Inheritance of Protein-Losing Enteropathy and Nephropathy
A common syndrome of protein-losing enteropathy (PLE) and/or protein-losing nephropathy (PLN) has been documented in male and female Soft Coated Wheaten Terriers (SCWT). PLE is associated with eosinophilic or lymphocytic/plasmacytic inflammatory bowel disease and is usually diagnosed at an earlier age (mean 4.5 years) than PLN (mean 6 yrs), which is the result of focal mesangioproliferative and sclerosing glomerulonephritis. Although analysis of pedigrees from SCWT at large revealed a common male ancestor, the exact mode of inheritance could not be determined.
of Soft Coated Wheaten Terriers
Published in the Journal of Veterinary Internal Medicine, 2002, 16:352.
The purpose of this study was to further characterize the mode of inheritance of PLE and PLN in SCWT by three types of breedings: Group 1 consists of a litter of 6 SCWT (3 M, 3 F) born to an affected female x affected male SCWT. Group 2 represents a litter of 4 SCWT (1 M, 3 F) born to a brother x sister mating of Group 1 dogs. Group 3 involves an outcross of an affected male SCWT from Group 1 to a healthy Beagle and resulted in 8 (4 M, 4 F) offspring. These dogs were maintained at the North Carolina State University, fed a standard diet, and were serially evaluated through serum biochemical profiles, CBCs, urinalyses, urine protein:creatinine ratios (normal <1) and Fecal Alpha1 Protease Inhibitor (AlphaPI; normal <5.67 ug/g) every 3 months, small intestinal biopsy and renal ultrasound every 6 months and renal biopsy every 12 months. In order to make a diagnosis of PLE and/or PLN, dogs needed to have both laboratory and histopathologic abnormalities that were consistent with the diseases.
In Group 1 SCWT, 6 of 6 developed PLE (n=1), PLN (3) or both (2); the present age of 2 survivors (PLE/PLN, 1; PLN, 1) is 7 yrs. Both of the parents of Group 2 but none of the 4 dogs in Group 2, developed PLN at 4.5 yrs of age, however, 3 of them have PLE (1 dog appears to have no evidence of disease, but has just reached the mean age to develop PLE). Of the 8 outcross dogs in group 3 (present age 5.5 yrs), 1 has PLE/PLN and 1 has PLN.
Although the breeding of affected to affected SCWT in Group 1 and 2 producing primarily affected SCWT is consistent with an autosomal recessive mode of inheritance, the development of PLE and PLN in the SCWT x Beagle outcross suggests an autosomal dominant trait. The risk for SCWT to develop PLE and PLN is clearly inherited, however, further breeding and clinical follow up is needed to reach a definitive conclusion.