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Hurler syndrome with severe complication in post‐bone marrow transplantation course: Life threatening interstitial pneumonitis and hypertension
Author(s) -
OHASHI TOYA,
TAHARA TAKAHIRO,
FUJISAWA KOUJI,
HOSHI YASUTAKA,
ITO FUMIYUKI,
SUZUKI NAOKI,
ETO YOSHIKATSU,
MAEKAWA KIHEI
Publication year - 1995
Publication title -
pediatrics international
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.49
H-Index - 63
eISSN - 1442-200X
pISSN - 1328-8067
DOI - 10.1111/j.1442-200x.1995.tb03407.x
Subject(s) - medicine , hurler syndrome , bone marrow , complication , bone marrow transplantation , urinary system , pneumonitis , surgery , gastroenterology , lung , disease
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.