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特殊營養食品 | 遺傳暨罕見疾病中心
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特殊營養食品
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#
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適應症
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產品名稱
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罕病介紹
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衛教單張
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1
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3-羥基-3-甲基戊二酸血症(3-Hydroxy-3-methylglutaric acidemia)
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I-Valex-2
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(28).png) (29).png) |
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2
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3-羥基-3-甲基戊二酸血症(3-Hydroxy-3-methylglutaric acidemia) (一歲以下)
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LEU1
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3
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3-羥基-3-甲基戊二酸血症(3-Hydroxy-3-methylglutaric acidemia) (一歲以上)
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LEU2 Prima
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IVA ANAMIX JUNIOR
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4
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3-羥基-3-甲基戊二酸血症(3-Hydroxy-3-methylglutaric acidemia)
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IVA ANAMIX INFANT
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5
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三甲基巴豆醯輔酶 A 羧化酵素缺乏症(3- Methylcrotonyl-CoA carboxylase deficiency)
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LEU1
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(30).png) (31).png) |
(13).png) |
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6
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腎上腺腦白質失養症(Adrenoleukodystrophy)
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Lorenzo’s oil
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(32).png) (33).png) (34).png) |
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7
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阿拉吉歐症候群(Alagille Syndrome)
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Alfare
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(35).png) (36).png) (37).png) |
(14).png) |
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8
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胺基酸代謝疾病(Amino acid metabolic disorders)
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Energivit
ESSENTIAL AMINO
ACID MIX POWDER
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Glutamic Acid
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L-CITRULLINE
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L-GLYCINE
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L-ISOLEUCINE
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| L-LEUCINE | ||||
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L-VALINE
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Pro-Phree
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9
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生物素酶缺乏症(Biotinidase Deficiency)
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BIOTIN 5000 MCG
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(38).png) (39).png) (40).png) |
(15).png) |
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10
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瓜胺酸血症(Citrullinemia)
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ESSENTIAL AMINO
ACID MIX POWDER
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(41).png) (42).png) (43).png) |
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PFD 2
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11
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瓜胺酸血症(Citrullinemia)
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Alfare
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12
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先天性全身脂質營養不良症(Congenital generalized lipodystrophy)
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MONOGEN
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(44).png) (45).png) (46).png) |
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13
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先天性尿素循環代謝障礙(Congenital Urea cycle disorders)
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ESSENTIAL AMINO
ACID MIX POWDER
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(47).png) |
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PFD 2
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14
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家族性高乳糜微粒血症(Familial Hyperchylomicronemia)
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MONOGEN
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(48).png) (49).png) |
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15
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家族性高乳糜微粒血症(Familial Hyperchylomicronemia)
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MCT OIL (液體)
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16
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脂肪酸氧化作用缺陷(Fatty acid oxidation defect)
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MONOGEN
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(50).png) (51).png) (52).png) |
(16).png) |
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Pregestimil
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ProViMin
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17
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脂肪酸氧化作用缺陷 (長鏈)(Fatty acid oxidation defect, long chain)
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MCT OIL (液體)
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Portagen
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18
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半乳糖血症(Galactosemia)
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Prosobee
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(53).png) (54).png) (55).png) |
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19
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戊二酸尿症，第一型(Glutaric aciduria type I)
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GA1 ANAMIX INFANT
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(56).png) (57).png) (58).png) (59).png) |
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Glutarex-1
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Glutarex-2
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L-ARGININE
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PFD Toddler
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PFD 2
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20
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戊二酸尿症，第一型(Glutaric aciduria type I) (一歲以上)
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GA1 ANAMIX JUNIOR
XLYS LOW TRY
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Maxamaid
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21
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戊二酸尿症，第二型(Glutaric aciduria type II)
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ProViMin
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22
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肝醣儲積症(Glycogen storage disease)
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Glycosade
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(60).png) (61).png) (62).png) (63).png) (64).png) (65).png) |
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23
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遺傳性高酪胺酸血症(Hereditary tyrosinemia)
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PFD Toddler
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(66).png) (67).png) |
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Tyrex-1
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Tyrex-2
