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RENAL UNIT CASE 7 |
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Clinical History: A 9 year old girl had been in good health until June of 1976 when her mother observed that she was tired and listless. Her appetite was poor and she would awaken in the morning with puffy eyes. Later in the day, her feet would swell. She had nicturia but no other symptoms referable to the urinary tract. On examination the BP was 95/60 mmHg. Pulse -100/min. T -37.2°C. Her weight was in the 99th percentile, having been in the 55th percentile only six months previously. She had a slightly distended abdomen and ankle edema was confirmed. Urinalysis: protein 4+; Sp. Gr. 1.024. The sediment contained waxy casts but no cells. Serum creatinine was 0.9 mg/dl; serum albumin 1.9 g/dl. Chest X-ray showed small pleural effusions. Questions: 1. What is the clinical diagnosis? How do you explain the various clinical manifestations? 2. What additional clinical information would you want in order to narrow the differential diagnosis? Your instructor will have some of this data. 3. Is it necessary to do a kidney biopsy? 4. A biopsy was done and five glomeruli were available for light microscopy. They all had the appearance shown in Figure 1 (H&E) and Figure 2 (PAS). Can you exclude membranous nephropathy on the basis of the light microscopy? 5. Immunofluorescence microscopy showed that there was no Ig or complement in the glomeruli. Figure 3 shows a representative electron micrograph. What diagnosis would you make at this point? Can you exclude membranous nephropathy? How would you treat? What are the possible outcomes? 6. The patient was treated with Prednisone 1 mg/kg per day and diuretics. The edema decreased and proteinuria decreased to 2.1 g/24 hours. Over the next two years she continued to have intermittent problems with edema and proteinuria. Steroid dosage varied but steroids could never be stopped without the return of severe edema. What would you do? What problems would you be anticipating? |
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