Hurler syndrome with severe complication in post‐bone marrow transplantation course: Life threatening interstitial pneumonitis and hypertension

TOYA OHASHI*, TAKAHIRO TAHARA, KOUJI FUJISAWA, YASUTAKA HOSHI, FUMIYUKI ITO, NAOKI SUZUKI, YOSHIKATSU ETO, KIHEI MAEKAWA

*この研究の対応する著者

研究成果: Article査読

抄録

Bone marrow transplantation (BMT) was performed in a 3 year old patient with Hurler syndrome. The post‐BMT course was complicated by interstitial pneumonitis and severe hypertension, which were life threatening. The patient responded well to therapy and recovered. BMT in this patient resulted in significant clinical improvement in the signs and symptoms of Hurler syndrome. Biochemical improvement, including elevated α‐l‐iduronidase activity in white blood cells and decreased urinary glycosaminoglycan excretion was significant. However, skeletal and neurological impairment were not improved. We conclude that BMT for Hurler syndrome should be performed at an earlier stage, before severe neuronal damage has occurred. Moreover, BMT is a high risk procedure and there will always be a possibility that life threatening complications will occur, as in our case. 1995 Japan Pediatric Society

本文言語English
ページ(範囲)697-700
ページ数4
ジャーナルPediatrics International
37
6
DOI
出版ステータスPublished - 1995 12月
外部発表はい

ASJC Scopus subject areas

  • 小児科学、周産期医学および子どもの健康

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