The detail information of Fanconi syndrome
Basic Information

Disease Description: A kidney tubular dysfunction of basenjis which leads to glycosuria

Inherit Mode: This is a significant disorder in the Basenji breed, affecting upwards of 10% of dogs. The mode of inheritance is presumed to be autosomal recessive. This disorder has also been seen in other breeds (as below) - mode of inheritance unknown.

Disease Symptom: Dogs that are affected with this disorder usually show signs anywhere from 3 to 7 years of age. The earliest signs are excessive drinking and urination, because the kidneys are unable to conserve water. Because of the loss of nutrients and electrolytes in the urine, your dog may experience weight loss and muscle weakness. The condition may worsen slowly over several years, or may progress rapidly to kidney failure with signs such as poor appetite, extreme loss of weight, lethargy, vomiting, and pale gums due to anemia.Dogs with Fanconi syndrome are prone to urinary tract infections due to the sugar in the urine.

Disease Cause: -

Disease Diagnose: The signs described above can have several causes. Your veterinarian will do blood and urine tests that will point to your dog's kidneys as the cause of the problem. Laboratory findings include lowurine specific gravity, glucosuria with normoglycemia (diagnostic in a Basenji), phosphaturia, and aminoaciduria. The defective urine concentrating ability represents aform of nephrogenic diabetes insipidus; isosthenuria or hyposthenuriamay precede glucosuria. There may be mild proteinuria. As the disease progresses, dogs will developazotemia, hypophosphatemia, variable metabolic acidosis(hyperchloremic), and hypokalemia (which may contribute to the muscleweakness sometimes seen).Unlike other hereditary kidney diseases, theprimary abnormality is functional with little apparent structuralchange. The kidneys are generally grossly normal, and lesions with lightmicroscopy are variable and non-specific.

Treat Method: There is no cure, but there are ways to slow the progression of kidney disease, which your veterinarian will discuss with you. These include making sure your dog always has access to fresh drinking water and, depending on the stage of the disease, feeding a special (high quality, low-protein) diet and the use of some medications which help to support kidney function.Regular monitoring of kidney function is important. For the veterinarian:Management of this disease includes regular monitoring; vitamin, mineral and amino acid replacement; control of metabolic acidosis with sodium bicarbonate; and medical management of uremia of chronic renal failure as necessary. Potassium citrate therapy provides both alkalinization and potassium supplementation.

Breeder Advice: Fanconi syndrome is an important problem in the Basenji breed, affecting 10% or more of dogs. Affected dogs should not be bred and, because the mode of inheritance is unconfirmed, it is safest to avoid breeding either parent or any siblings. One problem for breeders is that the condition may not become evident until 6 or 7 years of age. There is a linked marker DNA test available for Basenjis through the OFA that gives results of probable normal, probable carrier, probable affected, and indecisive.

Disease Description Source: Link

Associated Diseases
Disease Name Other Name Mode of inheritance Link ID Possible OMIM ID Gene
Fanconi syndrome - Autosomal Recessive FAN1
Associated Breeds
iDog Breed Number Breed Name Personality Height Weight Breed Source
CB20 Basenji(African Barkless Dog) Smart, independent, alert, and poised; affectionate with friends, aloof with strangers 43.2 cm (male), 40.6 cm (female) 10.9 kg (male), 10 kg (female) Democratic Republic of the Congo
CB44 Border Terrier Plucky, happy, affectionate 30.5-38.1 cm 5.9-7 kg (male), 5.2-6.4 kg (female) United Kingdom (Scotland, England)
CB147 Labrador Retriever Friendly and outgoing, Labs play well with others 57.2-62.2 cm (male), 54.6-59.7 cm (female) 29.5-36.3 kg (male), 24.9-31.8 kg (female) Canada, United Kingdom (England)
CB170 Norwegian Elkhound Confident, dependable; dignified but friendly 52.1 cm (male), 49.5 cm (female) 24.9 kg (male), 21.8 kg (female) Norway
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