Cone-Beam computed tomography evaluation of maxillary expansion in twins with cleft lip and palate

OBJECTIVE: The establishment of normal occlusal relationships in patients with cleft lip and palate using rapid maxillary expansion may promote good conditions for future rehabilitation. OBJECTIVE: This study describes the clinical case of monozygotic twins with unilateral cleft lip and palate at the age of mixed dentition, who were treated using the same rapid maxillary expansion protocol, but with two different screws (conventional and fan-type expansion screw). Results were evaluated using plaster models, intraoral and extraoral photographs, and Cone-Beam computed tomography (CBCT) scans obtained before the beginning of the treatment, (T1). METHODS: The patients were followed up for 6 months after maxillary expansion, when the same tests requested at T1 were obtained again for review (T2). T1 and T2 results were compared using lateral cephalometric tracings and measurements of the intercanine and intermolar distances in the plaster models using a digital caliper. RESULTS: The two types of expansion screws corrected the transverse discrepancy in patients with cleft lip and palate. The shape of the upper arches improved at 10 days after activation. CONCLUSION: CBCT scans provide detailed information about craniofacial, maxillary and mandibular changes resulting from rapid maxillary expansion. The most adequate screw for each type of malocclusion should be chosen after detailed examination of the dental arches

Cysteamine revisited: repair of arginine to cysteine mutations.

Cysteamine is a small aminothiol endogenously derived from coenzyme A degradation. For some decades, synthetic cysteamine has been employed for the treatment of cystinosis, and new uses of the drug continue to emerge. In this review, we discuss the role of cysteamine in cellular and extracellular homeostasis and focus on the potential use of aminothiols to reconstitute the function of proteins harboring arginine (Arg) to cysteine (Cys) mutations, via repair of the Cys residue into a moiety that introduces an amino group, as seen in basic amino acid residues Lys and Arg. Cysteamine has been utilized in vitro and ex vivo in four different genetic disorders, and thus provides "proof of principle" that aminothiols can modify Cys residues. Other aminothiols such as mercaptoethylguanidine (MEG) with closer structural resemblance to the guanidinium moiety of Arg are under examination for their predicted enhanced capacity to reconstitute loss of function. Although the use of aminothiols holds clinical potential, more studies are required to refine specificity and treatment design. The efficacy of aminothiols to target proteins may vary substantially depending on their specific extracellular and intracellular locations. Redox potential, pH, and specific aminothiol abundance in each physiological compartment are expected to influence the reactivity and turnover of cysteamine and analogous drugs. Upcoming research will require the use of suitable cell and animal models featuring Arg to Cys mutations. Since, in general, Arg to Cys changes comprise about 8% of missense mutations, repair of this specific mutation may provide promising avenues for many genetic diseases.QNR