Mucopolysaccharidosis type IVA (MPS IVA, or Morquio syndrome type A) is an inherited metabolic lysosomal disease caused by the deficiency of the N-acetylglucosamine-6-sulfate sulfatase enzyme. The deficiency of this enzyme accumulates the specific glycosaminoglycans (GAG), keratan sulfate, and chondroitin-6-sulfate mainly in bone, cartilage, and its extracellular matrix. GAG accumulation in these lesions leads to unique skeletal dysplasia in MPS IVA patients. Clinical, radiographic, and biochemical tests are needed to complete the diagnosis of MPS IVA since some clinical characteristics in MPS IVA are overlapped with other disorders. Early and accurate diagnosis is vital to optimizing patient management, which provides a better quality of life and prolonged life-time in MPS IVA patients. Currently, enzyme replacement therapy (ERT) and hematopoietic stem cell transplantation (HSCT) are available for patients with MPS IVA. However, ERT and HSCT do not have enough impact on bone and cartilage lesions in patients with MPS IVA. Penetrating the deficient enzyme into an avascular lesion remains an unmet challenge, and several innovative therapies are under development in a preclinical study. In this review article, we comprehensively describe the current diagnosis, treatment, and management for MPS IVA. We also illustrate developing future therapies focused on the improvement of skeletal dysplasia in MPS IVA.

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This paper presents the preliminary results of different trials carried out with two species of mysids from Gran Canaria: Leptomysis lingvura (G.O. Sars, 1866) and Paramysis nouvel. Experiments lasting 21 days showed significantly higher fecundity and survival in L. lingvura than in P. nouveli (P<0.05). We also report the biochemical profile of both species fed 48‐h‐Artemia nauplii enriched with Easy‐DHA‐Selco® for 7 days. A comparison of our results with those of for Artemia and rotifers, organisms frequently used as live food in aquaculture, showed that mysids have a high percentage of protein per dry mass (73.38% in P. nouveli, and 74.19% in L. lingvura). Furthermore, the percentage of docosahexaenoic acid (DHA), eicosapentaenoic acid (EPA) and arachidonic acid (AA) in total fatty acids was higher in both species than that reported by Roo and colleagues for rotifers and Artemia. In addition to the content of these fatty acids, their ratios between them are also important for normal growth and larval development. We found that the ratio, DHA:EPA, was 0.85 0.02 and 0.89 0.01; the ratio, DHA: AA, 6.25 0.26 and 4.74 0.14; and the ratio, EPA:AA, 7.32 0.26 and 5.32 0.2, respectively, for P. nouveli and L. lingvura in cultures and these ratios do not significantly differ (P>0.05) from organisms in the wild. Here, we argue that as mysids are prey for many commercially important fish, cephalopods and rays, it is likely that the biochemical composition of mysids in their natural environment is “optimal” for these predators. Therefore, we studied the lipid profile of both species as they naturally occur in their environment. The results indicate that these mysids could be used to develop high quality live fish food.