Sickle Cell Disease In Nigeria Research Articles

The fatty acid composition of the serum phospholipids of children with sickle cell disease in Nigeria

The purpose of this study was to determine the fatty acid composition of the serum phospholipids of children with sickle cell disease (SCD) in Nigeria and to compare the relative fluidity of the acyl chains of the serum phospholipids of controls versus the subjects with SCD. It is widely accepted that the fatty acid composition of an individual's serum phospholipids reflects that of their tissue phospholipids. An alteration in the fatty acid composition of membrane phospholipids could affect critical membrane-dependent enzymes and processes (e.g., ion and solute transport, hormone–receptor interactions, signal transduction pathways). We found a significant reduction in the content of polyunsaturated n-3 fatty acids in the phospholipids of subjects with SCD which could result in a reduction of the fluidity of their tissue membranes. Specifically, there was a 40–50% reduction in the proportion of total n-3 fatty acids in subjects with SCD. On the basis of calculated melting points and double bond indices of the acyl chains of the serum phospholipids, the phospholipids of the children with SCD are less fluid relative to those of their healthy counterparts. In addition, we determined that linoleic acid, arachidonic acid, and stearic acid were the major determinants of the fluidity of the acyl chains of the serum phospholipids of the healthy controls and children with SCD.

Assessment of the bone status of Nigerian children and adolescents with sickle cell disease using calcaneal ultrasound and serum markers of bone metabolism.

Growth and skeletal maturation are impaired in sickle cell disease (SCD). SCD is also associated with decreased bone mineral density (BMD) as determined by dual X-ray and photon absorptiometry. Quantitative ultrasound (US), which is as good a predictor of fracture as absorptiometry, provides additional information about bone architecture and elasticity. It is not known if the quantitative US parameters, broadband ultrasound attenuation (BUA) and speed of sound (SOS), are affected in children and adolescents with SCD. We therefore compared the bones of 80 children with SCD in Nigeria to those of age- and gender-matched controls using calcaneal ultrasound and the serum bone markers N-telopeptide of type1 collagen (NTx) and bone-specific alkaline phosphatase (BSAP), which are indicators of bone resorption and formation, respectively. BUA, which is reflective of BMD, was significantly lower for both the male and female SCD subjects compared with controls (86 vs 113 dB/MHz, P < 0.001 and 87 vs 100 dB/MHz, P < 0.001, respectively). However, SOS, which is more indicative of bone elasticity, was significantly different only for the male SCD subjects. Both NTx and BSAP were significantly reduced in the serum of the male and female SCD subjects. Correlations between BUA and serum NTx were found for both female controls and SCD subjects (r = 0.58, P < 0.001 and r = 0.32, P = 0.05, respectively), but not for the male subjects or controls. Significant correlations between BUA and BSAP were observed only for the female controls. In summary, we have shown that US analysis, in combination with serum markers of bone metabolism, can be used to distinguish bone development in children with SCD from that of nonaffected controls.

Leg ulceration in a population of Nigerian patients with sickle cell anaemia – twenty years experience

This retrospective study was conducted to determine the incidence of leg ulcers among a cohort of patients presenting with sickle cell disease at the University of Benin Teaching Hospital, Benin City. A total of 1163 patients with various haematological conditions were seen over a period of twenty years (1973–1992). The case files of these patients were examined with a view to identifying those with leg ulcers. Of the 1163 cases of haematological disorders seen over the study period, 686 (58.9%) were sickle cell disease. The patients were aged between 11 and 42 years (mean age = 28 years). Skin ulcers were the commonest long-term complications, representing 63 (9.2%) cases. Fifty two (7.6%) were leg ulcers with a slight female preponderance (male: female ratio 1:1.08), and the left leg was more commonly affected. Two cases were reported healed following EUSOL toileting and dressing. Also routine EUSOL toileting and dressing with antibiotic powder, though simple, appears more likely to improve ulcer healing than surgical approaches. Leg ulcer is the commonest long-term complication of sickle cell disease in Nigeria, and Staphylococcus aureus is most frequently cultured. [Jnl Medicine & Biomedical Res. Vol.1(1) 2002: 18-21]

Malevolent Ogbanje: recurrent reincarnation or sickle cell disease?

