No abstract

Patients with inborn errors of metabolism (IEMs) have become an emerging and challenging group in the adult healthcare system whose needs should be known in order to implement appropriate policies and to adapt adult clinical departments. We aimed to analyze the clinical characteristics of adult patients with IEMs who attend the most important Spanish hospitals caring for these conditions. A cohort study was conducted in 500 patients, categorized by metabolic subtype according to pathophysiological classification. The most prevalent group of IEMs was amino acid disorders, with 108 (21.6%) patients diagnosed with phenylketonuria. Lysosomal storage disorders were the second group, in which 32 (6.4%) and 25 (5%) patients had Fabry disease and Gaucher disease respectively. The great clinical heterogeneity, the significant delay in diagnosis after symptom onset, the existence of some degree of physical dependence in a great number of patients, the need for a multidisciplinary and coordinated approach, and the lack of specific drug treatment are common features in this group of conditions.

No abstract

Prescription rates of anti-ulcer drugs in hospitals and their spill-over to general practice have risen over the last few years, increasing pharmaceutical expenses. The aim of this study was to analyze gastroprotective drug prescription habits in our hospital by assessing both prevalence and adherence to approved indications. An observational study of prescription-indication was performed with 2 cross sections separated by 6 months to avoid selection bias. We found overprescription of gastroprotective drugs, mainly proton pump inhibitors for the prevention of non-steroidal antiinflammatory drug-induced ulcer (17.1%). Overall, 77.6% of prescriptions had no acceptable indication, despite the availability of a specific protocol produced by the Pharmacy and Therapeutics Committee in our center. There was a high prevalence of prescriptions for non-approved indications such as prophylaxis in patients administered corticosteroids without non-steroidal anti-inflammatory agents (25.7%). New programs to train clinicians and clinical managers in rational drug use are required.

Clinical characteristics were similar for both HFPEF and HFREF patients. Women were predominant in the HFPEF group, as was hypertensive etiology. No significant differences in mortality were observed between the groups.