A case of Ullrich disease with distinct pathological change of muscular dystrophy

A case of Ullrich disease was presented. The patient was a 3-year-old girl with torticollis, generalized muscle weakness and acroatonia since birth. High-arched palate, protruded calcaneus, and mild contracture of proximal joints were also recognized. Intellectual development was normal. Serum level of CPK was slightly increased. In histological and histochemical examinations of quadriceps femoris muscle, proliferated connective tissue, marked variation in the muscle fiber diameter, and a lot of degenerated and regenerated fibers were recognized. Minimal injury easily causes subcutaneous hemorrhage, but no abnormality was found in the structure of collagen.     

Properties of membranes from polyelectrolyte complexes consisting of methyl glycol chitosan, [2-(diethylamino)ethyl]dextran,and poly(potassium vinyl sulfate)

Water-insoluble polyelectrolyte complexes (PEC) were prepared by mixing methyl glycol chitosan (MGC, 1 ) and [2-(diethylamino)ethyl]dextran (EA, 2 ) with poly(potassium vinyl sulfate) (PVSK, 3 ) in aqueous solution at various hydrogen ion concentrations. Elemental analyses, IR spectroscopy, and solubilities of PEC reveal that PEC differ in molecular structure and properties according to pH. It seems that the degree of dissociation and the conformation of MGC, EA, and PVSK change with pH. PEC membranes were made by casting from solutions of all kinds of PEC, and transport phenomena through the membrane of PEC prepared in 4 wt.-% HCl solution were investigated under various conditions. The driving force of the transport depends on the membrance potential, Donnan potential, and diffusion potential, according to measurements of the transport ratio of Na⁺ and the electric potential difference between the left- and right-hand side of the membrane. Moreover, the permeability of K⁺ is higher than that of Na⁺.