The prevalence of hemoglobin S and glucose-6-phosphate dehydrogenase deficiency in Jordanian newborn

Talafih, Khalid; Hunaiti, Abdelrahim A.; Gharaibeh, Nayef S.; Gharaibeh, Mohammad; Jaradat, Saied

doi:10.1016/s0378-3782(97)90571-5

Cited by 3 publications

(2 citation statements)

References 7 publications

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“…Thus no significant difference was found in the bilirubin levels in sickle cell patients with and without G6PD deficiency. It was consistent with the findings of Gibbs WN et al, Steinberg MH et al and Talafih K et al 4,5,20 Nouraie M et al observed that the G6PD deficiency was not associated with increased hemolysis as measured by lactate dehydrogenase, serum bilirubin, aspartate amino transferase or a haemolytic component derived from these markers (P > 0.09) and concluded that G6PD deficiency may be associated with lower hemoglobin concentration in sickle cell anemia by a mechanism other than increased hemolysis which was consistent with the present study. 13 However, Smits HL et al demonstrated increased jaundice as a result of accelerated hemolysis.…”

Section: Serum Bilirubinsupporting

confidence: 93%

Influence of glucose-6 phosphate dehydrogenase (G6-PD) deficiency upon clinico-haematological and biochemical expression of patients with sickle cell disease

et al. 2017

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Background: According to world health organization, G6PD deficiency constitute 7.5% of world population as carriers whereas 2.9% as deficient. It has been reported from India that the prevalence varies from 0-27% in different caste, ethnic and linguistic groups. Various studies have revealed strong interaction between G6PD deficiency and sickle cell genes on one hand and environment on the other, one may therefore expect changes in their frequencies over a period of time in the population. We studied the influence of G6PD deficiency upon the clinico-haematological and biochemical expression of sickle cell disease.

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Section: Serum Bilirubinsupporting

confidence: 93%

Influence of glucose-6 phosphate dehydrogenase (G6-PD) deficiency upon clinico-haematological and biochemical expression of patients with sickle cell disease

et al. 2017

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“…Combined hemoglobinopathy and enzyme deficiency have been reported [14]. Hb D trait can be symptomatic when associated with other causes for hemolysis like enzyme deficiency as suspected in one our cases.…”

Section: Hbd Traitmentioning

confidence: 65%

Hb D: A Not So Rare Hemoglobinopathy

Devi

Rameshkumar

Sitalakshmi

2014

Indian J Hematol Blood Transfus

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Dear Editor, Hb D is a b chain haemoglobin variant. It was first described by Itano in 1951 [1]. It differs in structure from Hb A at 121 position on b chain where glutamine replaces glutamic acid [2]. Hb D is known variously as Hb D Punjab, Hb D Los Angeles. It is the fourth most frequently occurring Hb variant [3].Hb D Punjab occurs with greatest prevalence (2 %) in Sikhs of Punjab and in Gujarat (1 %). It is also found sporadically in blacks and Europeans, the latter usually seen in countries that have close association with India in the past [3].HbD heterozygotes are clinically normal and homozygotes have a clinically mild phenotype. Hb D attains clinical significance in association with either b thalassemia or HbS.We describe 12 patients, 9 from South Indian population (three cases of Hb D trait and six cases of Hb SD) and 3 from West Bengal (one case homozygous HbDD, one case of HbD b thalassemia and one case of HbD trait).Blood samples collected in EDTA were subjected to complete blood counts, reticulocyte count and red cell indices on the Sysmex XT 1800i/XT 2000i/XT 4000i. Peripheral blood smears were prepared stained with Leishman's stain and examined. Sickling test was done where indicated by using 1 % sodium metabisulphite. G6PD screening was done using dye reduction test. The HPLC was done on BioRAD Variant II. The clinical data was retrieved from the medical records department.HPLC was done on 1,460 cases over a period of 2 years from September 2009 to February 2012. Five hundred and eighty-nine cases of haemoglobinopathies were detected. b Thalassemia trait was most common with 373 cases (63 %) being detected. b Thalassemia major was seen in 36 cases (6.1 %) and b thalassemia intermedia in 13 cases (2.2 %). Sickle cell disease was the next common with 28 cases of Sickle cell trait (4.7 %), 25 cases of sickle cell anemia (4.2 %) and 18 cases of sickle b thalassemia (3 %). HbE was seen significantly in the migrant population from Bengal with 24 cases of HbEE disease (4 %), 27 cases of HbE trait (4.5 %) and 14 cases of HbE b thalassemia (2.3 %). Twelve cases (2.0 %) showing Hb D were detected of which six cases were double heterozygous HbSD, one case of homozygous HbDD, one case of HbD b thalassemia and four cases of HbD trait. Eleven cases (1.8 %) of hereditary persistence of foetal haemoglobin (HPFH), four cases (0.6 %) of HPFH trait and four cases (0.6 %) of a thalassemia were also seen.

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Sickle cell disease in Middle East Arab countries

El‐Hazmi¹,

Al-Hazmi²,

Warsy³

2011

Indian Journal of Medical Research

125

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The sickle cell (HbS) gene occurs at a variable frequency in the Middle Eastern Arab countries, with characteristic distribution patterns and representing an overall picture of blood genetic disorders in the region. The origin of the gene has been debated, but studies using β-globin gene haplotypes have ascertained that there were multiple origins for HbS. In some regions the HbS gene is common and exhibits polymorphism, while the reverse is true in others. A common causative factor for the high prevalence and maintenance of HbS and thalassaemia genes is malaria endemicity. The HbS gene also co-exists with other haemoglobin variants and thalassaemia genes and the resulting clinical state is referred to as sickle cell disease (SCD). In the Middle Eastern Arab countries, the clinical picture of SCD expresses two distinct forms, the benign and the severe forms, which are related to two distinct β-globin gene haplotypes. These are referred to as the Saudi-Indian and the Benin haplotypes, respectively. In a majority of the Middle Eastern Arab countries the HbS is linked to the Saudi-Indian haplotype, while in others it is linked to the Benin haplotype. This review outlines the frequency, distribution, clinical feature, management and prevention as well as gene interactions of the HbS genes with other haemoglobin disorders in the Middle Eastern Arab countries.

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The prevalence of hemoglobin S and glucose-6-phosphate dehydrogenase deficiency in Jordanian newborn

Cited by 3 publications

References 7 publications

Influence of glucose-6 phosphate dehydrogenase (G6-PD) deficiency upon clinico-haematological and biochemical expression of patients with sickle cell disease

Influence of glucose-6 phosphate dehydrogenase (G6-PD) deficiency upon clinico-haematological and biochemical expression of patients with sickle cell disease

Hb D: A Not So Rare Hemoglobinopathy

Sickle cell disease in Middle East Arab countries

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