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58 Cards in this Set
- Front
- Back
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Responses to glomerular injury |
Hypercellularity Basement membrane thickening Hyalinisation Sclerosis |
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Responses to glomerular injury Hyperceullarity |
Proliferation - mesangial, endothelial Leukocyte infiltration Crescents - severe - epithelial cells (mostly parietal and leukocytes) Acute, inflammatory diseases |
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Responses to glomerular injury Basement membrane thickening |
Appears as thickening of capillary walls Deposition of amorphous electron-dense material on the endothelial or epithelial side of the basement membrane or within the GBM itself Chronic |
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Responses to glomerular injury Hyalinisation |
Accumulation of homogenous pink material (plasma proteins) Fills capillary lumens Consequence of endothelial or capillary wall injury - end results of various forms of glomerular damage Chronic |
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Responses to glomerular injury Sclerosis |
Extracellular collagenous matrix - mesangial, capillary loops -> obliteration of capillary lumens -> fibrous adhesions between the sclerotic portions of the glomeruli and nearby parietal epithelium Chronic |
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Patterns of glomerular disease |
Focal - <50% of glomeruli affected Diffuse - ≥50% of glomeruli affected Segmental - Involving only part of the glomerular tuft Global - involving all of the glomerular tuft |
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Characteristics of Nephrotic syndrome |
Massive proteinuria >3.5 g/day Hypoalbuminaemia <3g/dL Generalised oedema Hyperlipidaemia and lipiduria Hypercoagulatbility Hypogammaglobulinaemia Hypocalcaemia |
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Mechanism of Nephrotic syndrome |
Structural and/or charge change in glomerulus capillary wall --> increased permeability --> heavy proteinuria --> loss of albumin, immunoglobulins, loss of antithrombin 3 Loss of albumin --> hypoalbuminaemia --> oedema |
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Glomerular disease with nephrotic syndrome |
Minimal change disease Focal Segmental Glomerulosclerosis Membranous glomerulonephropathy Membranoproliferative glomerulonephritis Diabetic glomerulosclerosis |
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Minimal change disease Presentation and pathology |
Nephrotic with selective loss of albumin and not immunoglobulins Most common cause of nephrotic syndrome in children Usually idiopathic Often associated with atopic conditions, respiratory infection, vaccines, Hodgkin lymphoma and NSAID use Cytokines cause podocyte injury --> effacement of foot processes |
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Minimal change disease LM, IF, EM |
LM - normal, may see lipid in PCT IF - Negative EM - foot process effacement, no deposits |
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Minimal change disease Management and prognosis |
Excellent response to steroids Exclude precipitative factors Manage symptoms Remission in majority |
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Focal segmental glomerulosclerosis Presentation and pathology |
More common in adults than in children Nephrotic syndrome 10-15% primary, 85-95% secondary May be associated with HIV, heroin use, sickle cell disease, and/or obesity Secondary to glomerulonephritis End-point for many diseases due to loss of nephrons and hyperfiltration injury Endothelial cell damage --> entrapment of plasma proteins --> sclerosis and hyalinisation |
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Focal segmental glomerulosclerosis LM, IF, EM |
LM - Focal and segmental sclerosis and hyalinisation Increased mesangial matrix Obliteration of capillary lumina Lipid droplets Collapsing glomerulopathy IM Focal, IgM and C3 EM Foot process effacement |
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Focal segmental glomerulosclerosis Management and prognosis |
Primary - poor response to steroids Treat proteinuria Treat underlying disease if secondary 50% renal failure in 10 years 20% renal failure in 2 years High recurrence rate in transplants |
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Membranous glomerulopathy Presentation and pathology |
Nephrotic Most common cause of nephrotic syndrome in adults 75% primary - antibodies against PLA2R on podocytes 25% secondary - SLE, Drugs (e.g. NSAIDs), underlying malignancy, chronic infections other autoimmune diseases (e.g. diabetes, thyroiditis) Chronic immune complex-mediated capillary wall injury --> thickening of basement membrane --> thickening of GBM |
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Membranous glomerulopathy LM, IF, EM |
LM Diffuse thickening of GBM Open capillary lumen Siver stain reveals spikes on GBM IF Granular IgG and C3 EM Subepithelial deposits Spikes of BM material - spike and dome appearance Foot process effacement |
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Membranous glomerulopathy Management and prognosis |
Treatment of underlying disease ACEI or ARB Immunosuppression Natural history - 30% spontaneous remission, 30% persistent proteinuria, 30% progressive renal failure |
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Membranoproliferative glomerulonephritis Presentation and pathology |
Nephrotic and/or nephrotic syndrome 10% of cases of nephrotic syndrome in children Type 1 (80%) - circulating immune complexes associated with Hep B and Hep C Type 2 (dense deposit disease) - excessive complement activation due to C3 nephritic factor (autoantibody) More likely to be nephritic Both lead to complement activation |
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Membranoproligerative glomerulonephritis LM, IF, EM |