The Igbo of Nigeria believe that everyone is ogbanje (reincarnates) but malevolent ogbanje differ from others in being revenge-driven, chronically ill and engaging in repeated cycles of birth, death and reincarnation. This study examined culturally defined symptoms of 100 children classified as malevolent ogbanje; and investigated their family history and child mortality experience. There was concordance between cultural descriptions of malevolent ogbanje and symptoms as manifested in sickle cell patients. Hemoglobin analysis showed that 70 of the 100 children had sickle cell disease (SCD); while 68 families had death-related names. The symptoms associated with Igbo cases of reincarnation, high child mortality rates, and the high prevalence of sickle cell disease among children classified as malevolent ogbanje all support the conclusion that the symptomatology and early mortality experience are related to sickle cell. Names with themes of death were prevalent in families of children described as malevolent ogbanje. The findings are discussed with reference to cultural resistance to SCD as an explanation for malevolent ogbanje and the implications for the health care of children with SCD in Nigeria.

Hypofibronectinaemia and severity of sickle cell anaemia in Nigeria.

Plasma fibronectin levels in sickle cell anaemia and apparently healthy Nigerians were investigated to determine any correlation with disease severity. A cheaper in-house plasma fibronectin assay was also developed that could be adapted for use in Africa and elsewhere. Plasma fibronectin assay was concurrently carried out using the newly developed inhibition enzyme-linked immunosorbent assay (ELISA) and a commercial competitive binding ELISA. The in-house assay compared favourably with that of the commercial kit. The mean plasma fibronectin levels in sickle cell anaemia subjects were significantly lower than that of control subjects (P < 0.001). Plasma fibronectin concentration could therefore be useful in assessing the severity of sickle cell anaemia.

Hypofibronectinaemia and severity of sickle cell anaemia in Nigeria

Plasma fibronectin levels in sickle cell anaemia and apparently healthy Nigerians were investigated to determine any correlation with disease severity. A cheaper in‐house plasma fibronectin assay was also developed that could be adapted for use in Africa and elsewhere. Plasma fibronectin assay was concurrently carried out using the newly developed inhibition enzyme‐linked immunosorbent assay (ELISA) and a commercial competitive binding ELISA. The in‐house assay compared favourably with that of the commercial kit. The mean plasma fibronectin levels in sickle cell anaemia subjects were significantly lower than that of control subjects (P &lt; 0·001). Plasma fibronectin concentration could therefore be useful in assessing the severity of sickle cell anaemia.

Self-help in sickle-cell disease in Nigeria

Self-help in sickle-cell disease in Nigeria

A profile of sickle cell disease in Nigeria.

Nigeria has a population of 112 million with an annual growth rate of 3.2%. About 25% of adults throughout the country have the sickle cell trait, AS, while the Hb C trait is largely confined to the Yoruba people of southwestern Nigeria in whom it occurs in about 6%. Other variant hemoglobins including beta thalassemia are rare, but alpha thalassemia occurs in 39% (32% with 3 alpha-globin genes; 7% with 2 alpha-globin genes). Of a total of 5.4 million expected live births in 1988, about 90,000 will have SCD and 1.1 million the trait, AS. The clinical phenotype of sickle cell anemia is severe with manifestations occurring very early in childhood and mean Hb level 7.6 g/dl with HbF 5.9%. A very high infant mortality due to infections occurs especially in rural areas. Gallstones, leg ulcerations, and stroke appear less common than in American sicklers, and aplastic crises have not been described. Poor availability of resources to the public health and welfare sectors and economic inflation are severely curtailing access to appropriate medical and social services. This situation is frustrating to the families of a growing number of surviving patients in urban or middle to upper income groups. Efforts to create more awareness of SCD are paradoxically increasing frustration and stigmatization in the absence of a commensurate improvement of services. Any measures aimed at enhancing the sensitization of health professionals, policy makers, and resource allocators to the pertinent issues in the control of SCD would seem to be at this stage an important step in the right direction.

Coagulation changes in homozygous sickle cell disease in Nigeria.

Coagulation changes in homozygous sickle cell disease in Nigeria.

Ocular findings in children with homozygous sickle cell anemia in Nigeria.

The ophthalmological findings in 91 Nigerian children with homozygous sickle cell anemia is reported. While the most constant sign was abnormality of the conjunctival vasculature, seen in 74(81%) of the patients, retinal lesions were found in a total of 53(58%) patients. Only tortuosity of major vessels was seen in some patients but several others had more than one type of retinal lesion.

Family study in sickle cell disease in Nigeria.

A pilot survey has been made of Nigerian families at risk of producing SS haemoglobin homozygotes. Genotypes of parents and off-spring were investigated in twenty-five families. There is a suggestion that more SS children are produced where the first born suffers from haemoglobin SS disease. Mortality among offspring was higher in the study population than in the general population.