腎性骨病 ppt課件

1、腎性骨病,Renal Osteodystrophy(ROP),腎性骨病的定義,分為狹義腎性骨病和廣義腎性骨病。狹義腎性骨病是指慢性腎衰竭伴發(fā)代謝性骨病。廣義腎性骨病是指和腎臟相關(guān)的疾病。The ROP has the narrow sense definition and the broad sense definition. The narrow sense definition of ROP is referred to chr

2、onic renal failure with metabolism bone diseases. The broad sense definition of ROP is referred to diseases related to the kidney.,腎性骨病的分類：,高轉(zhuǎn)化性骨病High tumover bone disease低轉(zhuǎn)化性骨?。篖ow tumover bone disease 1、非動

3、力性骨??；Adynamic bone disease 2、骨軟化癥；Osteomalacia混合型骨病 Mix renal osteodystrophyβ2-微球蛋白淀粉樣變　β2-microglobulin amyloidosis,腎性骨病的定義,一：高轉(zhuǎn)化性骨?。倚岳w維性骨炎Osteitis fibrosa）　以甲狀旁腺機(jī)能亢進(jìn)，成骨細(xì)胞和破骨細(xì)胞增殖活躍及骨小梁周圍纖維化為特征。二：低轉(zhuǎn)化型腎性骨病

4、：骨軟化指新形成類骨質(zhì)礦化缺陷，常由鋁沉積所致。非動力性骨病指骨形成降低，多與高鈣血癥，維生素D過度抑制PTH分泌等有關(guān)。三：β2-微球蛋白淀粉樣變 β2-微球蛋白在關(guān)節(jié)處沉積引起疼痛和骨折。,發(fā)病機(jī)制Pathogenesis,高轉(zhuǎn)化性骨?。倚岳w維性骨炎）1 低鈣血癥，腎功能減退時，腎臟合成1，25（OH）2D3和排磷能力降低,導(dǎo)致低鈣血癥，而低鈣血癥增加PTH的分泌2 高磷血癥，抑制1ａ羥化酶的活

5、性，增加骨骼對ＰＴＨ的抵抗，刺激PTH的分泌。Hypocalcaemia, with reduced kidney function. The low synthesis of 1,25( OH)2 D3 and hyperphosphatemia will cause hypocalcaemia, and hypocalcaemia increase the secretion of the PTH.Hyperphosphate

6、mia, hyperphosphatemia will repress the activity of the 1-αhydroxylase , increasing the resistance ability of the bone to the PTH and increase the secretion of the PTH.,3 活性維生素Ｄ3的變化，腎功能減退時，近端腎小管細(xì)胞內(nèi)磷含量增高抑制線粒體1ａ羥化酶，使1，25（O

7、H）2D3合成減少，PTH基因轉(zhuǎn)錄和表達(dá)增加。4 骨骼對PTH的抵抗5 甲狀旁腺自主性增生。Changes of the active form of Vit D. The impression of the activity of the 1-αhydroxylase causes the low synthesis of 1,25( OH)2 D3.The resistance ability of the bone to

8、the PTHThe independent hyperplasia of the parathyroid gland,發(fā)病機(jī)制Pathogenesis,低轉(zhuǎn)化性骨病 Low tumover bone disease,1 非動力性骨??；機(jī)制尚未闡明，多與糖尿病、甲狀旁腺切除抑制PTH分泌有關(guān)2 骨軟化癥；多與1，25（OH）2D3缺乏和鋁中毒有關(guān)。 The pathogenesis is

9、still not very clear, but mainly related to the impression of the secretion of the PTH It has something with the deficit of 1,25( OH)2 D3 and the aluminium intoxication.,β2-微球蛋白淀粉樣變。正常人每日產(chǎn)生β2-微球蛋白150-200毫克，當(dāng)腎功能衰竭時β2-微

10、球蛋白排泄減少在血中蓄積，并沉積于骨、關(guān)節(jié)及肌腱等處，引起骨的囊性損害，彌漫性脫鈣及腕管綜合癥。,高轉(zhuǎn)化性骨病的臨床表現(xiàn)The clinical performance of high tumover bone disease,肌肉骨骼癥狀骨痛和骨折，疼痛部位多見于腰背部、下肢等。表現(xiàn)為深部劇痛。自發(fā)性肌腱撕裂，多發(fā)于四頭肌、三頭肌、跟腱、常發(fā)生于行走、下樓梯、和顛倒時骨骼畸形和生長障礙，常見于小兒尿毒癥患者關(guān)節(jié)炎和關(guān)節(jié)周圍炎

11、，常表現(xiàn)為類似痛風(fēng)性關(guān)節(jié)炎的紅、腫、痛Muscle and skeleton symptomsBone ache and the bone fracture.Spontaneous tendon pulled.Arthritis and periarthritis.Skeletal deformity and growth retardation.,皮膚瘙癢，常未見皮疹，鈣磷在皮膚沉積所致皮膚潰瘍和組織壞死，少見，后發(fā)于手指

