肾病综合征英文PPT课件

1、.,1,Nephrotic Syndrome,.,2,The outlines,Definition of Nephrotic syndrome Etiology Pathogenesis Clinical picture Diagnostic workup Pathological picture Complication Managements,.,3,What is Nephrotic syndrome?,proteinuria (3.5g/day), hypoalbuminemia (30g/L) edema hyperlipidemia,.,4,Types of nephrotic

2、syndrome (1) Idiopathic nephrotic syndrome: Etiology of the disease is unknown, accounting for approximately 90% of nephrosis in childhood. (2) Secondary nephrosis: NS resulted from systemic disease such as anaphylactoid purpura, systemic lupus Erythematosus (SLE), and so on.,Etiology,.,5,Etiology,.

3、,6,Etiology,.,7,Etiology,.,8,Etiology,In children Minimal change disease is predominant In adults Systemic disease related: 30% Primary renal disorders: 70% Membranous nephropathy Focal glomerulosclerosis Minimal change disease Amyloidosis In elderly Increased incidence of amyloidosis and decreased

4、incidence of SLE,.,9,Pathogenesis,DAMAGED,Proteinuria,.,10,(1) Proteinuria: Massive proteinuria is the most chief characteristics of nephrosis resulting from an increase in glomerular capillary wall permeability to plasma protein. The mechanism may be related to Molecular barrier injury :holes on GB

5、M become larger; Charge barrier injury: loss of negative,.,11,.,12,(2) Hypoproteinemia Plasma protein is lost by urine; Protein catabolism , so total plasma protein concentration, especially albumin.,.,13,(3)Hyperlipidemia All serum lipid (cholesterol, triglycerides) and lipoprotein levels are eleva

6、ted. A. Hypoproteinemia stimulates generalized protein synthesis in the liver, including the lipoprotein; B. Lipid catabolism is diminished.,.,14,(4) Edema A. Hypoalbuminemia leads to a decrease in plasma osmotic pressure, which permits the translation of fluid from intravascular compartment to inte

7、rstitial space. B. The intravascular volume make renal perfusion pressure activating rennin-angiotensin-aldosterone system, which stimulates distal tubular reabsorption of sodium. C. Reduced intravascular volume also stimulates the release of antidiuretic hormone, which enhances there absorption of

8、water in the collecting duct. D. Because of plasma osmotic pressure , the sodium and water enter interstitial space,.,15,.,16,Pathology,In adults, the nephrotic syndrome is a common condition leading to renal biopsy. In many studies, patients with heavy proteinuria and the nephrotic syndromes have b

9、een a group highly likely to benefit from renal biopsy in terms of a change in specific diagnosis, prognosis, and therapy. Selected 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,.,17

10、,(1) Minimal change disease (78%), the glomeruli appear normal. The epithelial cell foot processes fused. More than 95% of children with MCD, and better responding to corticosteroid therapy. Light Microscopy Either normal or reveals only mild mesangial cell proliferation EM Diffuse fusion of the epi

11、thealial cell foot processes,Pathology,.,18,Pathology,.,19,Pathology,.,20,(2) Focal Segmental Glomerulosclerosis(6.7%), sclerosis and hyalinosis involving a portion of glomerular tuft, even only one of the glomeruli, accompanied tubular atrophy. IgM and C3 within sclerotic areas. (3)Mesangial prolif

12、eration:Only mesangial proliferation. Immunoglobulin and complement deposits in the mesangial area.,Pathology,.,21,Pathology,Segmental sclerosis; focal segmental glomerulosclerosis.,.,22,Pathology,.,23,(4) Membrane nephrosis: GBM thicker, immune complex deposits. (5) Membranoproliferative glomerulon

13、ephritis: Diffuse proliferation of mesangial cells and mesangial matrix. Electronic density deposits and C3 deposit in mesangial and GBM.,Pathology,.,24,Secondary to: DM (the leading cause of secondary nephrotic syndrome) SLE Amyloidosis Infections: Hepatitis B and C, HIV,syphilis, post-streptococca

14、l Malignancy: multiple myloma , Hodgkin lymphoma, solid tumor Drugs (NSAIDs, gold, penicillamine ,heavy metals etc).,Pathology,.,25,Clinical Presentation,Generalized Odema -The predominant feature -The face, particularly the periorbital area, is swollen in the morning139(29-30):416-422. -Knowledge o

