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系膜毛细血管性肾小球肾炎
全网发布： 21:49
系膜毛细血管性肾小球肾炎是一类具有以下组织学特征的原发性肾小球疾病：肾小球基底膜弥漫性增厚伴内皮下或基底膜内沉积物；系膜细胞增生，系膜基质增多，插入到内皮下而呈现“双轨”征；肾小球外观呈分叶状。临床表现为综合征、血尿，常有、肾功能损害及血清补体降低。本病的其他病名有分叶性肾炎、慢性低补体性肾炎等。
系膜毛细血管性肾小球肾炎的基本病变使肾小球基膜增厚伴沉积物，系膜增生伴插入。根据电子致密物的沉积部位，系膜毛细血管性肾小球肾炎可以分为两型，B型65%~80%，其余为C型。
B型系膜毛细血管性肾小球肾炎： 光镜与电镜：肾小球基底膜弥漫性增厚，成“双轨”征。系膜细胞增生、系膜基质增多、系膜区增宽，使肾小球呈分叶状。“双轨”征的形成是由于高度增生的系膜组织（细胞、基质）插入系膜与内皮细胞之间，而不是基膜分裂。系膜区及内皮下有沉积物，在电子显微镜下为电子致密物，内皮细胞轻度增生，肾小球常有中性粒细胞、单核细胞浸润，约10%的病例浸润明显。少数病例的典型病变仅见于肾小球的部分节段。小管间质病变可见于疾病早期，严重与否与肾小球硬化的程度相吻合，间质纤维化和肾小球硬化均很明显时，临床上往往有进行性肾功能损害。免疫荧光检查：C3沿肾小球基膜和系膜区呈弥漫性颗粒状沉积，约2/3病例伴IgG、IgM沉积。常常有纤维连接蛋白阳性。1/3的病例早期有补体成分沉积。部分病例沉积物仅见于基膜，系膜区阴性。
C型系膜毛细血管性肾小球肾炎： 光镜与电镜：光镜下肾小球的改变与B型相似，基膜弥漫性增厚，呈明亮的嗜伊红色，折光性强，呈缎带状。B型的各种改变均可以见到，唯系膜细胞及内皮细胞增生不及B型明显。中性粒细胞浸润及新月体形成比B型突出，可以有血管袢坏死，纤维素沉积。上皮下“驼峰”状沉积物较B型多见。电镜下见基膜内大量、大块、均质的电子致密物呈条带状沉积，分布可呈节段性、不连续或弥漫性，故本病又称为“致密物沉着病”。系膜区、肾小球囊、肾小管基膜、小血管壁均可见电子致密物。上皮细胞足突融合，系膜插入现象常见，但程度不及B型严重。免疫荧光检查：C3沉积为主，沿肾小球基膜呈光滑、不连续的线状沉积，或颗粒状沉积。高分辨率的显微镜下可以见到C3沿正常基膜致密层内外缘分布，包围膜内阴性的电子致密物。常有系膜区颗粒状、结节状、团块状沉积，或环状分布。C3也呈线状分布于肾小球囊、肾小管基膜和小血管壁。不到一半的病例有C4 、C1q沉积，其阳性强度较弱。可见C5b-9沉积于毛细血管襻。IgG、IgA很少阳性，IgM见于约50%的病例。
其它类型的系膜毛细血管性肾小球肾炎：III型系膜毛细血管性肾小球肾炎具有B型系膜毛细血管性肾小球肾炎的特点，同时有明显的上皮下沉积物，有“钉突”形成，类似与膜性肾病的病理改变。基膜致密层断裂、分层。现趋向于将此型归入B型系膜毛细血管性肾小球肾炎。因为B、C型均有上皮下沉积物，而且重复活检时上皮下沉积物的情形和数量会有变化。具有上皮下沉积物的病例在病理、临床表现和临床经过等方面并没有独到之处。
