Case Study of a 15-Year-old Boy with McCune-Albright Syndrome Combined with Pituitary Gigantism : Effect of Octreotide-long Acting Release (LAR) and Cabergoline Therapy

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著者

    • TSUBAKI JUNKO
    • Department of Pediatrics, Hokkaido University School of Medicine
    • ISHIZU KATSURA
    • Department of Pediatrics, Hokkaido University School of Medicine
    • JO WAKAKO
    • Department of Pediatrics, Hokkaido University School of Medicine
    • ISHI NOBUAKI
    • Department of Neurosurgery, Hokkaido University School of Medicine

抄録

The use of octreotide-LAR and cabergoline therapy has shown great promise in adults with acromegaly; however, the experience in pediatric patients has rarely been reported. We described a clinical course of a 15-year-old boy of McCune-Albright syndrome (MAS) with pituitary gigantism. At the age of 8 years, a growth hormone (GH) and prolactin (PRL) producing pituitary adenoma was diagnosed at our hospital. He also had multiple fibrous dysplasia, so that he was diagnosed as having MAS. The tumor was partially resected, and GNAS1 gene mutation (R201C) was identified in affected tissues. We introduced octreotide to suppress GH secretion (100 μg 2/day s.c). During therapy with octreotide, IGF-1 and GH levels could not be suppressed and the patient frequently complained of nausea from octreotide treatment. Therefore, the therapy was changed to monthly injections of octreotide-LAR at the age of 12.3 years and was partially effective. However, as defect of left visual field worsened due to progressive left optic canal stenosis, he underwent second neurological decompression of the left optic nerve at 13.4 years of age. After surgery, in addition to octreotide-LAR, cabergoline (0.25 mg twice a month) was started. This regimen normalized serum levels of GH and IGF-1; however, he showed impaired glucose tolerance and gallstones at 15.7 years of age. Therefore, the dose of octreotide-LAR was reduced to 10 mg and the dose of cabergoline increased. This case demonstrated the difficulty of treating pituitary gigantism due to MAS. The use of octreotide-LAR and cabergoline should be considered even in pediatric patients; however, adverse events due to octreotide-LAR must be carefully examined.<br>

収録刊行物

  • Endocrine journal  

    Endocrine journal 55(3), 595-599, 2008-06-01 

    The Japan Endocrine Society

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各種コード

  • NII論文ID(NAID)
    10021266702
  • NII書誌ID(NCID)
    AA10901436
  • 本文言語コード
    ENG
  • 資料種別
    NOT
  • ISSN
    09188959
  • データ提供元
    CJP書誌  CJP引用  J-STAGE 
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