Clinical Pediatric Endocrinology
Online ISSN : 1347-7358
Print ISSN : 0918-5739
ISSN-L : 0918-5739
Volume 23, Issue 1
Displaying 1-3 of 3 articles from this issue
Review Article
  • Toshimi Michigami
    2014 Volume 23 Issue 1 Pages 1-8
    Published: 2014
    Released on J-STAGE: February 03, 2014
    JOURNAL OPEN ACCESS
    Abstract. Endochondral bone formation involves multiple steps, consisting of the condensation of undifferentiated mesenchymal cells, proliferation and hypertrophic differentiation of chondrocytes, and then mineralization. To date, various factors including transcription factors, soluble mediators, extracellular matrices (ECMs), and cell-cell and cell-matrix interactions have been identified to regulate this sequential, complex process. Moreover, recent studies have revealed that epigenetic and microRNA-mediated mechanisms also play roles in chondrogenesis. Defects in the regulators for the development of growth plate cartilage often cause skeletal dysplasias and growth failure. In this review article, I will describe the current understanding concerning the regulatory mechanisms underlying chondrogenesis.
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Original Articles
  • Keiichi Ozono, Yukihiro Hasegawa, Masanori Minagawa, Masanori Adachi, ...
    2014 Volume 23 Issue 1 Pages 9-15
    Published: 2014
    Released on J-STAGE: February 03, 2014
    JOURNAL OPEN ACCESS
    Abstract. Oral sodium phosphate formulations indicated for hypophosphatemia are commercially available worldwide. In Japan, however, many medical institutes have used hospital dispensary or foreign over-the-counter formulations because no such medication with an indication covered by the health insurance system is domestically available. To address this problem, we initiated the development of Phosribbon®. The present study evaluated the efficacy and safety of Phosribbon® in 16 patients with hereditary hypophosphatemic rickets. The optimal dosage and an administration pattern were also investigated. Administration of the agent resulted in an increase in the level of serum phosphorus in all patients, which implied that the employed dosage was appropriate. The dosage and administration pattern were adjusted based on comprehensive considerations, including changes in clinical laboratory values such as serum phosphorus, alkaline phosphatase and intact PTH, the dosage of a concomitantly administered activated vitamin D formulation and characteristics of individual patients. Adverse drug reactions were observed in 2 patients, neither of which were serious or necessitated therapy dose reduction or discontinuation. We conclude that Phosribbon® is a safe and effective treatment for patients with hypophosphatemic rickets and that dose adjustment in this therapy can be guided by the results of regular clinical examination and renal ultrasonography. (ClinicalTrials.gov Identifier: NCT01237288)
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  • Satoshi Takakuwa, Yoko Kina
    2014 Volume 23 Issue 1 Pages 17-25
    Published: 2014
    Released on J-STAGE: February 03, 2014
    JOURNAL OPEN ACCESS
    Abstract. The initial treatment of childhood-onset Graves’ disease is based on the result of clinical trials of adult-onset disease. The major adverse events associated with methimazole, the only medication approved for childhood-onset disease in Japan, are considered to depend on the dose, and the risk of adverse events is increased in patients requiring higher doses for initial treatment. The serum levels of thyroid hormones are partially dependent on the enterohepatic circulation, especially under thyrotoxicosis. Cholesterol absorption inhibitors suppressing the enterohepatic circulation have the possibility of controlling thyrotoxicosis. In this clinical trial, 13 patients with childhood-onset Graves’ disease (5.5 to 15.3 yr old) were divided into three treatment groups: low-dose (0.25 mg/kg/d) methimazole monotherapy, high-dose (1.0 mg/kg/d) methimazole monotherapy, and combination (low-dose methimazole + a cholesterol absorption inhibitor) therapy. The therapeutic efficacy was determined based on the rates of decrease of thyroid hormones for four weeks. The high-dose methimazole regimen was superior in efficacy to the low-dose methimazole regimen, while the combination therapy demonstrated effects equal to those of the high-dose monotherapy. Therefore, combination therapy with a cholesterol absorption inhibitor can improve thyrotoxicosis, and the dose of methimazole can be reduced in the initial treatment of child-onset Graves’ disease.
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