Somatic deletion of the imprinted 11p15 region in sporadic persistent hyperinsulinemic hypoglycemia of infancy is specific of focal adenomatous hyperplasia and endorses partial pancreatectomy

Sporadic persistent hyperinsulinemic hypoglycemia of infancy (PHHI) or nesidioblastosis is a heterogeneous disorder characterized by profound hypoglycemia due to inappropriate hypersecretion of insulin. An important diagnostic goal is to distinguish patients with a focal hyperplasia of islet cells of the pancreas (FoPHHI) from those with a diffuse abnormality of islets (DiPHHI) because management strategies differ significantly. 16 infants with sporadic PHHI resistant to diazoxide and who underwent pancreatectomy were investigated. Selective pancreatic venous sampling coupled with peroperative surgical examination and analysis of extemporaneous frozen sections allowed us to identify 10 cases with FoPHHI and 6 cases with DiPHHI. We show here that in cases of FoPHHI, but not those of DiPHHI, there was specific loss of maternal alleles of the imprinted chromosome region 11p15 in cells of the hyperplastic area of the pancreas but not in normal pancreatic cells. This somatic event is consistent with a proliferative monoclonal lesion. It involves disruption of the balance between monoallelic expression of several maternally and paternally expressed genes. Thus, we provide the first molecular explanation of the heterogeneity of sporadic forms of PHHI such that it is possible to perform only partial pancreatectomy, limited to the focal somatic lesion, so as to avoid iatrogenic diabetes in patients with focal adenomatous hyperplasia.     

Effects of jumping skill training on walking balance for children with mental retardation and Down's syndrome

In the present study, we hypothesized that the enhancements obtained from the practice of jumping activity could be transferred to improve the walking balance in children with mental retardation (MR) and Down's syndrome (DS). SUBJECTS: Fourteen children with the diagnosis of MR or DS, aged 3 to 6 years, were recruited from a day care institution. They were ambulant but without jumping ability. Sixty-one non-handicapped children was used to serve as a normative comparison group. METHOD: Before the training program, the performances of walking balance, jump skills and jumping distances were assessed individually by one physical therapist. The balance sub-test in the Bruininks Oseretsky Test of Motor Proficiency (BOTMP) was administered to assess the walking balance. Motor Skill Inventory (MSI) was used to assess the qualitative levels of jumping skills. A jumping skill training lesson that included horizontal jumps and vertical jumps was designed and integrated into the educational program. The recruited children received 3 sessions of training per-week for 6 weeks. A post-training test and a follow-up test were administered to the handicapped children. RESULT: In BOTMP scores, statistical differences exited between the pre-training and post-training tests in the tested items of floor walk and beam walk. However, no significant difference was found in the items of floor stand, beam stand and floor heel-toe walk. MSI scales revealed there were significant differences between pre-training and post-training tests. There was no significant difference between the scores of post-training test and the follow-up test. DISCUSSION: The results implicated that the jumping activity might effectively evoke the automatic and dynamic postural control. Moreover, the significant improvements of the floor walk and beam walk performances might be due to the transferred effects via the practice of dynamic jumping activity. Furthermore, implications and suggestions are discussed.     

Early patterning of prelimbic cortical axons to the striatal patch compartment in the neonatal mouse

The striatum receives excitatory input from virtually the entire cerebral cortex. In the adult, this input is segregated into two functionally distinct compartments of the striatum, the patch (striosome) and matrix regions. This study determined whether the patterning of corticostriatal afferents from the prelimbic cortex to the striatal patch compartment develops during the early period of collateral formation or instead at the time of peak synaptogenesis. Initial formation of corticostriatal axon collaterals was observed by embryonic day (E) 19. Quantification of corticostriatal collaterals revealed a significant increase in the number and complexity of collateral branches at postnatal day 6 as compared to E19. Concomitant with the increase in collateral branching, a heterogeneous pattern of collateralization consisting of parallel rows of corticostriatal collaterals was observed in the medial striatum. In addition to the rows, clusters of corticostriatal axons occurred more laterally. These clusters colocalized with patches of dense tyrosine hydroxylase-positive fibers, a marker for the striatal patch compartment in the neonatal mouse. Together, these data indicate that corticostriatal patterning occurs during the period of early axon collateralization resulting in a segregation of corticostriatal axon collaterals from the prelimbic cortex to the striatal patch compartment.     

Toughening of Dental Porcelain by Tetragonal ZrO2 Additions

The effect on mechanical behavior of ZrO₂ additions to a dental porcelain was investigated. The ZrO₂ was introduced into the glassy matrix phase of the porcelain by refritting the all-glass porcelain constituent. X-ray diffraction indicated that a sizeable fraction of the ZrO₂ was retained in the tetragonal form after the porcelain was fired. Zirconia additions to the porcelain produced substantial improvements in fracture toughness, strength, and thermal shock resistance.