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XPTM TYROSIDON
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24
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遺傳性高酪胺酸血症(Hereditary tyrosinemia) (一歲以下)
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TYR Anamix Infant
TYR1
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25
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遺傳性高酪胺酸血症(Hereditary tyrosinemia) (一歲以上)
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TYR2 SECUNDA
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26
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遺傳性高酪胺酸血症(Hereditary tyrosinemia) (一歲至八歲)
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XPXT MAXAMAID UNFLAV
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27
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遺傳性高酪胺酸血症(Hereditary tyrosinemia) (八歲以上)
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XPHEN TYR MAXAMUM
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28
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高胱胺酸尿症(Homocystinuria)
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Methionine-removed powdered milk
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(68).png) (69).png) (70).png) |
(17).png) |
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PFD Toddler
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PFD 2
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29
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高胱胺酸尿症(Homocystinuria) (一歲以下)
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HCU ANAMIX INFANT HOM1-INFANT
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HCY1
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30
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高胱胺酸尿症(Homocystinuria) (一歲以上)
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HCY2
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HOM2
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XMET Maxamaid
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Hominex-2
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31
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高胱胺酸尿症(Homocystinuria)
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Hominex-1
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32
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高離胺酸血症(Hyperlysinemia) (一歲以下)
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LYS1
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(71).png) (72).png) |
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33
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高離胺酸血症(Hyperlysinemia) (一歲以上)
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LYS2 PRIMA
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34
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高甲硫胺酸血症(Hypermethioninemia)
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Methionine-removed powdered milk
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(73).png) (74).png) |
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35
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高甲硫胺酸血症(Hypermethioninemia) (一歲以下)
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Hominex-1
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HCU ANAMIX INFANT
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SOD Anamix Infant
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36
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高甲硫胺酸血症(Hypermethioninemia) (一歲以上)
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XMET Maxamaid
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37
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先天性膽酸合成障礙(Inborn errors of bile acid synthesis)
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Alfare
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(75).png) (76).png) |
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Generaid Plus
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Medium Chain Triglyceride Portagen
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Pregestimil
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38
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異戊酸血症(Isovaleric acidemia)
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LEU1
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(77).png) (78).png) (79).png) |
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| 39 | 異戊酸血症(Isovaleric acidemia)(一歲至十歲) | IVA ANAMIX JUNIOR | ||
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40
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異戊酸血症(Isovaleric acidemia)
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L-GLYCINE
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PFD Toddler
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41
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異戊酸血症(Isovaleric acidemia)
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I-Valex-2
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42
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異戊酸血症(Isovaleric acidemia)
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I-Valex-1
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43
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Leigh 氏童年期腦脊髓病變(Leigh disease)
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L-ARGININE
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(80).png) |
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44
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楓糖尿症(Maple syrup urine disease)
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BCAD 1
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(81).png) (82).png) (83).png) |
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BCAD 2
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PFD Toddler
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PFD 2
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Ketonex-1
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S-20
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45
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楓糖尿症(Maple syrup urine disease) (一歲以下)
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MSUD 1
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MSUD Anamix Infant
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46
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楓糖尿症(Maple syrup urine disease) (一歲以上)
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Ketonex-2
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MSUD 2
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MSUD Maxamaid
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47
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MELAS 症候群(MELAS)
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L-ARGININE
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(84).png) |
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48
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甲基丙二酸血症(Methylmalonic acidemia)
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MMA/PA Anmix Junior
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(85).png) (86).png) (87).png) |
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PFD Toddler
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PFD 2
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OA1
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OA2
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49
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甲基丙二酸血症(Methylmalonic acidemia) (一歲以下)
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Milupa OS1
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50
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甲基丙二酸血症(Methylmalonic acidemia) (一歲以上)
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OS2 Secunda XMTVI Maxamaid
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Propimex-2
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51
