A 10 y/o female is brought to the pediatrician because her mother noticed blood in her urine and diminished vision acuity.
HPI Her family is Mormon. Her mother suffers from chronic renal failure (CRF)
PE VS: normotension. Patient appears well-nourished; bilateral sensorineural hearing loss; bilateral cataracts.
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Labs CBC/PBS: normochromic, normocytic anemia. Elevated serum creatinine and Blood Urea Nitrogen (BUN). Patient has proteinuria; hematuria with RBC casts (as explained in class!)
Gross pathology Small kidneys
Micro Pathophysiology: Longitudinal thinning and splitting of glomerular basement membrane, producing characteristic laminated appearance with glomerular sclerosis; interstitial infiltrate containing fat-filled macrophages(=LARGE FOAM CELLS). The disease is also called hereditary chronic nephritis. it is mostly caused by a defect in the alpha chain of type IV collagen.
2) Please provide a short term protocol for proper management of this patient. (Your pharmacology should help).