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:
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 diseaseThe Patient in question is a case of Minimal change disease because:Age of PatientPeak age of onset of Minimal change disease is 6 - 8 yearsPresenting symptomsPeripheral edema / Generalized edema is the characteristic presenting feature of minimal change disease.Hematuria is uncommon in Minimal change diseaseLaboratory FeaturesProteinuria (Nephrotic range) is characteristicHyaline casts and Fatty casts may be seen in minimal change disease.Occasional Hyaline casts and Fatty casts may be seen in Minimal change diseaseHyaline CastsHyaline casts are composed almost exclusively of protein alone and they pass almost unchanged down the urinary tractSmall 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 diseaseFatty CastsFatty casts are a special type of cellular cast associated with fatty degeneration of tubular epitheliumThese casts are composed of fatty droplets and proteinPresence 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: ReviewMost 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 : InsidiousClinical featuresPeripheral Edema: Presenting FeatureHematuria : Uncommon (may be seen in 20-30% of patients)Hypeension V. RareNephrotic syndrome is the typical presentationPeripheral edema is the hallmark of Nephrotic syndrome occurring when serum albumin levels become less than 3g/dlInitially dependent Edema > Generalized edema May develop Pleural effusion, pulmonary edema, ascitisLaboratory (Features of Nephrotic syndrome)ProteinuriaHypoalbuminemiaHyperlipidemia/Hyper cholesterolemia (Increased hepatic production of lipids)HypercoagulabilnySerum Complement `C3' levels are NormalRenal pathology (Biopsy)InvestigationLight microscopy QElectron microscopy QlmmunofluorescenceQObservationNo abnormality hence the term minimal changeFusion of foot processesAbsence of immunoglobulin or complemenNo immunoglobulin deposit (IgG) are seen as Renal biopsyPrognosisPrognosis is GoodResponse to steroids is ExcellentDoes not progress to Renal FailureTreatmentsCoieosteroids form the mainstay for treatment of MCD
No IgG deposits or C3 deposition on Renal biopsy
Her C3 levels will be low
IgA Nephropathy is the likely diagnosis
Alpo's syndrome is the likely diagnosis