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甲基丙二酸血症(Methylmalonic acidemia)
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MMA/PA Anamix Infant
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Propimex-1
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52
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甲基丙二酸血症之特殊輔助治療(Methylmalonic acidemia-special nutritional supplements)
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L-ISOLEUCINE
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53
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甲基丙二酸血症之特殊輔助治療(Methylmalonic acidemia-special nutritional supplements)
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L-VALINE
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54
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粒線體缺陷(Mitochondrial defect)
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L-ARGININE
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(88).png) (89).png) |
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55
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多發性羧化酶缺乏症(Multiple carboxylase deficiency)
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BIOTIN 5000 MCG
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(90).png) (91).png) |
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56
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非酮性高甘胺酸血症(Nonketotic hyperglycinemia)
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PFD Toddler
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(92).png) (93).png) (94).png) |
(18).png) |
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PFD 2
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57
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有機酸血症(白胺酸代謝異常引起之有機酸血症) (Organic acidemias (Organic acidemias associated with leucine metabolism)
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LEU1
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(95).png) |
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58
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典型苯酮尿症合併蔗糖酶同麥芽糖酶缺乏症(PAH type PKU combine with Sucrase-isomaltase deficiency)
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Calogen
Fructose Module Phlexy-Vits
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PK AID-4
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59
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苯酮尿症(Phenylketonuria)
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Phenyl-Free 1
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(96).png) (97).png) (98).png) |
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Phenyl-Free 2
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PK AID-4
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60
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苯酮尿症(Phenylketonuria) (一歲以下)
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Phenex-1
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PKU 1 Mix
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61
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苯酮尿症(Phenylketonuria) (一歲以上)
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Phenex-2
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Phenylalanine-removed powdered milk
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PKU 2
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PKU 3
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62
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苯酮尿症之特殊輔助治療(Phenylketonuria-special nutritional supplements)
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PKU Lophlex Powder(柳橙及莓果口味)
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63
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進行性家族性肝內膽汁滯留症(Progressive Familial intrahepatic cholestasis, PFIC)
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Alfare
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(99).png) (100).png) (101).png) |
(19).png) |
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Generaid Plus
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Medium Chain Triglyceride Portagen
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Pregestimil
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64
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丙酸血症(Propionic acidemia)
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OA1
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(102).png) (103).png) |
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OA2
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PFD Toddler
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PFD 2
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MMA/PA Anamix Junior
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65
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丙酸血症(Propionic acidemia) (一歲以下)
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Milupa OS1
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66
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丙酸血症(Propionic acidemia) (一歲以上)
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OS2 Secunda
XMTVI Maxamaid
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Propimex-2
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67
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丙酸血症(Propionic acidemia)
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MMA/PA ANAMIX INFANT
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Propimex-1
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68
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丙酮酸鹽脫氫酶缺乏症(Pyruvate dehydrogenase deficiency)
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RCF
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(105).png) (106).png) |
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69
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亞硫酸鹽氧化酶缺乏症(Sulfite oxidase deficiency)
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Xmet XCys Maxamaid
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(107).png) (108).png) |
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70
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髮-肝-腸症候群(Tricho-hepato-enteric syndrome)
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Neocate Junior
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(109).png) (110).png) |
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Neocate LCP
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71
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尿素循環代謝異常(Urea cycle disorders)
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L-ARGININE
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(111).png) (112).png) |
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PFD Toddler
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WND1
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WND2
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72
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尿素循環代謝異常(Urea cycle disorders) (一歲以下)
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UCD1
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73
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尿素循環代謝異常(Urea cycle disorders)
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Cyclinex-1
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74
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尿素循環代謝異常(Urea cycle disorders) (一歲以上)
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Cyclinex-2
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UCD2 SECUNDA
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75
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Wiskott-Aldrich 氏症候群(Wiskott-Aldrich syndrome)
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Neocate Junior
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(113).png) (114).png) (115).png) |
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Neocate LCP
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