LM Large hypercellular glomeruli with lobular appearance Tram-track appearance - GBM thickening and splitting Proliferation of mesangial and endothelial cells IF Type 1: granular - IgG, C3/C1q Type 2: discontinuous linear - C3 EM Type 1: subendothelial deposits Type 2: intramembranous deposits |
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Membranoproliferative glomerulonephritis Management and prognosis |
Poor response to steroids Slowly progress, unremitting course 50% renal failure within 10 years Can recur in transplants |
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Diabetic glomerulonephritis Presentation and pathology |
Nephrotic, microalbuminuria, azotaemia ESRF Non-enzymatic glycosylation of vascular basement membrane proteins -> hyaline arteriosclerosis Haemodynamic changes -> increase GFR -> hyperfiltration -> glomerular hypertrophy |
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Diabetic glomerulonephritis LM, IF, EM |
LM Global and diffuse Capillary basement membrane thickening Mesandial sclerosis and expansion - Kimmelstiel-wilson nodules Nodular glomerulosclerosis Accumulation of hyaline material IF Linear homogenous weak to moderate reactivity for IgG and albumin EM Diffuse thickened GBM, no deposits |
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Diabetic glomerulonephritis Management and prognosis |
ACEI - dilation of efferent arteriole ~30-40% of diabetes mellitus patients develop sever or end stage renal insufficiency |
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Characteristics of nephritic syndrome |
Haematuria + red blood cell casts in urine Azotaemia Oliguria Mild-moderate hypertension and periorbital oedema Mild proteinuria |
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Mechanism of nephritic syndrome |
Inflammation of glomeruli -> glomerular cell proliferation -> obstruction of capillary lumen -> capillary rupture -> haematuria and proteinuria (mild) -> decreased GFR -> acute renal failure and sodium and water retention |
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Post-Streptococcal Glomerulonephritis Presentation and Pathology |
Nephritic Most common in children aged 6-10 years Occurs 2-3 weeks after infection with Group A, beta-haemolytic streptococcal infection (skin or pharynx) Trapped immune complexes - streptococcal Ag and specific Ag |
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Post-streptococcal glomerulonephritis LM, IF, EM |
LM Enlarged hypercellular glomeruli = leukocytes, mesangium, endothelial proliferation +/- crescent formation if severe IF "Lumpy bumpy" Granular IgG and C3 in GBM and mesangium EM Subepithelial humpsPost |
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Post-streptococcal Glomerulonephritis Management and prognosis |
Supportive therapy >95% of children recover completely, 1% progress to renal failure 60% of adults fully recover, some go on to develop RPGN |
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Alport syndrome |
Inherited defect in type IV collagen; most commonly X-linked Results in thinning and splitting of glomerular basement membrane Isolated haematuria, sensory hearing loss, ocular disturbances |
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IgA Nephropathy (Berger disease) Presentation and pathology |
Children and young adults Recurrent Haematuria and RBC casts Usually follows an URTI, GIT infection or UTI Most common glomerulonephritis worldwide Primary or secondary - HSP, Liver disease, psoriasis, coeliac disease, IBD, HIV Increased plasma IgA -> IgA deposition in mesangium -> mesangial widening and proliferation |
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IgA nephropathy LM, IM, EM |
LM Mesangial expansion with some increase in cellularity, capillary loops still open IF Granular IgA +/- C3 in mesangium EM Mesangial and paramesangial deposits |
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IgA nephropathy management and prognosis |
Depends on clinical parameters and indication of severity/progression Management of blood pressure and proteinuria 30-40% microscopic haematuria only 5-10% acute nephritic syndrome 15-40% slow progression to CRF over 20 years Can recur in transplanted kidney Increased risk of progression: onset in old age, heavy proteinuria, hypertension, extent of glomerulosclerosis |
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Type 1 Rapidly progressive glomerulonephritis Presentation and pathology |
Anti-GBM disease Young adult males Haemoptysis Rapid and progressive loss of renal function - severe oliguria, nephritic syndrome Good pasture syndrome Circulating IgG against collagen in glomerular and alveolar basement membranes Also associated with lung pathology |
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Type 1 Rapidly progressive glomerulonephritis LM, IF, EM |
LM Focal and segmental Crescents - fibrin and macrophages Segmental necrotising lesions Silver stain - discontinuous GBM IF IgG linear pattern EM GBM wrinkling and rupture |
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Type 1 Rapidly progressive glomerulonephritis Management and prognosis |
Steroids, cytotoxic drugs, plasmapheresis 20% die 60% end stage renal disease 20% recover Can recur in transplants |
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Type 2 Rapidly Progressive Glomerulonephritis Presentation and pathology |
Immune Complex Rapid and progressive loss of renal function - severe oliguria, nephritic syndrome Idiopathic, and immune complex nephritides (eg.Post-infectious GN (most common), IgA nephropathy) or systemic immunologicdisease (e.g. SLE) |
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Type 2 Rapidly Progressive Glomerulonephritis LM, IF, EM |
LM Focal and segmental Crescents - fibrin and macrophages Segmental necrotising lesions Silver stain - discontinuous GBM IF IgA granular - HSP/IgA glomerulonephritis IgG+C1q - SLE EM GBM wrinkling and rupture |
