内科学肾病综合征(英文)

Nephrotic Syndrome,Learning objectives,Clinical manifestation, pathology and treatment of nephrotic syndrome Pathophysiology of nephrotic syndrome Complications of nephrotic syndrome,Outline,Definition Pathophysiology Pathology and clinical manifestation Complications Diagnosis and differential diagnosis Treatment,Mr. O,19 years old university student Noticed foaming urine during the past 3-4 weeks Puffing face, swelling feet for 1 week Had gain 9 kg body weight Shoes too tight to wear Slightly fatigue and SOB, otherwise normal,Figure 1. Nephrotic edema.,Figure 2. Nephrotic edema.,Diagnosis:,Proteinuria: 3.5g/d Hypoalbuminemia: SAlb 30g/L Edema Hyperlipidemia,Pro +,What will you do for Mr. O ?,What will you do ?,Urine routine 24h urinary protein Kidney function Liver function and serum albumin Lipids ,Lets go back to Mr. O,Urine protein +, RBC (-), WBC (-) 24h urinary protein 7.8g Normal kidney and Liver function Serum albumin 20g/L Total cholesterol 10.2mmol/L,Mr. O was diagnosed with nephrotic syndrome,Proteinuria (albuminuria),Figure 3.,Hypoalbuminemia,Albumin Immunoglobulins Metal binding proteins Erythropoietin urinary loss Transferrin Complement deficiency Coagulation components,Mechanisms leading to nephrotic hypoalbuminemia,Hyperlipidemia,Most NS 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. Nephrotic patients often have a hypercoagulable state and are predisposed to deep vein thrombophlebitis, pulmonary emboli, and renal vein thrombosis.,Mechanisms of Hyperlipidemia,Increased hepatic synthesis of LDL, VLDL and lipoprotein (a) in response to hypoalbuminemia Urinary loss of HDL Enzymatic changes with abnormal lipid biosythesis and degradation,Edema,Two different major mechanisms: In 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.,Edema,How many pathological types in nephrotic syndrome ? For Mr. O, which pathological type is the most likely ?,Pathology,Minimal change disease (MCD) Mesangial proliferative glomerulonephritis (MsPGN) Membranoproliferative glomerulonephritis (MPGN) Membranous nephropathy (MN) Focal segmental glomerulosclerosis (FsGs),Minimal change disease (MCD),Epidemiology: It is the most common cause of NS in children, accounting for 80-90% of young patients with NS, while only 10-20% in adults. There appears to be a male preponderance, especially in children, in whom the male- to- female ratio is 23 :1,Minimal change disease (MCD),Pathology No glomerular lesions by light microscopy No staining with antibodies specific for immunoglobulins or complement components Effacement of visceral epithelial cell foot processes,Figure 5a. Pathology of glomerular disease. Light microscopy. (a) Normal glomerulus; minimal change disease.,Minimal change disease (MCD),Clinical manifestations: The cardinal clinical feature of MCD in children is the relatively sudden onset of proteinuria and development of the NS. Hematuria, hypertension and impaired kidney function are not common.,Mesangial proliferative glomerulonephritis (MsPGN),Epidemiology: It is a common reason of NS in our country, accounting for 30% of primary nephrotic syndrome, higher than those in western.,Mesangial proliferative glomerulonephritis (MsPGN),Pathology Diffuse proliferation of mesangial cells and ECM Positive staining with IgA, IgG, IgM or C3 in mesangial area Dense deposits in mesangial area,Mesangial proliferative glomerulonephritis (MsPGN),Clinical manifestations: 50% has infection before onset of kidney disease. Non-IgAN: 50% with NS, 70% with hematuria IgAN:15% with NS, almost all with hematuria,Membranoproliferative glomerulonephritis (MPGN),Epidemiology: It is accounting for 10-20% of primary nephrotic syndrome patients in our country .,Membranoproliferative glomerulonephritis (MPGN),Pathology Severe diffuse proliferation of mesangial cells and ECM, demonstrating doubling and more complex replication of glomerular basement membranes Peripheral granular to bandlike staining for C3 and IgG Dense deposits in mesangial subendothelial area,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).,Membranoproliferative glomerulonephritis (MPGN),Clinical manifestations: 50-60% present as a nephrotic syndrome. Almost all patients with hematuria Early onset of impairment of kidney function, hypertension, anemia Progressive disease (10 year renal survival rate 50%),Membranous nephropathy (MN),Epidemiology Membranous nephropathy is the lea