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

Autori
Ćorić, Marijana ; Brčić, Iva ; Brčić, Luka ; Dotlić, Snježana ; Kuzmanić, Duško

Vrsta, podvrsta i kategorija rada
Sažeci sa skupova, sažetak, stručni

Izvornik
Virchows Archiv / - , 2009, S404-S404

Skup
22nd European Congress of Pathology

Mjesto i datum
Firenca, Italija, 04.09.2009. - 09.09.2009

Vrsta sudjelovanja
Poster

Vrsta recenzije
Međunarodna recenzija

Ključne riječi
glomerulopathy; fibronectin

Sažetak
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.

Izvorni jezik
Engleski

POVEZANOST RADA