原發(fā)性腎小球疾病英文

1、原發(fā)性腎小球疾病英文原發(fā)性腎小球疾病英文概述概述腎小球疾?。℅lomerular disease）是指病變位于腎小球的一類以血尿，蛋白尿，水腫和髙血壓等為主要表現(xiàn)的腎臟疾病，可導(dǎo)致終末期腎臟病(End Stage Renal Disease, ESRD)，分原發(fā)性，繼發(fā)性和遺傳性。原發(fā)性病因常不明確，故又稱之為特發(fā)性，腎小球病變是唯一或主要病變。繼發(fā)性病因常比較明確（如系統(tǒng)性紅斑狼瘡和糖尿病等），腎小球病變?yōu)槿硇约膊〉囊粋€(gè)部分，病理變化類型并不單一。而遺傳性腎小球疾病，是遺傳基因變異所致的腎小球疾?。ㄈ鏏lport綜合征和Fabry病等）。原發(fā)性腎小球疾病英文分類分類原發(fā)性腎小球疾病病理分型

2、(根據(jù)WHO 1995年制定的標(biāo)準(zhǔn))：n微小病變性腎小球病n局灶節(jié)段性腎小球腎炎/腎小球硬化n彌漫性腎小球腎炎膜性腎病增生性腎炎 系膜增生性腎小球腎炎 毛細(xì)血管內(nèi)增生性腎小球腎炎 腹增生性腎小球腎炎/系膜毛細(xì)血管性腎小球腎炎 新月體(和壞死)性腎小球腎炎增生硬化性/硬化性腎小球腎炎原發(fā)性腎小球疾病英文n免疫反應(yīng)n腎小球免疫損傷的機(jī)制n腎小球疾病的進(jìn)展 發(fā)病機(jī)制發(fā)病機(jī)制 (The mechanisms of glomerular injury)原發(fā)性腎小球疾病英文原發(fā)性腎小球疾病英文原發(fā)性腎小球疾病英文臨床表現(xiàn)臨床表現(xiàn)n蛋白尿 Proteinurian血尿 Hematurian水腫 Edeman

3、高血壓 Hypertensionn腎功能損害 Renal Insufficiency原發(fā)性腎小球疾病英文Nephrotic syndrome原發(fā)性腎小球疾病英文Figure 1. Nephrotic edema. 原發(fā)性腎小球疾病英文Figure 2. Nephrotic edema. 原發(fā)性腎小球疾病英文Nephrotic syndrome This is characterized by proteinuria (Typically 3.5g/24h), hypoalbuminemia ( less than 30g/dL ) and edema. Hyperlipidaemia is a

4、lso present. Primary and secondary causes are summarized in Table 2, 3a,b In practice, many clinicians refer to “nephrotic range” proteinuria regardless of whether their patients have the other manifestations of the full syndrome because the latter are consequences of the proteinuria.原發(fā)性腎小球疾病英文Patho

5、physiology原發(fā)性腎小球疾病英文ProteinuriaProteinuria can be caused by systemic overproduction, tubular dysfunction, or glomerular dysfunction. It is important to identify patients in whom the proteinuria is a manifestation of substantial glomerular disease as opposed to those patients who have benign transien

6、t or postural (orthostatic) proteinuria.原發(fā)性腎小球疾病英文Heavy proteinuria (albuminuria)Figure 3.原發(fā)性腎小球疾病英文Zandi-Nejad K et al. Kidney Int. Suppl 2004;92:S76-S89圖1、導(dǎo)致蛋白尿腎病的小管間質(zhì)炎癥及腎硬化的某些事件原發(fā)性腎小球疾病英文圖2、腎臟對(duì)濾過(guò)白蛋白處理(回吸收) 的旁路Russo LM et al. Kidney Int. Suppl 2004;92:S67-S68原發(fā)性腎小球疾病英文HypoalbuminemianHypoalbuminem

7、ia is in part a consequences of urinary protein loss. It is also due to the catabolism of filtered albumin by the proximal tubule as well as to redistribution of albumin within the body. This in part accounts for the inexact relationship between urinary protein loss, the level of the serum albumin,

8、and other secondary consequences of heavy albuminuria .原發(fā)性腎小球疾病英文 The salt and volume retention in the NS may occur through at least two different major mechanisms. nIn the classic theory, proteinuria leads to hypoalbuminemia, a low plasma oncotic pressure, and intravascular volume depletion. Subequ

9、ent underperfusion of the kidney stimulates the priming of sodium-retentive hormonal systems such as the RAS axis, causing increased renal sodium and volume retention, In the peripheral capillaries with normal hydrostatic pressures and decreased oncotic pressure, the Starling forces lead to transcap

10、illary fluid leakage and edema .Edema原發(fā)性腎小球疾病英文nIn some patients, however, the intravascular volume has been measured and found to be increased along with suppression of the RAS axis. An animal model of unilateral proteinuria shows evidence of primary renal sodium retention at a distal nephron site,

