Ebook Manual of nephrology (8/E): Part 2
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Ebook Manual of nephrology (8/E): Part 2
The Patient with Glomerular Disease or VasculitisSarah E. Panzer and Joshua M. ThurmanI. OVERVIEW. The glomerular diseases are defined by their clinic Ebook Manual of nephrology (8/E): Part 2cal presentations and the histologic findings associated with the diseases. Glomerular diseases can also be categorized as primary processes in which the disease is confined to the kidney or as secondary processes in which a systemic disease impacts the kidney. Many glomerular diseases are autoimmun Ebook Manual of nephrology (8/E): Part 2e in nature. Injury to the kidney may be caused by the deposition of immune complexes within the glomeruli or by autoantibodies directed against antigEbook Manual of nephrology (8/E): Part 2
ens present within the kidney. The small vessels of rhe kidney and the glomerular capillaries are also frequently the target of small vessel vasculitiThe Patient with Glomerular Disease or VasculitisSarah E. Panzer and Joshua M. ThurmanI. OVERVIEW. The glomerular diseases are defined by their clinic Ebook Manual of nephrology (8/E): Part 2e considered when hematuria and/or proteinuria is present. Therefore, the approach to rhe patient with possible glomerular disease should begin with an assessment of the protein excretion in the urine and a microscopic analysis of the urine for dysmorphic red blood cells and/or red blood cell casts. Ebook Manual of nephrology (8/E): Part 2When hematuria and/or proteinuria has been identified and glomerular disease is determined to be the most likely etiology, further clinical informatioEbook Manual of nephrology (8/E): Part 2
n and serologic testing can assist in the classification of the renal disorder before invasive testing. Although it is often difficult to predict the The Patient with Glomerular Disease or VasculitisSarah E. Panzer and Joshua M. ThurmanI. OVERVIEW. The glomerular diseases are defined by their clinic Ebook Manual of nephrology (8/E): Part 2rome and the nephrotic syndrome. The recognition of these syndromes can guide further serologic testing.II. CLINICAL PATTERNS OF GLOMERULAR DISEASEA. The Nephritic Syndrome. Patients with the nephritic syndrome typically present with hematuria, dysmorphic red blood cells and/or red blood cell casts, Ebook Manual of nephrology (8/E): Part 2 and proteinuria. The proteinuria can range from 200 mg/day to heavy proteinuria (greater than 10 g/day). Clinically, it is accompanied by hypertensioEbook Manual of nephrology (8/E): Part 2
n and edema. Renal insufficiency is common and typically progressive. The term vapidly progressive glomerulonephritis (RPGN) refers to diseases with aThe Patient with Glomerular Disease or VasculitisSarah E. Panzer and Joshua M. ThurmanI. OVERVIEW. The glomerular diseases are defined by their clinic Ebook Manual of nephrology (8/E): Part 2atient with Glomerular Disease or Vasculitis I 181of proteinuria per day) is usually the most prominent renal abnormality. Dysmorphic red blood cells and casts are typically absent, but exceptions do exist. Focal segmental glomerulosclerosis (FSGS), for example, usually presents with nephrotic range Ebook Manual of nephrology (8/E): Part 2 proteinuria but can be associated with low-grade hematuria. Additional complications of the nephrotic syndrome include hyperlipidemia, thrombosis, anEbook Manual of nephrology (8/E): Part 2
d infection. The diseases that cause the nephrotic syndrome can lead to chronic, progressive renal injury, but typically are more slowly progressive tThe Patient with Glomerular Disease or VasculitisSarah E. Panzer and Joshua M. ThurmanI. OVERVIEW. The glomerular diseases are defined by their clinic Ebook Manual of nephrology (8/E): Part 2istologic pattern defined by light microscopy, immunofluorescence staining for immunoglobulins (Igs) and complement proteins, and examination of the glomerular ultrastructure by electron microscopy. The primary glomerular diseases are listed in Table 9-1, with the prominent histologic findings on bi Ebook Manual of nephrology (8/E): Part 2opsy that define rhe disorder. There is a general correlation between the pattern of histologic injury' and the clinical presentation. Thus, the cliniEbook Manual of nephrology (8/E): Part 2
cal findings can suggest rhe underlying pathologic process, although definitive diagnosis requires a biopsy. The clinician must also consider if thereThe Patient with Glomerular Disease or VasculitisSarah E. Panzer and Joshua M. ThurmanI. OVERVIEW. The glomerular diseases are defined by their clinic Ebook Manual of nephrology (8/E): Part 2pattern seen in systemic diseases, so this distinction is usually made clinically.The nephritic syndrome is usually caused by glomerular inflammation and manifests with an “active urine sediment (e.g., cells and/or casts). Immune complexes which deposit in the mesangium or in the subendothelial spac Ebook Manual of nephrology (8/E): Part 2e [membranoproliferative glomerulonephritis (MPGN), IgA nephropathy, and many forms of lupus nephritis] generate inflammatory mediators that have acceEbook Manual of nephrology (8/E): Part 2
