Zinc in sickle cell disease: A narrative review.

Sickle cell disease (SCD) is an inherited disease caused by hemoglobin S mutated hemoglobin S. It is characterized by chronic hemolysis, intermittent vaso-occlusive crises followed by ischemia-reperfusion, and organ damage. These patients have an increased risk of multiple micronutrient deficiencies, such as zinc. The reduced zinc bioavailability in sickle cell patients may lead to several complications such as growth retardation, delayed wound healing, increased vaso-occlusive crises, and infections. This narrative review aims to analyze the literature concerning the zinc status in SCD and their possible consequences on the patients' clinical evolution. We found in children and adolescents a direct association between zinc insufficiencies/deficiencies with increased disease severity in SCD. Monitoring zinc status in children and adolescent SCD appears essential for reducing disease-associated morbidity and infections. Zinc supplementation is a safe therapeutic modality for treating SCD patients. New research must be carried out, especially for adults, to ensure more remarkable survival for this population.

miRNA profile and disease severity in patients with sickle cell anemia.

Identification of biomarkers associated with severity in sickle cell anemia is desirable. Circulating serum microRNAs (miRNA) are targets studied as diagnostic or prognostic markers, but few studies have been conducted in sickle cell anemia. The purpose of this study is to identify specific signatures of miRNAs in plasma samples from sickle cell anemia patients according to severity indexes. Screening of the miRNAs expression was performed in 8 patients, classified by tricuspid regurgitation velocity (TRV) measure: 4 with TRV ≥ 2.5 m/s and 4 with TRV < 2.5 m/s. The samples were analyzed by real-time PCR using Megaplex RT Human Pool A and Pool B comprising 667 distinct miRNAs. Seventeen miRNAs were differentially expressed between the two groups (p < 0.05). Five differentially expressed miRNAs (miR15b, miR502, miR510, miR544, miR629) were selected for validation in a cohort of 52 patient samples, 26 with TRV ≥ 2.5 m/s. Another two severity scores were also used: organ injury score (OIS) and Bayesian score (BS). Univariate binary logistic regressions were performed to analyze the data. Five out of 17 differentially expressed miRNAs were selected for validation in 52 patient samples: miR15b, miR502, miR510, miR544, and miR629. Two miRNAs (miR510 and miR629) were significantly decreased in cases of greater severity. Whereas miR510 expression discriminated the patients according to TRV and OIS, miR629 expression did it according to BS. This is the first study investigating plasma miRNAs as possible biomarkers for SCA severity. Our data suggest that low levels of miR510 and miR629 expression are associated with greater SCA disease severity. Further studies are still necessary to elucidate mechanism of these miRNAs and their related proteins.

Manejo quirúrgico de dientes supernumerarios en adolescente con Tetralogía de Fallot: Reporte de caso / Manejo cirúrgico para remoção de dentes supranumerários em adolescente com Tetralogia de Fallot: Relato de caso / Surgical Management of supernumerary teeth in adolescent with Tetralogy of Fallot: Case report

La tetralogía de Fallot (TOF) se ha asociado con varios defectos genéticos y puede presentarse simultáneamente con ma-nifestaciones craneofaciales. El paciente odontológico con TOF puede requerir algunos cambios en el plan de tratamiento. El objetivo del presente reporte de caso es mostrar que las extracciones dentales se pueden llevar a cabo en personas con TOF bajo anestesia local, una vez que se realiza la correcta planificación. Cinco dientes supernumerarios fueron extraídos de un paciente adolescente con TOF y se realizó profilaxis antibiótica para evitar la endocarditis infecciosa. La paciente regresó para una nueva evaluación al 7mo día postoperatorio. Él informó que no había sentido molestias o dificultades para comer. El examen bucal postoperatorio confirmó la reparación eficiente de los tejidos en todas las regiones expuestas al procedimiento quirúrgico, sin ninguna señal de infección.

