Self-inflicted oral trauma in a baby with Moebius syndrome

Moebius syndrome (MS) is a rare disorder mainly characterized by bi- or unilateral palsy of the facial and abducens nerves. Among the various orofacial characteristics related to this syndrome, the following are pointed out, difficulty with sucking, tongue malformation, mandibular hypoplasia, saliva drooling, arched palate and micrognathia. In spite of the diversity of oral features described for these patients, self-inflicted oral trauma has not yet been reported. Therefore, the purposes of the present report were to describe an unusual case of a baby with Moebius syndrome who had a parafunctional habit that resulted in a persistent traumatic lesion of the lower lip, and discuss the treatment performed.     

Peripheral cemento-ossifying fibroma in a child: a case report

Peripheral cemento-ossifying fibroma is a gingival lesion of reactive nature and unknown etiology. Such a lesion can either interfere with normal tooth eruption or become a factor in plaque development, which usually results in caries formation in newly erupted teeth. Therefore, the objective of the present article was to report a case of peripheral cemento ossifying fibroma occurring in the mandibular posterior region of a 11-year-old girl in association with both absence of premolar eruption and presence of severely caried first permanent molar.     

Evaluation of antigens from various Leishmania species in a Western blot for diagnosis of American tegumentary leishmaniasis

A Western blot method that uses antigens from culture promastigote forms of Leishmania (Viannia) braziliensis, L. (Leishmania) amazonensis, L. (Leishmania) tropica, and a trypanosomatid (strain 268T) isolated from naturally infected tomatoes was evaluated for laboratory diagnosis of American tegumentary leishmaniasis (ATL). Serum samples were obtained from 108 patients with ATL (group I), 23 chagasic patients (group II), 32 patients with other diseases (group III), and 78 healthy individuals (group IV). The overall analysis showed a sensitivity of 76.90%, 90.40%, 78.50%, and 87.90%, a specificity of 100%, 93.80%, 87.80%, and 77.10%, a positive predictive value of 100%, 94.00%, 89.50%, and 72.50%, a negative predictive value of 75.70%, 90.00%, 75.40%, and 90.20%, and a concordance coefficient kappa of 0.7358, 0.8400, 0.6491, and 0.6287 for L. (V.) braziliensis, L. (L.) amazonensis, L. (L.) tropica, and strain 268T antigens, respectively. The antigenic profile recognized by serum samples from patients with ATL and with Chagas' disease permits serologic distinction between these infections.     

Prospective evaluation comparing the effects of enalapril and losartan in left ventricular remodeling after acute myocardial infarction

Background

Previous studies have compared angiotensin receptor blockers and angiotensin-converting enzyme inhibitors in patients with heart failure, but there are few data about the effect of these drugs regarding left ventricular remodeling after myocardial infarction.

Methods

Fifty-two consecutive patients with first anterior wall myocardial infarction within 24 hours of evolution were randomized to receive enalapril (as much as 20 mg; mean, 14.6 mg), or losartan (as much as 50 mg; mean, 48 mg). Left ventricular ejection fraction and ventricular volumes were analyzed in 2 serial radionuclide ventriculograpies, carried out within 4 days after the infarction (mean, 97.4 ± 114.2 hours) and after 6 months (mean, 177.7 ± 16.7 days). Ventriculographies were analyzed by a single blinded observer. Mainly because of the unexpected large SD values obtained, the power of the study to demonstrate equivalence between the groups was only 15.7%.

Results

The differences obtained between the first and the second ventriculographies, for the enalapril and losartan groups, were: for left ventricular ejection fraction, −0.4% ± 6.6% versus −1.1% ± 5.9% (P = . 67; 95% CI, 2.77-4.23); for final systolic volume, 0.07 ± 7.7 mL/m² versus −0.2 ± 6.1mL/m² (P = . 85; 95% CI, −3.57-4.26); for final diastolic volume −0.7 ± 12.1 mL/m² versus −3.6 - 9.9 mL/m² (P = . 34; 95% CI, −3.22-9.17).

Conclusion

This study, although underpowered, suggests that neither enalapril nor losartan was superior as compared with each other for left ventricular remodeling after myocardial infarction; however, powerful evidence of equivalence was not provided.     

Ca(2+)-regulated structural changes in troponin

Troponin senses Ca2+ to regulate contraction in striated muscle. Structures of skeletal muscle troponin composed of TnC (the sensor), TnI (the regulator), and TnT (the link to the muscle thin filament) have been determined. The structure of troponin in the Ca(2+)-activated state features a nearly twofold symmetrical assembly of TnI and TnT subunits penetrated asymmetrically by the dumbbell-shaped TnC subunit. Ca ions are thought to regulate contraction by controlling the presentation to and withdrawal of the TnI inhibitory segment from the thin filament. Here, we show that the rigid central helix of the sensor binds the inhibitory segment of TnI in the Ca(2+)-activated state. Comparison of crystal structures of troponin in the Ca(2+)-activated state at 3.0 angstroms resolution and in the Ca(2+)-free state at 7.0 angstroms resolution shows that the long framework helices of TnI and TnT, presumed to be a Ca(2+)-independent structural domain of troponin are unchanged. Loss of Ca ions causes the rigid central helix of the sensor to collapse and to release the inhibitory segment of TnI. The inhibitory segment of TnI changes conformation from an extended loop in the presence of Ca2+ to a short alpha-helix in its absence. We also show that Anapoe, a detergent molecule, increases the contractile force of muscle fibers and binds specifically, together with the TnI switch helix, in a hydrophobic pocket of TnC upon activation by Ca ions.     

Evaluating NRC’s Recommendations for Educating Children with Autism a Decade Later

Background

Over a decade ago, the National Research Council (NRC) published the influential book, Educating Children with Autism.

Objective

To survey parents and state-level special education administrators to evaluate how NRC’s recommendations, as published in Educating Children with Autism, have impacted educational practices for children with autism in the US.

Methods

149 parents of children with autism and 35 state-level special education administrators from 35 states were surveyed on the degree to which they agreed with NRC’s recommendations and the degree to which they believed NRC’s recommendations have been implemented.

Results

Parents and administrators agreed with most NRC recommendations; however, disagreements between parents and administrators were evident regarding labeling of students with autism, intensity of services, and training of staff. Conversely, parents and administrators concurred that the recommendations had been poorly to modestly implemented, overall. Lowest levels of implementation were found for training of qualified staff, research-based curricula, and mental health supports for families. Qualitative analysis of parents’ comments highlighted that vigorous advocacy was necessary for their children to receive appropriate services consistent with NRC’s recommendations.

Conclusions

Over a decade following publication of Educating Children with Autism, most of NRC’s recommendations have not been fully implemented. Policy makers should focus particular attention on improving professional training, disseminating research-based practices, and providing mental health supports for families.