Background. Thiamine-responsive megaloblastic anemia syndrome (TRMA), also known as Rogers syndrome, is characterized by megaloblastic anemia, sensorineural hearing loss, and diabetes mellitus. Disturbances of the thiamine transport into the cells results from homozygous or compound heterozygous mutations in the SLC19A2 gene. Case presentation. We report a girl which presented with sensorineural deafness treated with a hearing prosthesis, insulin requiring diabetes, macrocytic anemia, treated with thiamine (100 mg/day). Hemoglobin level improved to 12.1 g/dl after dose of thiamine therapy increased up to 200 mg/day. Conclusion. Patients with TRMA must be evaluated for megaloblastic anemia, sensorineural hearing loss, and diabetes mellitus. They must be followed for response of hematologic and diabetic after thiamine therapy. It should be kept in mind that dose of thiamine therapy may be increased according to the clinical response. Genetic counseling should be given.

Antecedentes. El síndrome de anemia megaloblástica sensible a la tiamina (TRMA, por sus siglas en inglés), también conocido como síndrome de Rogers, se caracteriza por presentar anemia megaloblástica, hipoacusia neurosensorial y diabetes mellitus. Las alteraciones en el transporte de la tiamina hacia las células se deben a mutaciones homocigotas o heterocigotas compuestas en el gen SLC19A2. Presentación de un caso. Presentamos el caso de una niña que manifestaba sordera neurosensorial tratada con una prótesis auditiva, diabetes con necesidad de insulina y anemia macrocítica, tratada con tiamina (100 mg/día). El nivel de hemoglobina mejoró hasta alcanzar 12,1 g/dl después de aumentar la dosis terapéutica de tiamina hasta 200 mg/día. Conclusión. Se debe evaluar a los pacientes con TRMA para detectar anemia megaloblástica, hipoacusia neurosensorial y diabetes mellitus. Se les debe dar seguimiento para determinar la respuesta de la enfermedad hematológica y de la diabetes después de la terapia con tiamina. La dosis terapéutica de tiamina puede aumentarse según la respuesta clínica. Debe proporcionarse asesoramiento genético.

PURPOSE: To investigate the effect of platelet rich fibrin (PRF) and beta tricalcium phosphate (β-TCP), alone or in combination, on bone regeneration in pig tibial defects.METHODS: Four standardized defects were prepared in both tibias of three adult male pigs. The first defect was left unfilled as a control; the others were grafted with either PRF, β-TCP or PRF mixed with β-TCP. All animals were sacrificed on the 12th postoperative week and the tibial bones were removed, histologic sections were prepared and the experimental sites were examined microscopically and stereologically.RESULTS: Histologic and stereologic examination revealed more new bone formation in the defects filled with PRF mixed β-TCP than in the defects grafted with either β-TCP or PRF alone.CONCLUSION: The platelet rich fibrin and beta tricalcium phosphate combination effectively induces new bone formation.

OBJETIVO: Comparar os efeitos da heparina não fracionada (HNF) e da heparina de baixo peso molecular (HBPM) na cicatrização de feridas cutâneas de ratos. MÉTODOS: Quarenta e oito ratos machos Sprague-Dawley foram submetidos à ferida na pele dorsal com espessura total de 8mm e foram distribuídos aleatoriamente em três grupos iguais. No grupo experimental A, a heparina sódica foi injetada a uma concentração de 1000U/kg. No grupo experimental B, a enoxaparina foi injetada a uma concentração de 1mg/kg. Solução salina fisiológica (0,5ml) foi administrada para o grupo controle. As injeções foram feitas por via subcutânea, uma vez por dia, durante sete dias. Nos dias 7º e 10º amostras de tecido foram obtidas de todos os ratos. O exame histológico destes tecidos foi realizado em microscópio de luz. RESULTADOS: O exame histológico mostrou uma diferença significativa entre os grupos no 7º e 10º dias na cicatrização das feridas. Observou-se que a cicatrização de feridas do grupo com heparina de baixo peso molecular foi melhor. Esta diferença foi estatisticamente significante no 10º dia. CONCLUSÕES: A administração diária de doses únicas de heparina não fracionada e de heparina de baixo peso molecular melhora a cicatrização de feridas. A heparina de baixo peso molecular induz melhor a cicatrização de feridas do que a heparina não fracionada.

