Children with protein-losing enteropathy after the Fontan operation are at risk for abnormal bone mineral density.

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.

Self-assessment of sexual maturity status in children with Crohn's disease.

OBJECTIVE: To evaluate the validity of self-assessment of sexual maturity status (SMS) in children with Crohn's disease (CD), a pediatric chronic disease that often affects growth and development. STUDY DESIGN: Self-assessment of SMS in 100 children (34 girls) ages 8 to 18 years with CD from the Children's Hospital of Philadelphia was compared with an independent assessment by one well trained pediatrician, using drawings and written descriptions of Tanner stages of breast, genital, and pubic hair development. RESULTS: Overall, subjects with CD (age, 14.3 +/- 2.8 years) had delayed growth status based on height-for-age (HAZ) and weight-for-age Z scores (WAZ). Boys were more growth delayed (HAZ, -0.90 +/- 1.1; WAZ, -0.75 +/- 1.3) than girls (HAZ, -0.45 +/- 1.3; WAZ, -0.20 +/- 1.1). Kappa coefficients (kappa) showed excellent agreement between child and physician assessment of SMS, ranging from kappa = 0.74 to 0.85, depending on sex and SMS component, corresponding to a 79% to 88% agreement. When not in agreement, children tended to overestimate their SMS. Boys who overestimated (n = 8) had significantly higher weight and BMI status than boys who assessed accurately. CONCLUSIONS: Self-assessment of SMS was a reliable and valid method in children with CD and is useful in screening for maturational delay in children with chronic disease.