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

Research output: Contribution to journalArticlepeer-review

Abstract

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

Original languageEnglish
Pages (from-to)697-700
Number of pages4
JournalPediatrics International
Volume37
Issue number6
DOIs
Publication statusPublished - 1995 Dec
Externally publishedYes

Keywords

  • bone marrow transplantation
  • high blood pressure
  • Hurler syndrome
  • interstitial pneumonitis
  • mucopolysaccharidosis
  • transplantation

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

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