Japanese Patent Publication No. 5B-23400), but the purity of GNP separated by this method is low, with a Phe content of 2 to 3%.
Special powder Phe-containing device for PKU patients currently on the market that cannot be used as a composition material for PKU treatment 67■/10
0g powder, and in order to adjust the Phe content to this level, the Phe content of GNP should be 1.0% or less, preferably 0.
．． Such GNP, which must be less than 5%, is
Rennet casein can be obtained by adjusting the pH of the waste liquid obtained during the preparation of rennet casein to an acidic pH, removing the generated precipitate, and desalting the resulting supernatant (Japanese Patent Application Laid-Open No. 63-284199), or by adjusting the pH of cheese whey. It became possible to do this manually for the first time by ultrafiltration (Japanese Patent Application No. 1-97583) after adjusting it to acidity.
このようにして得られたＧ？ＩＰの代表的なアミノ酸組
成を表１に示す。G obtained in this way? Table 1 shows the typical amino acid composition of IP.
表１
ＧＭＰのアミノ酸組成（ｇ／１００ｇ粉末）ＧＭＰは、
ＰＫＵ患者用栄養調製物１００ ｇ当りＰｈｅ含量が８
０■以下、現在市販されている特配と同じＰｈｅ含量と
するためには６７■となるように配合する。この際必要
な蛋白質の全量をＧＭＰで置換し、不足するアミノ酸は
アミノ酸粉末で補う、あるいは、ＬＰＰや全脂粉乳をＰ
ｈｅ含量が前記量を越えない限度でＧ？ＩＰと共に配合
してもよい。調製物中のアミノｆｌｉ＆１１成は、現在
市販されているものと同し様な組成となるように、ＧＭ
Ｐ、 ＬＰＰ、全脂粉乳、あるいはアミノ酸粉末を配
合することが望ましい。Table 1 GMP amino acid composition (g/100g powder) GMP is
Phe content per 100 g of nutritional preparation for PKU patients is 8
In order to have the same Phe content as the currently commercially available special products, the Phe content should be 67. At this time, replace the entire amount of necessary protein with GMP and supplement the missing amino acids with amino acid powder, or replace LPP or whole milk powder with GMP.
G as long as the he content does not exceed the above amount? It may be blended with IP. The amino fli & 11 components in the preparation are GM
It is desirable to incorporate P, LPP, whole milk powder, or amino acid powder.
現在市販されているＰＫＩＩ患者用特粉に特配蛋白質の
他に脂肪、＃！質、ミネラルおよびビタミンが配合され
ている。脂肪、Ｗ質、ミネラルおよびビタミンは、現在
市販されているＰ Ｋ Ｕ 、ｉ音用特配の組成をめど
に配合すればよい０表２に現在市販されているＰＫＵ恵
者用特粉特配分組成を示す。脂肪は油脂糖質粉末によっ
て供給される。ｍ質は油脂糖質粉末ならびに澱粉や乳糖
を配合することによって供給される。ビタミンおよびミ
ネラルは、これらを混合した粉末を所定量配合すればよ
い。Currently available on the market, special flour for PKII patients contains special protein and fat, #! Contains nutrients, minerals and vitamins. Fat, W quality, minerals, and vitamins should be blended with the composition of currently commercially available PKU special powder for i-sound. Table 2 shows the currently commercially available special powder composition for PKU Keisha. shows. Fat is supplied by fat-carbohydrate powder. Substances are supplied by blending oil/fat/carbohydrate powder, starch, and lactose. For vitamins and minerals, a predetermined amount of a powder mixture of these may be blended.
