X-Linked Hereditary Nephropathy, XLHN

Kidney and Bladder

Protein Losing Nephropathy causes inappropriate loss of protein in the urine. Certain parts of the kidney act as a sieve where ions, small molecules like urea, and water are filtered out of the blood into the urine. In PLN, the sieve is compromised and protein moves in the urine. This leads to muscle wasting, abnormal fluid accumulation in the skin and limbs, and kidney failure.

  • Signs and symptoms

    Affected dogs exhibit muscle wasting, abnormal accumulation of fluids in tissue (usually first observed as edema in the limbs), and excessive thirst and urination. PLN dogs are at high risk of clotting problems due to a loss of clotting proteins. Finally, excessive protein is toxic to the kidney, so dogs develop signs of kidney failure: vomiting, poor appetite, lethargy, and changes in thirst and urination.

    Signs typically develop as early as 2-3 months of age and rapidly progress in severity.

    Uncover health risks with Embark

    Embark Breed + Health Test

    Original price:

    $199

    Sale price:

     $159

    Shop

    Embark for Breeders Dog DNA Test

    $129 - $159

    Shop

  • Diagnosis

    PLN is diagnosed through genetic, urine and blood testing. Additional tests, including blood pressure and abdominal ultrasound, may be recommended based on your dog’s clinical signs. There are secondary causes of PLN not associated with genetic mutations.

  • Treatment

    Current therapies for PLN are aimed at reducing the protein load on the kidney and fighting the toxic effects of protein wasting. Dietary and medication recommendations are based on how clinically advanced the PLN is. Dialysis, kidney transplants, and gene therapy are being explored as potential treatment options.

  • What to do if your dog is at risk

    Actions

    • Regular veterinary visits and laboratory testing will be vital to catching this disease in any early stage where it can be more easily managed.
    • Closely monitor your dog for changes in their drinking and urination patterns.

  • Genetic Information

    This mutation was first described in Samoyeds.

    This mutation has a sex-linked recessive inheritance pattern, which means that the gene is on the X chromosome. Females have two X chromosomes and must inherit two abnormal copies to be affected with this disease. Males have only one X chromosome and either have a normal copy of the gene or the mutation. Males with the mutation typically die at 2-15 months of age. Carrier females tend to have excessive protein in their urine, but a normal lifespan.

    Gene names:

    COL4A5 (Exon 35) ‐ chr

    Inheritance type:

    X-linked recessive

  • Breeds affected

    This health condition affects the following breeds

Learn about your dog’s unique genetic health

Dog owners

Breed identification, health and trait insights, personalized care recommendations, and the world’s first canine relative finder—all in one leading dog DNA test.

Learn about the report for dog owners
Shop the test
Breeding programs

Embark’s test for breeding programs is one comprehensive DNA test designed with your needs in mind.

Learn about the report for breeders
Shop the test