Sexual Maturation of Female Adolescent Patients with Sickle Cell Anaemia in Lagos, Nigeria.

BACKGROUND: Sickle cell anaemia (SCA) has been shown to have adverse effects on growth and sexual development. Recent Nigerian studies have reported a declining trend in the age of sexual maturation and menarche in haemoglobin AA (HbAA) individuals. OBJECTIVE: To evaluate the sexual maturation of female patients with SCA aged 10 to 19 years seen at Lagos University Teaching Hospital Idi-Araba, Lagos and Sickle Cell Foundation. METHODS: It was a cross-sectional study involving 140 girls with SCA (subjects) and an equal number of girls with HbAA (controls) who were matched for age and socio-economic class over a fivemonth period. The evaluation involved anthropometry, calculation of BMI and determination of breast and pubic hair development using Tanner staging. RESULTS: The mean BMI for the subjects was 17.2±2.39 kg/m2and 20.2±3.09 kg/m2 for the controls (p < 0.001). The mean age at onset of breast development (B2) was 13 ± 0.39 years in the subjects and 10.5 ± 0.51 years in the controls (p < 0.001). The corresponding mean age for pubic hair development (PH2) was also higher at 13.8 ± 0.59 years for the girls with SCA and 10.4 ± 0.51 years for the controls (p <0.001), while menarche was achieved in the subjects at 14.7 ± 1.68 years and in the controls at 12.3 ± 1.33 years (p < 0.001). Upper socioeconomic class was associated with earlier menarche and faster transition from pre-pubertal stage to pubertal stage of sexual maturation in the controls but was not observed in the SCA subjects. CONCLUSION: Sexual maturity in patients with SCA lagged behind those of the HbAA controls.

CONTEXTE: Il a été démontré que la drépanocytose (ACS) a un effet néfaste sur la croissance et le développement sexuel. Des études nigérianes récentes ont rapporté une tendance à la baisse de l'âge de maturation sexuelle et de la ménarche chez les individus hémoglobine AA (HbAA). OBJECTIF: Évaluer la maturation sexuelle de patientes atteintes d'ACS âgées de 10 à 19 ans vues à l'hôpital universitaire de Lagos IdiAraba, Lagos et à la Fondation drépanocytaire. MÉTHODOLOGIE: Il s'agissait d'une étude transversale portant sur 140 filles atteintes d'ACS (sujets) et un nombre égal de filles atteintes d'AAb (témoins) qui ont été appariées pour l'âge et la classe socio-économique sur une période de cinq mois. L'évaluation comprenait des mesures anthropométriques, le calcul de l'IMC et la détermination du développement des poils mammaires et pubiens à l'aide de la stadification Tanner. RÉSULTATS: L'IMC moyen pour les sujets était de 17,2±2,39 kg/m2 et de 20,2±3,09 kg/m2 pour les témoins (p<0,001). L'âge moyen au début du développement mammaire (B2) était de 13±0,39 ans chez les sujets et de 10,5±0,51 ans chez les témoins (p<0,001). L'âge moyen correspondant pour le développement des poils pubiens (PH2) était également plus élevé à 13,8±0,59 ans pour les filles atteintes d'ACS et à 10,4±0,51 ans pour les témoins (p<0,001) tandis que la ménarche a été atteinte chez les sujets à 14,7±1,68 ans et chez les témoins à 12,3±1,33 ans (p <0,001). La classe socio-économique supérieure a été associée à une ménarche plus précoce et à une transition plus rapide du stade prépubertaire au stade pubertaire de maturation sexuelle chez les témoins, mais n'a pas été observée chez les sujets SCA. CONCLUSION: La maturité sexuelle chez les patients atteints d'ACS était inférieure à celle des témoins de l'HbAA. MOTS CLÉS: Hémoglobine SS, Maturité sexuelle, Ménarche.

Blood pressure, hematologic and biochemical changes following L-arginine supplementation in children with sickle cell anaemia already on hydroxyurea therapy.

