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The integration and exchange of information among health organizations and system providers are currently regarded as a challenge. Each organization usually has an internal ecosystem and a proprietary way to store electronic health records of the patient’s history. Recent research explores the advantages of an integrated ecosystem by exchanging information between the different inpatient care actors. Many efforts seek quality in health care, economy, and sustainability in process management. Some examples are reducing medical errors, disease control and monitoring, individualized patient care, and avoiding duplicate and fragmented entries in the electronic medical record. Likewise, some studies showed technologies to achieve this goal effectively and efficiently, with the ability to interoperate data, allowing the interpretation and use of health information. To that end, semantic interoperability aims to share data among all the sectors in the organization, clinicians, nurses, lab, the entire hospital. Therefore, avoiding data silos and keep data regardless of vendors, to exchange the information across organizational boundaries. This study presents a comprehensive systematic literature review of semantic interoperability in electronic health records. We searched seven databases of articles published between 2010 to September 2020. We showed the most chosen scenarios, technologies, and tools employed to solve interoperability problems, and we propose a taxonomy around semantic interoperability in health records. Also, we presented the main approaches to solve the exchange problem of legacy and heterogeneous data across healthcare organizations.

Mucopolysaccharidosis type IVA (MPS IVA) is an inborn error of glycosaminoglycan (GAG) catabolism due to the deficient activity of N-acetylgalactosamine-6-sulfate sulfatase that leads to accumulation of the keratan sulfate and chondroitin 6-sulfate in body fluids and in lysosomes. The pathophysiology of this lysosomal storage disorder is not completely understood. The aim of this study was to investigate oxidative stress parameters, pro-inflammatory cytokine and GAG levels in MPS IVA patients. We analyzed urine and blood samples from patients under ERT (n=17) and healthy age-matched controls (n=10-15). Patients presented a reduction of antioxidant defense levels, assessed by a decrease in glutathione content and by an increase in superoxide dismutase activity in erythrocytes. Concerning lipid and protein damage, it was verified increased urine isoprostanes and di-tyrosine levels and decreased plasma sulfhydryl groups in MPS IVA patients compared to controls. MPS IVA patients showed higher DNA damage than control group and this damage had an oxidative origin in both pyrimidine and purine bases. Interleukin 6 was increased in patients and presented an inverse correlation with GSH levels, showing a possible link between inflammation and oxidative stress in MPS IVA disease. The data presented suggest that pro-inflammatory and pro-oxidant states occur in MPS IVA patients even under ERT. Taking these results into account, supplementation of antioxidants in combination with ERT can be a tentative therapeutic approach with the purpose of improving the patient's quality of life. To the best of our knowledge, this is the first study relating MPS IVA patients with oxidative stress.

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