Botulinum toxin blocks mast cells and prevents rosacea like inflammation

Background

Rosacea is a chronic inflammatory skin condition whose etiology has been linked to mast cells and the antimicrobial peptide cathelicidin LL-37. Individuals with refractory disease have demonstrated clinical benefit with periodic injections of onabotulinum toxin, but the mechanism of action is unknown.

Classification and cyclic fatigue evaluation of new kinematics for endodontic instruments

This study evaluated the effect of new motions of the motor TriAuto ZX2 on the cyclic fatigue of endodontic instruments. Vortex Blue 35.06 instruments were divided into four groups (n = 10) and tested for fatigue in a curved artificial canal (90° and 2 mm radius) using the following motions: continuous rotation (CR), Optimum Torque Reverse (OTR) set at 180° and the Optimum Glide Path (OGP), which was tested at 90° and 240°. The time to fracture (TTF) and the lengths of the fractured fragments were recorded. The mean TTF was significantly different among the groups (anova , P < 0.05): OGP 90° (213.39 ± 27.45), OTR 180° (121.24 ± 17.03), OGP 240° (45.24 ± 5.61) and CR (8.43 ± 1.27). Weibull analysis confirmed the shortest life expectancy for CR and the longest survival for OGP at 90°. The resistance to fatigue was affected by motions and pre‐set angles. The proprietary movements that are currently available for endodontic instruments were classified according to their kinematics.     

Analysis of the epsilon-sarcoglycan gene in familial and sporadic myoclonus-dystonia: evidence for genetic heterogeneity.

The epsilon-sarcoglycan gene (SGCE) on human chromosome 7q21 has been reported to be a major locus for inherited myoclonus-dystonia. Linkage to the SGCE locus has been detected in the majority of families tested, and mutations in the coding region have been found recently in families with autosomal dominant myoclonus-dystonia. To evaluate the relevance of SGCE in myoclonus-dystonia, we sequenced the entire coding region of the epsilon-sarcoglycan gene in 16 patients with either sporadic or familial myoclonus-dystonia. No mutations were found. This study suggests that epsilon-sarcoglycan does not play an important role in sporadic myoclonus-dystonia and supports genetic heterogeneity in familial cases.     

Identical mutations in the CSB gene associated with either Cockayne syndrome or the DeSanctis-cacchione variant of xeroderma pigmentosum

Xeroderma pigmentosum (XP) and Cockayne syndrome (CS) are two hereditary disorders in which photosensitivity is associated with distinct clinical and cellular phenotypes and results from genetically different defects. We have identified the primary molecular alteration in two patients in whom clinical manifestations strongly reminiscent of a severe form of XP were unexpectedly associated with the CS cellular phenotype and with a defect in the CSB gene. Sequencing of the CSB -coding region in both cDNA and genomic DNA showed that these patients had identical alterations to those in a patient with the clinical features of the classical form of CS. These data, together with fluorescence in situ hybridization analysis, demonstrated that the two siblings with XP as well as the CS patient were homozygous for the same CSB mutated allele, containing a silent C2830T change and a nonsense mutation C2282T converting Arg735 to a stop codon. The finding that the same inactivating mutation underlies different pathological phenotypes indicates that there is no simple correlation between the molecular defect and the clinical features. Therefore, alterations in the CSB gene give rise to the same repair defect at the cellular level but other genetic and/or environmental factors determine the pathological phenotype.     

Paroxysmal dystonia and paroxysmal tremor in a young patient with multiple sclerosis

A 16-year-old patient with multiple sclerosis (MS) showed paroxysmal movement disorders during a recurrence of the disease. The paroxysms took the form ot brief unilateral dystonic posturings of the right body suggestive of paroxysmal dystonia (PD); they completely receded with acetazolamide. A single episode of a high amplitude, rythmic slow and coarse generalized tremor, present at rest and increasing with movement, particularly involving the head in a no-no movement, occurred soon after recovery from PD and lasted three hours. The present report provides evidence that MS has to be considered in the diagnostic approach to symptomatic childhood PD and underlines the efficacy of acetazolamide in the treatment of PD attacks. It also describes a rare paroxysmal movement disorder, defined as paroxysmal dystonic tremor, that can be considered as falling within the spectrum of PD.This study was partially supported by the Paolo Zorzi Association for Neurosciences, and the Harry De Jur Foundation.     

Surgical repair of pectus excavatum by internal metal strut fixation. Clinical experience in 51 cases

BACKGROUND: Broad is the spectrum of congenital chest wall deformities. Fortunately the severe life-threatening deformities (i.e. ectopia cordis and Jeune's disease) are rarer than the more frequent pectus excavatum and carinatum. Funnel chest is the most common anterior chest wall deformity, it results from posterior depression of the sternum and cartilages, from the third to the eighth; it occurs more frequently in boys than in girls (4:1 ratio). While the deformity may be recognizable at birth, especially during crying spells, it progressively worsens during childhood, producing, sometimes, respiratory and cardiac impairment. METHODS: The authors report their experience, from April 1970 to December 1998, in correction of Pectus Excavatum using a single surgical technique, at the Borgo Roma Hospital of Verona. During this period 51 patients (mean age 14 years) affected by this deformity, were treated performing a sterno-chondroplasty with internal fixation. Most of the patients (94%) required operation for the esthetic and psychological reasons only. The grade of funnel chest (according to the Chin Classification) was: type I in 59.6% of patients, type II in 31.4% and type III in 11.7%. The surgical technique consisted in the modification of the Ravitch's technique (subperichondrial cartilage resection, transverse osteotomy with internal stainless steel strut fixation). We considered the intervention advisable to correct an existing defect or to prevent its progression. We don't think, in fact, that spontaneous recession of the deformity may occur. RESULTS: The follow-up varied from 4 to 18 years. The result was classified according to the classification of Humphreys and it was excellent in 64.7%, good in 17.7%, fair in 9.8% and poor in 7.8%. CONCLUSIONS: This technique proved to be safe and effective, it can be performed with no mortality, very low complication rate and satisfactory results.