Hydroxyurea is an approved therapy in the management of children suffering from sickle cell disease (SCD). In adults co-administration of hydroxyurea and L-Arginine in adult sufferers of SCD had shown some benefits. This study examined the effect of co-administration of hydroxyurea (15-35mg/kg/day) and L-Arginine (500 mg/day) for 6 weeks on blood pressure, haematological parameters, liver and antioxidant enzymes levels. The levels of these parameters when the subjects were on hydroxyurea alone were taken as control values. Results showed that the combined therapy (HU + L-Arginine) decreased SBP, DBP, MAP and PP (p <0.01 in each case) but increased %HbF, Hb and PCV (p< o.001 in each case). It elevated CAT, SOD, GPX (p < 0.001 in each case) but depressed MDA, AST, ALT and ALP (p < 0.001 in each case). The study shows that L-Arginine used as an adjunct to hydroxyurea therapy may be beneficial to children suffering from sickle cell anaemia.

Prevalence of Skin Diseases in Children with Human Immunodeficiency Syndrome Infection in Paediatric HIV Clinic of A Tertiary Hospital in Nigeria.

BACKGROUND: HIV infection/AIDS being a multi-systemic disease affects the skin at various stages in course of the illness. A knowledge of the common skin diseases associated with HIV infection can lead to early detection, appropriate staging and commencement of appropriate care in the infected patients. OBJECTIVE: The study was to document the prevalence and pattern of skin diseases in children with HIV infection seen at the Paediatric department of a tertiary centre in Nigeria. METHODS: HIV positive children attending the PEPFAR HIV clinic of Lagos University Teaching Hospital, Nigeria were examined for the presence of skin lesions. Anthropometric measurements were also obtained. Blood samples Were taken for CD4+ cell count, viral load and full blood count. RESULTS: Two hundred and fourteen (214) patients were studied, consisting of 107 HIV infected children and 107 uninfected children as controls. Skin lesions were observed in 89 (83%) of the HIV infected patients, while only 72 uninfected controls had skin lesions. (p = 0.035). The predominant skin disease in the HIV infected children was Pruritic papular eruption (PPE) with a frequency of 25.9%, followed by fungal infections (24.6%). Herpes zoster was found only in HIV infected children (p = 0.041). There was a strong correlation between the degree of immunosuppression (as reflected by the value of age dependent CD4+ cell count/CD4+ percentage). and the prevalence of skin disease in the HIV infected patients. The presence of pruritic papular eruptions and Herpes zoster was associated with advanced immunosuppression. CONCLUSION: Skin diseases are common in HIV infection in our environment. Early detection of HIV infection can be made in the presence of skin diseases like Pruritic papular eruption and Herpes zoster.

Immune Suppression and Oral Manifestations of HIV in a Group of Nigerian Children.

BACKGROUND: Oral manifestations are common in HIV positive children and have been reported as possible predictors of HIV disease progression. This study assessed the prevalence of oral manifestations of HIV/AIDS and its association with immune suppression in a group of HIV positive Nigerian children. STUDY DESIGN: One hundred and twelve HIV positive children were examined for oral manifestations of HIV. The manifestations were compared with CDC Immune suppression categories using age specific CD4 lymphocyte counts. RESULTS: A total of 85 (76%) children had oral lesions. Oral candidiasis (65.2%) and parotid gland swelling (33%) were the most common lesions. Presence of oral lesions was significantly associated with declining immune status, p<0.05. CONCLUSION: The presence of oral lesions was significantly associated with lower immune status.

Orofacial and dental injuries associated with seizures in paediatric patients in Lagos University Teaching Hospital.

