Cystic fibrosis of pancreas and nephrotic syndrome: a rare association
A nine-year-old male patient diagnosed with CF, lung disease, and pancreatic insufficiency had been monitored for three years. A gradual increase in the ratio of decline in the urine and complaints of swelling of the face and both ankles had begun two years previous. Physical examination revealed the following: temperature, 36.8℃; pulse, 92/min; blood pressure, 109/71 mmHg (95th percentile); edemas in both lower extremities and the sacral region; and more prominent edemas around the face. Other system examinations were normal. Liver function tests, viral hepatitis panel results, alpha-1 antitrypsin levels, and abdominal ultrasonography were normal but sweat test was 98 mmol/L (chloride concentration).\nAt that time he was detected hypoalbuminemia and proteinuria on a random urine sample. Complement factors 3 and 4 were normal, antinuclear antibody was negative, serum urea was 11 mg/dL, triglyceride was 175 mg/dL, total cholesterol was 230 mg/dL, creatinine was 0.6 mg/dL, and albumin was 0.5 g/dL in diagnosis time and 24-hour urine collection revealed nephrotic range proteinuria (>960 mg/m2/day).\nThe patient began steroid therapy following NS diagnosis. At follow-up, the patient was found to have steroid-resistant NS, and a renal biopsy was performed under light microscopy and immunofluorescence microscopy. The renal biopsy showed mesangial proliferation without AA. Partial remission was achieved using low-dose steroids (15 mg/day prednisolone, for a period 1 year) and cyclosporine A therapy (3 mg/kg/day; for a period 4 months). Serum albumin and total protein levels in serum were 3.2 g/dL, 5.5 g/dL after treatment, respectively. Renal biopsy was the second time in the last 6 months. Serum Amyloid A protein was negative. At the moment, our patient has treated with 0.5 mg/kg/every other day prednisone and his proteinuria (30 g/day) has continued. The patient is still in partial remission.\nThe patient's radiological imaging showed destruction around the peripheral airways, and bronchiectasis was detected. Pulmonary function tests provided the following values: forced expiratory volume in 1 second (FEV1) 78% and forced vital capacity (FVC) 85%; FEV1/FVC values were low. The patient was treated with recombinant human DNase, followed by treatment with inhaled bronchodilators and pulmonary rehabilitation.