Increased Laterality of the Thalamus in Children and Adolescents with Asperger's Disorder: An MRI and Proton Spectroscopy Study

Objective

Thalamic abnormalities have been reported in people with pervasive developmental disorders (PDD) including Asperger''s Disorder (ASP). The aim of the present study was to compare the volume and volume fraction of the thalamus and the metabolite concentrations in children and adolescents with ASP using the magnetic resonance imaging and proton magnetic resonance spectroscopy. Additionally, the relationships between thalamic abnormalities and clinical features were examined.

Methods

Volume and volume fractional and metabolic measurements of bilateral thalamus were collected from 15 boys with ASP with a total IQ over 70 (age range 7-18 years, mean age 11.6±3.79 years), and 15 healthy controls matching age, sex and IQ. The thalamic volumes, hemisphere volumes and total brain volumes (TBV) were estimated using the stereological methods on magnetic resonance images. Chemical metabolites of thalamus were evaluated by ¹H spectroscopy.

Results

No differences in thalamic volumes, volume fractions and metabolites were observed between the groups. There were significant correlation between thalamic volume and total brain volume in both groups. The ASP group showed a significant left-minus-right thalamus difference as well as a significantly greater laterality index. In addition, a significant correlation between the laterality index and Autism Behavior Checklist language scores was observed.

Conclusion

Findings from this investigation point to a significant increase in laterality of the thalamus and a relationship with language problems in individuals with ASP. Our findings suggest that thalamic abnormalities may be related to mild language problems observed in ASP.     

Exercise tolerance,lung function abnormalities,anemia, and cardiothoracic ratio in sickle cell patients

Many patients with sickle cell disease (SCD) have a reduced exercise capacity and abnormal lung function. Cardiopulmonary exercise testing (CPET) can identify causes of exercise limitation. Forty‐four consecutive SCD patients (27 HbSS, 11 HbSC, and 6 HbS‐beta thalassemia) with a median age (interquartile range) of 26 (21–41) years underwent pulmonary function tests, CPET, chest x‐ray, and echocardiography to further characterize exercise limitation in SCD. Peak oxygen uptake (V′O₂‐peak), expressing maximum exercise capacity, was decreased in 83% of the studied patients. V′O₂‐peak correlated with hemoglobin levels (R = 0.440, P = 0.005), forced vital capacity (FVC) (R = 0.717, P < 0.0001). Cardiothoracic ratio on chest x‐ray inversely correlated with FVC (R = ?0.637, P < 0.001). According to criteria for exercise limitation, the patients were limited in exercise capacity due to anemia (n = 17), cardiovascular dysfunction (n = 2), musculoskeletal function (n = 10), pulmonary ventilatory abnormalities (n = 1), pulmonary vascular exercise limitation (n = 1), and poor effort (n = 3). In the present study we demonstrate that anemia is the most important determinant of reduced exercise tolerance observed in SCD patients without signs of pulmonary hypertension. We found a strong correlation between various parameters of lung volume and cardiothoracic ratio and we hypothesize that cardiomegaly and relative small chest size may be important causes of the impairment in pulmonary function, that is, reduced long volumes and diffusion capacity, in SCD. Taking into account anthropomorphic differences between SCD patients and controls could help to interpret lung function studies in SCD better. Am. J. Hematol. 89:819–824, 2014. © 2014 Wiley Periodicals, Inc.     

Depression severity is associated with increased risk behaviors and decreased CD4 cell counts

Depression is a common comorbidity among HIV-infected individuals. We studied the relationship between depressive symptoms, risk behaviors (risky-sexual behavior, tobacco, alcohol, and illicit drug use) and HIV outcomes. This cross-sectional study conducted in 2009 at the Washington University HIV Clinic included screening for depression with patient health questionnaire, survey of sexual behavior, illicit drug, alcohol, and tobacco use within 30 days. Sociodemographics, plasma HIV RNA levels, CD4 cell counts, and sexually transmitted disease test results were obtained from medical records. Multivariate logistic and linear regression models were used to assess the association between depressive symptoms severity and risk behaviors, HIV outcomes and combination antiretroviral therapy (cART) adherence. A total of 624 persons completed the assessment of whom 432 (69%) were male and 426 (68%) African-American. The median CD4 cell count was 410 cells/mm³ and 479 persons (77%) were on cART of whom 112 (23%) had HIV RNA level > 400 copies/mL. Overall, 96 (15%) had symptoms of major depressive disorder. Depressive symptom severity was associated with increased likelihood of high-risk drinking (odds ratio [OR], 2.4; 95% confidence interval [CI], 1.1–5.1), current tobacco use (OR, 1.8; 95% CI, 1.1–2.9), illicit drug use (OR, 1.7; 95% CI, 1.0–2.8), and risky-sexual behavior (OR, 1.5; 95% CI, 0.8–2.7). Suboptimal cART adherence (visual analog scale < 95%) was also associated with depressive symptoms severity (p < 0.05). After adjustment for age, sex, race, receipt of cART, and cART adherence, depressive symptoms severity was independently associated with lower CD4 cell count (p < 0.05) but not with higher HIV RNA level (p = 0.39). Depression adversely affects HIV-infected individuals, requiring greater effort at utilizing multidisciplinary interventions.     

