engleski

Fibronectin glomerulopathy in a 34-year old male: case report.

Background. Fibronectin glomerulopathy is a rare autosomal dominant familial kidney disease. It presents with proteinuria, microscopic hematuria, and hypertension that lead to end-stage renal failure in the second to sixth decade of life. Methods. The patient is a male who developed low-grade proteinuria at 25 years of age. At 34 years of age he presented with nephrotic-range proteinuria (6g per day), low serum albumin, and severe hypertension (220/130 mmHg). His father had a renal disease and died of myocardial infarction at the age of 33. Renal biopsy showed enlarged glomeruli with massive deposits of fibronectin in the mesangium and subendothelial spaces. These deposits also showed scant immunoreactivity for immunoglobulins and complement proteins. Fibronectin deposits showed focal fuchsinophilia with the SFOG trichrome stain. Fibronectin deposits were detected in the peripheral loop and mesangium by immunohistochemistry. By electron microscopy the deposits in the mesangium and the subendothelial spaces appeared to be arranged in a fine granular pattern. Result. He received a multidrug treatment titrated against urinary protein excretion and after 20 months, urinary protein excretion is about 2 g per day ; blood pressure is 160/90 mm Hg ; and his renal function is now stable. Conclusion. Immunohistochemistry and electron microscopy is essential for diagnosing fibronectin glomerulopathy in patients with positive family history and distinctive clinical findings. If the disease is properly diagnosed the patient's condition can be improved, at least temporarily, with multidrug treatment.

glomerulopathy; fibronectin

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