Diffusion tensor imaging of the brain in Pompe disease

Journal of Neurology - Enzyme replacement therapy has drastically changed prospects of patients with Pompe disease, a progressive metabolic myopathy. As classic infantile patients survive due to...     

Treatment of class III multiple gingival recessions: a randomized-clinical trial

Background: The aim of this controlled randomized split-mouth study was to evaluate whether a modified tunnel/connective tissue graft (CTG) technique – enamel matrix derivative (EMD) combination will improve the treatment of multiple class III recession when compared with the same technique alone.
Materials and Methods: Twenty healthy subjects with a mean age of 31.7 years, were enrolled for the trial in a university periodontal clinic. Patients with at least three adjacent gingival recessions on both sides of the mouth were treated with a modified tunnel/CTG technique. On the test side, an EMD was used in addition. Clinical parameters were measured at baseline, 28 days, 3, 6 and 12 months after the surgery. Results are presented at the subject level.
Results: The mean root coverage from baseline to 1 year post-surgery was 82% for the test group and 83% for the control group. Complete root coverage was achieved at 1 year in eight (38%) of the 20 surgeries (experimental and control group).
Conclusions: One-year results indicate that the modified tunnel/CTG technique is predictable for the treatment of multiple class III recession-type defects. The addition of EMD does not enhance the mean clinical outcomes.     

IL‐36γ has proinflammatory effects on human endothelial cells

Interleukin‐36 cytokines are predominantly expressed by epithelial cells. Significant upregulation of epidermal IL‐36 is now a recognised characteristic of psoriatic skin inflammation. IL‐36 is known to induce inflammatory responses in dendritic cells, fibroblasts and epithelial cells. Although vascular alterations are a hallmark of psoriatic lesions and dermal endothelial cells are well known to play a critical role in skin inflammation, the effects of IL‐36 on endothelial cells are unexplored. We here show that endothelial cells including dermal microvascular cells express a functionally active IL‐36 receptor. Adhesion molecules VCAM‐1 and ICAM‐1 are upregulated by IL‐36γ stimulation, and this is reversed by the presence of the endogenous IL‐36 receptor antagonist. IL‐36γ‐stimulated endothelial cells secrete the proinflammatory chemokines IL‐8, CCL2 and CCL20. Chemotaxis assays showed increased migration of T‐cells following IL‐36γ stimulation of endothelial cells. These results suggest a role for IL‐36γ in the dermal vascular compartment, and it is likely to enhance psoriatic skin inflammation by activating endothelial cells and promoting leucocyte recruitment.     

The role of isotretinoin in acne therapy: why not as first-line therapy? facts and controversies

Acne is one of the most prevalent diseases in dermatology: Millions of people worldwide experience this distressing condition. To determine the appropriate therapeutic strategy, there is a strong need for a standardized classification system of acne. The exact molecular mechanism of action of isotretinoin is not completely understood; however, oral isotretinoin targets simultaneously at all major mechanisms of acne pathogenesis. Various mass media reports about the risk of teratogenicity and depression from isotretinoin usage as well as the creation of intense prevention programs have created an obstacle to the use of the most active available drug against acne, presenting isotretinoin as a very dangerous regimen. According to recommendations of several international experts, which we share, oral isotretinoin may be prescribed not only to patients with severe disease but indications should be broadened to also include patients with less severe forms of acne, especially in cases with scarring, significant psychologic stress, or failure to respond to conventional therapy.     

Developmental,familial and educational characteristics of a sample of children with Autism Spectrum Disorders in Greece

The aim of this study is to describe the developmental, familial and educational characteristics of 91 children with a clinical diagnosis of autism spectrum disorders (ASDs), from one educational district of Athens, Greece. Records of the 91 children, aged 4–14 years old, were examined with respect to sex, age of diagnosis, type of ASDs, school placement, co-existing disorders, parental ages, type of conception, prematurity and birth order. The results indicated that the boy:girl ratio was 4.3:1 while the average age of diagnosis was 6 years which implies a delay in the early detection of ASDs. Children with typical autism constituted 80% of the sample, while the Asperger group constituted 20%. The majority of pupils were served in general education, and only a small percentage attended special schools. The preferable type of support was one-to-one support in the classroom (47%). Among the most common disorders that coexist with the ASDs was ADHD, epilepsy, dyslexia and learning difficulties. Interesting findings were obtained for the advanced parental age, birth order and the relationship of assisted conception and premature birth with the presence of autism. The present study offers some useful insights about the characteristics of ASDs in a Greek school-based sample.