临床表现多种多样。病前可有前驱感染，特别是儿童，如，15%有链球菌感染病史，两型系膜毛细血管性肾小球肾炎的临床表现没有明显差别。50-60%的病理表现为肾病综合征，常常伴有镜下血尿，少数有肉眼血尿；急性肾炎综合征见于15-20%的病例，而且更常见于C型；其余表现为无症状性血尿和/或蛋白尿。起病时30%的病例有轻度高血压，20%出现肾功能损害。上述各组症状可同时存在。
实验室检查显示非选择性蛋白尿。蛋白尿程度不一，轻度至肾病范围的蛋白尿均都可能出现，常有各种管型。尿红细胞为多形性（肾小球源性），有红细胞管型。持续性低补体血症是本病的特征，系膜毛细血管性肾小球肾炎是唯一具有这一现象的原发性肾小球疾病。
本病虽然疗效欠佳，但根据其临床表现及病理学改变早期采用联合治疗，可一定程度上改善或稳定肾功能，提高生存率。在治疗上应积极控制高血压，血肌酐在小于265μmol/L（3mg/dl）时适当应用血管转换酶抑制剂及/或长效钙离子拮抗剂，尚能延缓肾功能减退。尽管激素和/或环磷酰胺冲击治疗大多无效，且副作用大，较少选用。但多主张长期隔日泼尼松、环磷酰胺及抗凝联合治疗，激素治疗早晚影响预后，发病后一年内治疗反映良好，而五年后治疗者大多无效。抗凝治疗常选用尿激酶及肝素静脉滴注（用法与膜性肾病相似），有时起到改善肾小球滤过功能的作用。
临床过程虽有差异，但本病预后差，长期随访中，自然缓解率&50%，平均存活率5年65%~85%，10年50%~55%，15年30%~40%。预后与下列因素有关：①年龄：儿童较成年人发展缓慢；②临床表现：病初有肾功能不全、早期有高血压、肉眼血尿、持续性肾病综合征者预后差；③类型：III型较B型佳，C型最差，C型肾移植中，1~3年内几乎全部复发；④病理中若有新月体形成大于30%，多在4年内死亡。处于膜增殖炎症和增殖硬化期，肾功能损害均发生早且重。
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肾小球疾病（各种肾炎、肾病综合征），急慢性肾功能衰竭，肾小管间质性肾炎，糖尿病肾病，高血压性肾损害，尿路感染，多囊肾，肾结石及各种中医肾病。适用课程:&人体生理学（一）(),人体生理学（二）(),人体生理学(),人体生理学(),生理学(),生理学（一）(),生理学（二）(),生理学（三）(),生理学(),生理学（二）(),解剖生理学(),生理学（一）(),生理学（二）(),生理学（三）(),生理学（四）(),解剖生理学(),生理学(),生理学(),生理学(),生理学（四）(BIOT1012),生理学（一）(CLMB1004),生理学（三）(CLMD3012),生理学（二）(MEIM1002),解剖生理学(PHAR1007)【访问量：909174】
苏州大学医学部生理学系
2005 (87)Physiology (7 8 year length of schooling), edited by Yaotai, 1st edition, published by People`s Medical Publishing House, 2005.
TEXTBOOK OF PHYSIOLOGY(), edited by Yan Jianqun & Wu Bowei, published by Science Publishing Company, 2006.
清除率及尿液的排放clearance and urine excretion
【教学目的】
【教学重点】
【教学难点】
【教学方法】 Discussed Manner.