本発明における油脂Ｗｔ絹粉末、コーンサラダ１１、ヤ
シ油１３、α−デンプン２１、乳糖１３．５、粉末水あ
め５８、乳化剤他２．４を配合して製造したものである
。蛋白質としては、ＬＰＰ、全脂粉乳、ホエーＰＣ等が
用いられ、＃ｉ質としては澱粉、乳糖、デキス） ＩＪ
ン、蔗糖等が、脂肪は油脂糖質粉末から供給されるがそ
の油脂としてはサフラワー油、コーン油、大豆油、ヤシ
油等の植物油、ラード等の動物油脂、中鎖脂肪酸トリグ
リセリド（ＭＣＴ）等が用いられる。また微量成分のビ
タミン、無機質等は表２に記載される成分等が用いられ
る。蛋白質、脂質及び糖質の配合割合は栄養調製物１０
０ｇに対し、蛋白質１４〜１８ｇ、脂１ｉ１６〜２１ｇ
及び糖質５５〜６７ｇにすることが望ましく、この蛋白
質の一部または全部をκ−カゼイングリコマクロペプチ
ドで置換し、製品中のＰｈｅ含量を８０■以下、望まし
くは６７■あるいはそれ以下とする。It was produced by blending the oil and fat Wt silk powder of the present invention, 11 parts of corn salad, 13 parts of coconut oil, 21 parts of α-starch, 13.5 parts of lactose, 58 parts of powdered starch syrup, 2.4 parts of emulsifier and others. As the protein, LPP, whole milk powder, whey PC, etc. are used, and as the #i quality, starch, lactose, dextrose) IJ
Fats are supplied from fats and carbohydrate powders, including vegetable oils such as safflower oil, corn oil, soybean oil, and coconut oil, animal fats and oils such as lard, and medium-chain fatty acid triglycerides (MCTs). etc. are used. Further, as the trace components such as vitamins and minerals, the components listed in Table 2 are used. The blending ratio of protein, lipid and carbohydrate is 10% of the nutritional preparation.
0g, protein 14-18g, fat 1i16-21g
It is desirable to have 55 to 67 g of carbohydrates, and some or all of this protein is replaced with κ-casein glycomacropeptide, so that the Phe content in the product is 80 μ or less, preferably 67 μ or less.
本発明の栄養調製物は、通常粉末状に混合されで製造さ
れる。また、投与時には濃度１３〜１５％となるように
調乳し、飲用される。The nutritional preparation of the present invention is usually prepared by mixing it into powder form. In addition, at the time of administration, milk is prepared to a concentration of 13 to 15% and drunk.
血１
本発明によるＰＫＩＩ症および母性ＰＫＵ治療用調製物
は、通常の食品と比べても殆ど遜色がない程淡白な味で
あるばかりでなく、ＧＮＰの分子量が高いので、腸管内
における浸透圧の上昇も殆ど問題にならない。このよう
に、長年不快な味と下痢に苦しんできたＰＫＵ乳幼児お
よび妊娠等のＰＫＵ患者にとって、本発明による治療用
調製物は多大なる貢献をなすものである。Blood 1 The preparation for treating PKII disease and maternal PKU according to the present invention not only has a bland taste that is almost comparable to that of ordinary foods, but also has a high molecular weight of GNP, which reduces the osmotic pressure in the intestinal tract. Rising is hardly a problem. Thus, the therapeutic preparation according to the present invention makes a great contribution to PKU patients such as PKU infants and pregnant women who have been suffering from unpleasant taste and diarrhea for many years.
以下の実施例は本発明を例示するものであるが、本発明
を限定するものではない、実施例に示す配合例の成分組
成は表２に示したものと同じである。The following examples illustrate the present invention, but do not limit the present invention. The compositions of the formulations shown in the examples are the same as those shown in Table 2.
実施例１
油脂糖質粉末６６．１ｇ、アミノ酸（表２の組成のもの
）４．３ｇ、 ＧＭＰ １３．０ ｇ、糖質１３．７
ｇ及びビタミン・ミネラル（表２の組成のもの）２．
９ ｇを混合して本発明のＰＫＵ治療用調製物を得た。Example 1 Fat and oil sugar powder 66.1g, amino acids (composition shown in Table 2) 4.3g, GMP 13.0g, sugar 13.7
g and vitamins and minerals (with the composition shown in Table 2)2.
9 g were mixed to obtain the PKU treatment preparation of the present invention.
実施例２
油脂糖質粉末６６、１ｇ、アミノ酸２．５ｇ、 ＬＰＰ
１２．３ｇ、ＧＭＰ ２．２ ｇ、 ｔＭ’ｌ１４．
Ｏｇ及ヒヒ９ ミ７ ・ミ＊ラル２．９ｇを混合して本
発明のＰＫＵ治療用調製物を得た。Example 2 Fat and oil sugar powder 66.1g, amino acid 2.5g, LPP
12.3g, GMP 2.2g, tM'l14.
2.9 g of Og and Baboon9 Mi7.mi*ral were mixed to obtain the PKU treatment preparation of the present invention.
