日本未熟児新生児学会雑誌　第21巻　第2号　50～54頁（2009年）

日本未熟児新生児学会雑誌　21(2)：226-230；2009　

受付日：平成17．09．13

受理日：平成20．10．20

McCune-Albright症候群に伴う高Ca血症をincadronateにより治療した極低出生体重児の一例

Successful Treatment of Hypercalcemia with Bisphosphonate in a very Low Birth Weight Infant with McCune-Albright syndrome；Case Report

＊1群馬大学周産母子センター　NICU，＊2群馬大学大学院医学系研究科　小児生体防御学分野，＊3現・Neoこどもクリニック，＊4現・希望の家療育病院

＊1 Neonatal Intensive Care Unit, Perinatal Care Center, Gunma University，＊2 Department of Pediatrics and Developmental Medicine, Gunma University Graduate School of Medicine，＊3 Neo Pediatric Clinic，＊4 Kiboh-noie Hospital

中島一恵＊1＊2・吉澤幸弘＊1＊2・大木康史＊1＊2・塩島　健＊1＊2・名古　靖＊1＊2＊3・森川昭廣＊1＊2＊4

Kazue NAKAJIMA＊1＊2，Yukihiro YOSHIZAWA＊1＊2，Yasushi OHKI＊1＊2， Takeshi SHIOJIMA＊1＊2，Yasushi NAKO＊1＊2＊3，Akirihiro MORIKAWA＊1＊2＊4

Key Words：Bisphosphonate，hypercalcemia，McCune-Albright syndrome，very low birth weight infant

　我々は，難治性高Ca血症を伴ったMcCune-Albright症候群の極低出生体重児の治療に，bisphosphonate製剤を使用し，良好な治療成績を得た本邦初の症例を経験したので報告する。症例は在胎35週，出生体重1,442gの男児。副甲状腺機能亢進症に伴う単純X線写真上の複数の不規則骨吸収像，高Ca血症を認めた。高Ca血症に対し，低Caミルク，利尿剤とcalcitoninにより治療したが無効で，日齢58に血清Ca値16.7mg/dLと著明な高Ca血症を認めたため，骨吸収抑制剤であるbisphosphonate製剤（incadronate）を投与したところ，血清Ca値の改善がみられ，本製剤が新生児高Ca血症の一治療として有効であることが示唆された。

　McCune-Albright syndrome is a syndrome that may involve multiple endocrine and non-endocrine organs due to signal transducting guanine nucleotide binding protein gene abnormality. Recently, we experienced a preterm newborn case with this disorder who showed multiple endocrine disorders, severe bone lesions, and intractable hypercalcemia, On 55 days of age, serum calcium level reached to 16.7mg/dL. Despite of the treatment including low calcium formula, furosemide, and salmon-derived calcitonin, hypercalcemia had persisted, Incadronate disodium was administered. Two days after the first dose of incadronate, serum calcium level decreased to 11.0 mg/dL. To our knowledge, this is the smallest and youngest case with hypercalcemia treated with bisphosphonates. Although the long-term significance should be determined, bisphosphonates may be a choice as a treatment of intractable hypercalcemia in preterm infants.