A case of sickle cell an˦mia in the Sudan

Archibald, R. G.

doi:10.1016/s0035-9203(26)90485-9

Cited by 27 publications

(9 citation statements)

References 5 publications

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“…SCA was first reported by Archibald [19]. Three foci of the disease were subsequently described: western Sudan, where a prevalence rate of up to 30.4% was reported among the Messeryia [20].…”

Section: Discussionmentioning

confidence: 99%

Relationship of the Sickle Cell Gene to the Ethnic and Geographic Groups Populating the Sudan

Mohammed

Attalla

Bashir

et al. 2006

Public Health Genomics

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The presence of a geographical pattern in the distribution of the sickle cell gene (S gene) and its association with malaria is well documented. To study the distribution of the S gene among various ethnic and linguistic groups in the Sudan we analyzed a hospital-based sample of 189 sickle cell anemia (SCA) patients who reported to the Khartoum Teaching Hospital between June 1996 and March 2000 and 118 controls with other complaints, against their ethnic and linguistic affiliations and geographic origin. Electrophoresis for hemoglobin S and sickling tests were carried out on all patients and controls as a prerequisite for inclusion. The majority of patients (93.7%) belonged to families of single ethnic descent, indicating the high degree of within-group marriages and thus the higher risk of augmenting the gene. SCA was found to be predominant among the Afro-Asiatic-speaking groups (68.4%) including nomadic groups of Arab and non- Arab descent that migrated to the Sudan in various historical epochs. Those patients clustered in western Sudan (Kordofan and Darfur) from where 73% of all cases originate. The proportion of patients reporting from other geographic areas like the south (3.1%), which is primarily inhabited by Nilo-Saharan-speaking groups (19% of the whole sample) who populated the country in previous times, is disproportionate to their total population in the country (χ² = 71.6; p = 0.0001). Analysis of the haplotypes associated with the S gene indicated that the most abundant haplotypes are the Cameroon, Benin, Bantu and Senegal haplotypes, respectively. No relationship was seen between haplotypes and the various hematological parameters in the sub-sample analyzed for such association. These results provide an insight into the distribution of the sickle cell gene in the Sudan, and highlight the strong link of the middle Nile Valley with West Africa through the open plateau of the Sahel and the nomadic cattle herders and also probably the relatively young age of the S gene.

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Section: Discussionmentioning

confidence: 99%

Relationship of the Sickle Cell Gene to the Ethnic and Geographic Groups Populating the Sudan

Mohammed

Attalla

Bashir

et al. 2006

Public Health Genomics

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“…His patient was a 20-year-old dental student from Granada. In Africa, the disease went largely unnoticed until 1926, when Archibald reported the first case from the Sudan [2]. Nwokolo [3] drew attention to the presence of the disease in Eastern Nigeria in 1960, but he confined his report to discussing diagnosis and management.…”

Section: Introductionmentioning

confidence: 99%

Clinical presentation of severe anemia in pediatric patients with sickle cell anemia seen in Enugu, Nigeria

2003

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Anemia is a major cause of morbidity and mortality among patients with sickle cell anemia. In this study, 108 episodes of severe anemia were prospectively evaluated in 108 patients with hemoglobin SS disease attending the pediatric sickle cell clinic of the University of Nigeria Teaching Hospital, Enugu, Nigeria. Young children between the ages of 2 and 4 years were found to be at the greatest risk of developing anemic crises (severe anemia). There was a gradual but progressive decline in the incidence of severe anemia in the age range 8-16 years old. Upper respiratory tract infections are the most commonly associated infections in patients with severe anemia. Others included malaria, septicemia, urinary tract infection, acute chest syndrome, and osteomyelitis. Their role in precipitating episodes of severe anemia among the patients studied could not be fully evaluated. Pallor, jaundice, and fever were the most commonly encountered symptoms in patients with severe anemia on admission. About half of the parents/guardians failed to notice severe anemia among the patients studied, perhaps due to the dark color of the African skin. Caregivers need to be educated on how to recognize anemia among patients with sickle cell anemia when they develop febrile episodes. Am. J. Hematol. 72:185-191, 2003.

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“…There was controversy on splenic size. Atrophy was characteristic of early autopsy reports (Graham, 1924; Jaffe, 1927; Bennett, 1929; Steinberg, 1930; Yater & Mollari, 1931; Corrigan & Schiller, 1934), but splenomegaly was common in young patients (Jamison, 1924; Archibald, 1926; Alden, 1927), intermittent in others (Dreyfoos, 1926) and, in some, the spleen appeared to enlarge during painful crises (Stewart, 1927; Josephs, 1928; Wollstein & Kreidel, 1928). Occasionally, marked enlargement extended to the iliac crest (Hahn & Gillespie, 1927) and spleens weighing 180–210 g were removed in children under 3 years of age (Bell et al , 1927; Wollstein & Kreidel, 1928).…”

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confidence: 99%