OBJECTIVE: To determine the prevalence and pattern of presentation of orofacial and dental injuries in children with seizures at the Children's Emergency Unit of the Lagos University Teaching Hospital (LUTH), Lagos, Nigeria. METHODS: This was a prospective study of children with febrile or non-febrile convulsion who presented at the Children's Emergency Unit of LUTH between July 2008 and August 2009. The age, gender, type of convulsion and the presence/absence of orofacial and other bodily injuries were recorded in a proforma. Mechanism, type and classification of injury were recorded for patients with orofacial injuries. RESULTS: A total of 257 children (148 males and 109 females) with febrile/non-febrile convulsion were included in the analysis. The mean age (SD) of patients was 32.8 ± 40.5 months. There were 223 (86.8%) cases of febrile convulsion and 34 (13.3%) cases of non-febrile convulsion. Thirteen children sustained orofacial injuries giving a prevalence of 5.1%. There was no significant difference in the prevalence of orofacial injury between patients with febrile convulsion (4.5%) and those with non-febrile convulsion (8.8%) (P=0.282). The most common cause of orofacial injuries was forceful insertion of hard object into the mouth during convulsive episodes. Twelve (92.3%) patients sustained soft tissue injury, while one sustained both soft and hard tissue injuries. The most common site of injury was the lip. CONCLUSIONS: Oro-facial and dental injuries may occur in children with seizures. It is therefore important that these injuries be looked for by the paediatrician and the dentist/oral and maxillofacial surgeons should be involved in managing these children.

Haemoglobin and ferritin concentrations in cord blood in a tertiary health centre in Nigeria.

BACKGROUND: Haemoglobin(Hb) and serum ferritin (SF) concentrations of cord blood of babies born at term at the Lagos State University Teaching Hospital, Maternity Centre (Ayinke House), Ikeja in the South-Western part of Nigeria were determined to establish mean values for these substances in our locality. OBJECTIVES: To establish the mean values for haemoglobin and serum ferritin concentrations of cord blood of babies born at term in our environment and to determine the prevalence of foetal anaemia and low iron store in cord blood in our locality. METHODS: Haemoglobin and ferritin levels in cord blood of 142 newborns were determined. Two millilitres of blood was collected from the cord of each newborn into EDTA bottle for complete blood count analysis and another 2mls into a plain bottle for serum ferritin assay. Cut-off values for cord blood Hb and serum ferritin concentrations were 12.5g/dL and 60 microg/L respectively. RESULTS: The mean Hb and ferritin values were 13.024 +/- 2.41 g/dL and 70.85 +/- 97.07 microg/dL respectively. The prevalence of foetal anaemia is 32.4 %. About 59.2% of full term newborns had low iron store. Birth weight was significantly associated with Hb concentration (p=0.039) and apga sscore (p=0.002). CONCLUSION: The prevalence of foetal anaemia was 32.4%. More than half (59.2%) of the newborns had low cord blood serum ferritin.

The influence of socioeconomic status on the hemoglobin level and anthropometry of sickle cell anemia patients in steady state at the Lagos University Teaching Hospital.

BACKGROUND: Sickle cell anemia (SCA) has multisystemic manifestations and is associated with severe morbidity and high mortality. It commonly affects growth leading to wasting and stunting. AIMS AND OBJECTIVES: This study aimed to determine the influence of socioeconomic status on the nutritional status using anthropometric measurements and steady-state hemoglobin, of children with homozygous SCA, aged 1 year to 10 years in steady state at the Lagos University Teaching Hospital. MATERIALS AND METHODS: This is a cross-sectional study involving 100 children with SCA and 100 age-, sex-, and social class-matched controls that fulfilled the inclusion criteria. Social class was assessed using educational attainment and occupation of parents. Hemoglobin concentration was determined using the oxy-hemoglobin method. RESULTS: This study demonstrated a significantly lower mean weight and weight-for-height in the SCA patients than those of controls (P < 0.001). By contrast, this study did not demonstrate any statistical significant difference in the mean height and mean body mass index of SCA patients and controls (P = 0.06) and (P = 0.12), respectively. The mean weight, height, and body mass indices of the subjects and controls were consistently below those of the NCHS standards. The magnitude of the difference from the NCHS standard was also more pronounced in the subjects, increased with advancing age and affected male subjects more than females. Progressive declines in the anthropometric attainment and hemoglobin concentration were observed from social class 1 to 4; this was statistically significant in controls (P = 0.00) but not in subjects (P > 0.1). However, SCA patients had significantly lower values than controls in each of the social classes. CONCLUSION: Poor socioeconomic status has an adverse effect on the nutritional status and hemoglobin of SCA patients.

