Mesangial sclerosis, diffuse

Diffuse Mesangial Sclerosis (DMS) is a rare, progressive kidney disease characterized by the hardening (sclerosis) of the mesangial cells in the glomeruli of the kidney. This condition is part of a spectrum of diseases that affect the glomeruli, the tiny blood vessels in the kidneys that filter waste from the blood.

Causes

The exact cause of Diffuse Mesangial Sclerosis is not fully understood, but it is believed to be related to genetic mutations in certain genes, such as the WT1 gene. These mutations can disrupt the normal development and function of the kidney's filtration system. DMS can occur as a sporadic condition or may be part of a genetic syndrome, such as Denys-Drash syndrome or Frasier syndrome.

Symptoms

Symptoms of Diffuse Mesangial Sclerosis typically begin in infancy or early childhood. They may include:

• Proteinuria - excessive protein in the urine, which is a sign of kidney damage.
• Nephrotic syndrome - a collection of symptoms including proteinuria, low blood protein levels, high cholesterol levels, and swelling.
• Hypertension - high blood pressure.
• Progressive kidney failure, leading to the need for dialysis or kidney transplantation in severe cases.

Diagnosis

Diagnosis of Diffuse Mesangial Sclerosis involves a combination of clinical evaluation, laboratory tests, and kidney biopsy. Laboratory tests may show signs of nephrotic syndrome and kidney function impairment. A kidney biopsy, where a small sample of kidney tissue is examined under a microscope, is essential for confirming the diagnosis. The biopsy typically shows sclerosis and thickening of the mesangial areas within the glomeruli.

Treatment

There is no cure for Diffuse Mesangial Sclerosis, and treatment focuses on managing symptoms and slowing the progression of the disease. Treatment options may include:

• Medications to control blood pressure and reduce proteinuria, such as ACE inhibitors or angiotensin II receptor blockers.
• Diuretics to manage swelling.
• Dietary modifications to manage kidney function.
• In cases of advanced kidney failure, dialysis or kidney transplantation may be necessary.

Prognosis

The prognosis for individuals with Diffuse Mesangial Sclerosis varies. The disease tends to progress to end-stage kidney disease (ESKD) in most affected individuals, often within the first decade of life. However, the timing of progression can vary, and some individuals may have a slower course of disease.

See also

• Nephrotic syndrome
• Kidney transplantation
• Dialysis
• Genetic disorders

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