12、，足趾，股和踝部等軟組織鈣化，包括血管、關(guān)節(jié)周圍、內(nèi)臟、皮下和眼睛等內(nèi)臟鈣化，常發(fā)生于心肌和肺，如廣泛的肺鈣化引起肺纖維化Itch of skin: often without rashes.Dermal ulcer and tissue necrosis: seldom happen.Soft tissue calcificationInternal organs calcification: often seen in

13、the cardiac muscle and the lung.,高轉(zhuǎn)化性骨病的診斷The diagnosis of the high tumover bone disease,實驗室檢查低鈣、高磷、高鎂骨形成的生物學(xué)標(biāo)記物，血清堿性磷酸酶（TAB）總活力下 降骨吸收的生物學(xué)標(biāo)記物，血清膠原分解產(chǎn)物的酸性磷酸酶升高血清PTH升高血漿1，25（OH）2D3水平降低 Lab Examinations: Low level o

14、f calcium, high level of phosphours, high level of magnesium;The biological marker of the bone’s formation: the total vitality in serum alkaline phosphatase ( TAB) goes up;The biological marker of the bone’s resorption

15、: the level of the decomposition product of the serum collagen, ACPase, goes up;The level of the PTH in the serum goes up;The 1,25( OH)2D3 level lowers.,X線檢查，對腎性骨病的敏感性不高，其特征常為骨吸收、侵蝕和硬化骨密度的測定 是目前檢測ROD可靠的理想的診斷方法The X-

16、ray checks shows little sensitive often has a character of bone resorption, erosion and sclerosisThe measurement of the bone density is a dependable and ideal diagnosis in ROP of current examination methods.,骨活檢，是ROD惟一可

17、靠的診斷依據(jù),不僅可作出早期診斷,而且能根據(jù)組織學(xué)分型進(jìn)行有針對性的治療并觀察療效.其特征是骨轉(zhuǎn)化增快，成骨和破骨細(xì)胞數(shù)量活性增加，骨小梁周圍纖維化Bone biopsy is the only reliable diagnosis prove, which can not only make an earlier diagnosis, but also can give a corresponding treatment accor

18、ding to the histology typing and observe the curative effects.,ROD同位素99m锝骨掃描　為ROD的診斷提供了一個有價值的輔助檢查方法[6]。 The isotope Te-99 bone scans: shows people a new valuable method to diagnosis ROP.,高轉(zhuǎn)化性骨病的治療The treatment of the h

19、igh tumover bone disease,內(nèi)科治療減少磷的儲留，可通過限制磷的食入，如低磷飲食限制蛋白和乳類食品。給予磷結(jié)合劑和充分透析等方法。血磷?？刂圃?.45～1.95mmol/L(4.5～6ng/dl)水平。常用的磷結(jié)合劑有氫氧化鋁，碳酸鈣和醋酸鈣等。而氫氧化鋁以液體效果最佳,5～10ｍｌ/次,片劑2～3片/次,每日3次,為防止低磷導(dǎo)致軟骨病,每2個月查血磷1次。為防止鋁中毒,在血磷正常后可改用碳酸鈣補(bǔ)充鈣劑 最理

20、想的是碳酸鈣。有效劑量為4～12g/d,分3～4次服用。治療過程中應(yīng)定期監(jiān)測血鈣、磷水平,以防鈣磷乘積過高,引起軟組織及其他器官的轉(zhuǎn)移性鈣化Medicine treatments:Reduce the deposition of the phosphours.sufficient dialysis.Give some calcium,補(bǔ)充維生素D，有常規(guī)口服，口服沖擊和靜脈注射療法，如表,外科治療Surgical treatm

21、ents:,甲狀旁腺切除的指征：1、有顯著癥狀的持續(xù)性高鈣血癥2、頑固性瘙癢，透析和一般治療無效3、進(jìn)行性骨外鈣化4、嚴(yán)重和進(jìn)行性骨痛和骨折5、缺血性軟組織潰瘍和壞死The indications of the thyroid ablation:1, Continuously hypercalcemia, with severe symptoms;2, pruritus, and the dialysis and reg

22、ular treatments show little effects;3, Progressive ectosteal calcification;4, Severe and progressive bone ache and fracture;5, Ischemic soft tissue ulcer and necrosis.,方法：1、次全切除2、全切除并把一個甲狀旁腺移植到前臂3、全部切除Method:1, s

23、ub-total excision;2, total excision and transplant a thyroid gland in the forearm;3, total excision.,纖維性骨炎與骨軟化癥狀臨床表現(xiàn)的區(qū)別,骨軟化的防治The prevention and treatments of the bone soften:,減少鋁的攝入，服用氫氧化鋁不宜超過3克以及凈化透析用水清除組織中鋁，常用去鐵胺(

24、DFO)，按30毫克/公斤體重溶于5%的葡萄糖溶液250毫升，在透析結(jié)束前30分鐘靜脈滴注，每周1次，療程6-12個月使用高通透性透析器進(jìn)行血透，如丙烯腈透析膜，以及定期進(jìn)行血液濾過(HF)或血液透析(HDF)濾過1, Reduce the intake of the Aluminum 2, Clear the Aluminum in the tissue3, Use high permeability dialyzer to