Patients with myotonic dystrophy (DM) have recently been reported to be at increased risk of tumor development, but clinical associations related to this observation are unknown. We calculated the odds ratios (ORs) and 95% confidence intervals (CI) of self-reported tumor development by patients’ demographic and clinical characteristics to evaluate factors associated with tumor development in DM patients, using data from the National Registry of Myotonic Dystrophy and Facioscapulohumeral Dystrophy Patients and Family Members. Of the 911 participants, 47.5% were male and 85.7% had DM type 1 (DM1). Compared with DM1, patients with DM type 2 (DM2) were older at Registry enrollment (median age =55 vs. 44 years, p<0.0001) and at DM diagnosis (median age= 48 vs. 30 years, p<0.0001); and more likely to be females (p=0.001). At enrollment, 95 (10.4%) DM patients reported a history of benign or malignant tumor. Tumors were associated with female gender (OR=1.9, 95% CI=1.2–3.1, p=0.007) and DM1 (OR=2.1, 95% CI=1.1–4.1, p=0.03). In a subgroup analysis of patients with blood-based DNA testing results (397 DM1, 54 DM2), repeat expansion size was not associated with tumor risk in DM1 (p=0.26) or DM2 (p=0.34). In conclusion, female gender and DM1 subtype, but not DNA repeat expansion size, were associated with increased risk of tumors in DM. Follow-up studies are warranted to determine if oncogenes associated with dystrophia myotonica-protein kinase (DMPK) are altered in DM, and to determine if repeat expansion size, as in our study, is not associated with tumor development.

This work reports the facile, controlled, and low-cost synthesis of a nickel oxide and polyaniline (PANI) nanocomposites-based electrode material for supercapacitor application. PANI-NiO nanocomposites with varying concentrations of NiO were synthesized via in-situ chemical oxidative polymerization of aniline. The XRD and FTIR support the interaction of PANI with NiO and the successful formation of the PANI-NiO-x nanocomposite. The SEM analysis showed that the NiO and PANI were mixed homogenously, in which the NiO nanomaterial was incorporated in porous PANI globular nanostructures. The multiple phases of the nanocomposite electrode material enhance the overall performance of the energy-storage behavior of the supercapacitor that was tested in 1 M H2SO4 using cyclic voltammetry (CV), galvanostatic charge-discharge (GCD), and electrochemical impedance spectroscopy (EIS). Among the different nanocomposites, PANI-NiO-3 exhibit the specific capacitance of a 623 F g−1 at 1 A g−1 current density. Furthermore, the PANI-NiO-3 electrode retained 89.4% of its initial capacitance after 5000 cycles of GCD at a 20 A g−1 current density, indicating its significant cyclic stability. Such results suggest that PANI-NiO nanocomposite could be proposed as an appropriate electrode material for supercapacitor applications.

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Mononuclear cells of malnourished children contain diminished activity of phosphoglycerate kinase and pyruvate kinase (PK). The PK activity of these cells correlates well with the percentage of circulating thymus-derived lymphocytes (T-cells). Phytohemagglutinin causes an immediate increase in PK activity of mononuclear cells of malnourished patients. The correlation of PK activity with T-cells and the response of PK activity to phytohemagglutinin are in distinct contrast to observed perturbations of neonatal mononuclear cell metabolism. The relationship of the metabolic alterations to the pathophysiology of the immune system in malnutrition has not yet been defined.

SummaryThe metabolic capacity of neonatal monocytes was compared to the metabolic capacity of adult monocytes by two entirely different methods: the selective diminution of monocyte contamination of whole mononuclear cells and the isolation of relatively purified populations of monocytes.Monocyte removal from whole mononuclear cells produced a diminution in the pyruvate kinase (PK) activity (from 28.6 +: 1.1 to 15.6 + 1.2 nmoles/min/107 cells) and an increase in adenosine triphosphate (ATP) content (from 7.9 + 1.0 to 9.5 + 0.8 nmoles/ 10' cells) in adult cells. No change in PK activity (from 13.5 2 1.3 to 14.0 f 1.3) was observed in cord cells, but the ATP content of cord cells was higher after monocyte depletion (from 4.7 f 0.5 to 6.2 + 0.7).The suggestion of metabolic vulnerability was confirmed by metabolic analysis of isolated adult and cord monocytes. The PK activity of adult monocytes was greater than that of cord monocytes (57 + 9 and 25 + 0.3, respectively) and the ATP content of adult mopocytes (5.7 f 0.2) was greater than that of cord monocytes (2.3 * 0.1).The data confirm prior observations of diminished energy metabolism in neonatal mononuclear cells and suggest that the metabolic perturbations may, in part, correlate with functional immaturity of the neonatal monocyte. SpeculationMonocytes have a glycolytic rate that far exceeds that of other mononuclear cells. The presence of an increased rate of energy synthesis, however, is not accompanied by an increase in ATP content. The metabolic requirements of monocytes are, therefore, greater than for other mononuclear cells. This suggests that monocytes may be susceptible to dysfunction secondary to metabolic stress (increased metabolic requirements or diminished metabolic capacity). The neonatal monocyte appears to be much more metabolically vulnerable than the adult monocyte and this may. in part, account for the immune dysfunction observed in the newborn infant.Observations of increased frequency and severity of infections among newborn infants suggest that the immature immune system may not function as well as that of older individuals. A variety of data, reviewed previously (5), suggests that the cell-mediated immune function of newborns is impaired compared to that of older children and adults, but the specific nature of this dysfunction is unclear.In an attempt to clarify the nature and cause of neonatal immune dysfunction, we began a study of neonatal mononuclear cell metabolism. We demonstrated diminished content of several enzymes and ATP in the mononuclear cells of newborn infants (5). In particular, we noted decreased activity of PK (ATP: pyruvate phosphotransferase-EC: 2.7.1.40) activity, which increased during the first year of life to the level found in adult mononuclear cells. Furthermore, the PK activity of adult cells could be stimulated by exposure to high concentrations of phytohemagglutinin, while neonatal PK activity was unaffected by phytohemagglutinii. Glycolytic metabolism of neonatal mononuclear cells was reduced by 10-20% compar...