A Rare Presentation of Extramedullary Hematopoiesis in Post-polycythemic Myelofibrosis

Intravenous Immunoglobulin G (IVIG) therapy has been used as a component of the treatment of hemolytic disease of the newborn. There is still no consensus on its use in ABO hemolytic disease of the newborn routinely. The aim of this study is to determine whether administration of IVIG to newborns with ABO incompatibility is necessary. One hundred and seventeen patients with ABO hemolytic disease and positive Coombs test were enrolled into the study. The subjects were healthy except jaundice. Infants were divided into two groups: Group I (n = 71) received one dose of IVIG (1 g/kg) and LED phototherapy whereas Group II (n = 46) received only LED phototherapy. One patient received erythrocyte transfusion in Group I, no exchange transfusion was performed in both groups. Mean duration of phototherapy was 3.1 ± 1.3 days in Group I and 2.27 ± 0.7 days in Group II (p < 0.05). Mean duration of hospital stay was 5.34 ± 2.2 days in Group I and 3.53 ± 1.3 days in Group II (p < 0.05). Mean duration of phototherapy was 4.0 ± 1.5 days and 2.73 ± 1.1 days in double and single doses of IVIG respectively, and this was statistically significant (p < 0.05). IVIG therapy didn’t decrease neither phototherapy nor hospitalization duration in infants with ABO hemolytic disease. Meticulus follow-up of infants with ABO hemolytic disease and LED phototherapy decreases morbidity. IVIG failed to show preventing hemolysis in ABO hemolytic disease.     

Effect of methylphenidate treatment on appetite and levels of leptin,ghrelin, adiponectin,and brain-derived neurotrophic factor in children and adolescents with attention deficit and hyperactivity disorder

Objectives. We aimed to explore whether the use of methylphenidate relates leptin, ghrelin, adiponectin, and brain-derived neurotrophic factor (BDNF). In addition, the relationship between methylphenidate-related weight loss in attention deficit hyperactivity disorder (ADHD) patients and these biomolecules were evaluated. Methods. Thirty ADHD patients receiving methylphenidate and 20 healthy controls were included. Leptin, ghrelin, adiponectin, and BDNF levels were measured at baseline and after two-month treatment in both groups. Results. At baseline, leptin, ghrelin, adiponectin, and BDNF levels were similar in the ADHD and control groups. The most common adverse events occurring in the ADHD group after a 2-month treatment period included loss of appetite (70%) and weight loss (66.7%). A significant difference was found in body weight, BMI, and CGI scores of the ADHD patients after the treatment. While post-treatment ghrelin and adiponectin levels were significantly higher in the ADHD group, BDNF level was significantly lower. Post-treatment decrease in leptin levels was not significant. Conclusions. Leptin and BDNF were not associated with poor appetite and/or weight loss due to methylphenidate treatment. However, ghrelin and adiponectin might be biomolecules that play a role in underlying neurobiological mechanisms of methylphenidate-related appetite or weight loss.     

Subclinical cardiovascular disease and its association with risk factors in children with steroid-resistant nephrotic syndrome

Background

The aim of this study was to evaluate the presence of subclinical cardiovascular disease (CVD) and its relation to risk factors in pediatric patients with steroid-resistant nephrotic syndrome (NS).

Methods

Thirty-seven patients with normal renal function were compared with 22 healthy controls regarding the presence of subclinical CVD. Measurements included aortic pulse wave velocity (PWV), carotid intima media thickness (IMT), and left ventricular mass (LVM). Patients were additionally assessed for blood pressure (BP) pattern and the presence of hypertension by 24-h ambulatory blood pressure monitoring.

Results

Compared with the controls, patients had significantly higher mean aortic PWV-standard deviation scores (SDS), mean carotid IMT-SDS, and LVM index (p?<?0.001 for all). Increased aortic PWV was noted in 5 % of patients, increased carotid IMT in 22 %, and increased LVM index in 19 %. Five patients (14 %) were hypertensive, and mean BP indexes, SDS, and BP loads during nighttime were significantly higher than those during daytime (p?<?0.001 for all). Multivariate analysis revealed a significant relationship between PWV-SDS and ferritin (R ²?=?0.269, p?=?0.006) and between carotid IMT-SDS and proteinuria (R ²?=?0.141, p?=?0.022). The LVM index was independently associated only with higher body mass index SDS (R ²?=?0.317, p?<?0.001). In addition, six patients (16 %) had multiple abnormal subclinical CVD markers, and increased subclinical CVD risk was independently associated only with higher low-density lipoprotein cholesterol (R ²?=?0.292, p?=?0.044).

Conclusions

Based on these results, steroid-resistant NS children generally are at high risk of cardiovascular complications, but the increased risk is likely to be multifactorial.