Diagnostic interest of whole-body MRI in early- and late-onset LAMA2 muscular dystrophies: a large international cohort

Journal of Neurology - LAMA2-related muscular dystrophy (LAMA2-RD) encompasses a group of recessive muscular dystrophies caused by mutations in the LAMA2 gene, which codes for the alpha-2 chain of...     

Home,school, and community violence exposure and emotional and conduct problems among low-income adolescents: the moderating role of age and sex

Purpose

The purpose of this study is to assess whether violence exposure is associated with emotional/conduct problems, when adjusting for confounders/covariates and controlling for comorbidity, and to investigate interactions between violence exposure and sex and/or age.

Methods

This cross-sectional study evaluated a community-based sample of 669 in-school 11–15-year-olds. A three-stage probabilistic sampling plan included a random selection of census units, eligible households, and target child. Multivariable logistic regression investigated the effect of severe physical punishment by parents, peer victimization at school, and community violence on the study outcomes (adolescent-reported emotional/conduct problems identified by the Strengths and Difficulties Questionnaire/SDQ) when controlling for confounders (resilience, parental emotional warmth, maternal education/unemployment/anxiety/depression) and covariates (age, sex, stressful life events, parental rejection).

Results

Considering interactions, emotional problems were associated with community violence victimization among girls, while conduct problems were associated with severe physical punishment among the younger, suffering peer aggression among the oldest, bullying victimization among girls, and witnessing community violence among boys. Desensitization (less emotional problems with greater violence exposure) was noted among the youngest exposed to severe physical punishment and the oldest who witnessed community violence.

Conclusion

Age and sex are moderators of the association between violence exposure and emotional/conduct problems. Interventions at local health units, schools, and communities could reduce the use of harsh physical punishment as a parental educational method, help adolescents deal with peer aggression at school and keep them out of the streets by increasing the usual five hours in school per day and making free sports and cultural/leisure activities available near their homes.

     

Prevalence of delayed gastric emptying in diabetic patients and relationship to dyspeptic symptoms: a prospective study in unselected diabetic patients

OBJECTIVE: Data on the prevalence of abnormal gastric emptying in diabetic patients are still lacking. The relation between gastric emptying and dyspeptic symptoms assessed during gastric emptying measurement has not yet been investigated. The aim was to investigate the prevalence of delayed gastric emptying in a large cohort of unselected diabetic patients and to investigate the relation between gastric emptying and gastrointestinal sensations experienced in the 2 weeks before and during the test meal, prospectively. RESEARCH DESIGN AND METHODS: Gastric emptying was evaluated in 186 patients (106 with type 1 diabetes, mean duration of diabetes 11.6 +/- 11.3 years) using 100 mg (13)C-enriched octanoic acid added to a solid meal. RESULTS: Gastric emptying was significantly slower in the diabetic subjects than in the healthy volunteers (T(50): 99.5 +/- 35.4 vs. 76.8 +/- 21.4 min, P < 0.003; Ret(120 min): 30.6 +/- 17.2 vs. 20.4 +/- 9.7%, P < 0.006). Delayed gastric emptying was observed in 51 (28%) diabetic subjects. The sensations experienced in the 2 weeks before the test were weakly correlated with the sensation scored during the gastric emptying test. Sensations assessed during the gastric emptying test did predict gastric emptying to some extent (r = 0.46, P < 0.0001), whereas sensations experienced in the previous 2 weeks did not. CONCLUSIONS: This prospective study shows that delayed gastric emptying can be observed in 28% of unselected patients with diabetes. Upper gastrointestinal sensations scored during the gastric emptying tests do predict the rate of gastric emptying to some extent and sensation experienced during daily life does not.     

Soluble interleukin 2 receptor and soluble CD8 levels in previously treated human immunodeficiency virus-negative hemophiliacs multiply transfused with a monoclonal antibody-purified factor VIII concentrate

BACKGROUND : Serum levels of the soluble interleukin 2 receptor (sIL-2R) and soluble CD8 (sCD8) may be used as markers of T-cell activation. The course of serum levels of sIL-2R and sCD8 in hemophiliacs who were treated first with an intermediate-purity factor VIII concentrate and then with a monoclonal antibody (MoAb)-purified factor VIII concentrate are reported. STUDY DESIGN AND METHODS : Serum samples taken before the administration of the MoAb-purified concentrate and after 2 and 5 years of its administration to 20 human immunodeficiency virus-negative patients with hemophilia A were analyzed. Eighteen healthy age-matched men were used as controls. RESULTS : The sIL-2R and sCD8 levels were higher in patients treated with intermediate-purity concentrates than in controls (p = 0.006 and p = 0.0005, respectively). The sIL-2R levels showed a decrease after 5 years of treatment with the MoAb-purified concentrate (p = 0.018 for the difference between 2 and 5 years), to levels that were not significantly different from those in controls. Although sCD8 levels tended to decrease at 5 years (p = 0.09, for the difference between 2 and 5 years), they remained higher than those in controls (p = 0.0005 and p = 0.0016 at 2 and 5 years, respectively). The ratio of sCD8 and sIL-2R tended to increase between 2 and 5 years (p = 0.07). The sIL-2R and sCD8 levels were not related to the numbers of T-lymphocytes and HLA-DR-positive T-lymphocytes in peripheral blood. Nor was a relation demonstrated between sIL-2R levels and CD4-positive cell numbers or between sCD8 levels and CD8-positive cell numbers. Although a relation with chronic hepatitis C cannot be excluded, it seems more likely that changes in sIL-2R levels are due to the use of the MoAb-purified concentrate. CONCLUSION : Elevated levels of sIL-2R and sCD8 were found in multiply transfused human immunodeficiency virus- negative hemophiliacs. After treatment was changed to the use of a MoAb- purified concentrate. sIL-2R levels decreased. These findings suggest a change in immune stimulation that is remarkable, because signs of activation in the effector phase seem to have continued despite normalization in the proliferative phase.     

Partitioning of nongaussian-distributed biochemical reference data into subgroups

BACKGROUND: The aim of this study was to develop new methods for partitioning biochemical reference data, covering in particular nongaussian distributions. METHODS: We recently proposed partitioning criteria for gaussian distributions. These criteria relate to proportions of the subgroups outside each of the reference limits of the combined distribution (proportion criteria) and to distances between the subgroup distributions as correlates of these proportions (distance criteria). However, distance criteria do not seem to be ideal for nongaussian distributions because a generally valid relationship between proportions and distances cannot be established for these. RESULTS: Proportion criteria appear preferable to distance criteria for two additional reasons: (a) The prevalences of the subgroup populations may have a considerable effect on stratification, but these are hard to account for by using distance criteria. Two methods to handle prevalences are described, the root method and the multiplication method. (b) Tied reference values, another complication of the partitioning problem, could also be hard to take care of using distance criteria. Some solutions to the problems caused by tied reference values are suggested. CONCLUSIONS: Partitioning of biochemical reference data should preferably be based on proportion criteria; this is particularly true for nongaussian distributions. Both of the described complications of the partitioning problem, the prevalences of the subgroups and tied reference values, are hard to deal with using distance criteria, but the proposed methods make it possible to account for them when proportion criteria are applied.