ÿþ<html> <head> <meta http-equiv="Content-Language" content="en-us"> <meta http-equiv="Content-Type" content="text/html; charset=windows-1252"> <title>Hurler syndrome</title> <base href="http://www.brown.edu/Courses/Digital_Path/systemic_path/cardio/"> </head> <body link="#FFFFFF" vlink="#FFFFFF" alink="#FFFFFF" text="#FFFFFF" bgcolor="#000000"> <p style="margin-top: 0px; margin-bottom: 0px" align="center">&nbsp;</p> <p style="margin-top: 0px; margin-bottom: 0px" align="center"><b> <font face="Arial" size="4">Hurler syndrome (MPSI H)</font></b></p> <p style="margin-top: 0px; margin-bottom: 0px" align="center">&nbsp;</p> <p style="margin-top: 0px; margin-bottom: 0px" align="center"> <img border="0" src="CV72.jpg" width="314" height="375"></p> <p style="margin-top: 0px; margin-bottom: 0px" align="center"> &nbsp;</p> <p class="MsoNormal" align="center" style="margin-top: 0; margin-bottom: 0"> <font face="Arial">Among the cells in which mucopolysaccharides accumulate in Hurler syndrome are endothelial cells. In this </font></p> <p class="MsoNormal" align="center" style="margin-top: 0; margin-bottom: 0"> <font face="Arial">photo one sees glistening and thickened endocardium in the left atrium and ventricle and on the mitral valve.</font></p><p align="center" style="margin-top: 0; margin-bottom: 0">&nbsp;</p> <p align="center" style="margin-top: 0; margin-bottom: 0">&nbsp;</p> <p style="margin-top: 0; margin-bottom: 0" align="center"> <span id="link0" onMouseOver="displayAd(0);" onMouseOut="hideAd();"> <b><font face="Arial" color="#FFFF00" style="font-size: 9pt"> 1 minute clinical correlation</font></b></span><p align="center"><span style="font-size: 12.0pt; font-family: Arial"> Contributed by Dr. Don B. Singer</span></p> <p class="MsoNormal" align="center" style="margin-top: 0; margin-bottom: 0"> <b><font face="Arial" size="2"><a href="interstitial-fibrosis.html">Previous</a> | <a href="http://www.brown.edu/Courses/Digital_Path/systemic_path/index.html"> Home</a> | <a href="hurler2.html">Next</a></font></b><p class="MsoNormal" align="center" style="margin-top: 0; margin-bottom: 0"> &nbsp;<p class="MsoNormal" align="center" style="margin-top: 0; margin-bottom: 0"> <font face="Arial" size="2"> <a href="http://www.brown.edu/Courses/Digital_Path/systemic_path/cardio.html"> Back to cardiovascular section</a></font> </body> <!--PASTE JUST AFTER OPENING BODY TAG: ============================================================== --> <script language="JavaScript" type="text/javascript"> // // IntelliTXT FORCED-DEMO SCRIPT // ----------------------------- // VER: 3.0 // UPDATED: 03/03/06 // // IntelliTXT BASIC // (no creative) // //............................................................ // BEGIN EDITABLE TOOLTIP VARIABLES //TOOLTIP TITLES var titleArray = new Array(); titleArray[0] = ""; //TOOLTIP BODY TEXT var bodyArray = new Array(); bodyArray[0] = "The mucopolysaccharidoses (MPS) result from deficiencies of lysosomal enzymes related to the degradation of mucopolysaccharides which are long-chain carbohydrates linked with proteins to form proteoglycans which are constituents of the ground substance of connective tissue. The deficiencies are determined on a genetic basis. Hurler syndrome (MPS I H), one of the most severe forms of MPS, is due to a deficiency of að-1-iduronidase. Normal at birth, affected infants or children develop hepatosplenomegaly, growth retardation, coarse facial features, and skeletal deformities. They usually die within the first decade of life and death is often secondary to cardiac involvement. 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