【教具准备】
【授课内容】
Outline of this chapter
1 Functional anatomy of the kidney and renal blood circulation
1.1 Functional anatomy of the kidney
1.2 Renal blood circulation and its regulation
2 Glomerular filtration
2.1 Effective filtration pressure
2.2 Factors affecting glomerular filtration
3 Transportation function in the renal tubule and collecting duct
3.1 Transportation mode in the renal tubule and collecting duct
3.2 Reabsorption and secretion in the renal tubule and collecting duct
4 Urinary concentration and dilution
4.1 Urinary dilution
4.2 Urinary concentration
4.3 Role of the recta for maintaining the high solute concentration in the medullary interstitial fluid
5 Regulation of urine formation
5.1 Autoregulation in kidney
5.2 Nervous regulation and humoral regulation
6 Plasma clearance
6.1 Concept of plasma clearance its calculating method
6.2 Physiological significance of plasma clearance test
7 Micturition
7.1 Innervation of the urinary bladder and urethra
7.2 Micturition abnormity
(functional anatomy and blood flow of kidney )
(Functional anatomy of kidney)
span style='mso-ignore:;z-index:4;margin-left:0margin-top:0width:514height:318px'
span style='mso-ignore:;z-index:3;margin-left:0margin-top:0width:499height:296px'
juxtaglomerular apparatus
(characteristics and regulation of renal blood flow)
1.(characteristics of renal blood flow)
(autoregulation of renal blood flow and GFR)
2.(regulation of renal blood flow)
(Glomerular filtration)
——(filtration membrane)
(construction of filtration membrane)
(permeability of filtration membrane)
—(effective filtration pressure)
(ultrafiltration fluid)
(factors influencing the process of glomerular filtration)
(change of effective filtration pressure)
(change of renal blood flow)
(change of filtration membrane)
一、(material transport passway of renal tubule and collecting duct)
reabsorption. Tubular reabsorption denotes the transport of substances from the tubular fluid through the tubular epithelium into peritubular capillary bloodSecretion is the process by which substances move from outside to inside the tubule.(tubular fluid)
span style='mso-ignore:;z-index:2;margin-left:0margin-top:0width:484height:288px'
1.Na+Cl-(reabsorption of Na+Cl-and water)10 min
1(proximal convoluted tubule)
Na+(reabsorption of Na+ in the first part of proximal convoluted tubule)
NaCl(reabsorption of NaCl in the last part of proximal convoluted tubule)
(reabsorption of water of proximal convoluted tubule)
2(loop of Henle)
3(distal convoluted tubule and collecting duct)
2.HCO3H+(reabsorption of HCO3and secretion of H+) 10 min
1(proximal convoluted tubule)Process:Characteristics
2(loop of Henle)
3(distal convoluted tubule and collecting duct)
3.NH3H+HCO3(relationship between secretion of NH3 and transport of H+HCO310 min
4.K+(reabsorption and secretion of K+)
K+(reabsortpion of K+)
K+(secretion of K+)
5.(reabsorption and secretion of Ca2+)
1(proximal convoluted tubule)
2(loop of Henle)
3(distal convoluted tubule and collecting duct)
6.(reabsorption of glucose)
Urinary Concentration and Urinary Dilution40min
Concentrated and Diluted Urine by Kidneys
(Formation of the Osmotic Gradient in Medulla of Kidneys)
(Function of Vasa Recta in Keeping the High Osmotic Pressure of Renal Medulla)
span style='mso-ignore:;z-index:1;margin-left:0margin-top:0width:496height:318px'
(Regulation of Urine Formation)
(Autoregulation in kidneys)
(The Concentration of Solvent have effects on Renal Regulation )
(Glomerulotubular Balance)
(Nervous and Humoral Regulation)
(Functions of Renal Sympathetic Nerve)
vasopressinVPantidiuretic hormoneADH.A product of neurohypophysis which, through its action on kidneys, promotes the conservation of body water.
3.--(Renin-Angiotensin System)
(Clearance)
(Definition and calculation of Clearance)
UxGFRPxUxGFR=Ux
UxRPF PxUxRPF=Ux
(micturition)
(Innervation of the urinary bladder and urethra)
(micturition reflex)
(abnormal micturition)
The urinary system is composed of the kidneys, bladder and accessory structures. The kidneys produce urine, a fluid waste product whose composition and volume vary.
The six functions of the kidneys are regulation of extracellular fluid volume, regulation of osmolarity, maintenance of ion balance, homeostatic regulation of pH, excretion of wastes and foreign substances, and production of hormone. The most important function of the kidneys is the homeostatic regulation of the water and ion content of the blood.
I. Structure of the kidneys
Each kidney has about 1 million nephrons. Each nephron in the kidneys consists of a renal corpuscle and a tubule.
1. Each renal corpuscle comprises a capillary tuft, termed a glomerulus, and a Bowman's capsule, into which the tuft protrudes.