Tetralogy of Fallot (TOF) has been associated with several genetic defects and may present concurrently with craniofacial manifestations. The dental patient with TOF may require some changes in the treatment plan. The aim of the present case report is to show that dental extractions can be carried out in TOF individuals under local anaesthesia, since the correct planning is done. Five supernumerary teeth were extracted in adolescent with TOF and antibiotic prophylaxis was performed to avoid infective endocarditis. The patient returned for re-evaluation on the 7th postoperative day. He reported that there had been no discomfort or difficulty in eating. An oral examination confirmed that all extraction sites were healing well, without any indication of infection.

A Tetralogia de Fallot (TOF) está associada a diversas alterações genéticas e pode concomitantemente apresentar manifes-tações craniofaciais. O paciente com comprometimento odontológico geralmente necessita de alterações em seu plano de tratamento. O objetivo do presente estudo, é mostrar, por meio de um relato de caso, que procedimentos cirúrgicos odon-tológicos podem ser realizados em pacientes em TOF sob anestesia local, desde que o correto planejamento seja realizado. Cinco dentes supranumerários foram extraídos de um paciente adolescente com TOF e a profilaxia antibiótica foi realizada previamente ao procedimento cirúrgico, devido ao risco de endocardite infecciosa. O paciente retornou para reavaliação após 7 dias da realização do procedimento relatando que não houve desconforto ou dificuldade para se alimentar. O exame bucal pós-operatório, mostrou reparação tecidual eficiente em todas as regiões expostas ao procedimento cirúrgico, sem qualquer sinal de infecção.

Molecular analysis and association with clinical and laboratory manifestations in children with sickle cell anemia.

OBJECTIVES: To analyze the frequency of ß(S)-globin haplotypes and alpha-thalassemia, and their influence on clinical manifestations and the hematological profile of children with sickle cell anemia. METHOD: The frequency of ß(S)-globin haplotypes and alpha-thalassemia and any association with clinical and laboratorial manifestations were determined in 117 sickle cell anemia children aged 3-71 months. The confirmation of hemoglobin SS and determination of the haplotypes were achieved by polymerase chain reaction-restriction fragment length polymorphism, and alpha-thalassemia genotyping was by multiplex polymerase chain reaction (single-tube multiplex-polymerase chain reaction). RESULTS: The genotype distribution of haplotypes was 43 (36.7%) Central African Republic/Benin, 41 (35.0%) Central African Republic/Central African Republic, 20 (17.0%) Rare/atypical, and 13 (11.1%) Benin/Benin. The frequency of the α3.7 deletion was 1.71% as homozygous (-α3.7/-α3.7) and 11.9% as heterozygous (-α3.7/αα). The only significant association in respect to haplotypes was related to the mean corpuscular volume. The presence of alpha-thalassemia was significantly associated to decreases in mean corpuscular volume, mean corpuscular hemoglobin and reticulocyte count and to an increase in the red blood cell count. There were no significant associations of ß(S)-globin haplotypes and alpha-thalassemia with clinical manifestations. CONCLUSIONS: In the study population, the frequency of alpha-thalassemia was similar to published data in Brazil with the Central African Republic haplotype being the most common, followed by the Benin haplotype. ß(S)-globin haplotypes and interaction between alpha-thalassemia and sickle cell anemia did not influence fetal hemoglobin concentrations or the number of clinical manifestations.

Molecular analysis and association with clinical and laboratory manifestations in children with sickle cell anemia

