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Childhood sarcoidosis: A rare but fascinating disorder

Overview of attention for article published in Pediatric Rheumatology, September 2008
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  • High Attention Score compared to outputs of the same age and source (81st percentile)

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2 tweeters
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2 Facebook pages

Citations

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122 Dimensions

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105 Mendeley
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Title
Childhood sarcoidosis: A rare but fascinating disorder
Published in
Pediatric Rheumatology, September 2008
DOI 10.1186/1546-0096-6-16
Pubmed ID
Authors

Avinash K Shetty, Abraham Gedalia

Abstract

Childhood sarcoidosis is a rare multisystemic granulomatous disorder of unknown etiology. In the pediatric series reported from the southeastern United States, sarcoidosis had a higher incidence among African Americans. Most reported childhood cases have occurred in patients aged 13-15 years. Macrophages bearing an increased expression of major histocompatibility class (MHC) II molecules most likely initiate the inflammatory response of sarcoidosis by presenting an unidentified antigen to CD4+ Th (helper-inducer) lymphocytes. A persistent, poorly degradable antigen driven cell-mediated immune response leads to a cytokine cascade, to granuloma formation, and eventually to fibrosis. Frequently observed immunologic features include depression of cutaneous delayed-type hypersensitivity and a heightened helper T cell type 1 (Th1) immune response at sites of disease. Circulating immune complexes, along with signs of B cell hyperactivity, may also be found. The clinical presentation can vary greatly depending upon the organs involved and age of the patient. Two distinct forms of sarcoidosis exist in children. Older children usually present with a multisystem disease similar to the adult manifestations, with frequent hilar lymphadenopathy and pulmonary infiltrations. Early-onset sarcoidosis is a unique form of the disease characterized by the triad of rash, uveitis, and arthritis in children presenting before four years of age. The diagnosis of sarcoidosis is confirmed by demonstrating a typical noncaseating granuloma on a biopsy specimen. Other granulmatous diseases should be reasonably excluded. The current therapy of choice for sarcoidosis in children with multisystem involvement is oral corticosteroids. Methotrexate given orally in low doses has been effective, safe and steroid sparing in some patients. Alternative immunosuppressive agents, such as azathioprine, cyclophosphamide, chlorambucil, and cyclosporine, have been tried in adult cases of sarcoidosis with questionable efficacy. The high toxicity profile of these agents, including an increased risk of lymphoproliferative disorders and carcinomas, has limited their use to patients with severe disease refractory to other agents. Successful steroid sparing treatment with mycophenolate mofetil was described in an adolescent with renal-limited sarcoidosis complicated by renal failure. Novel treatment strategies for sarcoidosis have been developed including the use of TNF-alpha inhibitors, such as infliximab. The long-term course and prognosis is not well established in childhood sarcoidosis, but it appears to be poorer in early-onset disease.

Twitter Demographics

The data shown below were collected from the profiles of 2 tweeters who shared this research output. Click here to find out more about how the information was compiled.

Mendeley readers

The data shown below were compiled from readership statistics for 105 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
Denmark 2 2%
Spain 1 <1%
Japan 1 <1%
Unknown 101 96%

Demographic breakdown

Readers by professional status Count As %
Researcher 14 13%
Student > Postgraduate 13 12%
Other 13 12%
Student > Master 12 11%
Student > Ph. D. Student 12 11%
Other 31 30%
Unknown 10 10%
Readers by discipline Count As %
Medicine and Dentistry 77 73%
Agricultural and Biological Sciences 5 5%
Nursing and Health Professions 2 2%
Psychology 2 2%
Economics, Econometrics and Finance 1 <1%
Other 7 7%
Unknown 11 10%

Attention Score in Context

This research output has an Altmetric Attention Score of 2. This is our high-level measure of the quality and quantity of online attention that it has received. This Attention Score, as well as the ranking and number of research outputs shown below, was calculated when the research output was last mentioned on 19 August 2020.
All research outputs
#10,720,713
of 18,694,771 outputs
Outputs from Pediatric Rheumatology
#283
of 579 outputs
Outputs of similar age
#137,104
of 271,705 outputs
Outputs of similar age from Pediatric Rheumatology
#8
of 37 outputs
Altmetric has tracked 18,694,771 research outputs across all sources so far. This one is in the 41st percentile – i.e., 41% of other outputs scored the same or lower than it.
So far Altmetric has tracked 579 research outputs from this source. They typically receive a little more attention than average, with a mean Attention Score of 5.3. This one is in the 48th percentile – i.e., 48% of its peers scored the same or lower than it.
Older research outputs will score higher simply because they've had more time to accumulate mentions. To account for age we can compare this Altmetric Attention Score to the 271,705 tracked outputs that were published within six weeks on either side of this one in any source. This one is in the 47th percentile – i.e., 47% of its contemporaries scored the same or lower than it.
We're also able to compare this research output to 37 others from the same source and published within six weeks on either side of this one. This one has done well, scoring higher than 81% of its contemporaries.