15、f renal histology alters patient management in over 40 percent of patients. Nephrol Dial Transplant 1994; 9:1255.,.,30,The indications for renal biopsy in diabetic patients,10% of nephrotic syndrome cases in diabetes are due to other renal diseases *Presence atypical features such as 1-A rapidly pro

16、gressive nephrotic syndrome 2-Acute renal failure 3-Presence of glomerular haematuria and/or absence of associated microvascular lesions (retinopathy, neuropathy),Management of patients with nephrotic syndrome. Swissmedwkly 2009 ;139(29-30):416-422.,.,31,Normal Kidney biopsy,.,32,Minimal change Dise

17、ase,.,33,Membranous Nephropathy,.,34,Focal Segmental Glomerulosclerosis,.,35,Membranoproliferative Glomerulonephritis,.,36,Renal biopsy results,.,37,COMPLICATIONS,Due to loss of proteins in the urine,Due to oncotic pressure,Immunoglobulin susceptibility to infection antithrombin III and proteins C a

18、nd SThromboembolism vit Dbinding protein vit D deficiency Transferrin Iron deficiency anemia,Hyperlipidaemia Hypovolemia Acute renal failure Anasarca risk of cellulitis, bacterial peritonitis with ascites ,large pleural effusions or pulmonary edema,.,38,Treatment,.,39,Management of symptoms,Oedema L

19、ow salt diet Diuretics serial measurement of body weight Proteinuria ACE inhibitors or ARBs Hypoalbuminaemia High protein diet not indicated 0.81 g/kg/day,Ref: Up to date online 17.3.,.,40,Management of complication,Hyperlipidaemia Regular Lipid profile Statin if severe long lasting nephrotic syndro

20、me Control other CVD risk factorstarget blood pressure 125/75 Thromboembolic risk Routin Prophylactic anticoagulation not recommend High index of suspicion for thromboemboli Infections High index of suspicion Antipneumococcal and influenza vaccinations,Ref: Up to date online 17.3.,.,41,Disease-Speci

21、fic Therapy,Minimal change disease Approximately 80% of adults with MCD respond to prednisone Failure to respond may reflect an error in diagnosis; MCD is most commonly confused with early FSGS Treatment with cytotoxic agents may be indicated in patients who are considered: A- steroid dependent rela

22、pse while on corticosteroid therapy or requirement for continuation of steroids to maintain remission B-steroid resistant No remission with using of steroid C-frequent relapsers 3 relapses/Y,.,42,Disease-Specific Therapy,Membranous Nephropathy Because of the generally good outcome, treatment usually

23、 is reserved for patients with poor prognostic factors : (age 50, male gender, hypertension, reduced GFR, proteinuria 10 g/d, or marked interstitial fibrosis on renal biopsy) or severe symptomatic nephrotic syndrome Treatment options include high-dose alternate-day glucocorticoids in conjunction wit

24、h a cytotoxic agent (e.g., chlorambucil or cyclophosphamide for 6-12 months and in nonresponders, cyclosporine for 12 months,.,43,Disease-Specific Therapy,Focal Segmental Glomerulosclerosis PrednisoneIt : not proven to be effective May reduce proteinuria and slow progression to ESRD. Resistant cases

25、 may respond to a combination of glucocorticoids and cytotoxic agents.,.,44,Disease-Specific Therapy,Membranoproliferative Glomerulonephropathy treatment has not been shown to improve disease-free survival the use of corticosteroids in children likely stabilizes disease. HCV-associated MPGN may impr

26、ove with successful antiviral therapy,.,45,Corticosteroids For Adults,Remains controversial with no proven benefit Cochrane reviews on the treatment of nephrotic syndrome in adults found: -weak benefit for disease remission and proteinuria in persons with membranous nephropathy -no benefit for morta

27、lity or need for dialysis with corticosteroid therapy for membranous nephropathy or minimal change disease,.,46,Corticosteroids For Children,It is more clearly established that children respond well to corticosteroid treatment. Classically, minimal change disease responds better to corticosteroids than FSGS.,Corticosteroid therapy for nephrotic syndrome in children.