Minimal change disease

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Minimal change disease:

Minimal change disease Dr Mohammed Abdelsattar

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defined by nephrotic syndrome With normal appearing light microscopy with foot process effacement on electron microscopy in the absence of cellular infiltrates or immune deposits. Most common form of nephrotic syndrome in children .

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25% of cases of nephrotic syndrome in adult third most common cause of nephrotic syndrome in adults after membranous nephropathy and focal, segmental glomerulosclerosis E xcellent prognosis

Pathophysiology of minimal change :

Pathophysiology of minimal change  disorder of T-lymphocytes . These T cells are thought to release a cytokine - so-called permeability factor - that injures the glomerular epithelial cells. The identity of this permeability factor is still uncertain. Epithelial cell damage may lead to albuminuria in MCNS

Criteria for diagnosis:

Criteria for diagnosis protein losses (more than 3.5 g in an adult or more than 40 mg/m2/hour in a child) hypoalbuminemia , hyperlipidemia , and usually edema .

Aetiology:

A etiology I diopathic Secondary Drugs - Nonsteroidal anti-inflammatory drugs (NSAIDs), rifampin , interferon, ampicillin /penicillin, trimethadione , mercury-containing cosmetic skin cream Toxins - Mercury, lithium, bee stings, fire coral exposure Infection - Infectious mononucleosis, HIV, immunization Tumor - Hodgkin lymphoma (most commonly), carcinoma, other lymphoproliferative diseases

C/P:

C/P Oedema A scites pleural and pericardial effustion F oamy urine P rotinuria A bdominal pain due to spontaneous peritonitis

histology:

histology Renal biopsy shows normal glomeruli by light microscopy but will show effacement of the podocyte foot processes by electron microscopy. Immunofluorescent microscopy typically is negative , although some patients may show staining for immunoglobulin M ( IgM ) in the mesangial regions of the glomeruli

Normal foot processes:

Normal foot processes

Effacement of foot processes:

Effacement of foot processes

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patient cannot be said to have MCD with certainty without having had a kidney biopsy many young children with nephrotic syndrome have MCD kidney biopsies are only performed in those children with atypical findings or in those who are resistant to immunosuppressive therapy

Initial therapy for MCD in adults:

Initial therapy for MCD in adults single morning dose of prednisone or prednisolone be given 1 mg/kg/day , maximum of 80 mg , is continued for a minimum of 8 weeks .

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For those patients not in remission at 8 weeks , daily prednisone may be continued for another 2 months until remission is attained. A gradual taper is then recommended on an every-other-day schedule until the patient is tapered off over many months.

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In patients who remit we suggest that corticosteroids be tapered slowly over a total period of up to 6 months after achieving remission For patients with relative contraindications or intolerance to high-dose corticosteroids (e.g. uncontrolled diabetes, psychiatric conditions, severe osteoporosis) suggest oral cyclophosphamide or CNIs .

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We suggest using the same initial dose and duration of corticosteroids for infrequent relapses MCD in children is exquisitely sensitive to corticosteroids; however , adults tend to respond more slowly, with responses occurring as late as 3–4 months after starting Therapy

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The optimal method to taper corticosteroids in adults is not known, but corticosteroids are commonly tapered by 5–10 mg/wk or less after achieving remission, for a total period of corticosteroid exposure of at least 24 weeks

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Corticosteroid-resistant MCD MCD patients are steroid-resistant failed 16 weeks of daily or alternate-day corticosteroids as Steroid resistance may be due to undetected FSGS (which may not be seen in a biopsy specimen because it is a focal lesion). A repeat biopsy could be considered and may show FSGS, which is associated with a worse prognosis than MCD Re- evalulate patients who are corticosteroidresistant for other causes of nephrotic syndrome .

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We suggest that MCD patients who have AKI be treated with renal replacement therapy as indicated, but together with corticosteroids, as for a first episode of MCD . (2D)

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We suggest that, for the initial episode of nephrotic syndrome associated with MCD , statins not be used to treat hyperlipidemia , and ACE-I or ARBs not be used in normotensive patients to lower proteinuria . (2D)

sources:

sources Oxford in nephrology and hypertention Nephrology secrets KDIGO guidelines GN

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THANKS

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