PURPOSE: To compare the effects of unfractionated heparin (UH) and a low molecular weight heparin (LMWH) on skin wound healing of rats. METHODS: Forty eight male Sprague-Dawley rats underwent 8mm full thickness dorsal skin wounds and were randomly assigned to three equal groups. In experimental group A, heparin sodium was injected at a concentration of 1000U/kg. In experimental group B, enoxaparin was injected at a concentration of 1mg/kg. Physiologic saline (0.5ml) was administered to the control group. Injections were made subcutaneously, once daily, for seven days. At 7th and 10th days tissue samples were taken from all rats. Histologic examination of these tissues was made under light microscope and scored. RESULTS: Histological examination showed a significant difference between the 7th and 10th day groups in wound healing. It was observed that wound healing of LMWH injected group is better. This difference is statistically significant at 10th day. CONCLUSIONS: Daily administration of single doses of unfractionated heparin and a low molecular weight heparin improves wound healing positively. Low molecular weight heparin induces wound healing more than unfractionated heparin.

Articular eminence morphology and inclination are reported to be two predisposing factors for the internal derangement (ID). The purpose of this study is to investigate the relationship between the inclination and morphology of the articular eminence and ID. The study included 70 temporomandibular joints with ID in 35 patients: 51 joints had disc displacements with reduction (DDWR) and 19 joints had disc displacements without reduction (DDWOR). All subjects underwent bilateral high resolution magnetic resonance imaging scans which were performed in the sagittal and coronal planes with mouths closed and opened. Articular eminence morphology was characterized as box, sigmoid, flattened, or deformed. Articular eminence inclination was measured at three positions: steep (from 60° to 90°), moderate (from 30° to 60°) and shallow (from 15° to 30°). The images were divided into two; DDWR and DDWOR, and these two criteria were compared. The images of the two groups were compared; while flattened form was occurred with the highest incidence in the DDWOR group, sigmoid form was the most frequent in the DDWR group and articular eminence inclination was found steeper than it was for the DDWOR group. The steepness of the articular eminence may not have a predisposing effect on the development of disc displacements.

La morfología e inclinación de la eminencia articular son reportados como dos factores predisponentes para el trastorno interno (TI). El propósito de este estudio fue investigar la relación entre la inclinación y la morfología de la eminencia articular y el TI. El estudio incluyó a 70 articulaciones temporomandibulares, con TI en 35 pacientes: 51 articulaciones con desplazamiento discal con reducción (DDCR) y 19 de las articulaciones tuvieron desplazamiento discal sin reducción (DDSR). Todos los sujetos fueron sometidos a imágenes de resonancia magnética bilaterales de alta resolución que se realizaron en los planos sagital y coronal con la boca cerrada y abierta. La morfología articular la eminencia se caracterizó como una caja, sigmoide, aplanada, o deforme. La inclinación de la eminencia articular se midió en tres posiciones: empinada (de 60 ° a 90 °), moderada (entre 30 ° a 60 °) y poco profunda (entre 15 ° y 30 °). Las imágenes fueron divididos en dos; DDCR y DDSOR, y estos criterios fueron comparados. Al comparar las imágenes de ambos grupos, la forma aplanada se observó con mayor incidencia en el grupo de DDSR, la forma sigmoide fue la más frecuente en el grupo de DDCR y la inclinación de la eminencia articular se encontró más pronunciada que en el grupo DDSR. La inclinación de la eminencia articular puede no tener un efecto predisponente en el desarrollo de los desplazamientos de disco.