2. The tubule extends out from Bowman's capsule and is subdivided into many segments, which can be combined for reference purposes into the proximal tubule, loop of Henle, distal convoluted tubule and collecting duct. Beginning at the level of the collecting ducts, multiple tubules join and empty into the renal pelvis, from which urine flows through the ureters to the bladder.
3. Each glomerulus is supplied by an afferent arteriole,and an efferent arteriole leaves the glomerulus to branch into peritubular capillaries, which supply the tubule.
II. Basic Renal processes
1.The three basic renal processes are glomerular filtration, tubular reabsorption, and tubular secretion. In addition, the kidneys synthesize and /or catabolize certain substances. The excretion of a substance is equal to the amount filtered plus the amount secteted minus the amount reabsorbed.
2. Urine formation begins with glomerular filtration - approximately 180L/day - of essentially protein-free plasma into Bowman's space.
(1) Glomerular filtrate contains all plasma substances other than proteins and substances bound to protein.
(2) Glomerular filtration is driven by the hydrostatic pressure in the glomerular capillaries and is opposed by both the hydrostatic pressure in Bowman's space and the osmotic force due to the proteins in the glomerular capillary plasma.
3. As the filtrate moves through the tubules, certain substances are reabsorbed into the peritubular capillaries.
(1) Substances to which the tubular epithelium is permeable are absorbed by diffusion because water reabsorption creates tubule-interstitium concentration gradients for them.
(2) Tubular reabsorption rates are generally very high for nutrients, ions,and water, but are lower for waste products. Reabsorption may occur by diffusion or by mediated transport.
(3) Many of the mediated-transport systems manifest transport maximums, so that when the filtered load of a substance exceeds the transport maximum, large amounts may appear in the urine.
4. Tubular secretion (movement from the peritubular capillary into the tubules), like glomerular filtration, is a pathway for entrance of a substance into the tubule.
Ⅲ. Renal regulation
Renal function is regulated by neural and hormonal influences. The most important of these are:
1. renal sympathic nerves
2. renin-angiotensin system
3. aldosterone
4. atrial natriuretic peptide
5. antidiuretic hormone
6. prostaglandins
7. parathyroid hormone
Ⅳ. Clearance
Clearance is an abstract concept that describes what volume of plasma passing through the kidneys has been totally cleared of a substance in a given period of time. For substances such as inulin which are neither actively absorbed nor secreted by the kidneys, clearance is equivalent to the glomerular filtration rate (GFR). In clinical settings, creatinine is used to measure GFR.
If a person's GFR is known, then it is possible to measure the filtration rate of a substance.
If less substance appears in the urine than was filtered, then some was reabsorbed by the nephrons. If more substance appears in the urine than was filtered, then there is net secretion of the substance. If the same amount of the substance is filtered and excreted, then the substance is neither reabsorbed nor secreted.
Clearance values are also used to determine how the nephron handles a substance filtered into it. If the clearance of a substance is less than the inulin or creatinine clearances, then the substance has been reabsorbed. Conversely, if the clearance rate of the substance is greater than inulin or creatinine then it has been actively secreted into the nephron.
V. Micturition
Urine is stored in the bladder until released by urination, also known as micturition.
1. In the basic micturition reflex, bladder distention stimulates stretch receptors that tri these reflexes lead to contraction of the detrusor muscle, mediated by parasympathetic neurons, and relaxation of the external urethral sphincter, mediated by inhibition of the motor neurons to this muscle.
2. Voluntary control is exerted via descending pathways to the parasympathetic nerves supplying the detrusor muscle and the motor nerves supplying the external urethral sphincter.
Berne RM, Levy MN, Koeppen BM, Stanton BA. Physiology, 5th ed, St Louis: Mosby, 2004.
Guyton AC, Hall JE. TEXTBOOK OF MEDICAL PHYSIOLOGY, 10th ed, Philadelphia: W.B. Saunders Co, 2000.
Charles Seidel. BASIC CONCEPTS IN PHYSIOLOGY: a student’s survival guide (Great for Course Prep and USMLE), Houston: McGraw-Hill Co Inc, 2002.