油脂糖質粉末、アミノ酸、ｃｎｐ、 ｔＰｔ質及びビタ
ミン・ミネラルは実施例１と同しものを使用し、またＬ
ＰＰは特開昭６１−６８４２６に開示された方法でホエ
ー蛋白質ａ縮物を酵素処理して調製したものを使用した
。The same oil and sugar powder, amino acids, CNP, tPt, and vitamins and minerals as in Example 1 were used, and L
The PP used was prepared by enzymatically treating whey protein a condensate by the method disclosed in JP-A No. 61-68426.
実施例３
油脂［Ｗ６６．１ｇ、アミノ酸４．１ｇ、全脂粉乳２．
２ｇ、ＧＮＰ １２．５ｇ、 Ｉ！ｆｆ１２．２ｇ及び
ビタミン・ミネラル２．９ｇを混合して本発明のＰＫＵ
治療用調製物を得た。Example 3 Fats and oils [W66.1g, amino acids 4.1g, whole milk powder 2.
2g, GNP 12.5g, I! PKU of the present invention by mixing 12.2 g of ff and 2.9 g of vitamins and minerals.
A therapeutic preparation was obtained.
油脂糖質粉末、アミノ酸、ＧＮＰ、糖質及びビタミン・
ミネラルは実施例１と同しものを、またＬＰＰは実施例
２と同しものを使用した。全脂粉乳は、市販品（雪印乳
業■製）を使用した。Oil and fat carbohydrate powder, amino acids, GNP, carbohydrates and vitamins
The same minerals as in Example 1 were used, and the same LPP as in Example 2 was used. A commercially available whole milk powder (manufactured by Snow Brand Milk Products) was used.
これら実施例による調製物の組成及びＧＭＰを配合しな
かった参考例の組成を表３に示す、これらの調製物を用
いて官能検査を行った。The compositions of the preparations according to these examples and the composition of the reference example in which GMP was not blended are shown in Table 3. Sensory tests were conducted using these preparations.
調製物を１５％濃度に調乳し、１０名のパネルによる官
能検査を行った０本発明の調製物は表４に示すように、
いずれも従来品に比べ不快味（苦味）がなく飲み易いも
のであった。The preparation of the present invention was prepared to a concentration of 15% and subjected to a sensory test by a panel of 10 people, as shown in Table 4.
All of them had no unpleasant taste (bitter taste) and were easy to drink compared to conventional products.
以下余白
表４
官能検査の結果
不快味は、全くない場合を−とじ、極めて不快味のある
場合を＋十＋とし５段階に分けて評価した。飲み易さは
、非常に飲み易い場合を＋３とし、極めて飲みにくい場
合を−３とし７段階に分けて評価した。総合得点は１は
現行製品と同し、２は現行品よりやや良い、３は現行品
よりかなり良い、４は現行品より極めてよい、５は通常
食品と比べても遜色がないとして評価した。Margin Table 4 Below: Results of the sensory test The unpleasant taste was evaluated on a five-point scale, from - if there was no unpleasant taste at all, to +10+ if there was an extremely unpleasant taste. The ease of drinking was evaluated on a seven-point scale, with +3 being very easy to drink and -3 being extremely difficult to drink. The overall score was 1 as the same as the current product, 2 as slightly better than the current product, 3 as much better than the current product, 4 as extremely better than the current product, and 5 as comparable to regular foods.
Claims (4)
クロペプチドを配合することを特徴とするフェニルケト
ン尿症患者用栄養調製物。(1) A nutritional preparation for phenylketonuria patients, characterized in that κ-casein macropeptide is added to the diet for phenylketonuria patients.
部または全部がκ−カゼイングリコマクロペプチドで置
換されたものであることを特徴とする請求項（１）によ
るフェニルケトン尿症患者用栄養調製物。(2) Nutrition for patients with phenylketonuria according to claim (1), characterized in that part or all of the protein components of the diet for patients with phenylketonuria are substituted with κ-casein glycomacropeptide. Preparation.
κ−カゼインマクロペプチドで置換し、これと低フェニ
ルアラニンペプチド、油脂糖質粉末、アミノ酸、糖質、
ビタミン及びミネラルである請求項（１）または（２）
に記載のフェニルケトン尿症患者用栄養鋼製物。(3) All the protein components of the diet for phenylketonuria patients are replaced with κ-casein macropeptide, and this is combined with low phenylalanine peptide, fat and oil carbohydrate powder, amino acids, carbohydrates,
Claim (1) or (2) which is a vitamin and mineral
A nutritional steel product for patients with phenylketonuria described in .
