石骨症又稱大理石骨、原發性脆性骨硬化、硬化性增生性骨病和粉筆樣骨。
是一種少見的骨發育障礙性疾病。
本病之特徵為鈣化的軟骨持久存在,引起廣泛的骨質硬化,重者事關大局髓腔封閉,造成嚴重貧血。
石骨症(英文:Osteopetrosis)係一種骨代謝病,症狀的骨又硬又脆。
石骨症
顯微鏡下見破骨細胞異常,失去不規則邊緣,說明不活躍。
僵直性脊椎炎,怎麼治?Part-III(缺鋅硒維它命C,鎂錳應該也有幫助,建議善存+豆漿+茶)
告訴我們:
IFN-γ刺激破骨細胞形成
IL-2刺激破骨細胞活性
能提高IL-2的元素:
鋅、銅、硒、維生素A、維生素C、大豆異黃酮、蜂膠黃酮、兒茶素、花青素、茄紅素
能提高IFN-γ的元素:
鋅、硒、錳、維生素C、高劑量維生素E、茄紅素、大豆異黃酮
鎂錳在含量高時也能提高IFN-γ,不過鎂錳都能降低IL-2。
Osteopetrosis
石骨症
Severe - autosomal recessive inheritance pattern (ARO – AutosomalRecessive Osteopetrosis):
嚴重-常染色體隱性遺傳模式(ARO-常染色體隱性石骨症):
ARO is caused by mutations inTCIRG1, CLCN7, and less frequently SNX10, OSTM1, RANK and RANKL.
ARO是由TCIRG1,CLCN7中的突變引起的,而較少發生於SNX10,OSTM1,RANK和RANKL。
Intermediate - dominant or recessive inheritance: This group of OP ischaracterised
by an intermediate, but still severe, course.
中級-顯性或隱性遺傳:這組OP的特徵是中級但仍然很嚴重。
An intermediate recessive form associated with braincalcifications and renal
tubular acidosis is due to mutations in the carbonicanhydrase enzyme (CAII) gene.
與腦鈣化和腎小管酸中毒相關的一種中間隱性形式是由於碳酸酐酶(CAII)基因的突變。
Carbonic anhydrase-II (CA-II) is a zinc metallo-enzyme that catalyzes
the reversible hydration of carbon dioxide to form carbonic acid (H 2 CO 3 ).
碳酸酐酶-II(CA-II)是一種鋅金屬酶,其催化二氧化碳的可逆水合以形成碳酸(H 2 CO 3)。
CA-II has a wide tissue distribution, being found in bone, kidney (proximal tubules and collecting duct), erythrocytes and glial cells, with osteoclasts being particularly rich in CA-II. Deficiency of CA-II impairs the production of H + by the osteoclasts and,thus, bone resorption is blocked, leading to the development of osteopetrosis.
CA-II具有廣泛的組織分佈,存在於骨,腎(近端小管和集合管),紅細胞和神經膠質細胞中,
破骨細胞特別富含CA-II。 CA-II缺乏會破壞破骨細胞產生H+,因此骨吸收被阻斷,
導致骨硬化病的發展。
In common with osteopetrosis due to CA-II deficiency
最常見的骨硬化症是由於CA-II缺乏
Congenital osteopetrosis is characterized by severe impairment of bone resorption.
先天性石骨症的特徵是骨骼吸收嚴重受損。
parathyroid hormone (PTH)
甲狀旁腺激素(PTH)
The response was most marked at the highest PTH dose level.
高劑量甲狀旁腺激素(PTH)的明顯反應
甲狀旁腺功能亢進症,怎麼治?(缺鋅、維它命D)
告訴我們:
甲狀旁腺激素(PTH)的產生增加
血清PTH和鋅水平之間存在顯著的負相關
總結以上:
由以上看來石骨症缺鋅(不知還有沒有缺少其它營養)