Sickle Cell Anaemia Prevalence among Newborns in the Brazilian Amazon-Savanna Transition Region

Souza, Rayane Cristina Faria de; Neto, Pedro Agnel Dias Miranda; Santos, Jessflan Rafael Nascimento; Monteiro, Sílvio Gomes; Gonçalves, Maria Cláudia; Silva, Fabrício Brito; Holanda, Rodrigo Assunção; Santos, Julliana Ribeiro Alves

doi:10.3390/ijerph16091638

Cited by 8 publications

(5 citation statements)

References 15 publications

(17 reference statements)

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“…It is estimated that around 5% of the world population has some structural and/or functional change that affects hemoglobin. The World Health Organization has identified SCD as a major public health concern due to its biopsychosocial incidence and complexity (1)(2) . Accurate data regarding the number of individuals with SCD in the world are scarce, however it is assumed that approximately 300,000 children are born with the disease each year.…”

Section: Introductionmentioning

confidence: 99%

Educational technology on COVID-19 for families of children and adolescents with sickle cell disease

et al. 2021

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Objective: to construct and validate educational technology on COVID-19 and essential care for families of children/adolescents with sickle cell disease. Methods: this is a methodological study, in three stages: 1) elaborated educational technology, using the Doak, Doak and Root theoretical-methodological model; 2) content and appearance validation by the content validity coefficient. Delphi technique was applied in two rounds (Delphi I [12 judges]/Delphi II [11 judges]); 3) conducting a pilot test with six families. Results: “Sickle cell disease and COVID-19: essential care” included: consequences of COVID-19 in sickle cell disease, guidelines for reducing the risks of contracting the virus and having complications, signs and symptoms of COVID-19, guidelines in case of child/adolescent with suspicion or symptoms of COVID-19. Global content validity coefficient (Delphi II): 0.98. Conclusion: educational technology presented content and appearance validity for families of children/adolescents with sickle cell disease, related to COVID-19.

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Section: Introductionmentioning

confidence: 99%

Educational technology on COVID-19 for families of children and adolescents with sickle cell disease

et al. 2021

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“…In addition, MSS prevalences ranging from 0.06% to 3% have been reported in Europe, South America, Central Africa, and other countries. These variations could be justified not only by the fact that MSS is endemic in some regions while in others, it is mainly imported cases but also by the lack of knowledge of sickle cell disease in regions of high prevalence and the existence of consanguineous marriages [ 5 – 7 , 9 , 11 , 12 , 14 , 15 , 21 , 23 , 24 , 28 ].…”

Section: Discussionmentioning

confidence: 99%

Neonatal Screening for Sickle Cell Disease in Congo

Dokékias

Gokaba

Louokdom³

et al. 2022

Anemia

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Introduction. Sickle cell disease is an autosomal recessive inherited disorder due to the mutation of a gene coding for the globin beta chain. The aim of this study is to update the epidemiological data on hemoglobinoses, in particular sickle cell disease in newborns in Congo. Materials and Methods. This was a descriptive cross-sectional study, conducted from October 1, 2019, to March 31, 2020, throughout the Congolese national territory. It involved all full-term newborns, without distinction of nationality, aged 5 days or less, and whose parents consented to participate in the study. The blood samples, taken at the heel and collected on Whatman blotting paper, were analyzed using the HPLC Variant NBS machine. Results. In 2897 newborns (NN) screened, hemoglobin abnormalities were found in 603 NN (20.81%). The mean age of these newborns was 1 day (extremes 0 and 5 days). The male-to-female ratio was 1.03. Abnormal hemoglobins were mainly Hb S (n = 597 (97.71%)); Hb C (n = 5 (0.82%)); and variants (n = 7 (1.15%)). The national prevalence of major sickle cell (MSC) syndromes and sickle cell trait was 1.35% and 19.43%, respectively. The prevalence ranged from 1.77% to 2.56% for MSS in four departments and from 20.5% to 25.8% for the sickle cell trait in six other departments. Conclusion. Data on homozygous sickle cell disease remain consistent with previous studies. However, further studies should clarify the molecular anomalies of the variants observed in our samples.

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“…The lack of reliable data in most countries makes it difficult to estimate the actual number of affected people worldwide [7][8][9][10][11][12]. There are over 300,000 affected births each year, with a high frequency of patients with SCD across most of sub-Saharan Africa, Saudi Arabia, and India, as well as in descendants from the Caribbean, Central America, South America, and Mediterranean countries [13].…”

Section: A Prevalence That Is Difficult To Estimate Despite the Evolumentioning

confidence: 99%

Descriptive analysis of sickle cell patients living in France: The PHEDRE cross-sectional study

et al. 2021

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Background Sickle cell disease (SCD) induces chronic haemolytic anaemia and intermittent vaso-occlusion that results in tissue ischaemia causing acute, severe pain episodes that can lead to frequent hospitalizations. These consequences can have repercussions on family, social, school and/or professional life. Here, we present some of the results of the PHEDRE study (Pharmacodépendance Et DREpanocytose—drug dependence and sickle-cell disease), which is the largest study of patients with SCD in France. This paper intends to describe characteristics of the French SCD population. We also aimed to assess the impact of the disease on the lives of patients using objective and subjective variables. Methods The PHEDRE study was a national multicentric observational study. Adults, adolescents and children with a confirmed SCD diagnosis were included in the study by their referring doctor. Then, they were interviewed by phone about their socioeconomic status, about the impact of the disease on their lives and about their analgesic and psychoactive drug use. Results The study population consisted of 872 patients (28% were minors). Seventy-two percent of adults were active, and all minors were in school. Many patients presented criteria of severe SCD. Seventy-five percent were homozygous SS, 15% were double heterozygotes SC and 8% were heterozygotes Sβthal, 87% received specific treatment, 58% were hospitalized at least once for vaso-occlusive crisis in the past 12 months, and the number of analgesic drugs taken averaged 3.8. Seventy-five percent of patients reported academic or professional consequences related to their SCD, and 52% reported social consequences. Conclusions The impact of SCD on patients’ lives can be significant, nevertheless their social integration seems to be maintained. We highlighted respect of recommendations regarding analgesic treatments and only a few patients used tobacco, alcohol or cannabis. Trial registration Clinical Trials, NCT02580565; https://clinicaltrials.gov/ Registered 16 October 2015.

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Sickle Cell Anaemia Prevalence among Newborns in the Brazilian Amazon-Savanna Transition Region

Cited by 8 publications

References 15 publications

Educational technology on COVID-19 for families of children and adolescents with sickle cell disease

Educational technology on COVID-19 for families of children and adolescents with sickle cell disease

Neonatal Screening for Sickle Cell Disease in Congo

Descriptive analysis of sickle cell patients living in France: The PHEDRE cross-sectional study

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