ABNORMAL VITAMIN D METABOLISM IN PAGET''S DISEASE OF BONE

We have studied several biochemical indices of bone turnover and vitamin D metabolism in 32 untreated patients with Paget's disease and in 32 age-matched control subjects. patients with Paget's disease, as expected, were characterized by high bone turnover, as judged by alkaline phosphatase and urinary excretion of hydorxyproline. Serum values of 24,25-dihydroxyvitamin D3 (24,25(OH)2D3) and the ratio of 24,25(OH)2D3 to 25-OHD were significantly lower in patients than in control subjects. Serum concentrations of 1,25(OH)2D3 were normal in Paget's disease. The distribution of values for 24,25(OH)2D3 was log normal. On the basis of the normal range computed from control subjects, patients were divided into those with low or normal values for 24,25(OH)2D3. Disease activity, as judged by biochemical indices was significantly higher in the patients with the lower values of 24,25(OH)2D3. We conclude that Paget's disease is characterized by low circulating concentrations of 24,25(OH)2D3, particularly in patients with more extensive or severe disease.     

Spermiogenesis and spermiation in a monotreme mammal, the platypus, Ornithorhynchus anatinus

Spermatogenesis in the platypus ( Ornithorhynchus anatinus ) is of considerable biological interest as the structure of its gametes more closely resemble that of reptiles and birds than marsupial or eutherian mammals. The ultrastructure of 16 steps of spermatid development is described and provides a basis for determining the kinetics of spermatogenesis. Steps 1–3 correspond to the Golgi phase of spermatid development, steps 4–8 correspond to the cap phase, steps 9–12 are the acrosomal phase, and steps 13–16 are the maturation phase. Acrosomal development follows the reptilian model and no acrosomal granule is formed. Most other features of spermiogenesis are similar to processes in reptiles and birds. However, some are unique to mammals. For example, a thin, lateral margin of the acrosome of platypus sperm expands over the nucleus as in other mammals, and more than in reptiles and birds. Also, a tubulobulbar complex develops around the spermatid head, a feature which appears to be unique to mammals. Further, during spermiation the residual body is released from the caudal end of the nucleus of platypus sperm leaving a cytoplasmic droplet located at the proximal end of the middle piece as in marsupial and eutherian mammals. Other features of spermiogenesis in platypus appear to be unique to monotremes. For example, nuclear condensation involves the formation of a layer of chromatin granules under the nucleolemma, and development of the fibrous sheath of the principal piece starts much later in the platypus than in birds or eutherian mammals.     

Stability and decline in gross motor function among children and youth with cerebral palsy aged 2 to 21 years

This paper reports the construction of gross motor development curves for children and youth with cerebral palsy (CP) in order to assess whether function is lost during adolescence. We followed children previously enrolled in a prospective longitudinal cohort study for an additional 4 years, as they entered adolescence and young adulthood. The resulting longitudinal dataset comprised 3455 observations of 657 children with CP (369 males, 288 females), assessed up to 10 times, at ages ranging from 16 months to 21 years. Motor function was assessed using the 66-item Gross Motor Function Measure (GMFM-66). Participants were classified using the Gross Motor Function Classification System (GMFCS). We assessed the loss of function in adolescence by contrasting a model of function that assumes no loss with a model that allows for a peak and subsequent decline. We found no evidence of functional decline, on average, for children in GMFCS Levels I and II. However, in Levels III, IV, and V, average GMFM-66 was estimated to peak at ages 7 years 11 months, 6 years 11 months, and 6 years 11 months respectively, before declining by 4.7, 7.8, and 6.4 GMFM-66 points, in Levels III, IV, and V respectively, as these adolescents became young adults. We show that these declines are clinically significant.     

A Comparison of Commercially Available Polymethylmethacrylate-Based Soft Tissue Fillers

BACKGROUND The rapid market expansion of filler treatment options requires physicians and health care providers to fully understand differences among comparable products.
OBJECTIVE The objective was to compare commercially available polymethylmethacrylate (PMMA)-based soft tissue fillers to determine if there are meaningful variations in these products that could result in significantly different therapeutic profiles, especially with respect to safety.
METHODS AND MATERIALS PMMA particles were evaluated for size and morphology using scanning electron microscopy (SEM) techniques. PMMA microsphere soft tissue filler products from the United States, Europe, Brazil, and Canada were compared with respect to size, homogeneity/irregularity, surface smoothness/roughness, and the presence or absence of sediment and particulate debris.
RESULTS Marked differences with respect to PMMA particle morphology and related particle characteristics from a variety of products were found. Of note, some products demonstrated potentially concerning significant variability in particle size and irregular morphology.
CONCLUSION It is anticipated that the variability detected in these products, based on the literature, could result in different therapeutic profiles, especially with respect to safety. Physicians and health care providers should be aware that "comparable" products that at a glance appear similar may not be equal.