Samoyed hereditary glomerulopathy

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Shuskey

Samoyed hereditary glomerulopathy is a genetic disorder that affects the kidneys of Samoyed dogs. This condition is characterized by the progressive deterioration of the glomeruli, which are the tiny filtering units within the kidneys. The disease is similar to Alport syndrome in humans.

Genetics

Samoyed hereditary glomerulopathy is an X-linked disorder, meaning the defective gene responsible for the condition is located on the X chromosome. As a result, male Samoyeds are more severely affected than females. Female carriers may show mild symptoms or remain asymptomatic, while affected males typically exhibit severe kidney dysfunction.

Pathophysiology

The disease primarily affects the glomerular basement membrane (GBM), leading to its thickening and splitting. This structural damage impairs the kidney's ability to filter blood properly, resulting in proteinuria (excessive protein in the urine) and progressive renal failure.

Clinical Signs

Symptoms of Samoyed hereditary glomerulopathy usually appear between 3 to 4 months of age in affected males. Common clinical signs include:

• Proteinuria
• Hematuria (blood in the urine)
• Edema (swelling)
• Hypertension (high blood pressure)
• Progressive renal failure

Diagnosis

Diagnosis is typically based on clinical signs, family history, and specialized tests such as:

• Urinalysis to detect proteinuria and hematuria
• Renal biopsy to examine the glomeruli and GBM
• Genetic testing to identify carriers and affected individuals

Treatment

There is no cure for Samoyed hereditary glomerulopathy. Treatment focuses on managing symptoms and slowing the progression of kidney damage. Common management strategies include:

• Angiotensin-converting enzyme inhibitors (ACE inhibitors) to reduce proteinuria and control blood pressure
• Dietary modifications to reduce protein intake
• Supportive care for renal failure, such as fluid therapy and dialysis

Prognosis

The prognosis for affected male Samoyeds is generally poor, with most developing end-stage renal disease by 1 to 2 years of age. Female carriers may have a normal lifespan but can pass the defective gene to their offspring.

Prevention

Breeding programs should aim to identify and exclude carriers from breeding to reduce the incidence of the disease. Genetic testing can help identify carriers and affected individuals.

See also

• Alport syndrome
• Glomerulonephritis
• Renal failure
• Proteinuria