Dataset Information

Diagnostic features, treatment, and outcome of dogs with inflammatory protein-losing enteropathy.

ABSTRACT:

Background

Protein-losing enteropathy (PLE) because of chronic inflammatory enteropathy (CIE) in dogs is often treated with a combination of glucocorticoids and second-line immunosuppressant (SLI). This combined approach might not be necessary in all dogs.

Hypothesis/objectives

To describe diagnostic features and outcomes of dogs with PLE treated with glucocorticoids alone (group P) or with glucocorticoids and SLI (group S).

Animals

Thirty-one dogs with PLE.

Material and methods

Retrospective analysis of signalment data from diagnostic procedures, treatment, and outcome of dogs with CIE/PLE (from 2015 to 2017), using the hospital's digital case database. Dogs with hypoalbuminemia and CIE were included. Because of a stepwise treatment algorithm, dogs were allocated to group P or S. Time to serum albumin concentrations ?20?g/L and survival data were collected. Dogs were additionally categorized by their albumin and cobalamin serum concentrations. Multivariate and univariate analysis as well as Pearson's correlation and Kaplan-Maier survival analysis were performed.

Results

Seventeen dogs were included in group P and 14 in group S. World Small Animal Veterinary Association score of the duodenum was different between groups (P = .05), but none of the other examined data. Median time until serum albumin reached >20?g/L was 13?days. Median survival time after start of treatment was 85?days (range, 13-463?days) in group P and 166?days (range, 8-390?days) in group S.

Conclusion and clinical importance

No routine diagnostic test was predictive of clinical response, treatment group, or outcome. Glucocorticoid treatment alone can be appropriate in dogs with PLE.

SUBMITTER: Salavati Schmitz S

PROVIDER: S-EPMC6766500 | biostudies-literature | 2019 Sep

REPOSITORIES: biostudies-literature

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Publications

Diagnostic features, treatment, and outcome of dogs with inflammatory protein-losing enteropathy.

Salavati Schmitz Silke S Gow Adam A Bommer Nick N Morrison Linda L Mellanby Richard R

Journal of veterinary internal medicine 20190805 5

<h4>Background</h4>Protein-losing enteropathy (PLE) because of chronic inflammatory enteropathy (CIE) in dogs is often treated with a combination of glucocorticoids and second-line immunosuppressant (SLI). This combined approach might not be necessary in all dogs.<h4>Hypothesis/objectives</h4>To describe diagnostic features and outcomes of dogs with PLE treated with glucocorticoids alone (group P) or with glucocorticoids and SLI (group S).<h4>Animals</h4>Thirty-one dogs with PLE.<h4>Material and ...[more]

PMID: 31381203

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Project description:BackgroundCamptodactyly-arthropathy-coxa vara-pericarditis (CACP, OMIM: #208250) syndrome is a rare autosomal recessive disease that can be difficult to recognise not only because of its wide clinical variability but also because of its clinical resemblance to juvenile idiopathic arthritis (JIA). PRG4 is the only gene so far known to be associated with CACP syndrome. Children with CACP syndrome lack the glycoprotein lubricin due to recessive mutations in PRG4. Lubricin serves as a lubricant in joints, tendons and visceral cavities (pleural cavity, pericardium) and inhibits synovial proliferation. Children with CACP syndrome suffer from congenital camptodactyly, arthropathy, coxa vara and sometimes pericarditis. This report concerns a child with CACP syndrome complicated by protein-losing enteropathy (PLE), caused by constrictive pericarditis and so contributes to knowledge of the presentation of CACP syndrome.Case presentationA 10- year-old girl with consanguineous parents suffered from congenital camptodactyly and progressive swollen and painful joints. Her father and his sister had similar childhood-onset joint complaints. Laboratory tests showed no signs of inflammation but showed persistent low protein- and IgG- levels, indicating a secondary immunodeficiency. Increased alpha antitrypsin clearance confirmed PLE. Abdominal ultrasound with Doppler showed hepatomegaly and portal hypertension. Echocardiography suggested constrictive pericarditis. However, heart catheterization could not confirm this. Ultrasound and X-ray examination of the joints combined with a puncture of the synovial fluid were performed. These results, combined with the clinical presentation and the consanguinity, suggested CACP syndrome. Due to excessive enteral protein losses, the patient was treated with Cotrimoxazol prophylaxis and immunoglobulin supplements. These supplements were inadequate to achieve normal IgG values. As constrictive pericarditis with subsequent PLE was the best explanation for the excessive IgG losses, pericardiectomy was performed with good results. Genetic testing in our patient was complicated but revealed a pathogenic mutation within the repeat sequence in exon 7 of the PRG4 gene.ConclusionPLE resulting from constrictive pericarditis can be a complication of CACP syndrome. As serious complications can arise from the resulting secondary immunodeficiency, we recommend regular evaluation of clinical symptoms of constrictive pericarditis and PLE in children with CACP syndrome.

Dataset Information

Diagnostic features, treatment, and outcome of dogs with inflammatory protein-losing enteropathy.

Background

Hypothesis/objectives

Animals

Material and methods

Results

Conclusion and clinical importance

Publications

Diagnostic features, treatment, and outcome of dogs with inflammatory protein-losing enteropathy.

Similar Datasets

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