The Journal of Clinical Pediatric Rheumatology
Online ISSN : 2434-608X
Print ISSN : 2435-1105
Volume 3 , Issue 1
Showing 1-7 articles out of 7 articles from the selected issue
  • Shumpei Yokota
    2010 Volume 3 Issue 1 Pages 3-9
    Published: 2010
    Released: October 30, 2020
    JOURNALS FREE ACCESS
    Download PDF (8693K)
  • Noriko Kinjo
    2010 Volume 3 Issue 1 Pages 11-13
    Published: 2010
    Released: October 30, 2020
    JOURNALS FREE ACCESS
    Download PDF (3657K)
  • Naomi Mitsuda, Mikiya Fujieda, Kaori Nishikubo, Takuma Hara, Masayuki ...
    2010 Volume 3 Issue 1 Pages 15-18
    Published: 2010
    Released: October 30, 2020
    JOURNALS FREE ACCESS
    We report on a case of pediatric MCTD accompanied by bamboo nodes. A 6-year-old girl admitted to our hospital complaining fatigue, fever and purpura. Blood examination showed low platelet count,high index of anti-nuclear antibody and anti-RNP antibody. At first,she was diagnosed as idiopathic thrombocytopenic purpura and she was treated with prednisolone. Platelet count elevated by prednisolone,but she complained arthralgia,erythema of cheeks,and hoarseness after the reduction of prednisolone.Raynaud phenomenon was also demonstrated by thermography.Thereafter,her diagnosis was changed to MCTD and she was treated with prednisolone and MMF in good contro1.Bamboo nodes show a transversal cream-yellow band lesion at the midpoint of the upper surface of vocal fold.They are named bamboo nodes because their appearance resembles bamboo and its nodes.These unique lesions are known as specific lesions related to autoimmune diseases.It is reported that there is a good correlation between the manifestations of these lesions with primary disease activity, and in some cases,hoarseness due to this lesion may be first and only subjective symptom of autoimmune disease.The incidence of the lesion is not clear,and there is no report on pediatric case to our knowledge.
    Download PDF (4584K)
  • Tomomi Sato, Shinobu Yoshida, Nobuhiko Okamoto, Kashirou Nishizawa, Ta ...
    2010 Volume 3 Issue 1 Pages 19-22
    Published: 2010
    Released: October 30, 2020
    JOURNALS FREE ACCESS
    Hemophagocytic syndrome(HPS)is a very rare complication of juvenile dermatomyosits(JDM).  We report a case of JDM with HPS. A l4-yr-old girl was admitted to our hospital complaining of remittent fever, appetite loss and body weight loss. She could not walk with arthralgia of the knee. Initially, she was suspected of juvenile idiopathic arthritis. We administered ibuprofen. Her appetite loss and the pain of knee immediately disappeared. However she showed heliotorope rash and Gottron sign. We finally make a diagnosis of JDM. Although we treated with ibuprofen for two weeks, her signs and symptoms relapsed. Laboratory examinations revealed anemia,decreased number of platelets and elevated transaminases.Bone marrow aspiration showed hemophagocytosis,indicating complicated HPS. Methylprednisolone pulse therapy was effectively administered. It is seemed that steroid pulse therapy is effective therapy against HPS with JDM.
    Download PDF (4356K)
  • Minoru Fukuda, Toshihito Nagata, Takafumi Niwa Niwa, Kanayo Ito, Midor ...
    2010 Volume 3 Issue 1 Pages 23-26
    Published: 2010
    Released: October 30, 2020
    JOURNALS FREE ACCESS
     Familial Mediterranean fever (FMF) is one of the auto-inflammatory diseases caused by mutations of in the MEFV gene.Recently,FMF variants characterized by distinct clinical symptoms,not typical of FMF,has been identified.We experienced a case of FMF variants with mutations in MEFV exon 3(MEFV exon3 variants).A 3-year-old boy was referred with persisting fever,erythema,arthralgia and myalgia. Although the administration of antibiotics has no effect,the fever went down 15 days later. Subsequently, periodic fever was observed every 2 to 4 weeks,and duration of the febrile attack was 5 to 15 days. He had neither chest pain nor abdominal pain,and no evidence of serositis was observed. MEFV analysis demonstrated the heterozygous mutation P369S/R408Q. Because periodic fever persisted thereafter, colchicine was started from 4 months after the onset of disease,and relieved him from febrile attacks. He ceased to take colchicine at 2 years and 7 months after the onset of disease. Beyond one year after discontinuation of colchicine,febrile attack has never recurred so far.
    Download PDF (4569K)
  • Kenichi Okumura, Kanta Kishi, Yasuhiko Mori, Nami Okamoto, Takuji Mura ...
    2010 Volume 3 Issue 1 Pages 27-31
    Published: 2010
    Released: October 30, 2020
    JOURNALS FREE ACCESS
     We encountered a case of systemic juvenile idiopathic arthritis(sJIA)with myocarditis. A 5-year-old boy was admitted to a regional hospital after exhibiting prolonged fever,joint pain and skin rash. A diagnosis of sJIA was made. His condition did not improve despite therapy,and he was transferred to our hospital. On hospital day 2,severe chest pain developed. Electrocardiography showed ST elevations in leads V2-V6 and heart fatty acid binding protein test was positive. Echocardiography showed reducing left ventricular wall motion and mild mitral regurgitation. Multiple and regional defects in the left ventricular lateral wall and inferior wall and apex were detected by quantitative gated single photon emission computed tomography(QGS). Myocarditis due to sJIA was diagnosed by QGS and therapy including administration of diuretics and a phosphodiesterase inhibitor was started. Three days after starting therapy, chest pain improved. The multiple defects were improved by hospital day l3. Cardiac involvement is more frequent with sJIA than with other subtypes of JIA, and is the main cause of death among JIA patients. Faster diagnosis and therapy can contribute to better outcomes for sJIA patients with myocarditis.
    Download PDF (5588K)
  • Shoko Furukawa, Yoshihiko Sakurai, Nobuyuki Eura, Yoshitaka Kitauchi, ...
    2010 Volume 3 Issue 1 Pages 33-37
    Published: 2010
    Released: October 30, 2020
    JOURNALS FREE ACCESS
    Neurological involvement is rare in sarcoidosis, and isolated neurosarcoidosis without any other organ involvement is often difficult to diagnose. We report the case of an 11-year-old boy who was clinically diagnosed with isolated neurosarcoidosis. Right facial nerve paralysis developed in the patient 2 months before admission. Although oral corticosteroid produced partial improvements in facial palsy, decreased visual acuity was identified 2weeks before admission. He saw an ophthalmologist and was diagnosed with acute optic neuritis l week before admission. Continuous oral corticosteroids failed to improve visual acuity. Following additional development of photophobia and paresthesia of the lower leg, he was admitted to our hospital for detailed investigations. Examination of the visual field revealed bitemporal inferior quadrantanopia, suggesting damage to the optic chiasm. We failed to obtain positive examination findings for general reactions, but magnetic resonance imaging (MRI)of the brain revealed signal hyperintensities on T2-weighted imaging and gadolinium enhancement in the posterior optic nerve and chiasm. No tissue diagnosis was made because of the difficulty of intracranial biopsy. After considering differential diagnoses, a clinical diagnosis of isolated neurosarcoidosis was made on the basis of clinical and imaging findings. Methylprednisolone pulse therapy was initiated, achieving gradual improvements in right facial palsy and visual acuity This case suggests the usefulness of diagnostic imaging with MRI for diagnosing and making therapeutic decisions for isolated neurosarcoidosis.
    Download PDF (5990K)
feedback
Top