A 7 year old girl is brought with complaints of generalized swelling of the body. Urinary examination reveals Grade 3 proteinuria and the presence of hyaline and fatty casts. She has no history of Hematuria. Which of the following statements about her condition is true:
Question Category:
Correct Answer:
No IgG deposits or C3 deposition on Renal biopsy
Description:
Answer is A (No IgG Deposits or C3 deposition on Renal biopsy) The presence of generalized edema significant proteinuria and absence of hematuria in a young (7 year old) girl suggests a diagnosis of Nephrotic syndrome. Minimal change disease (Lipoid nephrosis) is the commonest cause of Nephrotic syndrome in children and is the most likely diagnosis of the patient in question. Minimal chance disease is characterized by absence of IgG deposits and / or complement on immunofluorescence (biopsy specimen) and is therefore the single best answer of choice. IRA Nephropathy and Alpo's syndrome characteristically present with an Acute Nephritic syndrome with recurrent gross hematuria. The patient in question has no hematuria and hence these diagnosis can be excluded. Serum complement C3 levels are characteristically normal in Minimal change disease The Patient in question is a case of Minimal change disease because: Age of Patient Peak age of onset of Minimal change disease is 6 - 8 years Presenting symptoms Peripheral edema / Generalized edema is the characteristic presenting feature of minimal change disease. Hematuria is uncommon in Minimal change disease Laboratory Features Proteinuria (Nephrotic range) is characteristic Hyaline casts and Fatty casts may be seen in minimal change disease. Occasional Hyaline casts and Fatty casts may be seen in Minimal change disease Hyaline Casts Hyaline casts are composed almost exclusively of protein alone and they pass almost unchanged down the urinary tract Small number of these casts may be seen in normal urine and in any condition associated with proteinuria (including Minimal change disease) These casts carry no attached significance and do not necessarily imply an underlying renal disease Fatty Casts Fatty casts are a special type of cellular cast associated with fatty degeneration of tubular epithelium These casts are composed of fatty droplets and protein Presence of these casts suggests a disease associated with nephrotic syndrome such as primary lipoid nephrosis (minimal change) or nephrosis secondary to Diabetes, SLE, amyloidosis etc. Minimal change Disease: Review Most common cause of Nephrotic syndrome in children (80% in children; 20% in adults) Peak Age of onset is between 6-8 years of Age (usually < 10 years) Type of onset : Insidious Clinical features Peripheral Edema: Presenting Feature Hematuria : Uncommon (may be seen in 20-30% of patients) Hypeension V. Rare Nephrotic syndrome is the typical presentation Peripheral edema is the hallmark of Nephrotic syndrome occurring when serum albumin levels become less than 3g/dl Initially dependent Edema > Generalized edema May develop Pleural effusion, pulmonary edema, ascitis Laboratory (Features of Nephrotic syndrome) Proteinuria Hypoalbuminemia Hyperlipidemia/Hyper cholesterolemia (Increased hepatic production of lipids) Hypercoagulabilny Serum Complement `C3' levels are Normal Renal pathology (Biopsy) Investigation Light microscopy Q Electron microscopy Q lmmunofluorescenceQ Observation No abnormality hence the term minimal change Fusion of foot processes Absence of immunoglobulin or complemen No immunoglobulin deposit (IgG) are seen as Renal biopsy Prognosis Prognosis is Good Response to steroids is Excellent Does not progress to Renal Failure Treatments Coieosteroids form the mainstay for treatment of MCD
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