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Fibronectin Glomerulopathy is a Rare Cause of Nephrotic Syndrome

2012-12-15 11:47

Fibronectin Glomerulopathy is a hereditary kidney disease due to the deposition and accumulation of proteins in the glomeuruli and cause damages of the kidney structure and functions. It is a much rarer cause of nephrotic syndrome in clinic compared with other pathogenic factors.

There are two types of fibronectin---soluble and insoluble. The former is found in the blood circulation and the latter exists in the basement membranes and extracellular matrix. When there is more than normal fibronectin deposition in the kidneys, it will cause renal damages and decline of kidney functions.

It can occur to any ages and the peak age of its onset is adolescents and young adults. It has the typical symptoms of nephrotic syndrome including hypertension, proteinuria, hypoalbuminemia, edema (first appears around the eyes, ankles and legs and in serious conditions will affect the whole body) and microscopic hematuria. In addition, other common symptoms include excessive urine albumin level, renal tubular acidosis, etc. Renal functions will suffer from various degrees of damages and some can even progress into end stage renal disease.

Diagnosis for fibronectin glomerulopathy needs to be confirmed by renal biopsy. And since it is hereditary, family history of the disease can also help the diagnosis.

There is at present no specific treatment for fibronectin glomerulopathy besides symptomatic ones such as ACEI for hypertension and proteinuria, diuretics for swelling and corticosteroids. And when it develops into end stage renal disease, dialysis or kidney transplant will be required.

Since it is hereditary, genetic counseling can help parents who have fibronectin glomerulopathy find out weather the children have the risk of developing the disease later in life and offer early and proper prevention measures and treatments for slowing down the illness progression and protecting kidney functions.


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