BODY-MASS INDEX AND BLOOD PRESSURE AS DISEASE MODIFIERS IN DYSTROPHINIC CARDIOMYOPATHY

Abstract

Objective: Introduction: Duchenne muscular dystrophy (DMD) is one of many neuromuscular diseases, that commonly creates severe disability and early death. Cardiomyopathy associated with DMD is an increasingly acknowledged presentation of this neuromuscular disorder, significantly increasing morbidity and mortality, as well as the requirement for cardiological treatment. Design and method: Purpose: is to review literature data relatively the presence of elevated blood pressure values and body mass index in children with DMD and their impact on the development of heart disease. Results: Several studies have published cross-sectional data about the prevalence of hypertension and increased BMI in children with DMD. Wong et al., 2017 reported that 25.5% of patients on daily steroids aged 10–13 years had systolic hypertension. In patients aged 13–16 years, they reported systolic hypertension in 10.3% of patients. Corticosteroid treatment is thus an essential part of the standards of care in DMD and is recommended from the age of 4 to 5 years onward. Ricotti et al., 2013 observed hypertension in 5% of the patients aged 3–15 years on intermittent steroids while in a study by Braat et al., 2015, 45% of the patients had hypertension. In N.M. van de Velde et al., 2019, study increased BMI, but not systolic blood pressure, was related to early myocardial deformation defined by peak systolic GLS in young DMD patients < 11 years of age. Prevalence of obesity has been described up to 73% in steroid-native DMD patients < 13 years and higher BMI was associated with longer duration and higher cumulative dose in ambulant DMD patients using prednisone (Lamb et al., 2016). In Barber et al. study, 2013 use of corticosteroids has managed to prolongation of the ambulant stage in DMD by almost 3 years. It has also been correlated with a delayed onset of dystrophinic cardiomyopathy in Markham et al., 2008 and Davidson et al., 2014 studies. Conclusions: The results of this review suggest that factors influencing afterload, such as increased blood pressure and BMI, may play a role in the deterioration of cardiac function in DMD.