A child with uncomplicated idiopathic nephrotic syndrome usually presents with sudden onset edema without hypertension. Urinalysis reveals massive proteinuria, highly selective for albuminuria with little or no loss of larger serum proteins and no hematuria. Treatment with high dose steroids is usually effective. In a minority of patients, the nephrotic syndrome may relapse, requiring repetitive and sometimes combined immunosuppressive treatment, for which resistance may develop. These relapsing and therapy resistant forms of the nephrotic syndrome are often accompanied by less selective proteinuria, hematuria and systemic hypertension. A third expression pattern of the idiopathic nephrotic syndrome is found in children either at birth or within the first years of life, in whom there is often a family history of kidney disease and where urinary protein loss is therapy resistant from the start leading to end stage renal failure at an early phase in life.
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