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This classification represents an expansion on the strategy of Kissane116 and Elkin and Bernstein,118 employing key groupings of genetic disorders, such as the 2 polycystic kidney diseases, and entities with similar morphologic findings, such as metanephric dysgenesis of renal dysplasias, without mixing pathogenetically unrelated entities, such a medullary sponge kidney with the genetic medullary cystic diseases and nephronophthisis Unfortunately, the Elkin and Bernstein,118 and the Brisceglia et al (113) formulations suffer from unnecessary additional complexity by inclusion of diseases that lack true cyst formation or maldevelopment as defined in the glossary of terms in Table 3.
Two new fact sheets from the National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK), part of the National Institutes of Health, explain two of these disorders: kidney dysplasia and medullary sponge kidney.
The name medullary sponge kidney is derived from the appearance on x-ray of the small aggregates of fluid sacs, or cysts, in the renal papilla.