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Pediatric Cardiology

, Volume 33, Issue 8, pp 1264–1268 | Cite as

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

  • David J. Goldberg
  • Kathyrn Dodds
  • Catharine M. Avitabile
  • Andrew C. Glatz
  • Jill L. Brodsky
  • Edisio J. Semeao
  • Elizabeth B. Rand
  • Edna E. Mancilla
  • Jack Rychik
Original Article

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.

Keywords

Bone mineral density Dual-energy X-ray absorptiometry Fontan operation Protein-losing enteropathy 

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Copyright information

© Springer Science+Business Media, LLC 2012

Authors and Affiliations

  • David J. Goldberg
    • 1
    • 5
  • Kathyrn Dodds
    • 1
  • Catharine M. Avitabile
    • 1
  • Andrew C. Glatz
    • 1
    • 5
  • Jill L. Brodsky
    • 2
  • Edisio J. Semeao
    • 3
    • 5
  • Elizabeth B. Rand
    • 3
    • 5
  • Edna E. Mancilla
    • 4
    • 5
  • Jack Rychik
    • 1
    • 5
  1. 1.Division of CardiologyThe Children’s Hospital of PhiladelphiaPhiladelphiaUSA
  2. 2.The Mid-Hudson Medical GroupPoughkeepsieUSA
  3. 3.Division of GastroenterologyThe Children’s Hospital of PhiladelphiaPhiladelphiaUSA
  4. 4.Division of EndocrinologyThe Children’s Hospital of PhiladelphiaPhiladelphiaUSA
  5. 5.Department of PediatricsThe Perelman School of Medicine at The University of PennsylvaniaPhiladelphiaUSA

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