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Glomerular Diseases (DIABETIC NEPHROPATHY [in nephrotic syndrome]…

- - - - pathogenesis of idiopathic/primary FSGS unknown; probably multifactorial. Possible mechanisms: a T cell–mediated circulating permeability factor, soluble urokinase-type plasminogen activator receptor (suPAR) levels elevated in 2/3 of cases of primary FSGS, TGF-β–mediated cellular prolif and matrix synth, and podocyte abnormalities a. w/ genetic mutations.
      - Secondary causes of FSGS include genetic mutations in podocyte genes, viral infection (HIV, HBV,...), [drugs like heroin, pamidronate; chronic HTN, alport syndrome, lymphoma, radiation, reflux nephropathy which is more common in children; obesity a common cause] sickle cell disease, vesicoureteral reflux, obesity, unilateral renal agenesis, remnant kidneys, and
        aging.
  - - - Primary form: AutoAbs against phospholipase A2 R in podocytes found in 70% of Pxs
      - Secondary: caused by AI diseases (SLE, autoimmune thyroiditis), infection (HBV, HCV), drugs (penicillamine, NSAIDs), and solid malignancies (colon cancer, lung cancer).
- - - - a complication of <<subacute>> bacterial endocarditis, particularly in pxs who remain untreated for a long time, have negative blood cultures, or have right-sided endocarditis. Glomerulonephritis is unusual in <<acute>> bacterial endocarditis because it takes 10–14 days to develop immune complex–mediated injury, by which time the patient has been treated, often with emergent surgery.
        kidneys have subcapsular hemorrhages w/ a “flea-bitten” appearance, and microscopy on renal biopsy reveals focal proliferation around foci of necrosis a. w/ abundant mesangial, subendothelial, and subepithelial immune deposits of IgG, IgM, and C3
        آمبولی ناشی از Vegetation به صورت Stroke های متعدد در ریه و CNS می تواند دیده شود.ایجاد آبسه در نواحی مختلف هم دور از انتظار نیست.
      - Clinical presentation: gross or microscopic hematuria, pyuria, and mild proteinuria or, less commonly, RPGN with rapid loss of renal function. A normocytic anemia, elevated ESR, hypocomplementemia, high titers of rheumatoid factor, type III cryoglobulins, circulating immune complexes, and ANCAs may
        be present
        تغییرات در کلیه حاصل از subacute bacterial endocarditis می توانند به صورت Diffuse proliferative GN ، membranous proliferative GN ، mesangial proliferation باشند
      - Primary treatment: eradication of infection w/ 4–6 weeks of
        antibiotics
        درمان طولانی مدت باکتریال و وژتاسیون های موجود به مدت 6 - 14 هفته
    - - it u. affects children betw ages of 2-14 yrs; develops 2–6 w after skin infection and 1–3 w after streptococcal pharyngitis (by a specific - of group A beta hemolytic strep)
        renal biopsy: endocapillary proliferation; hypercellularity of mesangial and endothelial cells, glomerular infiltrates of PMN leukocytes, granular subendothelial immune deposits of IgG, IgM, C3 , C4, and C5–9 , and subepithelial deposits (which appear as “humps”)
        more prevalent in developing countries
      - Etiology and pathogenesis: an immune mediated disease involving putative streptococcal antigens, circulating immune complexes, and activation of complement in a. w/ cell-mediated injury
      - clinical presentation: an acute nephritic picture w/ hematuria, pyuria, RBC casts, edema, HTN, and oliguric renal failure,
        which may be severe enough to appear as RPGN
        cultures u. negative, but elevated titers of ASO, antihyaluronidase (40%) (these two elevated in pulmonary infection), antistreptokinase,and anti-DNAse B (70%) (elevated in cutaneous infection) abs --> recent streptococcal infection
        Activation of alternative complement pathway reflected by low C3 complement levels. C4 levels u. normal
        cryoglobulinemia
      - Treatment: supportive, aims to minimize fluid overload,
        optimize BP control, and eradicate ongoing infection; For children, the prognosis is excellent, w/ most pxs recovering renal function in 1 to 2 months
        Recurrent poststreptococcal glomerulonephritis is rare
        علائم عموماً بعد از یک تا دو هفته به طور خود به خودی بهبود مییابند ولی پروتئینوری و هماچوری ممکن است ماه ها ادامه یابد که تا یک سال معمولاً بر طرف میشود
- - - - hypogammaglobulinemia, vit D deficiency due to loss of vit D–binding pr, and iron deficiency anemia due to hypotransferrinemia.
      - Thrombotic complications more common, esp in pxs w/ greater pr loss (>10 g/24h) and serum [Alb] <2 g/dL.
      - Pxs w/ severe nephrotic syndrome may also have ARF when there is superimposed V depletion, sepsis, interstitial nephritis, or use of nephrotoxic agents such as NSAIDs
- - - - بیماری کلیوی ریوی. علائم ریوی: از یک rhinorrhea ساده و سینوزیت و ترشحات چرکی و خونی بینی و crusting تا خونریزی در بافت ریه متغیر باشد. علائم کلیوی: از هماچوری تا تخریب کامل کلیه
      - تست ANCA درخواست ولی تشخیص قطعی از طریق بیوپسی. با وجود تست ANCA باز هم تشخیص عموماً به سختی و دیر؛ زیرا سیر بیماری indolent کند و مخفی و ممکن است سالها فرد فقط علائم خفیف ریوی داشته باشد و تحت درمان بیماریهای تنفسی باشد ولی زمانی که کار از کار گذشت وکلیه خراب شد، تازه میفهمند وگنر بوده است. 70-80% بیماران با یافتهی upper respiratory مراجعه.
      - در اثر آنتی بادیهای ضد برخی از علل عفونی که با آنتی ژنهای غشای پایه واکنش متقاطع دارند، ایجاد میشود . سطح کمپلمان نیز نرمال است.
  - - - class I: prognosis excellent, no immunosuppression required.
      - class II
        
        w/ proteinuria<than 1 g/24h-->treated as dictated by extrarenal clinical manifestations of lupus.
        
        w/ proteinuria >3 g/24h--> treated w/ corticosteroids or calcineurin inhibitors.
      - class III or IV:
        induction therapy w/ corticosteroids plus cyclophosphamide or mycophenolate mofetil because both are considered equivalent.
      - Pure class V (membranous): u. benign prognosis, and initial therapy should be supportive. However, pxs w/ progressive or persistent nephrotic-range proteinuria should be treated w/ corticosteroids plus an additional immunosuppressive agent (e.g., cyclosporine, mycophenolate mofetil).
      - pxs w/ ESRD: renal transplantation