RESUMO
Background: Sickle Cell Disease (SCD) is an inherited condition that is widespread globally and especially in malaria-endemic West African countries. Limited epidemiological data on SCD are available for Guinea Bissau, where newborn screening is not yet implemented, routine diagnosis is not available, and care is case directed. Methods: Dried blood spots were collected from children accessing two hospitals managed by Italian Non-Governmental Organizations in the capital city of Bissau and sent to Padova for Hemoglobin (Hb) quantification through HPLC and molecular analysis. Beta globin gene analysis was performed in all; and Hb haplotype of the HbSS and HbSA patients was performed in South Africa. One hundred samples belonging to the most frequent ethnic groups were randomly selected for detection of G6PD mutations. Results: Samples from 848 consecutive children (498 males and 350 females, mean age 6.8 years) accessing the two hospitals were analyzed: 6.95% AS (4.42% allelic frequency), 0.94% SS, and 0.23% AC. 376G G6PD allelic frequency was 24%; 14.8% in AS individuals. The Senegal haplotype was the most prevalent (31%), and the proposition of chromosomes with the atypical haplotype was surprisingly high (56%). Conclusion: Our study demonstrates a significant frequency of the HbS allele in the population of Guinea Bissau supporting the implementation of screening strategies. The differences among ethnic groups can help guide targeted interventions for SCD awareness campaigns and determine priority areas for public health interventions. The pilot analysis on haplotypes reveals a large proportion of the atypical haplotype, which may be indicative of a genetically heterogeneous population.
RESUMO
CONTEXT: The circumcision is the most frequent procedure in paediatric surgery worldwide, performed for medical and ritual purposes. In developing countries, because of the difficult accessibility to healthcare, even a common procedure could be unsafe. AIMS: The aim of the article is to compare the perioperative and anaesthesiological management of circumcision in children between two Italian and two sub-Saharan African hospitals. MATERIALS AND METHODS: Medical records of paediatric circumcision from January 2014 to December 2016 have been reviewed. The involved hospitals were: Padua (Italy), Ferrara (Italy), Sao José em Bor (Guinea Bissau) and Yaoundé (Cameroun). RESULTS: In Padua, 77 circumcisions were performed, 19 of these (24.6%) were ritual. In 75 children (97.4%), locoregional anaesthesia (LRA) together with sedation was used; only one complication (1.3%) occurred. In Ferrara, 200 interventions were done, 140 (70%) ritual; general anaesthesia was administered to 183 (93.5%) patients. There were five complications (2.5%). In Bissau, 53 procedures were performed, 21 (39.6%) ritual; in 34 children (64.1%), LRA with sedation was preferred. Two complications (3.8%) were reported. In Yaoundé, 60 children were circumcised, 15 (25%) for ritual purposes; in 51 (85%), only LRA was performed; there was only one (1.7%) complication. In the African hospital, no post-operative analgesia was administered. CONCLUSION: Despite the different anaesthesiological techniques, the study shows no difference in rate of complications for the in-hospital setting. Training of the local medical team in pain management and post-operative care should be emphasised.
