克莱尔肾病、肾病综合征英文

Nephrotic and Nephritic Syndrome,Dr Claire GibbonsFY2C.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 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 injury.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 10mmol/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/MIEruptive 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 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 (most common cause in adults)Membranous glomerulonephritis,Systemic Causes,Secondary glomerulonephritisDiabetic nephropathySarcoidosisAutoimmune: SLE, SjogrensInfection: Syphilis, hepatitis B, HIVAmyloidosisMultiple myelomaVasculitisCancerDrugs: gold, penicillamine, captopril, NSAIDs,Investigations,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,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 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 prognosisEspecially 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,Clinical 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 differentials?,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 glomerulonephritisHenoch-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-streptolysin 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-infectious 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 uri