泡泡龍症怎麼治?Part-II
泡泡天使劉佩菁:眼睛不好對焦、口腔纖維化
對焦裝置→水晶體
維生素C是水晶體運作時不可或缺的營養素。
維他命C主要的功能,乃是負責將細胞與細胞之間連結起來(聯結組織)。
泡泡龍症怎麼治?Part-III(也許缺鋅,含鋅的基本食物:豆漿)
MIP / AQP0基因的缺失導致缺乏維持晶狀體纖維結構所需的縫合線形成,
導致干擾晶狀體的調節適應性和聚焦特性。
AQP0受鋅的強烈調節
泡泡龍症怎麼治?Part-IV(另一種狀況是缺乏維它命A。)
罕見遺傳性疾病表皮分解性水皰症的患者的皮膚會生水皰,即便輕碰也會脫落;
男童罹患的接合型肇因於LAMB3基因突變,導致表皮無法正確固定在真皮上。
最近的數據表明,維生素A缺乏的上皮細胞粘附層粘連蛋白的能力降低,
顯示層粘連蛋白結合蛋白的表達降低。
Blood vitamin and trace metal levels in epidermolysis bullosa.
表皮溶解水皰症中的血液維生素和微量金屬水平。
epidermolysis bullosa (EB)
表皮溶解水皰症(EB)
Notable abnormalities included low levels of plasma iron
and zinc(in junctional EB and recessive dystrophic EB),
vitamin C, vitamin A (primarily in EB simplex), vitamin A (in junctional
and recessive dystrophic EB), vitamin B12 (primarily in EB simplex),
and vitamin B6 (especially in recessive dystrophic EB).
顯著異常包括血漿鐵和鋅水平低(在交界性EB和隱性營養不良性EB中),
維生素C(主要在EB單純性的EB中),維生素A(交界性和隱性營養不良性EB),
維生素B12(主要在EB單純性的EB中)和維生素B6(尤其是隱性營養不良性EB)。
Nutritional aspects of children and adolescents with epidermolysis bullosa: literature review
兒童和青少年表皮溶解水皰症的營養方面:文獻綜述
The authors also observed reduced concentrations of insulin-like growth factor-1 (IGF-1) and IGF-binding protein-3 (IGFBP3), which are proteins produced and secreted by the pituitary gland to induce the increase of cells volume and numbers, enabling tissue,organ and consequently, body growth.
作者還觀察到了胰島素樣生長因子1(IGF-1)和胰島素樣生長因子結合蛋白3(IGFBP3)的濃度降低,這些蛋白是垂體產生和分泌的蛋白,可誘導細胞體積和數量的增加, 使組織,器官和身體得以生長。
Oral zinc supplementation in the experimental group (1) stimulated an increase
in the consumption of protein and fat (p = 0.0007, p < 0.0001, p < 0.0001, respectively),
(2) increased basal serum zinc (p < 0.0001), (3) increased plasma ALP(alkaline phosphatase)
(p = 0.0270), and (4) showed a positive correlation for IGF1( insulin-like growth factor 1),
IGFBP3(insulin-like growth factor binding protein 3), and OCN(osteocalcin),
comparing before and after oral zinc supplementation
(p = 0.0011, p < 0.0001, p < 0.0446, respectively).
實驗組補充口服鋅
(1)刺激蛋白質和脂肪的消耗量增加(分別為p = 0.0007,p <0.0001,p <0.0001),
(2)基礎血清鋅增加(p <0.0001),
(3)血漿ALP(鹼性磷酸酶)增加(p = 0.0270),並且
(4)與IGF1(胰島素樣生長因子1),IGFBP3(胰島素樣生長因子結合蛋白3)和OCN(骨鈣素)呈正相關 )
,比較口服補鋅前後(分別為p = 0.0011,p <0.0001,p <0.0446)。
Plasma GH(growth hormone) levels increased during intravenous zinc administration and IGF1 (insulin-like growth factor 1)and IGFBP3(insulin-like growth factor-binding protein 3)increased after oral zinc supplementation.
靜脈給予鋅後血漿GH水平升高,口服鋅補充後IGF1和IGFBP3升高。
Nutritional and metabolic profile of children with epidermolysis bullosa.
表皮溶解水皰症兒童的營養和代謝狀況。
Nutrient deficiencies were reported for zinc, magnesium, calcium, potassium,
and iron; vitamines A, D, E, B1, B12, and B6; protein, and calories.
據報導鋅,鎂,鈣,鉀和鐵營養不足。 維生素A,D,E,B1,B12和B6; 蛋白質和卡路里。
Comparison of laboratory values revealed significantly lower values
for hemoglobin, hematocrit, and zinc.
實驗室值的比較顯示血紅蛋白,血細胞比容和鋅的值明顯較低。
總結以上:
由上方看來,泡泡龍症患者主要應該缺鋅、維它命A,可能也缺維它命C。