The influence of socioeconomic status on the hemoglobin level and anthropometry of sickle cell anemia patients in steady state at the Lagos University Teaching Hospital

Background: Sickle cell anemia (SCA) has multisystemic manifestations and is associated with severe morbidity and high mortality. It commonly affects growth leading to wasting and stunting. Aims and Objectives: This study aimed to determine the influence of socioeconomic status on the nutritional status using anthropometric measurements and steady-state hemoglobin; of children with homozygous SCA; aged 1 year to 10 years in steady state at the Lagos University Teaching Hospital. Materials and Methods: This is a cross-sectional study involving 100 children with SCA and 100 age-; sex-; and social class-matched controls that fulfilled the inclusion criteria. Social class was assessed using educational attainment and occupation of parents. Hemoglobin concentration was determined using the oxy-hemoglobin method. Results: This study demonstrated a significantly lower mean weight and weight-for-height in the SCA patients than those of controls (P 0.001). By contrast; this study did not demonstrate any statistical significant difference in the mean height and mean body mass index of SCA patients and controls (P 0.1). However; SCA patients had significantly lower values than controls in each of the social classes. Conclusion: Poor socioeconomic status has an adverse effect on the nutritional status and hemoglobin of SCA patients

Adherence to Antiretroviral Therapy among HIV-Infected Children Attending a Donor-Funded Clinic at a Tertiary Hospital in Nigeria

Despite their high levels of knowledge about HIV and AIDS; young people ages 15-24 years in South Africa remain disproportionately affected by the epidemic. Young people's continued susceptibility to HIV infection has been consistently linked to intractable higher-risk sexual behaviours. This paper uses multivariate techniques and secondary data from two nationally representative surveys to illuminate individual and socio-structural factors that play a significant role in youths' continued engagement in higher-risk behaviour; despite their high awareness about HIV and AIDS. The findings show that notwithstanding progress in terms of increased condom use and reduced incidence of other sexually transmitted infections; the average age of sexual debut remains low; multiple sexual partnerships are prevalent; and inconsistent condom use is widespread among young people. Factors significantly associated with these risk behaviours occur at the individual and structural levels and include issues of race; gender; poverty and susceptibility to peer pressure. The paper concludes by recommending that future HIV-prevention interventions in South Africa should aim at building resilience among youths by promoting affirmative; supportive interventions that emphasize the potentials of young people

Severe idiosyncratic drug reaction (Lyells syndrome) after ingesting dihydroartemisinin.

Lyells syndrome also called Toxic epidermal necrolysis is the extreme form of idiosyncratic drug reaction that is called Steven Johnsons Syndrome: The condition results in an extensive loss of the skin with mucous membrane involvement. Lyells syndrome has been induced by many agents. The commonest agent in the literature being sulphonamides. However, in our search of the medical literature there was no report of dihydroarthemisinin as a cause of Lyells syndrome. We report three patients seen at two tertiary health institutions with Lyells syndrome after treatment for malaria with dihydroarthemisinin. This resulted from administration of dihydroarthemisinin with chloroquine in two patients and dihydroarthemisinin with Amodiaquine in one patient. The first patient was a seven year old child who developed 90% cutaneous involvement and died from hemorrhagic shock. The second was a 28 old female that developed a 76% body surface involvement and died from septicemia. The third patient was a pregnant 37 year old woman that developed 52% body involvement and died from septic shock. In these patients the earliest symptoms were not recognized and there was considerable delay before referral. In view of the recent WHO recommendation ofArthemisinin Combination Treatment (ACT) for malaria, we expect more cases of Steven Johnson Syndrome and Lyells syndrome from ACT treatment. The aim of this report is to raise the awareness of clinicians to this potentially fatal complication.

Anthropometric indices of infants born to HIV-1-infected mothers: a prospective cohort study in Lagos, Nigeria.

