First name
Andrew
Middle name
J
Last name
Klink

Title

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

Year of Publication

2012

Number of Pages

287-94

Date Published

2012 Jul

ISSN Number

1098-1101

Abstract

<p><strong>PURPOSE: </strong>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.</p>

<p><strong>METHODS: </strong>Using inpatient administrative data from 42 children's 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.</p>

<p><strong>RESULTS: </strong>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), Goodpasture's syndrome, and myasthenia gravis. TPE use in children's 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.</p>

<p><strong>CONCLUSION: </strong>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.</p>

DOI

10.1002/jca.21242

Alternate Title

J Clin Apher

PMID

22811262

Title

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

Year of Publication

2013

Number of Pages

43

Date Published

2013

ISSN Number

1546-0096

Abstract

<p><strong>BACKGROUND: </strong>This 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).</p>

<p><strong>METHODS: </strong>The 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.</p>

<p><strong>RESULTS: </strong>The 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.</p>

<p><strong>CONCLUSIONS: </strong>Our 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.</p>

DOI

10.1186/1546-0096-11-43

Alternate Title

Pediatr Rheumatol Online J

PMID

24206654

Title

Enthesitis in an inception cohort of enthesitis-related arthritis.

Year of Publication

2011

Number of Pages

1307-12

Date Published

2011 Sep

ISSN Number

2151-4658

Abstract

<p><strong>OBJECTIVE: </strong>To describe an enthesitis-related arthritis (ERA) inception cohort and determine which entheses and joints are most commonly affected.</p>

<p><strong>METHODS: </strong>We reviewed a retrospective inception cohort study of children with ERA who were diagnosed and treated at The Children's Hospital of Philadelphia between November 2007 and December 2009.</p>

<p><strong>RESULTS: </strong>During the study period, there were 32 newly diagnosed ERA patients. Fifty-nine percent were male, and the median age at the date of initial evaluation was 12.5 years (interquartile range [IQR] 10.2-14.3 years). The median number of tender entheses at presentation was 2 (IQR 0-5), and 21 subjects (66%) had at least 1 tender enthesis. The most prevalent tender entheses were the patellar ligament insertion at the inferior pole of the patella, the plantar fascial insertion at the calcaneus, the Achilles tendon insertion at the calcaneus, and the plantar fascial insertion at the metatarsal heads. Enthesitis was most often symmetric. The median number of active joints was 2 (IQR 0-4). The most commonly affected joints were the sacroiliacs, knees, and ankles. Sacroiliitis, which was defined clinically, was most often symmetric, while peripheral arthritis was most frequently asymmetric. The odds of having active enthesitis at 6 months increased significantly with each additional tender enthesis at the initial evaluation.</p>

<p><strong>CONCLUSION: </strong>Among pediatric patients with ERA, lower extremity enthesitis is prevalent at the time of diagnosis and is likely to persist 6 months later. Future studies should address standardization of the enthesitis examination, the pattern of enthesitis over time, enthesitis response to therapy, and the impact of enthesitis on quality of life.</p>

DOI

10.1002/acr.20508

Alternate Title

Arthritis Care Res (Hoboken)

PMID

21618453

Title

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

Year of Publication

2014

Number of Pages

218-27

Date Published

2014 Jan

ISSN Number

2326-5205

Abstract

<p><strong>OBJECTIVE: </strong>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.</p>

<p><strong>METHODS: </strong>We performed a prospective cross-sectional study of 30 patients with enthesitis-related arthritis (ERA) and 30 control subjects. The following tendon insertion sites were assessed by standardized physical examination with a dolorimeter and US: common extensor on the lateral humeral epicondyle, common flexor on the medial humeral epicondyle, quadriceps at the superior patella, patellar ligament at the inferior patella, Achilles, and plantar fascia at the calcaneus.</p>

<p><strong>RESULTS: </strong>Abnormal findings on US were detected most commonly at the insertion of the quadriceps (30% [18 of 60 sites]), common extensor (12% [7 of 60]), and Achilles (10% [6 of 60]) tendons. The intrarater reliability of US (kappa statistic) was 0.78 (95% confidence interval [95% CI] 0.63-0.93), and the interrater reliability was 0.81 (95% CI 0.67-0.95). Tenderness as detected by standardized dolorimeter examination had poor positive predictive value for US-confirmed enthesitis. In comparison to controls, patients with ERA reported more pain and had lower pain thresholds at every site, including control sites (P &lt; 0.001 for all comparisons). The interrater reliability of dolorimeter examination for detection of enthesitis was low (κ = 0.49 [95% CI 0.33-0.65]).</p>

<p><strong>CONCLUSION: </strong>Compared to US, standardized dolorimeter examination for the detection of enthesitis in children has poor accuracy and reliability. The decreased pain threshold of ERA patients likely contributed to the limited accuracy of the physical examination findings. Further research regarding the utility of US for identifying enthesitis at diagnosis of juvenile idiopathic arthritis, accurately predicting disease progression, and guiding therapeutic decisions is warranted.</p>

DOI

10.1002/art.38197

PMID

24449586

WATCH THIS PAGE

Subscription is not available for this page.