Brain and ventricular volume in patients with syndromic and complex craniosynostosis

textabstractPurpose: Brain abnormalities in patients with syndromic craniosynostosis can either be a direct result of the genetic defect or develop secondary to compression due to craniosynostosis, raised ICP or hydrocephalus. Today it is unknown whether children with syndromic craniosynostosis have normal brain volumes. The purpose of this study was to evaluate brain and ventricular volume measurements in patients with syndromic and complex craniosynostosis. This knowledge will improve our understanding of brain development and the origin of raised intracranial pressure in syndromic craniosynostosis. Methods: Brain and ventricular volumes were calculated from MRI scans of patients with craniosynostosis, 0.3 to 18.3 years of age. Brain volume was compared to age matched controls from the literature. All patient charts were reviewed to look for possible predictors of brain and ventricular volume. Results: Total brain volume in syndromic craniosynostosis equals that of normal controls, in the age range of 1 to 12 years. Brain growth occurred particularly in the first 5 years of age, after which it stabilized. Within the studied population, ventricular volume was significantly larger in Apert syndrome compared to all other syndromes and in patients with a Chiari I malformation. Conclusions: Patients with syndromic craniosynostosis have a normal total brain volume compared to normal controls. Increased ventricular volume is associated with Apert syndrome and Chiari I malformations, which is most commonly found in Crouzon syndrome. We advice screening of all patients with Apert and Crouzon syndrome for the development of enlarged ventricle volume and the presence of a Chiari I malformation

Development of Functional and Molecular Correlates of Vaccine-Induced Protection for a Model Intracellular Pathogen, F. tularensis LVS

In contrast with common human infections for which vaccine efficacy can be evaluated directly in field studies, alternative strategies are needed to evaluate efficacy for slowly developing or sporadic diseases like tularemia. For diseases such as these caused by intracellular bacteria, serological measures of antibodies are generally not predictive. Here, we used vaccines varying in efficacy to explore development of clinically useful correlates of protection for intracellular bacteria, using Francisella tularensis as an experimental model. F. tularensis is an intracellular bacterium classified as Category A bioterrorism agent which causes tularemia. The primary vaccine candidate in the U.S., called Live Vaccine Strain (LVS), has been the subject of ongoing clinical studies; however, safety and efficacy are not well established, and LVS is not licensed by the U.S. FDA. Using a mouse model, we compared the in vivo efficacy of a panel of qualitatively different Francisella vaccine candidates, the in vitro functional activity of immune lymphocytes derived from vaccinated mice, and relative gene expression in immune lymphocytes. Integrated analyses showed that the hierarchy of protection in vivo engendered by qualitatively different vaccines was reflected by the degree of lymphocytes' in vitro activity in controlling the intramacrophage growth of Francisella. Thus, this assay may be a functional correlate. Further, the strength of protection was significantly related to the degree of up-regulation of expression of a panel of genes in cells recovered from the assay. These included IFN-γ, IL-6, IL-12Rβ2, T-bet, SOCS-1, and IL-18bp. Taken together, the results indicate that an in vitro assay that detects control of bacterial growth, and/or a selected panel of mediators, may ultimately be developed to predict the outcome of vaccine efficacy and to complement clinical trials. The overall approach may be applicable to intracellular pathogens in general

The effect of traditional home remedies on glycemic control among people with type 2 diabetes mellitus (T2DM)

Traditional home remedy consumption is a typical ancient practice in India. These traditional home remedies are found to have beneficial effects on many chronic conditions. This study was designed to explore the effect of traditional home remedies on glycemic control in people with type 2 Diabetes Mellitus (DM). In this study, 148 type-2 DM patients aged between 35 and 70 of both genders, participated. Among 148 type-2 DM patients, 102 T2DM patients use traditional home remedies along with oral anti-diabetic drugs, while the remaining 46 are non-users. The details of age, duration of type-2 DM, glycated Hb (HbA1c) values, and use of traditional home remedies were obtained from a cross-sectional survey. The HbA1c value of 7-8% was considered an optimal target glycemic control, and ≤ 7% was considered poor control. A mean and SD were used to represent descriptive statistics. An independent sample test was used to compare the mean HBA1c between the fenugreek users and non-users by considering p<0.05 as statistically significant. The majority of type-2 DM in our study group used Trigonella foenum-graecum (Fenugreek) (76.47%). A small proportion of our study group is using Azadirachta indica (Indian lilac or neem) (7.84%), Momordica charantia (bitter guard) (3.93%), and Aegle marmelos L. (Bengal quince or bael) (2.94%). The HbA1c levels in the majority of the traditional home remedy users were within the recommended target levels. The mean HbA1c levels of fenugreek non-users were significantly higher (p<0.001) than fenugreek users. In conclusion, our study shows that type-2 DM traditional home remedy users have better glycemic control than non-users. Home remedies are potent natural food sources that can be used with anti-diabetic drugs. However, such a use should be done with the knowledge of treating doctors, which may help to achieve better glycemic control and prevent type-2 DM-related complications