The mucopolysaccharidoses (MPSs) are inherited lysosomal storage disorders caused by the absence of functional enzymes that contribute to the degradation of glycosaminoglycans (GAGs). The progressive systemic deposition of GAGs results in multi-organ system dysfunction that varies with the particular GAG deposited and the specific enzyme mutation(s) present. Cardiac involvement has been reported in all MPS syndromes and is a common and early feature, particularly for those with MPS I, II, and VI. Cardiac valve thickening, dysfunction (more severe for left-sided than for right-sided valves), and hypertrophy are commonly present; conduction abnormalities, coronary artery and other vascular involvement may also occur. Cardiac disease emerges silently and contributes significantly to early mortality.The clinical examination of individuals with MPS is often difficult due to physical and, sometimes, intellectual patient limitations. The absence of precordial murmurs does not exclude the presence of cardiac disease. Echocardiography and electrocardiography are key diagnostic techniques for evaluation of valves, ventricular dimensions and function, which are recommended on a regular basis. The optimal technique for evaluation of coronary artery involvement remains unsettled.Standard medical and surgical techniques can be modified for MPS patients, and systemic therapies such as hematopoietic stem cell transplantation and enzyme replacement therapy (ERT) may alter overall disease progression with regression of ventricular hypertrophy and maintenance of ventricular function. Cardiac valve disease is usually unresponsive or, at best, stabilized, although ERT within the first few months of life may prevent valve involvement, a fact that emphasizes the importance of early diagnosis and treatment in MPS.

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Proxemic theory emphasizes cultural uses of interpersonal distance to regulate intimacy via sensory exposure. However, research has confounded cultural differences in conversational distance with sex, language, and topic. This study assessed interpersonal distance between seated conversants from each of three cultures varying in purported contact norms. Thirty-five Japanese, 31 Venezuelan foreign students (assigned to speak either their native language or English), and 39 Americans had a 5-minute conversation on a common topic with a same-sex, same-nationality confederate. Three hypotheses were tested: (a) When speaking their native languages, Japanese will sit farther apart than Venezuelans, with Americans at an intermediate distance; (b) females will sit closer than males; and (c) foreign subjects, when speaking English, will more closely approximate American conversational distance than when speaking their native languages. The hypotheses were generally confirmed. According to proxemic theory (Hall, 1966), interpersonal distance helps to regulate intimacy by controlling sensory exposure. The possibility of visual, tactile, auditory, and olfactory stimulation is enhanced at closer distances. Differences in distancing within dyads are likely to be affected by a variety of sources of preferences for intimacy, including individual history of social deprivation and reinforcement, acquain-1979. We are grateful to

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