をκ−カゼイングリコマクロペプチドで置換し、これら
の成分、油脂糖質粉末、アミノ酸、糖質、ビタミン及び
ミネラルである請求項（１）〜（３）のいずれかに記載
のフェニルアラニンケトン尿症患者用栄養調製物。(4) A part of the whole milk powder in the diet for phenylketonuria patients is replaced with κ-casein glycomacropeptide, and these ingredients, oil and fat carbohydrate powder, amino acids, carbohydrates, vitamins and minerals ( The nutritional preparation for phenylalanine ketonuria patients according to any one of 1) to (3).
Priority Applications (1)
| Application Number | Priority Date | Filing Date | Title |
|---|---|---|---|
| JP2248170A JPH04126051A (en) | 1990-09-18 | 1990-09-18 | Nutritional preparation for patient suffering from phenylketonuria |
Applications Claiming Priority (1)
| Application Number | Priority Date | Filing Date | Title |
|---|---|---|---|
| JP2248170A JPH04126051A (en) | 1990-09-18 | 1990-09-18 | Nutritional preparation for patient suffering from phenylketonuria |
Publications (1)
| Publication Number | Publication Date |
|---|---|
| JPH04126051A true JPH04126051A (en) | 1992-04-27 |
Family
ID=17174255
Family Applications (1)
| Application Number | Title | Priority Date | Filing Date |
|---|---|---|---|
| JP2248170A Pending JPH04126051A (en) | 1990-09-18 | 1990-09-18 | Nutritional preparation for patient suffering from phenylketonuria |
Country Status (1)
| Country | Link |
|---|---|
| JP (1) | JPH04126051A (en) |
Cited By (5)
| Publication number | Priority date | Publication date | Assignee | Title |
|---|---|---|---|---|
| WO1994028126A3 (en) * | 1993-05-20 | 1995-01-26 | Univ Warwick | Phenylalanine-free protein and dna coding therefor |
| GB2294464A (en) * | 1993-05-20 | 1996-05-01 | University Of Warwick | Phanylalanine-free protien and dna coding therefor |
| WO1998008402A1 (en) * | 1996-08-30 | 1998-03-05 | Societe Des Produits Nestle S.A. | Nutritional formula for phenylketonuria patients |
| CN105286016A (en) * | 2015-11-10 | 2016-02-03 | 兰州天和生物催化技术有限公司 | Casein hydrolysate peptide food for phenylalanine metabolic disorders |
| JP2019533988A (en) * | 2016-09-01 | 2019-11-28 | メタックス インスティトゥート フュア ディエーテティック ゲーエムベーハーＭｅｔａｘ Ｉｎｓｔｉｔｕｔ Ｆｕｅｒ Ｄｉａｅｔｅｔｉｋ Ｇｍｂｈ | Phenylalanine-free protein for PKU treatment |
-
1990
- 1990-09-18 JP JP2248170A patent/JPH04126051A/en active Pending
Cited By (8)
| Publication number | Priority date | Publication date | Assignee | Title |
|---|---|---|---|---|
| WO1994028126A3 (en) * | 1993-05-20 | 1995-01-26 | Univ Warwick | Phenylalanine-free protein and dna coding therefor |
| GB2294464A (en) * | 1993-05-20 | 1996-05-01 | University Of Warwick | Phanylalanine-free protien and dna coding therefor |
| GB2294464B (en) * | 1993-05-20 | 1998-03-11 | Univ Warwick | Phenylalanine-free protein and DNA coding therefor |
| WO1998008402A1 (en) * | 1996-08-30 | 1998-03-05 | Societe Des Produits Nestle S.A. | Nutritional formula for phenylketonuria patients |
| AU717683B2 (en) * | 1996-08-30 | 2000-03-30 | Societe Des Produits Nestle S.A. | Nutritional formula for phenylketonuria patients |
| US6506422B1 (en) | 1996-08-30 | 2003-01-14 | Nestec S.A. | Nutritional formula for phenylketonuria patients |
| CN105286016A (en) * | 2015-11-10 | 2016-02-03 | 兰州天和生物催化技术有限公司 | Casein hydrolysate peptide food for phenylalanine metabolic disorders |
| JP2019533988A (en) * | 2016-09-01 | 2019-11-28 | メタックス インスティトゥート フュア ディエーテティック ゲーエムベーハーＭｅｔａｘ Ｉｎｓｔｉｔｕｔ Ｆｕｅｒ Ｄｉａｅｔｅｔｉｋ Ｇｍｂｈ | Phenylalanine-free protein for PKU treatment |
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