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The Kidney II.pdf pathology slides part 2
- 1. The Kidney and LowerUrinary Tract - II Dr A Rapsang
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- 3. • Glomerular diseases- diseases that primarily involve the renal glomeruli. • 2 groups: – Primary glomerulonephritis • The glomeruli are the predominant site of involvement. – Secondary glomerular diseases • Systemic and hereditary diseases which secondarily affect the glomeruli. • Clinical manifestations • Usually presented with – Proteinuria – Haematuria – Hypertension – Disturbed excretory function • Diagnosis – Renal biopsy
- 4. The following aresix major glomerular syndromes commonly found in different glomerular diseases • Nephritic syndrome • Nephrotic syndromes • ARF • CRF • Asymptomatic proteinuria • Haematuria.
- 5. ACUTE NEPHRITIC SYNDROME. •Acute onset following an infective illness • Haematuria - generally slight – Urine • Smoky appearance • Erythrocytes detectable by microscopy • Red cell casts • Proteinuria - mild (less than 3 gm per 24 hrs) • Hypertension - generally mild. • Oedema - usually mild – Results from sodium and water retention • Oliguria - variable and reflects the severity of glomerular involvement. • The underlying causes may be – Primary glomerulonephritic diseases • Acute glomerulonephritis – Systemic diseases
- 6. NEPHROTIC SYNDROME • Nephroticsyndrome is a constellation of features in different diseases having varying pathogenesis Characterised by • Heavy proteinuria (protein loss of more than 3 gm per 24 hrs) • Hypoalbuminaemia - due to urinary loss of albumin and inadequate hepatic synthesis of albumin. • Oedema – Usually peripheral but in children facial oedema may be more prominent – Due to • Fall in colloid osmotic pressure consequent upon hypoalbuminaemia. • Sodium and water retention
- 7. • Hyperlipidaemia • Lipiduria- due to excessive leakiness of glomerular filtration barrier. • Hypercoagulability – due to loss of anti-coagulants In children, primary glomerulonephritis is the main cause In adults, systemic diseases (diabetes, amyloidosis and SLE) are more frequent causes
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- 12. Acute Glomerulonephritis • AcuteGN follows acute infection and characteristically presents as acute nephritic syndrome. • 2 main groups: – Acute post-streptococcal GN – Acute non-streptococcal GN Acute post-streptococcal gn • Usually affects children between 2 to 14 years • Onset of disease is sudden after 1-2 weeks of streptococcal infection (streptococcal pharyngitis) • Etiopathogenesis. – Group A β-haemolytic streptococci infection
- 13. Grossly • Kidneys are symmetricallyenlarged • Petechial haemorrhages - appearance of flea- bitten kidney
- 14. M/E • Glomeruli -enlarged and hypercellular (inflammatory cells) • Tubules - swelling and hyaline droplets with red cell casts. • Interstitium - edema and leucocytic infiltration. • Vessels – no change
- 15. Acute glomerulonephritis (Electron microscopy) •Irregular deposits (‘humps’) on the epithelial side of the GBM (represent immune complexes)
- 16. Clinical features. • Usuallyyoung children presenting with acute nephritic syndrome • Sudden and abrupt onset following an episode of sore throat or skin infection 1-2 weeks prior to the development of symptoms. • The features include – Microscopic or intermittent haematuria – Red cell casts – Mild proteinuria (less than 3 gm per 24 hrs) – Hypertension – Periorbital oedema – Oliguria.
- 17. • In adults –Sudden hypertension – Edema – Azotaemia. • Complications – Rapidly progressive GN – Chronic GN – Uraemia – Chronic renal failure.
- 18. Membranous Glomerulonephritis • Widespreadthickening of the glomerular capillary wall • Causes – 85% - idiopathic – 15% - secondary to an underlying condition (e.g. SLE, malignancies, infections such as chronic hepatitis B and C, syphilis, malaria and drugs). Grossly • Kidneys are enlarged, pale and smooth.
- 19. M/E • Glomeruli –diffuse thickening • ‘duplication’ of GBM • No cellular proliferation • Tubules – vacuolation • Interstitium – fibrosis and scanty chronic inflammatory cells. • Vessels - hypertensive changes of arterioles may occur.
- 20. Membranous GN Electron microscopy •Subepithelial deposits of electron-dense material so that the basement membrane material protrudes between these deposits.
