1. 1. Glomerular diseases
2. 2. Glomerular diseases account for a significant proportion of acute and chronic kidney disease. There are many causes of glomerular damage, including immunological injury, inherited diseases such as ,metabolic diseases), and deposition of abnormal proteins such as amyloid in the glomeruli. The response of the glomerulus to injury and hence the predominant clinical features vary according to the nature of the insult. Most patients with glomerular disease do not present acutely and are asymptomatic until abnormalities are detected on routine screening of blood or urine samples.
3. 3. Diagnosis of glomerular diseases Urine tests The urinalysis may show red blood cells (which are seen when there is damage or inflammation in the glomeruli), white blood cells ( indicate inflammation), or increased protein levels ( indicator of glomerular damage). Blood tests Blood tests are used to measure the level of creatinine and blood
4. 4. Nephritic syndrome 1. Haematuria (red or brown urine) Oedema and generalised fluid retention Hypertension Oliguria Nephrotic syndrome 1. Overt proteinuria: usually > 3.5 g/24 hrs (urine may be frothy) 2. Hypoalbuminaemia (< 30 g/L) 3. Oedema and generalised fluid retention 4. Intravascular volume depletion with hypotension, or intravascular expansion with hypertension, may occur
5. 5. Clinical and laboratory features of glomerular injury Leakage of cells and macromolecules across the glomerular filtration barrier Proteinuria: characteristic of diseases that affect the podocyte, scarring and deposition of foreign material Haematuria: characteristic of inflammatory and destructive processes Impaired renal function and reduced GFR Hypertension
6. 6. glomerulonephritis describe all types of glomerular disease, even though some of these (such as minimal change nephropathy) are not associated with inflammation Glomerulonephritis is generally classified in terms of the histopathological appearances
7. 7. Minimal change nephropathy may occur at all ages but accounts for nephrotic syndrome in most children and about one- quarter of adults. caused by reversible dysfunction of podocytes. It is postulated that MCD is a disorder of T cells, which release a cytokine that injures the glomerular epithelial foot processes. This, in turn, leads to a decreased synthesis of polyanions that constitute the normal charge barrier to the filtration of macromolecules, such as albumin. When the polyanions are
8. 8. Focal segmental glomerulosclerosis Primary focal segmental glomerulosclerosis (FSGS) can occur in all age groups. In some patients, FSGS can have specific causes, such as HIV infection, podocyte toxins and massive obesity, but in most cases the underlying cause is unknown (primary FSGS). Patients with primary FSGS present with massive proteinuria and idiopathic nephrotic syndrome. Histological analysis shows
9. 9. Membranous glomerulonephritis
10. 10. Membranous glomerulonephritis, also known as membranous nephropathy _common cause of nephrotic syndrome in adults. _ antibodies (usually autoantibodies) directed at antigen(s) expressed on the surface of podocytes. _other causes, such as heavy metal poisoning, drugs, infections and tumours but most are idiopathic. Approximately one-third of patients with idiopathic membranous glomerulonephritis undergo spontaneous remission; one-third remain in a nephrotic state, and one-third go on to develop CKD. - _Short-term treatment with high doses of corticosteroids and cyclophosphamide may improve both the nephrotic syndrome and the long-term prognosis. _ toxicity of these regimens: nephrologists reserve such treatment for those with severe nephrotic syndrome or deteriorating renal function.
11. 11. IgA nephropathy
12. 12. sangial deposition pattern
13. 13. _One of the most common types of glomerulo nephritis and can present in many ways. _Haematuria is the earliest sign and is almost universal, and hypertension is also very common. _Proteinuria can also occur but is usually a later feature. In many cases, there is slowly progressive loss of renal function leading to ESRD. _Clinical presentations are protean and vary with age. _A particular hallmark of IgA nephropathy in young adults is the occurrence of acute self limiting exacerbations, often with gross haematuria, in association with minor respiratory
14. 14. HenochSchnlein purpura most commonly occurs in children but can also be observed in adults. It is characterised by a systemic vasculitis that often arises in response to an infectious trigger. characteristic petechial rash typically affecting buttocks and lower legs, and abdominal pain due to the occurrence of vasculitis involving the gastrointestinal tract. The presence of glomerulonephritis is usually indicated by the occurrence of haematuria.
15. 15. Mesangiocapillary glomerulonephritis " membranoproliferative " MPGN increase in mesangial cellularity with thickening of glomerular capillary walls and subendothelial deposition of immune complexes and/or components of the complement pathway. The typical presentation is with proteinuria and haematuria. can be classified into two main subtypes:
16. 16. Post-streptococcal glomerulonephritis case: 12 year old girl present in distress to the doctor : complaint: rusty color urine , puffy face on physical exam she had high blood pressure and a urine test stripe revealed proteinuria. On hearing past history the physician noted that she had recovered from a strep trhoat infection following an antibiotic treatment. Blood tests suggested an autoimmune response damaging the nephrons.
17. 17. _much more common in children than adults but is now rare in the developed world. _The latency is usually about 10 days after a throat infection or longer after skin infection, suggesting an immune mechanism rather than direct infection. _ An acute nephritis of varying severity occurs. Sodium retention, hypertension and oedema are particularly pronounced. _also reduction of GFR, proteinuria, haematuria and reduced urine volume. Characteristically, this gives the urine a red or smoky appearance. As in other causes of post-infectious glomerulonephritis, serum concentrations of C3 and C4 are typically reduced, reflecting complement consumption and evidence of streptococcal infection may be found. _ Renal function begins to improve spontaneously within 1014 days, and management by fluid and sodium restriction with diuretic and hypotensive agents is usually adequate. Remarkably, the renal lesion in almost all children and many adults seems to resolve com-pletely, despite the severity of the glomerular inflamma-tion and proliferation seen histologically.
18. 18. Rapidly progressive glomerulonephritis Rapidly progressive glomerulonephritis ( crescentic glomerulonephritis) is characterised by rapid loss of renal function over days to weeks. Renal biopsy shows crescentic lesions, often associated with necrotising lesions within the glomerulus, termed focal segmental (necrotising) glomerulonephritis. It is typically seen in Goodpastures disease, where the underlying cause is the development
19. 19. crescentic glomerulonephritis
20. 20. Inherited glomerular diseases AIports syndrome most important one affecting adults is Alports syndrome. -Most cases arise from a mutation or deletion of the COL4A5 ( X-linked recessive disorder.) -Mutations in COL4A3
21. 21. ACE inhibitors may slow but not prevent loss of kidney function. Patients with Alports syndrome are good candidates for RRT, as they are young and usually otherwise healthy. They can develop an immune response to the normal collagen antigens present in the GBM of the donor kidney and, in a small minority, anti-GBM disease develops and destroys the allograft.
22. 22. Thin glomerular basement membrane disease In thin glomerular basement membrane disease there is glomerular bleeding, usually only at the microscopic or dipstick level, without associated hypertension, proteinuria or a reduction in GFR. The glomeruli appear normal by light microscopy but, on electron microscopy, the GBM is abnormally thin. The condition may be familial and some patients are carriers of Alport mutations. This does not appear to account for all cases, and in many