• Title/Summary/Keyword: Juvenile dermatomyositis

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Juvenile Dermatomyositis Diagnosed by $^{99m}Tc$-HDP Three-phase Bone Scintigraphy (삼상 뼈스캔으로 진단된 소아기 피부근육염)

  • Kim, Ja-Hye;Song, Ho-Chun;Yoo, Su-Ung;Ha, Jung-Min;Chong, A-Ri;Oh, Jong-Ryool;Min, Jung-Joon;Bom, Hee-Seung;Jeong, Eun-Hui;Lee, Min-Chul
    • Nuclear Medicine and Molecular Imaging
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    • v.43 no.6
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    • pp.592-595
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    • 2009
  • Juvenile dermatomyositis is a common inflammatory muscle disease of childhood, characterized by weakness in proximal muscles and specific skin rash. In case of juvenile dermatomyositis without characteristic clinical features, non-invasive imaging tools such as $^{99m}Tc$-HDP three-phase bone scan are very helpful in diagnostic workup of myopathies. We report a case of 13-year old female with juvenile dermatomyositis, in which $^{99m}Tc$-HDP three-phase bone scan was useful in diagnosis and assessing therapy response.

Renal involvement in pediatric rheumatologic diseases

  • Kim, Seong Heon
    • Childhood Kidney Diseases
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    • v.26 no.1
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    • pp.18-24
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    • 2022
  • Pediatric rheumatologic diseases are rare systemic diseases that can involve various organs, including the kidneys. Each rheumatologic disease can exhibit characteristic renal involvement, which requires proper treatment and diagnosis. In this review, we discuss renal involvement in classic rheumatologic diseases, including juvenile idiopathic arthritis, Sjogren's syndrome, systemic sclerosis, and juvenile dermatomyositis. Reviews addressing lupus nephritis and antineutrophil cytoplasmic antibody-associated renal disease are complex and tend to cover a wide array of topics, and thus were excluded from this review.

A clinical analysis of juvenile dermatomyositis; focus on clinical manifestations at diagnosis (소아기 피부근염의 임상적 고찰; 진단시 임상증상을 중심으로 한 고찰)

  • Lee, So Young;Bang, Ji Seok;Kim, Hee Seok;Kim, Joong Gon
    • Clinical and Experimental Pediatrics
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    • v.50 no.11
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    • pp.1116-1124
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    • 2007
  • Purpose : Juvenile dermatomyositis (JDM) is the most common of the idiopathic inflammatory myopathies in children. The purpose of this study is to observe demographic, initial presentations, duration of time between disease onset and diagnosis, clinical manifestations and laboratory findings at diagnosis of patients with JDM. Methods : Forty seven patients identified at Seoul National University Children's Hospital from January 1986 to May 2007. Medical records were reviewed retrospectively focusing on initial presentations, clinical manifestations and laboratory findings at the time of diagnosis of patients with JDM. Results : Male and female patients were 25 and 22, respectively and sex ratio was 1.14:1. The average age at the time of diagnosis was 6.51 years. Skin rash (94%) was the most common symptom, followed by the proximal muscle weakness (89%). The disease activity score was 10.8. The duration between the onset of the skin rash and the muscle weakness and diagnosis was 7.18 and 4.70 months, respectively. The serum muscle enzymes, LDH, AST, CK and aldolase, were elevated in the patient with JDM. Autoimmune antibodies, antinuclear antibody, anti SSA antibody and anti SSB antibody, were negative findings. Electromyography findings were consistent with JDM in 88% of the patients, the muscle biopsy was in 91% and all MRI findings were compatible with those of patients with JDM. The most common symptom besides musculocutaneous lesions was the calcinosis (62.5%). The most common site of calcinosis was the pelvic area and buttocks. Conclusion : This study shows that the major symptoms are proximal muscle weakness and cutaneous lesion, and they are important to diagnose JDM.

Association of Diagnostic Criteria and Autoantibodies with Juvenile Dermatomyositis in Newly Diagnosed Children (소아기 피부근염의 진단 기준과 자가항체의 진단적 의의)

  • Shin, Kyung Sue;Kim, Joong Gon
    • Clinical and Experimental Pediatrics
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    • v.46 no.9
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    • pp.898-902
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    • 2003
  • Purpose : To determine the clinical association of diagnostic criteria and the prevalence of autoantibodies in newly diagnosed children with juvenile dermatomyositis(JDM). Methods : Thirty-two children with JDM were identified at Seoul National University Children's Hospital from March 1985 to March 1999 by retrospective review. The diagnosis of JDM was based on the criteria proposed by Bohan and Peter. We investigated for the presence of several autoantibodies: antinuclear(ANA), double-stranded DNA, anti-Sm, anti-ribonucleoprotein(RNP), anti-SSA/ SSB, anti-Jo1, anti-Scl-70 antibodies and rheumatoid factor(RF). Results : Sex ratio and age at diagnosis were similar to data published in other studies. All the newly diagnosed children with JDM had a typical rash(100%) and proximal muscle weakness(100%); 17(53%) had muscle pain or tenderness; 10(31%) calcinosis; eight(25%) dysphagia; eight(25%) arthritis, and seven(22%) fever. Muscle enzymes were elevated in 90% of the patients. Of the 27 patients who had an electromyogram, 20(70%) had diagnostic results. Sixteen(70%) of biopsied patients had appropriated results for JDM. Patients were negative for all autoantibodies except ANA and RF. ANA and RF were detected in 47% and 7% of the patients respectively. Conclusion : Although the sensitivity of the criteria proposed by Bohan and Peter is superior, each of these criteria has possible confounding factors. Additional criteria may be needed for early diagnosis of JDM. Based on our findings of autoantibodies in JDM, we do not recommend routine testing for autoantibodies in children with typical JDM.