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Children With Protein-Losing Enteropathy After the Fontan Operation Are at Risk for Abnormal Bone Mineral Density

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Abstract

Protein-losing enteropathy (PLE) is a rare but potentially devastating complication of single-ventricle physiology after the Fontan operation. Although abnormal bone mineral density (BMD) is a known complication of chronic disease and congenital heart disease, no reports have described BMD in patients with PLE. This study investigated a cross-sectional sample of children and young adults with a confirmed diagnosis of PLE. Serum levels of 25(OH)D, calcium, total protein, and albumin were recorded from the first outpatient encounter with each subject. Corrected calcium (cCa) was calculated from the serum calcium and albumin levels. Dual-energy X-ray absorptiometry (DXA) was used to measure BMD, and z-scores were generated using appropriate software. DXA results were available for 12 patients (eight males and four females). The age at DXA ranged from 7.2 to 25.2 years. The mean z-score was −1.73 standard deviation (SD) for the entire cohort, with 42 % z-scores below −2 SDs. Serum 25(OH)D levels were abnormal in 58 % of the patients. There was a positive correlation between cCa and DXA z-score and a negative correlation between total protein and DXA z-score. Patients receiving corticosteroid therapy had a significantly lower DXA z-score than those not receiving corticosteroids (−3.15 vs. −0.31; p = 0.02). Children with PLE are at risk for abnormal BMD compared with age- and sex-matched control subjects. In the study cohort, corticosteroid exposure, a marker of disease severity, appeared to be associated with decreased BMD. Routine bone health screening is warranted for children with PLE, particularly those receiving corticosteroid therapy.

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Authors and Affiliations

  1. Division of Cardiology, The Children’s Hospital of Philadelphia, 34th Street and Civic Center Blvd, Philadelphia, PA, 19104, USA

    David J. Goldberg, Kathyrn Dodds, Catharine M. Avitabile, Andrew C. Glatz & Jack Rychik

  2. The Mid-Hudson Medical Group, Poughkeepsie, NY, USA

    Jill L. Brodsky

  3. Division of Gastroenterology, The Children’s Hospital of Philadelphia, Philadelphia, PA, USA

    Edisio J. Semeao & Elizabeth B. Rand

  4. Division of Endocrinology, The Children’s Hospital of Philadelphia, Philadelphia, PA, USA

    Edna E. Mancilla

  5. Department of Pediatrics, The Perelman School of Medicine at The University of Pennsylvania, Philadelphia, PA, USA

    David J. Goldberg, Andrew C. Glatz, Edisio J. Semeao, Elizabeth B. Rand, Edna E. Mancilla & Jack Rychik

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  1. David J. Goldberg
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  2. Kathyrn Dodds
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  3. Catharine M. Avitabile
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  5. Jill L. Brodsky
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  6. Edisio J. Semeao
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  7. Elizabeth B. Rand
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  9. Jack Rychik
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Correspondence to David J. Goldberg.

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Goldberg, D.J., Dodds, K., Avitabile, C.M. et al. Children With Protein-Losing Enteropathy After the Fontan Operation Are at Risk for Abnormal Bone Mineral Density. Pediatr Cardiol 33, 1264–1268 (2012). https://doi.org/10.1007/s00246-012-0290-z

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  • DOI: https://doi.org/10.1007/s00246-012-0290-z

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