ABSTRACT
PURPOSE: Congenital myasthenic syndromes include a wide range of early-onset genetic neuromuscular transmission disorders. Mutations in any one of genes coding for proteins related to the neuromuscular junction synaptic transmission function, can lead to such rare recessive inherited disorders. CASE PRESENTATION: We present a report on recurrence of congenital myasthenic syndromes in two consecutive male siblings who were diagnosed on the basis of clinical findings. CONCLUSION: To minimize risks for having affected child/children with autosomal recessive disorders, consanguineous couples must undergo genetic risk assessment, counseling, and screening.
ABSTRACT
Statement of the Problem: Various factors have been introduced to achieve normal occlusion. One of them is anterior teeth torque that has a significant effect on orthodontic treatment outcomes. Purpose: The aim of this study was to investigate the effect of changes in anterior teeth torque on changes in the space occupied by six anterior teeth by using computer-aided three-dimensional interactive application (CATIA). Materials and Method: In this experimental finite element study, acrylic teeth with pre-adjusted MBT braces were aligned and three-dimensional (3D) scans were made by 3Dscaner. In the CATIA software program, upper incisors' torque was changed to -2, -4, -6, +2, +4 and+6 degrees and in the lower incisors to -1, - 3, -5, +3 and+5 degrees; the space was measured at 3 heights of maxillary incisor crowns and at incisal edges of mandibular incisors. Then maxillary incisors were then tapered and the measurements were made again. To evaluate the effect of tooth size, these procedures were carried out on teeth with +17% and -17% magnifications. Results: The results showed that by increasing anterior torque from 14.7 to 20.7 degrees in maxillary incisors, the space occupied by anterior teeth increased. Maximum changes were at cingulum height with 1.421mm. Reduction in anterior torque from 14.7 to 8.7 degrees resulted in a decrease in this space and maximum changes were observed in the cingulum height with 1.824mm. In the mandibular arch, a 10-degree change in anterior torque resulted in an -.752mm change in the space. Changes in the space occupied by anterior teeth was not significant in tapered and normal teeth in +6 and -6-degree torque (p Value= 0.78 and p Value=0.83). Conclusion: By increasing or decreasing the incisors' torque, the space occupied by anterior teeth increased and decreased, respectively. These changes were less in tapered teeth. Size variations had no significant effect on the results.
ABSTRACT
Kawasaki disease is an acute systemic vasculitis which can cause cardiac involvement among other symptoms. In this study we aimed to assess the relationship between the echocardiographic findings of Kawasaki disease with the clinical and paraclinical findings of the patients. In this cross-sectional study, the symptoms of 307 Kawasaki patients were registered and the association of the symptoms with paraclinical findings and echocardiographic studies was assessed. 190 (61.9%) of the patients were male and 117 (38.1%) were female. 193 patients (62.9%) did not have any abnormalities in their echocardiography, while others showed coronary artery aneurysms, perivascular brightness, coronary artery dilatation, and trivial Mitral Regurgitation in their echocardiography. A significant inverse relationship was seen with echocardiographic findings and age. Thrombocytosis, conjunctivitis, and oral and/or pharyngeal erythema and/or strawberry tongue were associated with higher rates of echocardiographic abnormalities. Echocardiographic abnormalities are associated with younger age, higher platelets, and the existence of conjunctivitis and oral and/or pharyngeal erythema and/or strawberry tongue.
