Acute glomerulonephritis 1[autosaved]

Acute glomerulonephritis 1[autosaved]

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  MAUREENCHARAGU by Maureen Charagu1
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  Definition Classification Acute streptococcal GN IgAnephropathy MembranoproliferativeGN Henoch Schonlein Purpura Lupus Nephrits Alport syndrome by Maureen Charagu 2
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  Glomerulonephritis is botha clinical and histopathologic term signifying inflammation and proliferation of the glomerulus Severe glomerular histopathologic and clinical entities, such as  anti-GBM antibody disease(Goodpasture syndrome)  Wegener granulomatosis  Idiopathic and Rapidly progressive GN  May be considered as differential diagnosis of GN, but are exceedingly rare in children. by Maureen Charagu 3
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  by Maureen Charagu4 AGN Acquired Infectious Noninfectious Primary renal dx Multisystemic dx Congenital Alport Syndrome
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  1. Primary glomerulonephritis Immune complex GN ▪ Post infectious glomerulonephritis ▪ IgA nephropathy(Berger disease) ▪ Membrenoproliferative glomerulonephritis ▪ Membranous GN (idiopathic)  Anti-GBM antibody mediated GN  Uncertain etiology- Minimal change GN, Focal segmental GN by Maureen Charagu 5
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  Systemic 2. GN associatedwith systemic disorders  Immunologically mediated ▪ Henoch-Schonlein purpura ▪ SLE and other collagen diseases e.g. scleroderma ▪ Vasculitides: polyateritis nodosa, wegener’s granolomatosis ▪ Systemic infections ( subacute bacterial endocarditis, shunt nephritis, syphilis, malaria, hepatitis B, HIV) 3. Hereditary disorders- Alport Syndrome by Maureen Charagu 6
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  1) Asymptomatic hematuriaand / or proteinuria 2) Acute nephritic syndrome  Hematuria  Oliguria  Hypertension  Edema 3) Nephrotic syndrome  Heavy proteinuria  Hypoalbuminemia  Edema with hyperlipidemia by Maureen Charagu 7
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  Follows a recenthistory (7-14days previously) of group A -β hemolytic streptococcal infection involving the pharynx and the skin(impetigo)  GFR= 38* ht (cm)/ plasma creatinine (umol/l) Clinical presentation  Gross hematuriacoffee/ tea colored  Edema (periorbital,facial and extremities) caused by sodium and water retention  Hypretension * headache  Oliguria  Azotemia- an increase in BUN and Creatinine related to a decrease in GFR  Fever is uncommon by Maureen Charagu 8
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  Investigations  Positive culture(throat swab)  Elevated Anti-streptolysinO titer  High titers of other antistreptococcal antibodies  Microscopy- dysmorphic RBC, heme granular casts, pyuria, white cell  Hypocomplementemia esp. C3, C4  CXR- cardiomegally, pleural effusion, pulmonary edema • Increase in serum creatinine • Severe glomerular injury may be accompanied by massive proteinuria (nephrotic) RENAL BIOPSY  In persistent hypocomplementemia and nephrotic syndrome by Maureen Charagu 9
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  Supportive, depends onthe degree of renal impairement and hypretension Antibiotic therapy (penicillin 10day course) if infection is still present Diuretics Antihypertnsives Close monitoring and reduction of salt and water intake In severe renal failure : hemodialysis or peritoneal dialysis Corticosteroids may also be administered  Good prognosis- resolves in 2-3 weeks by Maureen Charagu 10
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  Characterised by IgAdeposition in the mesangium Classic presentation is asymptomatic gross hematuria during acute unrelated illness IgA is elevated in most cases. complement is not depressed Gross hematuria resolves within days, there is no serious sequelae in 85% of cases by Maureen Charagu 11
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  Investigations Hematuria- exclude othercauses (SLE- ANA, Dnase, ASOT) Renal biopsy- presence of mesangial IgA, immunofluorescent deposits with lesser amounts of IgG,IgM Light microscopy – mesangial proliferation and increased mesangial matrix Treatment is not indicated and prognosis is good in most cases In severe proteinuria, hypertension, or renal insufficiency – corticosteroids, immunosuprressive drudg and omega-3 from fish oil are helpful by Maureen Charagu 12
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  Most common smallvessel vasculitis in childhood It is accompanied by arthritis, abdominal pain, bloody diarrhea Approximately 50% of patients develop reanl mainfestations, which vary from asymptomatic microscopic hematuria to severe, progressive glomerulonephritis pathogenesis Deposition of IgA in the glomerulus. This is analogous to the IgA deposits seen in systemic small vessels, primarily those of the skin and intestine The glomerular findings are indistigushable from those of IgA nephropathy by Maureen Charagu 13
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  Clinical presentation  Nephritisfollows onset of the rash, often weeks or months atfer initial presentation of the disease  Nephritic syndrome picture  Nephritic- nephrotic picture Prognosis  In most patients its excellent- spontaneous and complete resolution occurs in those with mild symptoms  Some my progress to chronis renal failure by Maureen Charagu 14
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  by Maureen Charagu15
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  Manifested histologically by alterationsof the GBM, mesangium and proliferation of the glomerular cells Type 1 – 80% of case  Characterised by subendothelial electron dense deposits of C3 $ IgG components in an irregular manner  Good prognosis Responds to corticosteroids Type 2 – Dense Deposit disease  Worst prognosis, less responsive to treatment MPGNType 1  Occurs with other diseases ▪ SLE ▪ Hepatitis B,C ▪ Chronic liver dx ▪ Chronic baterial infections by Maureen Charagu 16
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  systemic lupus erythematosus17
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  Geography. Race. Sex. Affects 1in 700 women of the child bearing age. Age- high incidence 20-29 years Family history Blood group – studies by Baart De La Faille systemic lupus erythematosus 18 Snoop’s daughter has lupusIt’s a disease of the young and old
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  renal involvement isindicated by varying degrees of hematuria and protenuria, accompanied by renal insufficiency and hypretension Significant renal involvement requires treatment with immunossupressive drugs and close monitoring ESRD develops in 10-15% of children with SLE by Maureen Charagu 19
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  Alport Syndrome  Itis a chornic form of GN and thus does not present with the clinical features seen in patients with an acute process  X linked  Related to mutation of gene that encode heterodimers (α3,α4,α5) of type 4 collagen found in ▪ Cochlea (nerve deafness) ▪ Eye- lens dislocation, posterior cataract, corneal dystrophy ▪ Nephritis  ESRD occurs  No treatment, supoortive management for associated hypertension by Maureen Charagu 20
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  by Maureen Charagu21