Koeppen BM, Stanton BA. Renal physiology, 3rd ed, Health Scicece Asia: Elsevier Science, 2002.
Question Thinking
【导航网站】Navigation for Web Address　　弥漫性毛细血管内增生性肾小球肾炎（diffuse endocapillary proliferative glomerulonephritis）病变为弥漫性，两侧肾几乎全部肾小球皆受累。这种肾炎常发生于感染以后，故有感染后肾小球肾炎之称。最常见的为A组乙型溶血性链球菌感染，其中以12型、4型、1型及49型与肾炎的关系最为密切。一般发生在链球菌感染后1～3周，是链球菌感染引起的变态反应性疾病，称为链球菌感染后肾炎。除链球菌外，其他如葡萄球菌、肺炎球菌和某些病毒及寄生虫等也可引起这种类型的肾炎。这种肾炎多见于儿童，成人也可发生，但病变往往比儿童严重。一般发病较急，临床主要表现为急性肾炎综合征。
　　病理变化
　　病变为弥漫性，两侧肾同时受累。病变进展较快，主要变化为肾小球内细胞增生。早期，肾小球毛细血管充血，内皮细胞和系膜细胞肿胀增生并有少量中性粒细胞浸润。毛细血管通透性增加，血浆蛋白质可以滤过而进入肾球囊。因此，病人的尿液中常有蛋白、红细胞及白细胞。轻型病人，病变可不再发展，以后逐渐痊愈；比较严重的病例，病变继续发展，肾小球内细胞增生加重。增生的细胞主要为系膜细胞和内皮细胞。增生细胞压迫毛细血管，使毛细血管腔狭窄甚至闭塞，肾小球呈缺血状（图12-7）。此外，肾小球内有多数炎性细胞浸润，主要为中性粒细胞，有时并有少数嗜酸性粒细胞、单核细胞、红细胞、浆液和纤维素性渗出液。镜下，肾小球内细胞数量增多，肾小球体积增大。病变严重时，毛细血管腔内可有血栓形成，毛细血管壁可发生纤维素样坏死。坏死的毛细血管袢破裂出血，大量红细胞进入肾球囊及肾小管腔内，可以引起明显的血尿。不同的病例病变表现形式可能不同。有的以渗出为主，称为急性渗出性肾小球肾炎。有些病变严重，肾小球毛细血管袢坏死，有大量出血者称为出血性肾小球肾炎。
图12-7 弥漫性毛细血管内增生性肾小球肾炎
肾小球内细胞数量增多，系膜细胞和内皮细胞增生并有少量中性白细胞浸润，毛血管腔狭窄&×320
　　上皮细胞一般无明显增生，少数严重的病例肾小球的壁层和脏层上皮细胞可增生，形成新月体。这种病变容易引起肾小球纤维化。如数量少，对功能影响不大。如病变广泛，可发展为新月体性肾炎。
　　在电子显微镜下可见肾小球系膜细胞和内皮细胞增生肿胀。基底膜和脏层上皮细胞间有致密物质沉积。这些沉积物大小不等，有的很大，在基底膜表面呈驼峰状或小丘状（图12-8，图12-9）。沉积物表面的上皮细胞足突多消失。基底膜变化不明显有时边缘稍不规则。沉积物一般在发病后几天就可出现，在4～6周内消失。有时基底膜内侧内皮细胞下和系膜内也可见小型沉积物。
图12-8 毛细血管内增生性肾小球肾炎
电镜下见肾小球毛细血管基底膜表面上皮细胞下有多数驼峰状电子致密沉积物&×5000
图12-9 毛细血管内增生性肾小球肾炎
电镜下见驼峰状沉积物位于毛细血管基底膜表面。沉积物表面有上皮细胞覆盖，上皮细胞足突消失&×16000
　　免疫荧光法检查显示，在肾小球毛细血管壁表面有免疫球蛋白和补体沉积（主要为IgG和C3），呈颗粒状荧光。系膜内也可有类似沉积物。
　　肾小球的病变可引起相应的肾小管缺血，肾小管上皮细胞常有浊肿、玻璃样变和脂肪变等。管腔内含有从肾小球滤过的蛋白、红细胞、白细胞和脱落上皮细胞。这些物质在肾小管内凝集，形成各种管型，如蛋白管型、透明管型、细胞管型（如红细胞、白细胞或上皮细胞管型）、颗粒管型。
