肾性骨病.ppt

单击此处编辑母版标题样式,单击此处编辑母版文本样式,第二级,第三级,第四级,第五级,*,单击此处编辑母版标题样式,单击此处编辑母版文本样式,第二级,第三级,第四级,第五级,*,肾性骨病,Renal Osteodystrophy(ROP),1,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.,2,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,3,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。,4,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.,5,3,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,6,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.,7,2-,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。,8,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.,9,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.,10,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 bone,s formation: the total vitality in serum alkaline phosphatase ( TAB) goes up;,The biological marker of the bone,s 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.,11,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.,12,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.,13,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.,14,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,1.45,1.95mmol/L(4.5,6ng/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,5,10,/,次,片剂,2,3,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,4,12g/d,分,3,4,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,15,补充维生素,D,，有常规口服，口服冲击和静脉注射疗法，如表,甲旁亢,IPTH,（,PG/ML,）,正常值上限的倍数,治疗选择,极轻度,小于,400,2-3,倍,不用,轻,-,中度,400-600,3-5,倍,常规口服疗法（,0.25-0.5 ug,Qd,）,中,-,重度,600-1200,6-10,倍,口服冲击,(2-4 ug,Biw),或静脉注射,极重度,大于,1200,大于,10,倍,局部注射或手术切除,16,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.,17,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.,18,纤维性骨炎与骨软化症状临床表现的区别,表现,纤维性骨炎,骨软化症,骨折,可发生,常见,近端肌肉无力,可发生,常见,关节周围炎,常发生,少见,肌腱撕裂,常发生,少见,骨外钙化,常见,可发生,血钙,可低可高常,2.5mmol/l,可低可高，常,2.5 mmol/l,血磷,常,2.1 mmol/l,常,3-4,倍的正常值,15-20,倍的正常值,4-5,倍的正常值,40-60,倍的正常值,血铝,常,5. umol/l,多数高，常,3.7 umol/l,注射去铁胺,(DFO),后血铝增高,多数,13 umol/l,多数,6.7 umol/l,罕有,3. umol/l,小细胞性贫血,罕见,流行性者多见，散发者少见,19,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，常用去铁胺,(DFO),，按,30,毫克,/,公斤体重溶于,5%,的葡萄糖溶液,250,毫升，在透析结束前,30,分钟静脉滴注，每周,1,次，疗程,6-12,个月,使用高通透性透析器进行血透，如丙烯腈透析膜，以及定期进行血液滤过,(HF),或血液透析,(HDF),滤过,1, Reduce the intake of the Aluminum,2, Clear the Aluminum in the tissue,3, Use high permeability dialyzer to dialysis, and perform HF and HDF regularly,20,2-,微球蛋白淀粉样变的治疗 选用高分子合成膜，进行血液滤过,(HF),和血液透析滤过，以增加,2-M,的清除，减少,2-M,的产生,21,谢谢,!,22,