Sickle Cell Anaemia Prevalence among Newborns in the Brazilian Amazon-Savanna Transition Region
Sickle cell anaemia is one of the most common hemoglobinopathies worldwide and an important public health problem in Brazil. This study evaluated the prevalence of sickle cell anaemia and its traits in newborns from the Amazon-Savanna Transition Region in the state of Maranhão, Brazil. A cross-sectional study was carried out, based on data from neonatal screening tests performed in 2013–2015 in Maranhão. The Hardy-Weinberg theorem was applied to analyse the frequency of expected homozygotes based on HbSS phenotype. A spatial-temporal distribution analysis was performed to delimit the regions with the greatest number of newborn cases with sickle cell anaemia. Of 283,003 newborns, 162 were found to have sickle cell anaemia, while 10,794 had a sickle cell trait, with a prevalence of 0.05% and 3.8%, respectively. The prevalence of expected homozygotes was higher in the North Region and in the state capital of Maranhão. This study may contribute to existing social and public health actions or the creation of new strategies for sickle cell disease in endemic areas in Brazil to improve the quality of life.
[Prevalence of hemoglobin S in the State of Paraná, Brazil, based on neonatal screening].
The Brazilian Ministry of Health created the National Neonatal Screening Program under ruling no. 822/2001, including neonatal screening for hemoglobinopathies. In the State of Paraná, neonatal screening is conducted by the Ecumenical Foundation for the Protection of the Handicapped. The prevalence rates were determined for homozygous and heterozygous hemoglobin S and Sbeta-thalassemia. Blood samples drawn on filter paper were examined by isoelectric focusing (IEF) and high-performance liquid chromatography (HPLC). From January 2002 to December 2004, 548,810 newborns were screened, with the detection of 21 with FS, two FSA/FS, and four FSA. After confirmatory tests at six months of age, 12 were defined as sickle-cell anemia, or a prevalence of 2.2:100,000 newborns; Sbeta-thalassemia was confirmed in 15 (2.7:100,000 newborns); and 8,321 newborns were diagnosed as heterozygous HbS (1,500:100,000 newborns). HbS prevalence in Paraná (in southern Brazil) is lower than in the Central-West, North, and Northeast of the country. Ethnic origin of the population, fetal deaths, and non-random procreation may contribute to the relatively low number of homozygous individuals in the State. Sbeta-thalassemia interaction suggests the presence of Euro-Mediterranean peoples in this population's miscegenation.
Surgical management of facial paralysis resulting from temporal bone fractures
Abstract Conclusion: To achieve good facial reanimation in cases with facial paralysis resulting from temporal bone fractures, the ideal timing for surgical intervention is at least within 1 month of injury and an appropriate surgical approach should be selected depending on the site of facial nerve injury. Objective: This paper aimed to address the ideal time for surgical intervention and the appropriate surgical approach for patients with facial paralysis resulting from temporal bone fractures. Methods: We retrospectively investigated 60 patients with facial paralysis due to temporal bone fractures who underwent facial nerve decompression via different operative approaches within 1 month after trauma, of which 48 were surgically treated by the middle cranial fossa approach (80%), 8 by a pure transmastoid approach (13.3%), and four by a combined transmastoid and middle cranial fossa approach (6.7%). The House–Brackmann (H-B) grading system was used to evaluate the recovery of facial nerve function. Results: The follow-up period for all the patients was 1 year. Among 60 patients who were surgically treated, 39 achieved grade I of facial nerve function, 18 achieved grade II, two achieved grade III, and one achieved grade IV according to the H-B grading system.