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Type 3 Rapidly Progressive Glomerulonephritis Presentation and Pathology |
Pauci Immune Rapid and progressive loss of renal function - severe oliguria, nephritic syndrome Neither immune complex or anti-GBM antibodies Majority are ANCA positive pANCA MPO - microscopic polyangitis cANCE PR3 - Wegeners granulomatosis Churg strauss - inflammation, eosinophils and asthma |
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Type 3 Rapidly Progressive Glomerulonephritis LM, IF, EM |
LM Focal and segmental Crescents - fibrin and macrophages Segmental necrotising lesions Silver stain - discontinuous GBM IF Negative EM GBM wrinkling and rupture |
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Type 3 Rapidly Progressive Glomerulonephritis Management and prognosis |
Steroids AND cytotoxic drugs Remission rate better the significant immunosuppressants than with corticosteroid alone |
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Congenital Renal Abnormalities |
10% of live births Agenesis Hypoplasia Ectopic kidney Horseshoe kidney Obstruction of pelviureteric junction Double ureters |
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Renal Agenesis |
Bilateral Not compatible with life Potter sequence - lung hypoplasia, flat face and low set ears, developmental defects of extremeties. Unilateral. Compatible with life unless other congenital abnormalities are severe/ Leads to hypertrophy of existing kidney -> hyper filtration -> increased risk of renal failure later in life |
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Renal hypoplasia |
Renal failure occurs in early childhood if bilateral. If unilateral - difficult to differentiate from atrophy from other causes - No scars, reduced number of renal lobes and pyramids. |
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Ectopic kidney |
Kidney in an abnormal location - usually lower in the abdomen. May or may not be smaller in size. Ureters can become kinked -> outflow obstruction --> hydronephrosis |
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Double ureters |
Results from split in the ureteric bud. Often associated with double renal pelvis. If unilateral - no clinical significance |
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Adult Polycystic kidney disease Inheritance, presentation and prognosis |
Autosomal dominant inheritance (PKD1- 85%, PKD2-15%) Asymptomatic until 20-30s Insidious onset haematuria Progressive kidney disease Flank pain UTI Hypertension 40% die of coronary or hypertensive heart disease 25% die of infection 15% die from ruptured berry aneurysm/hypertensive cerebral haemorrhage |
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Adult Polycystic kidney disease Gross morphology and extra-renal features |
Bilaterally enlarged Mass of cysts 3-4 cm Cysts filled with clear serious fluid to turbid red brown fluid No obvious intervening parenchyma Cysts visible on outside of kidney Polycystic liver disease Berry aneurysms Mitral valve prolapse Cysts in spleen, pancreas, lung |
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Childhood Polycystic Kidney disease Inheritance, presentation and prognosis |
Autosomal recessive PKHD1 - chromosome 6p - encodes an integral membrane protein Serious manifestations usually present at birth 4 subcategories - perinatal, neonatal, infantile, juvenile Variable prognosis |
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Childhood Polycystic Kidney disease Gross morphology and extra-renal features |
Bilaterally enlarged kidneys with smooth external appearance Numerous cysts in cotex and medulla give sponge-like appearance Liver cysts Portal fibrosis -> congenital hepatic fibrosis -> signs and symptoms of portal hypertension |
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Multicystic renal dysplasia Presentation and prognosis |
If bilateral - renal failure Unilateral - good prognosis, can be corrected with surgery |
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Multicystic renal dysplasia Gross morphology and extra-renal features |
Unilateral or bilateral enlarged kidney(s) Irregular with cysts of variable size Urinary tract abnormalities |
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Medullar sponge kidney Presentation, gross morphology and prognosis |
Haematuria, UTI, recurrent renal stones Multiple systemic dilatations of collecting ducts in medulla Excellent prognosis |
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Nephronothisis-medullary cystic disease complex Inheritance and presentation |
Can be sporadic, autosomal recessive or dominant Sporadic - 20% Familial juvenile - 40-50% - AR Renal-retinal dysplasia - 15% - AR Adult-onset medullar cystic disease - 15% - AD Polyuria and polydipsia |
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Nephronothisis-medullar cystic disease complex Gross morphology and extra-renal features |
Small kidneys with contracted granular surface Cysts in medulla - particularly at aorticomedullar junction Depending on variant Ocular motor abnormalities Retinitis pigmentosa Liver fibrosis |
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Acquired renal cystic disease |
Sporadic Haemorrhage, erythrocytosis, neoplasia Simple or multiple cysts, usually in cortex Occurs with dialysis |
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Haemolytic uraemic syndrome |
Triggered by shiga-like toxin produced by bacteria - e.g. E. coli Presents with influenza-like/diarrhoeal prodrome - sudden onset harmatemesis/malena, oliguria, haematuria, microangiopathic haemolytic uraemia, thrombocytopenia +/- neurological changes Excessive platelet activation |
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Malignant nephrosclerosis |
Malignant hypertension -> vascular injury -> increased endothelial permeability -> focal death of cells, platelet deposition -> fibrinoid necrosis and hyperplastic arteriolitis Onion skinning 'Flea-bitten' kidney |
