肾性骨病,Renal Osteodystrophy(ROP),肾性骨病的定义,分为狭义肾性骨病和广义肾性骨病。狭义肾性骨病是指慢性肾衰竭伴发代谢性骨病。广义肾性骨病是指和肾脏相关的疾病。 The ROP has the narrow sense definition and the broad sense definition. The narrow sense definition of ROP is referred to chronic renal failure with metabolism bone diseases. The broad sense definition of ROP is referred to diseases related to the kidney.,肾性骨病的分类：,高转化性骨病High tumover bone disease 低转化性骨病：Low tumover bone disease 1、非动力性骨病；Adynamic bone disease 2、骨软化症；Osteomalacia 混合型骨病 Mix renal osteodystrophy 2-微球蛋白淀粉样变 2-microglobulin amyloidosis,肾性骨病的定义,一：高转化性骨病（囊性纤维性骨炎Osteitis fibrosa） 以甲状旁腺机能亢进，成骨细胞和破骨细胞增殖活跃及骨小梁周围纤维化为特征。 二：低转化型肾性骨病：骨软化指新形成类骨质矿化缺陷，常由铝沉积所致。非动力性骨病指骨形成降低，多与高钙血症，维生素D过度抑制PTH分泌等有关。 三：2-微球蛋白淀粉样变 2-微球蛋白在关节处沉积引起疼痛和骨折。,发病机制 Pathogenesis,高转化性骨病（囊性纤维性骨炎） 1 低钙血症，肾功能减退时，肾脏合成1，25（OH）2D3和排磷能力降低,导致低钙血症，而低钙血症增加PTH的分泌 2 高磷血症，抑制1羟化酶的活性，增加骨骼对的抵抗，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. Hyperphosphatemia, hyperphosphatemia will repress the activity of the 1-hydroxylase , increasing the resistance ability of the bone to the PTH and increase the secretion of the PTH.,3 活性维生素3的变化，肾功能减退时，近端肾小管细胞内磷含量增高抑制线粒体1羟化酶，使1，25（OH）2D3合成减少，PTH基因转录和表达增加。 4 骨骼对PTH的抵抗 5 甲状旁腺自主性增生。 Changes of the active form of Vit D. The impression of the activity of the 1-hydroxylase causes the low synthesis of 1,25( OH)2 D3. The resistance ability of the bone to the PTH The independent hyperplasia of the parathyroid gland,发病机制 Pathogenesis,低转化性骨病 Low tumover bone disease,1 非动力性骨病；机制尚未阐明，多与糖尿病、甲状旁腺切除抑制PTH分泌有关 2 骨软化症；多与1，25（OH）2D3缺乏和铝中毒有关。 The pathogenesis is 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.,2-微球蛋白淀粉样变。正常人每日产生2-微球蛋白150-200毫克，当肾功能衰竭时2-微球蛋白排泄减少在血中蓄积，并沉积于骨、关节及肌腱等处，引起骨的囊性损害，弥漫性脱钙及腕管综合症。,高转化性骨病的临床表现 The clinical performance of high tumover bone disease,肌肉骨骼症状 骨痛和骨折，疼痛部位多见于腰背部、下肢等。表现为深部剧痛。 自发性肌腱撕裂，多发于四头肌、三头肌、跟腱、常发生于行走、下楼梯、和颠倒时 骨骼畸形和生长障碍，常见于小儿尿毒症患者 关节炎和关节周围炎，常表现为类似痛风性关节炎的红、肿、痛 Muscle and skeleton symptoms Bone ache and the bone fracture. Spontaneous tendon pulled. Arthritis and periarthritis. Skeletal deformity and growth retardation.,皮肤瘙痒，常未见皮疹，钙磷在皮肤沉积所致 皮肤溃疡和组织坏死，少见，后发于手指，足趾，股和踝部等 软组织钙化，包括血管、关节周围、内脏、皮下和眼睛等 内脏钙化，常发生于心肌和肺，如广泛的肺钙化引起肺纤维化 Itch of skin: often without rashes. Dermal ulcer and tissue necrosis: seldom happen. Soft tissue calcification Internal organs calcification: often seen in the cardiac muscle and the lung.,高转化性骨病的诊断 The diagnosis of the high tumover bone disease,实验室检查 低钙、高磷、高镁 骨形成的生物学标记物，血清碱性磷酸酶（TAB）总活力下 降 骨吸收的生物学标记物，血清胶原分解产物的酸性磷酸酶升高 血清PTH升高 血浆1，25（OH）2D3水平降低 Lab Examinations: Low level of calcium, high level of phosphours, high level of magnesium; The biological marker of the bones formation: the total vitality in serum alkaline phosphatase ( TAB) goes up; The biological marker of the bones resorption: 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线检查，对肾性骨病的敏感性不高，其特征常为骨吸收、侵蚀和硬化 骨密度的测定 是目前检测ROD可靠的理想的诊断方法 The X-ray checks shows little sensitive often has a character of bone resorption, erosion and sclerosis The measurement of the bone density is a dependable and ideal diagnosis in ROP of current examination methods.,骨活检，是ROD惟一可靠的诊断依据,不仅可作出早期诊断,而且能根据组织学分型进行有针对性的治疗并观察疗效.其特征是骨转化增快，成骨和破骨细胞数量活性增加，骨小梁周围纤维化 Bone biopsy is the only reliable diagnosis prove, which can not only make an earlier diagnosis, but also can give a corresponding treatment according to the histology typing and observe the curative effects.,ROD同位素99m锝骨扫描 为ROD的诊断提供了一个有价值的辅助检查方法6。 The isotope Te-99 bone scans: shows people a new valuable method to diagnosis ROP.,高转化性骨病的治疗 The treatment of the high tumover bone disease,内科治疗 减少磷的储留，可通过限制磷的食入，如低磷饮食限制蛋白和乳类食品。给予磷结合剂和充分透析等方法。血磷常控制在1.451.95mmol/L(4.56ng/dl)水平。常用的磷结合剂有氢氧化铝，碳酸钙和醋酸钙等。而氢氧化铝以液体效果最佳,510/次,片剂23片/次,每日3次,为防止低磷导致软骨病,每2个月查血磷1次。为防止铝中毒,在血磷正常后可改用碳酸钙 补充钙剂 最理想的是碳酸钙。有效剂量为412g/d,分34次服用。治疗过程中应定期监测血钙、磷水平,以防钙磷乘积过高,引起软组织及其他器官的转移性钙化 Medicine treatments: Reduce the deposition of the phosphours. sufficient dialysis. Give some calcium,补充维生素D，有常规口服，口服冲击和静脉注射疗法，如表,外科治疗 Surgical treatments:,甲状旁腺切除的指征： 1、有显著症状的持续性高钙血症 2、顽固性瘙痒，透析和一般治疗无效 3、进行性骨外钙化 4、严重和进行性骨痛和骨折 5、缺血性软组织溃疡和坏死 The indications of the thyroid ablation: 1, Continuously hypercalcemia, with severe symptoms; 2, pruritus, and the dialysis and regular 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、全切除并把一个甲状旁腺移植到前臂 3、全部切除 Method: 1, sub-total excision; 2, total excision and transplant a thyroid gland in the forearm; 3, total excision.,纤维性骨炎与骨软化症状临床表现的区别,骨软化的防治 The prevention and treatments of the bone soften:,减少铝的摄入，服用氢氧化铝不宜超过3克以及净化透析用水 清除组织中铝，常用去