java barcode scanner library IGA NEPHROPATHY & HENOCH-SCH NLEIN PURPURA 1 IgA Nephropathy in Objective-C

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IGA NEPHROPATHY & HENOCH-SCH NLEIN PURPURA 1 IgA Nephropathy
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IgA nephropathy (Berger s disease) is a primary renal disease of IgA deposition in the glomerular mesangium The inciting cause is unknown, but the same lesion is seen in Henoch-Sch nlein purpura IgA nephropathy is also associated with hepatic cirrhosis, celiac disease, and infections such as with HIV and cytomegalovirus IgA nephropathy is the most common form of acute glomerulonephritis in the United States and is even more prevalent worldwide, particularly in Asia It is most commonly seen in children and young adults, with males affected two to three times more commonly than females An episode of gross hematuria is the most common presenting complaint Frequently, this is associated with an upper respiratory infection (50%), gastrointestinal symptoms (10%), or a flu-like illness (15%) The urine becomes red or cola-colored 1 2 days after onset In contrast to postinfectious glomerulonephritis, this feature has been called synpharyngitic hematuria since there is no significant latent period Other findings include asymptomatic microscopic hematuria as an incidental finding and the nephrotic syndrome (see below) Approximately one-third of patients will experience a clinical remission Forty to 50 percent of patients will have progressive renal insufficiency The remainder will show chronic microscopic hematuria and a stable serum creatinine
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A Symptoms and Signs
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The patient is oliguric, edematous, and variably hypertensive
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B Laboratory Findings
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Serum complement levels are low; in postinfectious glomerulonephritis due to group A streptococcal infection, ASO titers can be high unless the immune response has been blunted with previous antibiotic treatment Classically, the urine is described as cola-colored Urinary red blood cells, red cell casts, and proteinuria under 35 g/d are present On microscopy, this entity appears as a diffuse proliferative glomerulonephritis Immunofluorescence shows IgG and C3 in a granular pattern in the mesangium and along the capillary basement membrane Electron microscopy shows large, dense subepithelial deposits or humps
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Treatment
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Treatment for this entity is supportive Appropriate antibiotics should be used Antihypertensives, salt restriction, and
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The most unfavorable prognostic indicator is proteinuria greater than 1 g/d; others include hypertension, persistent microscopic hematuria and proteinuria, glomerulosclerosis, and abnormal renal function The serum IgA level is increased in up to 50% of patients, and for that reason a normal serum IgA does not rule out the disease Serum complement levels are usually normal, and renal biopsy is the standard for diagnosis Glomeruli show a focal glomerulonephritis with diffuse mesangial IgA deposits and proliferation of mesangial cells IgG and C3 can also be seen in the mesangium of all glomeruli Skin biopsy often reveals granular deposits of IgA in dermal capillaries of affected patients In patients with significant proteinuria (> 1 g/d), ACE inhibitors or ARB drugs should be used to reduce proteinuria and hypertension The target blood pressure is less than 130/80 mm Hg In patients with proteinuria of 10 35 g/d, corticosteroid therapy has proven beneficial A recent regimen showed a 2% doubling of creatinine after 6 years in the treatment group versus a 21% doubling of creatinine in the control group The regimen consisted of giving methylprednisolone, 1 g/d intravenously, for 3 days during months 1, 3, and 5, plus prednisone in a dosage of 05 mg/kg every other day for 6 months This was aimed at patients with creatinine clearances greater than 70 mL/min Other treatments have included fish oil, with variable results in clinical trials Studies that have shown a benefit also show that low doses (2 5 g/d) are just as efficacious as high doses (9 12 g/d) A 1997 meta-analysis has showed no benefit from fish oil, but there was a trend toward benefit in patients with more proteinuria There are very few side effects of long-term fish oil administration aside from fishy breath and eructations Renal transplantation is an excellent option for patients with ESRD, but recurrent disease has been documented in 30% of patients 5 10 years posttransplant Fortunately, recurrent disease rarely leads to failure of the allograft
PAUCI-IMMUNE GLOMERULONEPHRITIS (ANCA-ASSOCIATED)
Pauci-immune glomerular lesions are seen with Wegener s granulomatosis, Churg-Strauss disease, and microscopic polyangiitis All are small-vessel vasculitides Wegener s granulomatosis also involves granulomatous inflammation of the respiratory tract with a necrotizing vasculitis of small and medium-sized vessels Microscopic polyangiitis (polyarteritis) is similar to Wegener s granulomatosis without granulomatous inflammation, but both commonly exhibit a necrotizing glomerulonephritis ANCA-associated glomerulonephritis can also present as a primary renal lesion The pathogenesis of these entities is unknown, but more than 80% of pauci-immune glomerulonephritis is associated with antineutrophil cytoplasmic antibodies
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