Andrew J. Klink

Corticosteroids May Improve Clinical Outcomes During Hospitalization for Henoch-Schönlein Purpura

OBJECTIVE: To characterize the effect of corticosteroid exposure on clinical outcomes in children hospitalized with new-onset Henoch-Schönlein purpura (HSP). PATIENTS AND METHODS: We conducted a retrospective cohort study of children discharged with an International Classification of Diseases, Clinical Modification code of HSP between 2000 and 2007 by using inpatient administrative data from 36 tertiary care childrens hospitals. We used stratified Cox proportional hazards regression models to estimate the relative effect of time-varying corticosteroid exposure on the risks of clinical outcomes that occur during hospitalization for acute HSP. RESULTS: During the 8-year study period, there were 1895 hospitalizations for new-onset HSP. After multivariable regression modeling adjustment, early corticosteroid exposure significantly reduced the hazard ratios for abdominal surgery (0.39 [95% confidence interval (CI): 0.17–0.91]), endoscopy (0.27 [95% CI: 0.13–0.55]), and abdominal imaging (0.50 [95% CI: 0.29–0.88]) during hospitalization. CONCLUSIONS: In the hospital setting, early corticosteroid exposure was associated with benefits for several clinically relevant HSP outcomes, specifically those related to the gastrointestinal manifestations of the disease.

Enthesitis in an inception cohort of enthesitis-related arthritis

To describe an enthesitis‐related arthritis (ERA) inception cohort and determine which entheses and joints are most commonly affected.

Variation in Inpatient Therapy and Diagnostic Evaluation of Children with Henoch Schönlein Purpura

OBJECTIVE To describe variation regarding inpatient therapy and evaluation of children with Henoch Schönlein purpura (HSP) admitted to childrens hospitals across the United States. STUDY DESIGN We conducted a retrospective cohort study of children discharged with a diagnosis of HSP between 2000 and 2007 by use of inpatient administrative data from 36 childrens hospitals. We examined variation among hospitals in the use of medications, diagnostic tests, and intensive care services with multivariate mixed effects logistic regression models. RESULTS During the initial HSP hospitalization (n = 1988), corticosteroids were the most common medication (56% of cases), followed by opioids (36%), nonsteroidal antiinflammatory drugs (35%), and antihypertensive drugs (11%). After adjustment for patient characteristics, hospitals varied significantly in their use of corticosteroids, opioids, and nonsteroidal antiinflammatory drugs; the use of diagnostic abdominal imaging, endoscopy, laboratory testing, and renal biopsy; and the use of intensive care services. By contrast, hospitals did not differ significantly regarding administration of antihypertensive drugs or performance of skin biopsy. CONCLUSIONS The significant variation identified may contribute to varying HSP clinical outcomes between hospitals, warrants further investigation, and represents a potentially important opportunity to improve quality of care.

Pediatric therapeutic plasma exchange indications and patterns of use in US children's hospitals.

Therapeutic plasma exchange (TPE) has been increasingly used over the past decade as a first‐line and lifesaving treatment for various conditions classified by the American society for apheresis (ASFA). To date, the degree to which utilization of TPE in pediatrics mirrors recommendations is unknown. Methods: Using inpatient administrative data from 42 childrens hospitals we conducted an 8‐year retrospective cohort study of children (≤18 years) with an international classification of diseases‐9‐clinical modification (ICD‐9‐CM) discharge diagnosis indicating an ASFA Category I or II condition, or a procedure code indicating receipt of TPE during hospitalization. Results: TPE was performed during 4,190 hospitalizations of 3,449 patients, of whom 310 (9.0%) and 77 (2.2%) had a primary discharge diagnosis of an ASFA Category I or II condition, respectively. Rates of TPE use for Category I conditions were highest for children with thrombotic thrombocytopenic purpura (TTP), Goodpastures syndrome, and myasthenia gravis. TPE use in childrens hospitals significantly increased from 2003 to 2010, but TPE was performed during only 13.4 and 9.3% of hospitalizations for ASFA Category I and II conditions, respectively. There was significant between‐hospital variation in the use of TPE for Category I conditions as a group and individual Category I conditions including TTP. Conclusion: We found low levels of TPE use across hospitals for key indications, including TTP, a condition for which TPE is considered a first‐line and life‐saving procedure. The variation identified may contribute to varying clinical outcomes between hospitals, warrants further investigation, and represents an important opportunity to improve quality of care. J. Clin. Apheresis 2012.