Objectives: To analyze the frequency of βS-globin haplotypes and alpha-thalassemia, and their influence on clinical manifestations and the hematological profile of children with sickle cell anemia. Method: The frequency of βS-globin haplotypes and alpha-thalassemia and any association with clinical and laboratorial manifestations were determined in 117 sickle cell anemia children aged 3–71 months. The confirmation of hemoglobin SS and determination of the haplotypes were achieved by polymerase chain reaction-restriction fragment length polymorphism, and alpha-thalassemia genotyping was by multiplex polymerase chain reaction (single-tube multiplex-polymerase chain reaction). Results: The genotype distribution of haplotypes was 43 (36.7%) Central African Republic/Benin, 41 (35.0%) Central African Republic/Central African Republic, 20 (17.0%) Rare/atypical, and 13 (11.1%) Benin/Benin. The frequency of the α3.7 deletion was 1.71% as homozygous (−α3.7/−α3.7) and 11.9% as heterozygous (−α3.7/αα). The only significant association in respect to haplotypes was related to the mean corpuscular volume. The presence of alpha-thalassemia was significantly associated to decreases in mean corpuscular volume, mean corpuscular hemoglobin and reticulocyte count and to an increase in the red blood cell count. There were no significant associations of βS-globin haplotypes and alpha-thalassemia with clinical manifestations. Conclusions: In the study population, the frequency of alpha-thalassemia was similar to published data in Brazil with the Central African Republic haplotype being the most common, followed by the Benin haplotype. βS-globin haplotypes and interaction between alpha-thalassemia and sickle cell anemia did not influence fetal hemoglobin concentrations or the number of clinical manifestations...

In vitro effect of 470 nm LED (Light Emitting Diode) in keloid fibroblasts / Efeito in vitro do LED (Light Emitting Diode) de 470 nm em fibroblastos de quelóide

Purpose: To quantify keloid fibroblasts after irradiation with 470nm blue LED, in vitro. Methods: Fibroblasts from keloid and adjacent skin have been obtained from 6 patients. Cells have been cultivated and maintained in DMEM culture medium. In Petri dishes, they were irradiated with energy doses of 6J, 12J and 18J. After 24 h, counting was done by the average of the triplicates for each sample. Results: There were no significant differences in the number of irradiated keloid fibroblasts at the studied doses (p=0.261). In adjacent skin fibroblasts, differences were observed (p=0.025) concerning the doses of 18 J and 6 J (p=0.03). Conclusions: There was a reduction in the number of adjacent skin fibroblasts irradiated with 470nm blue LED at the energy dose of 18 J compared to the ones irradiated at the energy dose of 6 J. There were no changes in keloid fibroblasts counting at any of the doses applied, 24 h after irradiation.

Objetivo: Quantificar fibroblastos de quelóide após irradiação com LED azul de 470nm, in vitro. Métodos: Foram obtidos fibroblastos de quelóide e pele adjacente, de seis pacientes. As células foram cultivadas e mantidas em meio de cultura DMEM. Em placas de Petri, receberam irradiação com doses de energia de 6J, 12J e 18J. Após 24 horas a contagem foi feita pela média da triplicata para cada amostra. Resultados: Não houve diferença na quantidade de fibroblastos de quelóide irradiados nas doses estudadas (p=0,261). Observou-se diferença nos fibroblastos de pele adjacente (p=0,025), com relação às doses de 18 J e 6 J (p=0,03). Conclusões: Houve redução dos fibroblastos de pele adjacente irradiados com LED azul de 470 nm na dose de energia de 18 J em relação à dose de 6 J. Não houve alteração na quantidade de fibroblastos de quelóide nas doses aplicadas após 24 horas da irradiação.

In vitro effect of 470 nm LED (Light Emitting Diode) in keloid fibroblasts.

PURPOSE: To quantify keloid fibroblasts after irradiation with 470nm blue LED, in vitro. METHODS: Fibroblasts from keloid and adjacent skin have been obtained from 6 patients. Cells have been cultivated and maintained in DMEM culture medium. In Petri dishes, they were irradiated with energy doses of 6J, 12J and 18J. After 24 h, counting was done by the average of the triplicates for each sample. RESULTS: There were no significant differences in the number of irradiated keloid fibroblasts at the studied doses (p=0.261). In adjacent skin fibroblasts, differences were observed (p=0.025) concerning the doses of 18 J and 6 J (p=0.03). CONCLUSIONS: There was a reduction in the number of adjacent skin fibroblasts irradiated with 470nm blue LED at the energy dose of 18 J compared to the ones irradiated at the energy dose of 6 J. There were no changes in keloid fibroblasts counting at any of the doses applied, 24 h after irradiation.