ss to the circulation and can cause an influx of inflammatory cells. Glomerular endothelial injury is also caused by autoantibodies to the glomerular The Patient with Glomerular Disease or VasculitisSarah E. Panzer and Joshua M. ThurmanI. OVERVIEW. The glomerular diseases are defined by their clinic Ebook Manual of nephrology (8/E): Part 2ediated vasculi tides. These two diseases frequently present with glomerular crescents and RPGN (Table 9-2).Diseases that present with the nephrotic syndrome disrupt rhe size and charge-selective barriers that ordinarily prevent the ultrafiltration of macromolecules across rhe glomerular capillary w Ebook Manual of nephrology (8/E): Part 2all. In general, these diseases disrupt the glomerular capillary wall without causing overt inflammation (FSGS, diabetic nephropathy, and amyloidosis)Ebook Manual of nephrology (8/E): Part 2
, or they affect the epithelial cells without causing endovascular inflammation [membranous nephropathy (MN) and minimal change disease (MCD)].III. CLThe Patient with Glomerular Disease or VasculitisSarah E. Panzer and Joshua M. ThurmanI. OVERVIEW. The glomerular diseases are defined by their clinic Ebook Manual of nephrology (8/E): Part 2 search for systemic diseases is warranted (Table 9-3). The history and physical examination should particularly focus on the assessment of rashes, lung disease, neurologic abnormalities, evidence ofPrimary Glomerular Diseases, Defined by HistologyNephriticHistologic FindingsNephroticHistologic Find Ebook Manual of nephrology (8/E): Part 2ingsRenal limited vasculitis/ microscopic pnlyangiilisNecrotizing capillary lesions, crescents; negative IF, FMMinimal change diseaseNormal light micrEbook Manual of nephrology (8/E): Part 2
oscopy, effaced 1CH1I processes rxi F MAnl (glomerular basemen 1 membrane disease1 inear IgG staining along glomerular basement membraneMembranous nepThe Patient with Glomerular Disease or VasculitisSarah E. Panzer and Joshua M. ThurmanI. OVERVIEW. The glomerular diseases are defined by their clinic Ebook Manual of nephrology (8/E): Part 2ial matrix, splilling (‘double contour*) of lire glomerular basement membrane. C3 granular staining on irTccal segmental glomcrulo sclerosisSclerosis in portions of glomeruli, C3 in areas ol sclerosis on IFFibrillary glomerulonephritisrItxillar deposits in mesangium, negative Ccngo red staining on I Ebook Manual of nephrology (8/E): Part 2FIgA nephropathyIgA in mesangium 1X1 IFFM. electron microscopy; GRM. gbmerular basement membrane. IF, immunofluorescence, Ig. immunoglobulin.The PatieEbook Manual of nephrology (8/E): Part 2
nt A ti Glcmerular CÌSMS3 or VjscjI tisChapter 9 • The Patient with Glomerular Disease or Vasculitis I 183MMVMHistologic Classification of Crescentic The Patient with Glomerular Disease or VasculitisSarah E. Panzer and Joshua M. ThurmanI. OVERVIEW. The glomerular diseases are defined by their clinic Ebook Manual of nephrology (8/E): Part 2ure’s disease Anti-GBM diseaseLupus nephritis IgA nephropathy Cryoglobulinemia Henoch-Schônlein purpuraANCA-associated vasculitis (GPA, Churg Strauss syndrome, microscopic polyangiitis)ANCA, antineutrophil cytoplasmic antibody; GBM, glomerular basement membrane; GPA, granulomatosis with polyangiitis Ebook Manual of nephrology (8/E): Part 2; Ig, immunoglobulin.clinically helpful to assist in the diagnosis of a specific renal disease (Table 9-4). Further laboratory assessment may be perfoEbook Manual of nephrology (8/E): Part 2
rmed based on these findings, and may include an antistreptolysin titer, antinuclear antibody (ANA), ANCA, cryoglobulins, and/or an anti-GBM antibody The Patient with Glomerular Disease or VasculitisSarah E. Panzer and Joshua M. ThurmanI. OVERVIEW. The glomerular diseases are defined by their clinic Ebook Manual of nephrology (8/E): Part 2le awaiting renal biopsy results, but they are not a substitute for renal biopsy. Proper management of the glomerular diseases requires a tissue diagnosis to confirm the clinical findings and provide information regarding rhe acuity and chronicity of rhe disease process.B. The Nephrotic Syndrome. Wi Ebook Manual of nephrology (8/E): Part 2th rhe identification of significant proteinuria, with or without other features of the nephrotic syndrome, secondary causes of proteinuria should beEbook Manual of nephrology (8/E): Part 2
considered (Table 9-5). History and physical examination should evaluate for the presence of viral and bacterial infections, malignancies (particularlThe Patient with Glomerular Disease or VasculitisSarah E. Panzer and Joshua M. ThurmanI. OVERVIEW. The glomerular diseases are defined by their clinic Ebook Manual of nephrology (8/E): Part 2oteinuria. Laboratory assessment initially includes CBC, electrolyte panel, 24-hour urine collection for protein and creatinine clearance, liver function tests, and a cholesterol panel, further assessment may include hepatitis and human immunodeficiency virus (HIV) serologies, ANA, rapid plasma reag Ebook Manual of nephrology (8/E): Part 2in, and serum and urine electrophoresis (Table 9-5). Renal biopsy should be performed in all cases in which no cause is evident, or to determine the eEbook Manual of nephrology (8/E): Part 2
xtent of renal disease to guide therapy or prognosis.IV. THERAPY FOR GLOMERULAR DISEASE. Treatment of glomerular disorders can be approached by manageGọi ngay
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