　　肾间质内常有不同程度的充血、水肿和少量淋巴细胞、中性粒细胞浸润。
　　肉眼观，早期变化不明显。以后肾轻度或中度肿大、充血、包膜紧张、表面光滑、色较红，故称大红肾。若肾小球毛细血管破裂出血，肾表面及切面可见散在的小出血点如蚤咬状，称蚤咬肾。切面可见皮质由于炎性水肿而增宽，条纹模糊与髓质分界明显。
　　临床病理联系
　　这种肾炎的主要临床症状为尿的变化，水肿和。
　　1．尿的变化由于肾小球毛细血管损伤，通透性增加，故常有血尿、蛋白尿、管型尿等。
　　（1）血尿：血尿常可反映肾小球毛细血管损伤的情况。轻度血尿需用显微镜才能发现。严重的血尿，肉眼可见尿呈鲜红色。有时尿中红细胞溶解，血红蛋白在酸性尿中转变成酸性血红素，使尿呈棕红色。
　　（2）蛋白尿：蛋白尿的程度不同，一般不很严重，但少数病人尿中可有大量蛋白质。
　　（3）管型尿：在肾小管内凝集形成的管型随尿液排出，尿液内可出现各种管型，称为管型尿。
　　（4）少尿：由于肾小球细胞增生肿胀，压迫毛细血管，致管腔狭小，肾血流受阻，肾小球滤过率降低，而肾小管再吸收无明显障碍，可引起少尿，致水钠在体内潴留。严重者并可有含氮代谢产物潴留，引起氮质血症。
　　2．水肿病人常有轻度或中度水肿，往往首先出现在级织疏松的部位如眼睑。水肿的原因主要是由于肾小球滤过减少，而肾小管再吸收功能相对正常，引起水钠潴留。此外，也可能与变态反应所引起的全身毛细血管痉挛和通透性增加有关。
　　3．病人常有轻至中度。过去认为这种肾炎时的与肾小球毛细血管阻塞，肾组织缺血引起肾素分泌增加有关。但病人血中肾素浓度多在正常范围之内。因此高血压的主要原因可能与水钠潴留引起的血量增加有关。严重的高血压可导致心力衰竭及高血压性脑病。
　　这种肾炎的预后与年龄和病因有一定关系。儿童链球菌感染后肾小球肾炎的预后很好，95％以上可在数周或数月内症状消失，病变消退，完全恢复。少数病人病变消退较慢，肾小球系膜增生，可持续数月甚至1～2年。临床上，病人常有迁延性蛋白尿和复发性血尿。有时无明显症状，成为隐匿性肾炎。这种病变大多以后仍可消退，恢复正常。少数病人（约占1％～2％）临床症状消失，但病变持续不退，以后症状可反复，逐渐发展为慢性硬化性肾小球肾炎。极少数病人病变严重，发展较快，同时有明显的肾球囊上皮细胞增生，形成大量新月体，可发展为新月体性肾小球肾炎。这些病人常迅速发生急性肾功能衰竭，预后差。还有极少数（＜1％）病人病变严重，发展迅速，在短期内发生肾功能衰竭、心力衰竭或高血压性脑病。
　　一般成人患感染后肾小球肾炎者预后较差，发生肾功能衰竭和转变为慢性肾炎者较多。此外，由其他感染引起的肾炎转变为慢性肾小球肾炎者，比链球菌感染后肾炎转为慢性者多见，预后也较差。
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肾小球毛系血管血压是什么
肾小球毛细血管是什么
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他的压力要比体内其它的毛细血管高，约在15--20mmHg左右他有分泌、吸收和过滤的功能
病情分析：你好,根据你所描述的情况,是属于肾炎的表现,建议到医院做个尿常规.指导意见：对于肾炎,可以试试下面方法,黄芪,母鸡1只（治净）,加水炖烂,饮汤食肉.疗效不错的.
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