肾脏及泌尿系疾病经常会引起一些临床症状课件

Nephrotic syndrome Figure 1. Nephrotic edema. Figure 2. Nephrotic edema. Clinical Syndrome n肾脏及泌尿系疾病经常会引起一些临床症 状、 体征和实验室表现相似的综合征。识 别患者属于哪一种综合征对诊断很有帮助 ，因为导致每个综合征的病因较之其包含 的个别临床症状和体征的致病原因要少， 故识别患者属于哪一种综合征对诊断有帮 助。 The most common syndrome of kidney disease nNephrotic syndrome nNephritic syndrome nAsymptomatic urinary abnormalities nAcute renal failure or Rapidly progressive renal failure nChronic kidney disease(Table 1) (一)肾病综合征 (二)肾炎综合征 (三)无症状性尿检异常 (四)急性及急进性肾衰竭 综合征 (五)慢性肾脏病(表1) 肾脏疾病常见综合征 Table 1. STAGES OF CHRONIC KIDNEY DISEASE* STAGEDESCRIPTIONGFR (mL/min/1.73m2) 1Kidney damage with normal or GFR90 2Kidney damage with mild or GFR60-89 3Moderate GFR30-59 4Severe GFR15-29 5Kidney failure 3.5g/24h), hypoalbuminemia ( less than 30g/dL ) and edema. Hyperlipidaemia is also present. Primary and secondary causes are summarized in Table 2, 3 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. NEPHROTIC SYNDROMENEPHROTIC SYNDROME n Pathophysiology -Proteinuria -Hypoalbuminemia -Edema -Hyperlipidemia n Cause (diagnosis and differential diagnosis) -Systemic renal disease hepatitis B associated glomerulonephritis, Henoch-Schonlein purpura, systemic lupus erythematosus, diatetes mellitus, amyloidosis -Idiopathic nephrotic syndrome n Complications -Infection -Coagulation disorders -Protein malnutrition and dyslipidemia -Acute renal failure Pathophysiology Proteinuria nProteinuria 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 transient or postural (orthostatic) proteinuria. Heavy proteinuria (albuminuria) Figure 3. Hypoalbuminemia nHypoalbuminemia 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, and other secondary consequences of heavy albuminuria . 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. Subequent 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 transcapillary fluid leakage and edema . Edema 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, 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 patient as well. Edema Figure 4. Hyperlipidemia nMost 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 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. Cause Table 2 CAUSES OF THE NEPHROTIC SYNDROME Table 3a NEPHROTIC SYNDROME ASSOCIATED WITH SPECIFIC CAUSES (“SECONDARY” NEPHROTIC SYNDROME) Table 3b NEPHROTIC SYNDROME ASSOCIATED WITH SPECIFIC CAUSES (“SECONDARY” NEPHROTIC SYNDROME) Pathology patterns and clinical presentations of idiopathic nephrotic syndome nIn adults, the nephrotic syndrome is a common 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 a slightly different spectrum of disease, but again the renal biopsy is the best guide to treatment and prognosis (Table 2, 3). Renal biopsy PRIMARY NEPHROTIC SYNDROMEPRIMARY NEPHROTIC SYNDROME n Minimal Change Disease n Focal Segmental Glomerulosclerosis n Membranous Nephropathy n Membranoproliferative Glomerulonephritis (MPGN) Figure 5a. Pathology of glomerular disease. Light microscopy. (a) Normal glomerulus; minimal change disease. Table 4 PRIMARY NEPHROTIC SYNDROMEPRIMARY NEPHROTIC SYNDROME n Minimal Change Disease n Focal Segmental Glomerulosclerosis n Membranous Nephropathy n Membranoproliferative Glomerulonephritis(MPGN) Figure 5b. Segmental sclerosis; focal segmental glomerulosclerosis. Figure 6. Light microscopic appearances in focal segmental glomerulosclerosis. Segmental scars with capsular adhesions in otherwise normal glomeruli. Table 5 PRIMARY NEPHROTIC SYNDROMEPRIMARY NEPHROTIC SYNDROME n Minimal Change Disease n Focal Segmental Glomerulosclerosis n Membranous Nephropathy n Membranoproliferative Glomerulonephritis(MPGN) 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). Figure 7b morphologically advanced MN Figure 7c. Morphologically more advanced MN (same patient as in (b) Table 6 PRIMARY NEPHROTIC SYNDROMEPRIMARY NEPHROTIC SYNDROME n Minimal Change Disease n Focal Segmental Glomerulosclerosis n Membranous Nephropathy n Membranoproliferative Glomerulonephritis(MPGN) Figure 8. Pathology of membranoproliferative glomerulonephritis type I. (a) Light microscopy shows a hypercellular glomerulus with accentuated lobular architecture and a small cellular crescent (methenamine silver). Table 7 Diagnosis and Differential diagnosis n Initial evaluation of the nephrotic patient includes laboratory tests to define whether the patient has primary, idiopathic nephrotic syndrome or a secondary cause related to a systemic disease. nCommon screening tests include the fasting blood sugar and glycosylated hemoglobin tests for diabetes, and antinuclear antibody test for rheumatoid disease, and the serum complement, which screen for many immune complex-mediated disease (Table 3), In selected patients, cryoglobulins, hepatitis B and C serology, anti- neutrophil cytoplasmic antibodies (ANCAS), anti GBM antibodies, and other tests may be useful. Once seco