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In the last 10 years enzyme replacement therapy (ERT) has become an alternative for the treatment of patients with Hunter disease (HD). Nevertheless, the information regarding efficacy and safety is scarce and mainly based on the pivotal trials. This scarcity is especially evident for adults and severe forms of HD.A systematic review of publications in the electronic databases PUBMED, EMBASE and Cochrane Central was undertaken. Clinical trials and observational studies were included. The data about efficacy and security were retrieved and analysed with Review Manager version 5.3.677 records were found, 559 remaining after the removal of duplicates. By title and abstract review, 427 were excluded. Full reading of the rest was made (122 publications) and 42 were finally included. It was not possible to perform meta-analysis of all the endpoints due to high heterogeneity in the reporting and measuring of variables in each publication. Eight clinical trials were included, 6 with high risk of bias. The quality of the other studies was low in 12%, average in 68% and good in 21%. Main findings were: a reduction in the elimination of glycosaminoglycans (GAG) in urine in all the studies (26/26), decrease in liver and spleen size (18/18), increase of 52.59 m (95% CI, 36, 42-68.76, p .001) in the 6-min walk test (TM6M), increase in forced vital capacity (FVC) of 9.59% (95% CI 4.77-14.51, p .001), reduction of the left ventricular mass index of 3.57% (95% CI 1.2-5.93) and reduction in mortality (OR) of 0.44 (0.27-0.71).The data suggests a clear and consistent effect of ERT in HD reducing the accumulation of GAGs in the body, demonstrated by the reduction of its urinary excretion, as well as by the reduction of its deposits (spleen, liver and heart). Likewise, there is an improvement in physical and respiratory function. In addition, a reduction in mortality has been observed. Lack of studies, small size of the samples, and methodological deficiencies are the main limitations to establish definite conclusions.The data suggests that ERT is effective and safe in the treatment of HD. There is a need to evaluate patient-centred outcomes and the impact on quality of life.

[EN] Reverse engineering and medical image-based modeling technologies allow manufacturing of 3D biomodels of anatomical structures of human body. These techniques are based on anatomical information from scanning data such as CT and MRI, whose scanners are used for scanning data acquisition of the external and internal geometry of anatomical structures. These 3D biomodels have many medical applications such surgical training, preoperative planning, surgical simulation, diagnosis and treatments. 3D virtual models of human body structures based on CT are increasingly being used in clinical practice. A data processing methodology is required to obtain an accurate 3D model suitable for manufacturing using AM, and specially the FDM technologies. This study shows a step-by-step methodology to process the CT information in bounded uncertainty conditions in order to obtain the STL models of the degenerated bone components, and to manufacture the 3D biomodels for surgery analysis with optimal design and details, and with an adequate accuracy to ensure proper results by surgeons analysis., The authors wish to acknowledge the support of Ms. Jerica Risent and Mr. Joan Ortiz of Ford Motor Company for his assistance in the scanning of printed models. This work was supported by the Polisabio Funding (UPV-Fisabio 2017)