克莱尔肾病、肾病综合征英文.ppt

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1、Nephrotic and Nephritic Syndrome,Dr Claire GibbonsFY2Claire.gibbonsdoctors.org.uk,Learning Objectives,Understand and define nephrotic and nephritic syndromes.Describe the initial investigations and management of nephrotic and nephritic syndromes.Describe the complications of nephrotic and nephritic

2、syndromes.,Draw a nephron!,Glomerulonephritis,Glomerulus capillary loop with basement membrane which allows passage of specific molecules into the nephronGlomerulonephritis inflammation/damage of the glomerular basement membrane resulting in altered function. Relatively uncommon cause of kidney inju

3、ry.Can present as nephrotic and/or nephritic syndrome.,What is nephrotic syndrome?,Increased permeability of the glomerulus leading to loss of proteins into the tubules.,Nephrotic Syndrome,Triad of:MASSIVE Proteinuria 3g/24hoursOr spot urine protein:creatinine ratio 300-350mg/mmolHypoalbuminaema 10m

4、mol/L),Presentation,New-onset oedemaInitially periorbital or peripheralLater genitals, ascites, anasarcaFrothy urineGeneralised symptoms lethargy, fatigue, reduced appetite,Further possible presentations.,OedemaBP normal/raisedLeukonychiaBreathlessness: Pleural effusion, fluid overload, AKIDVT/PE/MI

5、Eruptive xanthomata/ xanthalosmata,You are a GP with the following patients.,Young, fit 24 year old male complaining of frothy urine.10 year old boy with puffy eyes.74 year old female with multiple co-morbidities and swollen ankles.,Differential Diagnosis for Oedema,Congestive Cardiac Failure Raised

6、 JVP, pulmonary oedema, mild proteinuriaLiver disease Hypoalbuminaemia, ascites/oedemaWhat investigations can you do?You decide to send your patient to the renal clinic.,Causes of Nephrotic Syndrome,Primary glomerulonephritisMinimal change disease (80% paeds cases)Focal segmental glomerulosclerosis

7、(most common cause in adults)Membranous glomerulonephritis,Systemic Causes,Secondary glomerulonephritisDiabetic nephropathySarcoidosisAutoimmune: SLE, SjogrensInfection: Syphilis, hepatitis B, HIVAmyloidosisMultiple myelomaVasculitisCancerDrugs: gold, penicillamine, captopril, NSAIDs,Investigations,

8、Urine dipstick and send to labUrine microscopyBloods the usual ones, plus renal screenImmunoglobulins, electrophoresis (myeloma screen), complement (C3, C4) autoantibodies (ANA, ANCA, anti-dsDNA, anti-GBM)Renal ultrasoundRenal biopsy (all adults)Children generally trial of steroids first,Management,

9、ConservativeMonitor U&E, BP, fluid balance, weightSalt and fluid restrictionTreat underlying causeMedicalDiureticsACE-inhibitors/ARBsCorticosteroids/immunosuppressionDialysisAnticoagulationSurgicalRenal transplant,Complications,Increased susceptibility to infection20% adult casesDue to reduced serum

10、 IgG, reduced complement activity, reduced T cell functionThromboembolism40% adult casesPartly due to increased clotting factors and platelet abnormalitiesHyperlipidaemiadue to hepatic lipoprotein synthesis to restore osmotic pressure,Prognosis,VariesWith treatment, generally good prognosisEspeciall

11、y minimal change disease (1% progress to ESRF)Without treatment, very poor prognosisChildren under 5 or adults older than 30 = worse prognosis,What is nephritic syndrome?,Pathophysiology,Thin glomerular basement membrane with pores that allow protein and blood into the tubule.,Nephritic Syndrome,Cli

12、nical syndrome defined by:Haematuria/ red cell castsHypertension (mild)OliguriaUraemiaProteinuria (3g/24 hours),Signs and Symptoms,Haematuria (E.g. cola coloured)ProteinuriaHypertensionOliguriaFlank painGeneral systemic symptomsPost-infectious = 2-3 weeks after strep-throat/URTI,What are your differ

13、entials?,Malignancy (older patients) UTITraumaWhat bedside investigation would you like to do?You decide to refer to the renal clinic.,Causes,Post-infectious glomerulonephritisPrimaryIgA Nephropathy (Bergers disease)Rapidly progressive glomerulonephritisProliferative glomerulonephritisSecondary glom

14、erulonephritisHenoch-Schonlein purpuraVasculitis,Investigations,Urine dipstick and send sample to labUrine microscopy red cell castsBloods the usual plus renal screenImmunoglobulins, electrophoresis, complement (C3, C4) autoantibodies (ANA, ANCA, anti-dsDNA, anti-GBM); blood culture; ASOT (anti-stre

15、ptolysin O titre)Renal ultrasoundRenal biopsy,Red Cell Casts,Management,ConservativeMonitor U&E, BP, fluid balance, weightSalt and fluid restrictionTreat underlying causeMedicalDiureticsTreat hypertensionCorticosteroids/immunosuppressionDialysisSurgicalRenal transplant,Prognosis,VariesPost-infectiou

16、s usually self-resolving (95% recover renal function)Others are a bit more nasty,Example Case,Summary,Nephrotic syndrome = MASSIVE proteinuriaNephritic syndrome = haematuria/red cell castsMay be a mixed presentationNew oedema? Dipstick that urine!Haematuria? Exclude malignancy!,Any Questions?Claire.gibbonsdoctors.org.uk,Sources,Oxford Handbook of Clinical MedicineOxford Handbook for the Foundation ProgrammeEssential Revision Notes for the MRCPWW,

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