- 21. Clinical features. • Insidiousonset of nephrotic syndrome • Proteinuria • Microscopic haematuria • Hypertension • Progression to impaired renal function and endstage renal disease with progressive azotaemia occurs in approximately 50% cases • Renal vein thrombosis - due to hypercoagulability.
- 22. IgA Nephropathy (Synonyms:Berger’s Disease, IgA GN) • Characterised by aggregates of IgA, deposited principally in the mesangium. Etiopathogenesis. • Idiopathic in most cases. • Immune-mediated • Increased mucosal secretion of IgA. • HLA-B35 association • Genetically-determined
- 23. Clinical features. • Commonin children and young adults • Recurrent bouts of haematuria (often precipitated by mucosal infections) • Mild proteinuria • Occasionally nephrotic syndrome may develop.
- 24. Chronic Glomerulonephritis (Synonym:End-Stage Kidney) • Irreversible impairment of renal function. • Caused by different glomerular diseases which may progress to chronic GN – Rapidly progressive GN – Membranous GN – Membranoproliferative GN – Focal segmental glomerulosclerosis – IgA nephropathy – Acute post-streptococcal GN • About 20% cases of chronic GN are idiopathic without evidence of preceding GN of any type.
- 25. Grossly • Short contractkidney • The capsule is adherent to the cortex and has diffusely granular cortical surface. Cut section • Cortex is narrow and atrophic • Medulla is unremarkable.
- 26. M/E • Glomerular tuftsare acellular and completely hyalinised. • Blood vessels in the interstitium are hyalinised and thickened • Interstitium shows fine fibrosis and a few chronic inflammatory cells.
- 27. Clinical features. • Thepatients are usually adults. • The terminal stage of chronic GN is characterised by – Hypertension – Uraemia – Progressive deterioration of renal function. – Systemic manifestations of uraemia • These patients eventually die if they do not receive a renal transplant.
- 28. Diabetic Nephropathy • Complicationof diabetes mellitus. • More frequently in type 1 • Patients present with – Asymptomatic proteinuria – Nephrotic syndrome – Progressive renal failure – Hypertension. • Morphologic features - 4 types of renal lesions – Diabetic glomerulosclerosis – Vascular lesions – Diabetic pyelonephritis – Tubular lesions (Armanni-Ebstein lesions).
- 29. Diabetic glomerulosclerosis. • Pathogenesis –Hyperglycaemia → glomerular hypertension → renal hyperperfusion → deposiEon of proteins in the mesangium → glomerulosclerosis → renal failure. • Can be – Diffuse – Nodular Diffuse glomerulosclerosis. • Involvement of all parts of glomeruli. • There is thickening of the GBM • Diffuse proliferation of mesangial cells. • Capsular drop - eosinophilic hyaline thickening of the parietal layer of Bowman’s capsule and bulges into the glomerular space.
- 30. Nodular glomerulosclerosis. • Alsocalled as Kimmelstiel- Wilson (KW) lesions - nodules in the glomerulus. • Nodule – Ovoid acellular mass – Surrounded peripherally by glomerular capillary loops – Contain lipid and fibrin. • As the nodular lesions enlarge, they compress the glomerular capillaries As a result of glomerular and arteriolar involvement, renal ischaemia occurs leading to tubular atrophy and interstitial fibrosis and grossly small, contracted kidney.
- 31. Diabetic nephropathy • Nodular(Kimmelstiel- Wilson or KW) lesions.
- 32. Vascular lesions • Renalischaemia – Atheroma of renal arteries – Arteriolosclerosis affecting the afferent and efferent arterioles of the glomeruli • This results in – Tubular atrophy – Interstitial fibrosis
- 33. Diabetic pyelonephritis • Causedby bacterial infections. • Papillary necrosis result in acute pyelonephritis. • Chronic pyelonephritis can also develop. Tubular lesions (Armanni-Ebstein lesions) • Epithelial cells of the proximal convoluted tubules develop extensive glycogen deposits appearing as vacuoles (k/a Armanni-Ebstein lesions) • The tubules return to normal on control of hyperglycaemic state.
- 34. Assignment • What areglomerular diseases? How many types of glomerular diseases are there? • Differentiating features of nephritic and nephrotic syndrome • Pathogenesis, pathological and clinical features of – Acute glomerular nephritis – Chronic glomerular nephritis • Short notes on – IgA nephropathy – Diabetic nephropathy • What are Armanni-Ebstein lesions?