Sickle cell disease as a paradigm of immigration hematology: new challenges for hematologists in Europe

Roberts, Irene; Montalembert, Mariane de

doi:10.3324/haematol.11474

Cited by 89 publications

(58 citation statements)

References 53 publications

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“…In many countries SCD affects people belonging to minority communities, such as African Americans in the United States or African immigrants in Europe [10]. Immigrants present cultural, social, and financial barriers in accessing the health system and these factors have an effect on the management of chronic illnesses [11][12][13].…”

Section: Introductionmentioning

confidence: 99%

“…Even though comprehensive medical care has been shown to decrease health care resource utilization and to improve quality of life [3] for patients with SCD, in the United States only 36% of children with SCD perform at least one hematology visit per year for comprehensive care [4] and the rate of missed appointments is around 45% [5]. Moreover, although medical advances have resulted in availability of improved treatments for children with SCD, less than 50% are enrolled in Transcranial Doppler (TCD) screening programs for stroke prevention both in Europe [6][7] and the United States [8][9].In many countries SCD affects people belonging to minority communities, such as African Americans in the United States or African immigrants in Europe [10]. Immigrants present cultural, social, and financial barriers in accessing the health system and these factors have an effect on the management of chronic illnesses [11][12][13].…”

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confidence: 99%

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Comprehensive care for sickle cell disease immigrant patients: A reproducible model achieving high adherence to minimum standards of care

Colombatti

Montanaro

Guasti

et al. 2012

Pediatric Blood & Cancer

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INTRODUCTIONSickle Cell Disease (SCD) is the most common genetic disease worldwide. In the past decades advances in basic research and clinical investigations have produced a marked decrease in morbidity and mortality during early childhood. Newborn screening, prophylactic penicillin, effective vaccinations, and stroke prevention have largely contributed to these results [1][2]. Even though comprehensive medical care has been shown to decrease health care resource utilization and to improve quality of life [3] for patients with SCD, in the United States only 36% of children with SCD perform at least one hematology visit per year for comprehensive care [4] and the rate of missed appointments is around 45% [5]. Moreover, although medical advances have resulted in availability of improved treatments for children with SCD, less than 50% are enrolled in Transcranial Doppler (TCD) screening programs for stroke prevention both in Europe [6][7] and the United States [8][9].In many countries SCD affects people belonging to minority communities, such as African Americans in the United States or African immigrants in Europe [10]. Immigrants present cultural, social, and financial barriers in accessing the health system and these factors have an effect on the management of chronic illnesses [11][12][13].New models of comprehensive care need to be developed in order to ensure that all patients with SCD receive high quality care and benefit from the advances in clinical research, overcoming patient-related and health system-related barriers to specialized health care [14].Italy does not have a hemoglobinopathy newborn screening program and for many years pediatric hematology services have focused primarily on thalassemia. SCD has emerged as an important health condition in the last decade due to immigration, mainly from Africa and Albania, and the number of affected children is steadily increasing [15]. The majority of patients are diagnosed with SCD in the Emergency Room during acute events or because referred to a specialized center by the pediatric general practitioner, by friends or relatives. SCD is considered a rare disease and, according to the legislation of European countries, if a patient is registered as having a rare disease by a specialized reference center, patients and families can apply for disability benefits, care is free, and drugs are provided almost for free, with only a minimum contribution (prescription token). Children usually refer to their pediatric general practitioner for routine primary care, to local hospitals for emergencies and to tertiary care reference centers for specialized care.The Clinic of Pediatric Hematology-Oncology of the Azienda Ospedaliera-Università di Padova began assisting patients with SCD in 2003.From 2003 to 2005, several patient-related and health-operator related barriers to care had been noted. Patient-related barriers in seeking and accepting care regarded mainly language difficulties, socioeconomic factors (no permit of stay, precarious living conditions, low income, dif...

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Section: Introductionmentioning

confidence: 99%

mentioning

confidence: 99%

Comprehensive care for sickle cell disease immigrant patients: A reproducible model achieving high adherence to minimum standards of care

Colombatti

Montanaro

Guasti

et al. 2012

Pediatric Blood & Cancer

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“…Natural selection factors are the main reason for the high frequencies of the disease in tropical countries and in countries to which large populations have migrated from these regions. The World Health Organization reported that ~2% of newborns in Nigeria and Ghana were affected by sickle cell anemia, with a total of 150,000 affected children born each year in Nigeria alone (5,6). The frequency of SCD ranges from 10 to 40% across equatorial Africa, decreasing to 1-2% at the North African coast and <1% in South Africa (5).…”

