Profil épidémiologique des hémoglobinopathies: étude autour du cas index: étude transversale descriptive

Dahmani, Fatima; Benkirane, Souad; Kouzih, Jaafar; Woumki, Aziz; Mamad, Hassane; Masrar, Azlarab

doi:10.11604/pamj.2017.27.150.11477

Cited by 18 publications

(15 citation statements)

References 5 publications

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“…The frequency of other marriages was low (Table 3). These results were in agreement with others findings reporting a high rate of consanguinity in hemoglobinopathy population due to first cousin marriages that constitute more than 50% of the consanguineous marriages [12, 21–23].…”

Section: Discussionsupporting

confidence: 94%

Hemoglobinopathies in the North of Morocco: Consanguinity Pilot Study

Laghmich

Ismaili

Zian

et al. 2019

BioMed Research International

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Consanguinity is a social behavior characterized by the arrangement of marriages between relatives. It coincides generally with the geographic distribution of recessive genetic diseases as it increases the likelihood of homozygosis and, consequently, the incidence of their pathologies in the population. In this pilot study, we assess the effect of inbreeding on the burden of hemoglobinopathies in Northern Morocco. From January 2016 to December 2018, 197 children born in the studied region to three ancestral generations and diagnosed with hemoglobinopathies were subject to investigation. The rate of consanguinity in the parents' generation of children with hemoglobinopathies was 50.25%, with first cousin marriages accounting for 68.69% of consanguineous unions (FI = 0.02). The corresponding rates in the general population, based on a sample of N = 900, were 29.67% and 82.02%, respectively. The marriages between first cousins are the most common among the other types of consanguineous unions. Our study propounds that consanguinity substantially contributes to the hemoglobinopathy burden in the studied region and has changed little over time. Refraining from consanguineous marriages and detecting couples at risk could contribute to the reduction of the incidence of genetic diseases in our country.

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Section: Discussionsupporting

confidence: 94%

Hemoglobinopathies in the North of Morocco: Consanguinity Pilot Study

Laghmich

Ismaili

Zian

et al. 2019

BioMed Research International

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“…As patients with SCD are at a greater risk for severe comorbidities associated with vascular occlusion, hemolysis, and infection, in addition to oral disorders, their quality of life could be compromised (Fernandes et al, 2016). Hemoglobinopathies are often severe, present difficult management, and have a remarkable psychosocial impact on patients and on their families (Dahmani et al, 2017).…”

Section: Introductionmentioning

confidence: 99%

Health-related quality of life in children and adolescents with sickle cell disease

Veras

Lopes

Luna

et al. 2020

RSD

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To assess the health-related quality of life of children and adolescents with sickle cell disease (SCD). This is a cross-sectional study carried out with 97 male and female patients aged 5 to 18 years with clinical and laboratory diagnosis of SCD, seen at the Hematology and Hemotherapy Hospital of Pernambuco. The Pediatric Quality of Life InventoryTM questionnaires, version 4.0 – child/adolescent report – were applied. Most patients were female (76.3%), had sickle cell anemia (89.7%) and mixed race (59.8%). There was a significant association in the psychosocial domain between age groups 5 to 7 (72.10) and 8 to 12 years (p≤0.05), the latter of which had the worst quality of life score (65.36). There were significant differences between sexes only in patients aged 13 to 18 years for physical functioning and for the overall mean of the domains. The means in both cases were higher in female patients than in male ones (70.89 x 56.88 for physical functioning and 71.72 x 59.77 for the overall mean of the domains). Poor quality of life was observed among children and adolescents with SCD, with negative impacts according to age and sex.

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“…The prevalence of the sickle cell gene is high in Africa, in particular the Northern Tanzania, where it has been reported in 21.1% of births [19]. In a study from Morocco, sickle cell disease was the most common haemoglobinopathy with 23% having homozygous HbSS and additional 43.5% having the sickle gene [AS heterozygous AS (40.6%), S/ß-thalassemia composite heterozygosity (2.9%)] on a paediatric ward [20]. A cohort study from Kenya revealed that 69.3% of children that were admitted were undiagnosed sickle cell anemia patients [21].…”

Section: Discussionmentioning

confidence: 99%

Knowledge, beliefs and practices regarding sickle cell eye disease of patients at the sickle cell unit, Jamaica

Mowatt¹,

Ajanaku²,

Knight‐Madden³

2019

Pan Afr Med J

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Introduction Sickle cell disease can result in visually threatening eye disease (proliferative sickle cell retinopathy). This can be prevented with timely eye screening. It is important for patients to understand their role. Our research is to determine the knowledge, beliefs and practices (KBP) regarding eye disease of Sickle Cell patients and the impact of genotype, demographic and socio-economic status. Methods Cross-sectional study at the Sickle Cell Unit, Jamaica during May 2016. Consecutive non-pregnant adults (>18 years of age) attendees, who were not acutely unwell, were invited to participate. A 26-item single interviewer administered questionnaire was used to obtain socio-demographic data, highest level of education completed, employment status, sickle cell genotype, if known, frequency of clinic attendance and patients' knowledge, beliefs and practices. Ten of these were yes/no questions, whereas eight required that they choose correct answers from four choices. Results One hundred subjects were recruited, 72% had homozygous SS disease. Their ages ranged from 18-63 years (mean 34.1 years, SD11.3). Fifty six percent were female. Most (75%) had achieved at least secondary education. The majority (62%) were unemployed. The mean belief score was 3.6/6(60%) and the mean knowledge and practice scores were 3.3/7(47%) and 2.2/5(44%) respectively. Milder genotypes had higher knowledge scores vs the more severe genotypes (4.0 vs 3.2, P=0.013). Only 28% had regular eye examinations; less than 50% had seen an ophthalmologist in the past year. Practice scores were higher in employed than in unemployed patients (2.6 vs 1.9, (P=0.04)). Employed patients were more likely than the unemployed to see their eye doctor for regular eye “examinations” (42.1% vs 19.4%, χ2=6.0, P=0.02). The practice and knowledge scores correlated (r 2 =0.363, P<0.001) and belief score (r 2 =0.304, P =0.002), except where 98% believed they should see an ophthalmologist annually, but only 42% did, and 21% had never. Conclusion Knowledge scores were fair, however, the practice was not always in keeping with knowledge.

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Profil épidémiologique des hémoglobinopathies: étude autour du cas index: étude transversale descriptive

Cited by 18 publications

References 5 publications

Hemoglobinopathies in the North of Morocco: Consanguinity Pilot Study

Hemoglobinopathies in the North of Morocco: Consanguinity Pilot Study

Health-related quality of life in children and adolescents with sickle cell disease

Knowledge, beliefs and practices regarding sickle cell eye disease of patients at the sickle cell unit, Jamaica

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