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National trends in pediatric systemic lupus erythematosus hospitalization in the United States: 2000-2009.

ABSTRACT: In the setting of recent healthcare advances and emphasis on reduced spending, we aimed to characterize US trends in inpatient healthcare use and mortality for pediatric systemic lupus erythematosus (SLE).We performed a retrospective, serial, cross-sectional analysis of the national Kids' Inpatient Database (for 2000, 2003, 2006, and 2009). We identified patients with SLE aged 2 to 21 years using an International Classification of Diseases, 9th revision (ICD-9) code of 710.0 listed as a discharge diagnosis. Using sampling weights, we estimated trends in hospitalization, inpatient mortality, procedure rates, and length of stay (LOS). We analyzed patient and hospital-specific risk factors for mortality and LOS, and compared those outcomes to those without SLE.We identified 26,903 estimated pediatric SLE hospitalizations. The hospitalization rate of 8.6 (95% CI 7.6-9.6) per 100,000 population and mean LOS of 5.9 days (95% CI 5.6-6.2) were stable over time. We found a significant downward trend in mortality, decreasing from 1% to 0.6% (p = 0.04), which paralleled a less pronounced trend for those without SLE. The rate of dialysis, blood transfusions, and vascular catheterization procedures increased. Patients with SLE nephritis and non-white race were at risk for increased healthcare use and death.Pediatric SLE hospitalization rate and LOS remained stable, but inpatient mortality decreased as the rate of common therapeutic procedures increased. More research is needed to understand the drivers of these relationships.

SUBMITTER: Knight AM

PROVIDER: S-EPMC4789141 | biostudies-literature | 2014 Mar

REPOSITORIES: biostudies-literature

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National trends in pediatric systemic lupus erythematosus hospitalization in the United States: 2000-2009.

Knight Andrea M AM Weiss Pamela F PF Morales Knashawn H KH Keren Ron R

The Journal of rheumatology 20140201 3

<h4>Objective</h4>In the setting of recent healthcare advances and emphasis on reduced spending, we aimed to characterize US trends in inpatient healthcare use and mortality for pediatric systemic lupus erythematosus (SLE).<h4>Methods</h4>We performed a retrospective, serial, cross-sectional analysis of the national Kids' Inpatient Database (for 2000, 2003, 2006, and 2009). We identified patients with SLE aged 2 to 21 years using an International Classification of Diseases, 9th revision (ICD-9) ...[more]

PMID: 24488419

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Project description:Systemic lupus erythematosus (SLE) is a chronic, rare autoimmune disease. In recent years, multiple monogenic diseases with early onset autoimmunity and lymphoproliferation have been identified, such as autoimmune lymphoproliferative syndrome, rat sarcoma (RAS)-associated autoimmune leukoproliferative disease, signal transducer and activator of transcription 3 gain-of-function syndrome and interleukin-2 receptor α deficiency. Therefore, we performed whole-exome sequencing in children with SLE with lymphoproliferation to identify genes associated with these conditions.We enrolled 7 patients with SLE with lymphoproliferation from different families. Demographic data, clinical manifestations, laboratory and histopathologic findings, treatment, and outcome were documented. Whole-exome sequencing was performed in 7 patients and their families. Suspected variants were confirmed by Sanger sequencing. Protein levels were detected in patients with gene mutations by western blot.Four patients were male, and 3 were female. No consanguinity was reported within the 7 families. The average age at onset was 5.0 years (range: 1.2-10.0 years). The most common features were renal (7/7 patients) and hematologic (6/7 patients) involvement and recurrent fever (6/7 patients), while only 2 patients presented with skin involvement. Antinuclear antibodies at a titer of ≥1:320 were positive in all patients. All patients fulfilled four 2019 European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) criteria for the classification of SLE. We identified a somatic activating NRAS variant (c.38 A>G, p.G13C) in peripheral venous blood from 4 patients, at levels ranging from 8.8% to 42.8% in variant tissues that were absent from their parents. B cell lymphoma (BCL)-2-interacting mediator of cell death levels in peripheral blood mononuclear cells from 4 patients were markedly reduced, whereas those in the control were normal. Another 2 mutations, c.559C>T (p.Q187X) in the TNFAIP3 gene and c.3061G>A (p.E1021K) in the PIK3CD gene were detected in 2 patients.The SLE is a novel phenotype of somatic mutations in the NRAS gene and germline mutations in the PI3CKD gene. These genes, NRAS, TNFAIP3, and PIK3CD, should be considered candidates for children with SLE with lymphoproliferation. If patients with SLE and lymphoproliferation present with renal and hematologic involvement and recurrent fever, they need gene testing, especially male patients.

Dataset Information

National trends in pediatric systemic lupus erythematosus hospitalization in the United States: 2000-2009.

Publications

National trends in pediatric systemic lupus erythematosus hospitalization in the United States: 2000-2009.

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