Literature DB >> 21286938

Sub-pleural bullous changes in two adults with Mucopolysaccharidosis type I (Hurler-Scheie).

Michel C Tchan1, Nicole Graf, David O Sillence.   

Abstract

Two unrelated adult patients with MPS I (Hurler-Scheie) demonstrated sub-pleural bullous emphysema. This complication of MPS I should be looked for due to the risk of spontaneous pneumothorax.

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Year:  2011        PMID： 21286938     DOI： 10.1007/s10545-010-9273-5

Source DB:  PubMed          Journal:  J Inherit Metab Dis        ISSN： 0141-8955            Impact factor:   4.982


  3 in total

1.  Impaired elastogenesis in Hurler disease: dermatan sulfate accumulation linked to deficiency in elastin-binding protein and elastic fiber assembly.

Authors:  A Hinek; S E Wilson
Journal:  Am J Pathol       Date:  2000-03       Impact factor: 4.307

2.  Upregulation of elastase proteins results in aortic dilatation in mucopolysaccharidosis I mice.

Authors:  Xiucui Ma; Mindy Tittiger; Russell H Knutsen; Attila Kovacs; Laura Schaller; Robert P Mecham; Katherine P Ponder
Journal:  Mol Genet Metab       Date:  2008-05-13       Impact factor: 4.797

3.  Upregulation of elastase activity in aorta in mucopolysaccharidosis I and VII dogs may be due to increased cytokine expression.

Authors:  Jason A Metcalf; Bruce Linders; Susan Wu; Paul Bigg; Patricia O'Donnell; Meg M Sleeper; Michael P Whyte; Mark Haskins; Katherine P Ponder
Journal:  Mol Genet Metab       Date:  2009-12-11       Impact factor: 4.797
  3 in total

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