OBJECTIVE: The prevalence of steroid resistant nephrotic syndrome with histopathological finding of diffuse global glomerulosclerosis (DGGS) is 5 %. DGGS has poor outcome due to the progression of disease to renal failure. We present a twelve-year-and-two-month-old girl with diffuse global glomerulosclerosis steroid resistant nephrotic syndrome.
CASE: A twelve-year-and-two-month-old girl was admitted to Pediatrics Nephrology Ward of DR Wahidin Sudirohusodo Central Hospital, Makassar, with main complaint was swelling. Edema was appear in the eyelids, both legs, and ascites since 20 months before admission. She was treated with oral steroid (prednisone). Patient never resolved despite the treatment. Histopathological result of renal biopsy was DGGS. Laboratory findings revealed: hypoalbuminemia (1.3 gr/dL), proteinuria +3, protein esbach 3 gr/24 hours, CBC Hb 13.4 gr/dL, WBC 11,500/mm3, Plt 438,000/mm3. Kidney function test: (Urea 10 mg/dL, creatinine 0.59 mg/dL, eGFR 194.5 ml/minute/1.73 m2), hypercholesterolemia (total cholesterol 541 mg/dL), hypocalcemia (calcium 5.2 mg/dL), and severe vitamin D deficiency (Total vitamin D 25-OH is 4 ng/ml), normal serum cortisol 4.4 µg/dL. Anti dsDNA (14.6) and ANA profile were negative. Patient was treated with cyclophosphamide and oral steroid (methylprednisolone). Patient was still edematous at the end of therapy.
CONCLUSION: clinical finding and laboratories of DGGS are similar with FGGS’s. DGGS has a poor response either with cyclophosphamide and steroid therapy. It has a poor prognosis.