Autonomic neuropathy in Fabry disease: a prospective study using the Autonomic Symptom Profile and cardiovascular autonomic function tests

Background  

Fabry patients have symptoms and signs compatible with autonomic dysfunction. These symptoms and signs are considered to be due to impairment of the peripheral nervous system, but findings indicative of autonomic neuropathy in other diseases, such as orthostatic intolerance and male sexual dysfunction, are infrequently reported in Fabry disease. The aim of our study was to investigate autonomic symptoms and cardiovascular autonomic function in a large cohort of male and female Fabry patients.     

Treatment of Hypertension with Hydrochlorothiazide and Spironolactone

A comparison was made of the antihypertensive effect of a daily dose of 150 mg. of hydrochlorothiazide (HCT) with one of 150 mg. of spironolactone (SL) in 48 patients with essential hypertension.The blood pressure values after six weeks of therapy with HCT were not significantly different from those after SL. Treatment of 25 of these patients with the two agents combined did not reduce the blood pressure further. Blood urea nitrogen concentrations increased with both HCT and SL treatment.With combined treatment there was a further increase in blood urea nitrogen concentration and a decrease in serum sodium concentrations without weight loss beyond that achieved with either HCT or SL alone.     

MRI study of regional variations of pharyngeal wall compliance in cats

Upper airway compliance indicates the potentialof the airway to collapse and is relevant to the pathogenesis ofobstructive sleep apnea. We hypothesized that compliance would varyover the rostral-to-caudal extent of the pharyngeal airway. In aparalyzed isolated upper airway preparation in cats, we controlledstatic upper airway pressure during magnetic resonance imaging (MRI, 0.391-mm resolution). We measured cross-sectional area andanteroposterior and lateral dimensions from three-dimensionalreconstructed MRIs in axial slices orthogonal to the airway centerline.High-retropalatal (HRP), midretropalatal (MRP), and hypopharyngeal(HYP) regions were defined. Regional compliance was significantlyincreased from rostral to caudal regions as follows: HRP < MRP < HYP (P < 0.0001), and compliancedifferences among regions were directly related to collapsibility. Thusour findings in the isolated upper airway of the cat support thehypothesis that regional differences in pharyngeal compliance exist andsuggest that baseline regional variations in compliance andcollapsibility may be an important factor in the pathogenesis andtreatment of obstructive sleep apnea.