Temporal Association of Streptococcus, Staphylococcus, and Parainfluenza Pediatric Hospitalizations and Hospitalized Cases of Henoch-Schönlein Purpura

Objective. To determine if hospitalizations for specific infectious exposures are associated with hospital admissions for Henoch-Schönlein purpura (HSP). Methods. We conducted a retrospective cohort study using administrative data of children admitted to 40 children’s hospitals between January 1, 2002, and December 31, 2008. We examined the association of standardized rates of group A ß-hemolytic Streptococcus (GABS), Staphylococcus aureus, parainfluenza, influenza, adenovirus, and respiratory syncytial virus (RSV)-associated hospital admissions with standardized rates of HSP hospital admissions on a month by month basis using autoregressive moving average process models to account for temporal autocorrelation and clustering by hospital. Results. Among the 3,132 admissions for HSP observed over the 7-year study period, hospital admissions were most frequent September through April, but with substantial variability between hospitals for each month. Accounting for these month by month differences within each hospital, the rate of HSP admissions in a given month increased significantly as the standardized rates of GABS (p = 0.01), S. aureus (p < 0.01), and parainfluenza (p = 0.03) admissions increased. Conclusion. Our results demonstrate a local month by month temporal association between hospitalization for GABS, S. aureus, and parainfluenza and hospitalization for HSP. Future investigations will be required to determine causality.

Detection of enthesitis in children with enthesitis-related arthritis: dolorimetry compared to ultrasonography.

To evaluate the distribution of enthesitis and the accuracy of physical examination with a dolorimeter for the detection of enthesitis in children, using ultrasound (US) assessment as the reference standard.

The pediatric rheumatology quality of life scale: validation of the English version in a US cohort of juvenile idiopathic arthritis

BackgroundThis study aims to validate the English version of the Pediatric Rheumatology Quality of Life Scale (PRQL), a concise Health Related Quality of Life (HRQoL) measure, in a US cohort of children with juvenile idiopathic arthritis (JIA).MethodsThe PRQL is a 10-item HRQoL measure with two subscales: physical health and psychological health. The original version of this measure was validated using an Italian-speaking cohort of 472 JIA patients and 796 healthy controls and found to have acceptable psychometric properties. The English language version has not been validated in a US pediatric population. The English PRQL was administered to 161 JIA subjects from a US Rheumatology clinic. We assessed the reliability (internal consistency and test-retest) and validity (convergent, discriminative, and criterion) of the PRQL.ResultsThe English PRQL was feasible to administer and demonstrated good psychometric properties. Cronbach alpha (reliability) coefficients ranged from 0.72 to 0.81. Factor analysis yielded the existing subscales. The PRQL total and subscales were found to have moderate correlations with other HRQoL instruments, the Pediatric Quality of Life Inventory (PedsQL) generic core scale and the PedsQL rheumatology. The PRQL discriminated between subjects with active versus inactive disease and was responsive to an improvement or worsening in disease activity over time.ConclusionsOur results suggest that the English version of the instrument is suitable for use in JIA patients in the US. This tool provides a relatively easy method to integrate at least one patient-reported outcome into routine clinical or research assessment.

Detection of enthesitis in children with Enthesitis-related arthritis: dolorimeter examination compared to ultrasonography

To evaluate the distribution of enthesitis and the accuracy of physical examination with a dolorimeter for the detection of enthesitis in children, using ultrasound (US) assessment as the reference standard.

Histological Features of Ileitis Differentiating Pediatric Crohn Disease From Ulcerative Colitis with Backwash Ileitis

BACKGROUND/AIM Pediatric ileocolonic Crohn disease (CD) may be difficult to distinguish from ulcerative colitis (UC) with backwash ileitis (BWI). The primary aim of the study was to determine the probability of CD in children with a confluent colitis and ileitis when newly diagnosed with inflammatory bowel disease (IBD). METHODS A retrospective observational study of 100 newly diagnosed patients with IBD was performed. Two pathologists reviewed ileal biopsy specimens for 8 histological features. Biopsy and clinical features were evaluated for predictive ability of a final diagnosis of CD. RESULTS The presence of crypt distortion, lamina propria (LP) expansion, and acute LP inflammation combined with 4 clinical variables in multivariate regression analysis had adequate discriminative validity when comparing the mean probability of a final CD diagnosis between CD and not-CD groups (0.90 vs. 0.59, p value <0.001). When crypt distortion, LP expansion, and acute LP inflammation are present in any combination, the sensitivity and specificity for presence of CD ranges 38.4-57% and 92.9-100%, respectively. CONCLUSIONS Combining histological features of ileitis and clinical variables can adequately discriminate between the presence and absence of Crohn disease in children who present with confluent colitis and ileitis. Combined presence of certain histological features has high specificity for CD.

Detection of Enthesitis in Children With Enthesitis-Related Arthritis: Dolorimetry Compared to Ultrasonography: Ultrasound Versus Physical Examination for Pediatric Enthesitis

To evaluate the distribution of enthesitis and the accuracy of physical examination with a dolorimeter for the detection of enthesitis in children, using ultrasound (US) assessment as the reference standard.