11、 perhaps due to altered responsiveness to hormones such as atrial natriuretic factor. Here only the proteinuric kidney retains sodium and volume and at a time when the animal is not yet hypoalbuminemic. Thus, local factors within the kidney may account for the volume retention of the nephrotic patie

12、nt as well.Edema原發(fā)性腎小球疾病英文Figure 4.原發(fā)性腎小球疾病英文HyperlipidemianMost nephrotic patients have elevated levels of total and low-density lipoprotein (LDL) cholesterol with low or normal high-density lipoprotein (HDL) cholesterol . Lipoprotein (a) Lp(a) levels are elevated as well and return to normal with

13、remission of the nephrotic syndrome. Nephrotic patients often have a hypercoagulable state and are predisposed to deep vein thrombophlebitis, pulmonary emboli, and renal vein thrombosis.原發(fā)性腎小球疾病英文Cause原發(fā)性腎小球疾病英文Table 2 CAUSES OF THE NEPHROTIC SYNDROME原發(fā)性腎小球疾病英文Table 3a NEPHROTIC SYNDROME ASSOCIATED

14、WITH SPECIFIC CAUSES (“SECONDARY” NEPHROTIC SYNDROME)原發(fā)性腎小球疾病英文Table 3b NEPHROTIC SYNDROME ASSOCIATED WITH SPECIFIC CAUSES (“SECONDARY” NEPHROTIC SYNDROME)原發(fā)性腎小球疾病英文 Pathology patterns and clinical presentations of idiopathic nephrotic syndome原發(fā)性腎小球疾病英文nIn adults, the nephrotic syndrome is a common

15、condition leading to renal biopsy. In many studies, patients with heavy proteinuria and the nephrotic syndromes have been a group highly likely to benefit from renal biopsy in terms of a change in specific diagnosis, prognosis, and therapy. nSelected adult nephrotic patients such as the elderly have

16、 a slightly different spectrum of disease, but again the renal biopsy is the best guide to treatment and prognosis (Table 2, 3). Renal biopsy原發(fā)性腎小球疾病英文n Minimal Change Diseasen Focal Segmental Glomerulosclerosisn Membranous Nephropathyn Membranoproliferative Glomerulonephritis (MPGN)原發(fā)性腎小球疾病英文Figure

17、 5a. Pathology of glomerular disease. Light microscopy. (a) Normal glomerulus; minimal change disease. 原發(fā)性腎小球疾病英文Table 4原發(fā)性腎小球疾病英文n Minimal Change Diseasen Focal Segmental Glomerulosclerosisn Membranous Nephropathyn Membranoproliferative Glomerulonephritis(MPGN)原發(fā)性腎小球疾病英文Figure 5b. Segmental scleros

18、is; focal segmental glomerulosclerosis. 原發(fā)性腎小球疾病英文Figure 6. Light microscopic appearances in focal segmental glomerulosclerosis. Segmental scars with capsular adhesions in otherwise normal glomeruli. 原發(fā)性腎小球疾病英文Table 5原發(fā)性腎小球疾病英文n Minimal Change Diseasen Focal Segmental Glomerulosclerosisn Membranous

19、Nephropathyn Membranoproliferative Glomerulonephritis(MPGN)原發(fā)性腎小球疾病英文Figure 7a. Early MN: a glomerulus from a patient with severe nephrotic syndrome and early MN, exhibiting normal architecture and peripheral capillary basement membranes of normal thickness (Silvermethenamine 400). 原發(fā)性腎小球疾病英文Figure

20、7b Morphologically advanced MN原發(fā)性腎小球疾病英文Figure 7c. Morphologically more advanced MN (same patient as in (b)原發(fā)性腎小球疾病英文Table 6原發(fā)性腎小球疾病英文n Minimal Change Diseasen Focal Segmental Glomerulosclerosisn Membranous Nephropathyn Membranoproliferative Glomerulonephritis(MPGN)原發(fā)性腎小球疾病英文Figure 8. Pathology of m

21、embranoproliferative glomerulonephritis type I. (a) Light microscopy shows a hypercellular glomerulus with accentuated lobular architecture and a small cellular crescent (methenamine silver). 原發(fā)性腎小球疾病英文Table 7原發(fā)性腎小球疾病英文Diagnosis and Differential diagnosis原發(fā)性腎小球疾病英文n Initial evaluation of the nephrot

22、ic patient includes laboratory tests to define whether the patient has primary, idiopathic nephrotic syndrome or a secondary cause related to a systemic disease. 原發(fā)性腎小球疾病英文nCommon screening tests include the fasting blood sugar and glycosylated antibody test for rheumatoid disease, and the hemoglobin tests for diabetes, and antinuclear serum complement, which screen for many immune complex-mediated disease , In selected patients, cryoglobulins, hepatitis B and C serology, anti-neutrophil cytoplasmic antibodies (ANCA), anti GBM antibodies, and other tests may be useful. Once secondary cau