Section: Introductionmentioning

confidence: 99%

Rapid screening for sickle cell disease by polymerase chain reaction-high resolution melting analysis

Yue

Lin

Chen

et al. 2014

Molecular Medicine Reports

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Abstract. Each year, ~300,000 individuals with sickle cell disease (SCD), a hemoglobinopathy caused by β-globin gene mutation, are born, and >75% of those are in Africa. The present study examined 511 individuals on the island of Bioko (Equatorial Guinea) and attempted to establish a method for rapid sickle cell disease screening. Following DNA extraction and polymerase chain reaction (PCR) amplification, high resolution melting (HRM) analysis was used to assess the specificity of fluorescence signals of the PCR products and to differentiate various genotypes of these products. The analytical results of HRM were validated using DNA sequencing. By HRM analysis, 80 out of 511 samples were classified as hemoglobin S (Hb S) heterozygotes, while 431 out of 511 samples were classified as wild-type. No mutant homozygote was identified. DNA sequencing indicated that within the 431 wild-type samples as indicated by HRM analysis, one case was actually a Hb S heterozygote and another case was a rare hemoglobin S-C genotype (sickle-hemoglobin C disease). One out of 80 suspected Hb S heterozygotes as indicated by HRM was confirmed as wild-type by DNA sequencing and the results of residual 508 cases were consistent for HRM analysis and sequencing. In conclusion, HRM analysis is a simple, high-efficiency approach for Hb S screening and is useful for early diagnosis of SCD and particularly suitable for application in the African area.

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“…Sickling decreases the cells flexibility and results in a risk of various complications. The sickling occurs because of a mutation in the hemoglobin gene and life expectancy becomes shortened 1 . Sickle-cell disease, usually presenting in childhood, occurs more commonly in people (or their descendants) from parts of tropical and sub-tropical regions where malaria is or was common.…”

Section: Introductionmentioning

confidence: 99%

“…Sickle-cell disease (SCD) or sickle-cell anemia (SCA) or depranocytosis, is an autosomal recessive genetic blood disorder with over dominance, characterized by red blood cells that assume an abnormal, rigid, sickle shape 1 . Sickling decreases the cells flexibility and results in a risk of various complications.…”

Section: Introductionmentioning

confidence: 99%

Effects of Athrospira Platensis, Monodora Myristica and Helianthus Annuus on the Rate of Polymerization, Sickle Cell Reversion and Oxygen Affinity of Sickle Cell Hemoglobin

Nwaichi¹,

Monago²,

Amaraegbulem³

2013

J Pharm Sci Innov

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The in vitro effects of the cyanobacteria (Athrospira platensis), spices (Monodora myristica) and the achene (Hellianthus annuus) on the rate of polymerization, sickle cell reversion and oxygen affinity of sickle cell hemoglobin (HbS) was studied spectophotometrically using hemolysates of HbS containing erythrocytes treated with sodium metabisulphite in the presence of the different plant extracts. All fractions of the three plant extracts showed statistically significant decreases (p< 0.05) in the rate of polymerization therefore inhibited the rate of sickling with increasing time. The Benzene Soluble Extract (BESE) fraction of Hellianthus annuus demonstrated the highest antisickling activity (89.15% inhibition of HbSS), while the least was by the BESE fraction of Athrospira platensis (45.16% inhibition of HbSS). The crude aqueous extract (CAE) fraction of Athrospira platensis showed the highest antioxidant effect, while CAE fraction of Monodora myristica gave the least. The water soluble extract (WSE) fraction of Hellianthus annuus expressed maximum rate of sickling reversion. Alcohol extracts of these plants were found to exhibit a more significant antisickling activity relative to the aqueous extracts which is attributable to the presence of some lipophillic amino acids present in the alcoholic fraction of the extracts, and it could also be responsible for the observed high reversion capacity but low antioxidant effect of the lipophillic fraction of the plant extracts.

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Sickle cell disease as a paradigm of immigration hematology: new challenges for hematologists in Europe

Cited by 89 publications

References 53 publications

Comprehensive care for sickle cell disease immigrant patients: A reproducible model achieving high adherence to minimum standards of care

Comprehensive care for sickle cell disease immigrant patients: A reproducible model achieving high adherence to minimum standards of care

Rapid screening for sickle cell disease by polymerase chain reaction-high resolution melting analysis

Effects of Athrospira Platensis, Monodora Myristica and Helianthus Annuus on the Rate of Polymerization, Sickle Cell Reversion and Oxygen Affinity of Sickle Cell Hemoglobin

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