Hemoglobin Bart’s in Northern Algeria

Henni, T; Bachir, Dora; Tabone, P.; Jurdic, Pierre; Godet, Jacqueline; Colonna, P

doi:10.1159/000207187

Cited by 14 publications

(10 citation statements)

References 15 publications

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“…The observed (–α 3.7 ) chromosome frequency in the Congolese population was 0.41 which is higher than that reported for other African populations (table 5) [8, 9, 10, 11, 17, 18, 19, 20, 21, 22, 23, 24, 25, 26]. There appear to be two gradients of α + -thalassemia frequency, although malaria is hyperendemic in almost all sub-Saharan African countries.…”

Section: Discussionmentioning

confidence: 53%

α-Thalassemia in Bantu Population from Congo-Brazzaville: Its Interaction with Sickle Cell Anemia

et al. 1999

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Deletional α⁺-thalassemia (–α^3.7) was investigated in four groups of unrelated individuals from the Bantu population (newborns, normal adults, sickle cells trait carriers, sickle cell anemia patients) of Brazzaville, Congo. The frequency of the (–α^3.7) chromosome was similar between newborns (f = 0.40) and adult subjects (f = 0.36), and between sicklers and nonsickler subjects. The frequency of the (–α^3.7) chromosome in sickle cell anemia patients (SS patients) did not change when age was stratified. The hematological characteristics of SS patients with (–α/αα, –α/–α) and without (αα/αα) α⁺-thalassemia were similar to those reported in Jamaican and US sickle cell anemia patients. α⁺-Thalassemia had an effect on the percentage of hemoglobin S in sickle cell trait carriers. Thus, the high frequency of α⁺-thalassemia in the Congolese population presumably results from this disorder having a selective advantage favoring survival. However, the frequency of α⁺-thalassemia was not affected by age. Although in this selective tropical environment, α⁺-thalassemia as elsewhere markedly affects the hematological characteristics of sickle cell anemia patients, however our data provide no evidence that α⁺-thalassemia increases survival of SS patients.

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Section: Discussionmentioning

confidence: 53%

α-Thalassemia in Bantu Population from Congo-Brazzaville: Its Interaction with Sickle Cell Anemia

et al. 1999

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“…It is widespread in Africa and is thought to reflect a survival advantage against severe malaria [9][10][11][12] . While there have been reports of α+ -thalassaemia elsewhere in Africa, there is a dearth of information on its prevalence and interaction with sickle cell anaemia in Uganda [13][14][15] . In 1958 Raper described nine cases of thalassaemia major amongst Ugandans of Indian origin 16 .…”

Section: Corresponding Authormentioning

confidence: 99%

Alpha thalassemia among sickle cell anaemia patients in Kampala, Uganda

Lubega¹,

Ndugwa²,

Mworozi³

et al. 2015

Afr H. Sci.

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Abstract:Background: Sickle cell anaemia is prevalent in sub Saharan Africa. While α+-thalassaemia is known to modulate sickle cell anaemia, its magnitude and significance in Uganda have hitherto not been described. Objectives: To determine the prevalence of α+thalassaemia among sickle cell anaemia patients in Mulago Hospital and to describe the clinical and laboratory findings in these patients. Methods: A cross sectional study was carried out on patients with sickle cell anaemia in Kampala. Dried blood spots were used to analyze for the deletional α+ thalassaemia using multiplex polymerase chain reaction. Results: Of the 142 patients with sickle cell anaemia, 110 (77.5%) had the αα+thalassaemia deletion. The gene frequency of (-α) was 0.425. Ninety one percent (100/110) of those with α+thalassaemia were heterozygous (αα/α-). Amongst the patients older than 60 months, 15 (83.3%) of those without αα+thalassaemia had significant hepatomegaly of greater than 4 cm compared to 36 (45.6%) of those with α+thalassaemia (p=0.003). Conclusion:The gene frequency of (-α) of 0.425 noted in this study is higher than that reported from many places in Africa. Concurrent alpha thalassemia might be a protective trait against significant hepatomegaly in sickle cell anaemia patients more than 60 months of age at Mulago hospital.

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“…Among northern Algerians, the incid ence of a-thalassemias, as determined by the level of Hb Bart's in cord bloods, is approxi mately 10% [16] and 12 cases of Hb H dis ease have been reported [17]. An investiga tion at the genetic and molecular leve of one of these Algerian patients with Hb H dis ease is presented in this review.…”

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Hemoglobin H Disease from Algeria: Genetic and Molecular Characterization

Tabone¹,

Henni²,

Belhani³

et al. 1981

Acta Haematol

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A case of Hb H disease from Algeria was studied at the genetic and molecular level in order to delineate the pattern of α-thalassemia in the Mediterranean population. The family study indicated that both parents had the hematological and clinical manifestation of α-thalassemia trait and that the affected sibling had homozygous α-thalassemia with 5.6% Hb H, microcytosis and an α-/non-α-biosynthetic ratio of 0.64. Hybridization in globin cDNAα excess suggested that the molecular defect responsible for this form of α-thalassemia is a partial deletion of the haploid stock of α-globin genes. The Algerian case of Hb H disease studied thus differs from Asian and Negro cases by the mode of inheritance of the α-thalassemia mutation involved.

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Hemoglobin Bart’s in Northern Algeria

Cited by 14 publications

References 15 publications

α-Thalassemia in Bantu Population from Congo-Brazzaville: Its Interaction with Sickle Cell Anemia

α-Thalassemia in Bantu Population from Congo-Brazzaville: Its Interaction with Sickle Cell Anemia

Alpha thalassemia among sickle cell anaemia patients in Kampala, Uganda

Hemoglobin H Disease from Algeria: Genetic and Molecular Characterization

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