Numerous studies have reported that HIV-infected pregnant women are at increased risk of delivery of low birth weight (LBW) infants, of preterm deliveries and of intrauterine growth restriction. The objective of the study was to determine the effect of maternal HIV infection on the anthropometric characteristics of the babies at birth. A prospective study was carried out at the Lagos University Teaching Hospital, Nigeria. There were three times more LBW babies in the HIV-positive group than in the uninfected mothers (odds ratio = 3.47, 95% confidence interval = 1.69, 7.27; chi(2) = 12.99, P = 0.0003).The maternal weight (t = 15.85; P = 0.0001), maternal body mass index (BMI) (t = 15.07; P = 0.0003), birth weight of infants (t = 27.17; P = 0.0001) and birth length (t = 31.20; P = 0.001) were significantly less in HIV-positive mothers than in controls. In conclusion, poor maternal bodyweight and low BMI are significant contributors to LBW in HIV-infected women. Nutritional counselling, dietary intake and weight monitoring during pregnancy should be emphasized to improve pregnancy outcome in HIV-infected women.

Dental caries status of HIV infected children in Nigeria.

BACKGROUND: HIV positive children may be prone to developing Dental Caries due to prolonged and frequent use of sucrose containing medications and poor feeding practices. OBJECTIVE: To determine the caries status of primary and permanent dentition in HIV positive Nigerian children using dmft/DMFT indices. METHODS: One hundred and twelve HIV positive children aged 4 months to 13 years attending two dedicated HIV outpatient clinics were examined for dental caries of primary and permanent dentition using the WHO criteria. Carious lesions were recorded using the dmft and DMFT indices in the primary and permanent dentition respectively. RESULTS: Forty four [39.3%] of the 112 children examined had dental caries mainly in the primary dentition in 96.7% of the cases. Mean dmft was 1.15 +/- 1.88, mean DMFT was 0.17 +/- 0.57. Dental caries was not significantly associated with the immune status of HIV positive children; chi2 = 2.58, P = 0.275. CONCLUSIONS: Dental caries experience in the primary dentition of these HIV positive children was greater than in the Nigerian paediatric population. Dental caries prevalence in these HIV positive children although lower than that seen in developed countries was however higher than in other reports of healthy Nigerian children. There was no significant association between dental caries and immune status of HIV positive children.

Effect of antiretroviral therapy on asymptomatic malaria parasitaemia in HIV-1 infected children.

OBJECTIVE: To investigate the effect of antiretroviral ARV) therapy on the level of asymptomatic malaria parasitaemia in HIV-1 infected children. METHODS: Sixty-six HIV infected children had blood films prepared for malaria parasite identification and count. Mean parasite densities were compared across clinical stages and immunologic categories of disease and antiretroviral treatment status. RESULTS: Forty-five (68%) were less than 6 years old and 50 (75.7%) had advanced HIV disease. Twenty seven (41%) were on antiretroviral therapy. The prevalence of ASMP in the treated and untreated group was 44.4% and 15.4% respectively (p<0.01). The mean parasite density in the ARV treatment group was also significantly higher than in the untreated group (p=0.0071). CONCLUSIONS: ARV therapy seems to be associated with higher rates of ASMP and higher mean parasite counts.

Effect of antiretroviral therapy on asymptomatic malaria parasitaemia in HIV-1 infected children.

OBJECTIVE: To investigate the effect of antiretroviral (ARV) therapy on the level of asymptomatic malaria parasitaemia in HIV-1 infected children. METHODS: Sixty-six HIV infected children had blood films prepared for malaria parasite identification and count. Mean parasite densities were compared across clinical stages and immunologic categories of disease and antiretroviral treatment status. RESULTS: Forty-five (68%) were less than 6 years old and 50 (75.7%) had advanced HIV disease. Twenty seven (41%) were on antiretroviral therapy. The prevalence of ASMP in the treated and untreated group was 44.4% and 15.4% respectively (p<0.01). The mean parasite density in the ARV treatment group was also significantly higher than in the untreated group (p=0.0071). CONCLUSIONS: ARV therapy seems to be associated with higher rates of ASMP and higher mean parasite counts.

A 12-year retrospective study of non-burn skin loss (burn-like syndromes) at a tertiary burns unit in a developing country.

BACKGROUND: A retrospective study of the presentation, etiology, and prognosis of non-burn epidermal loss managed at the Lagos University Teaching Hospital Nigeria over a 12-year period. MATERIALS AND METHODS: Admission records of patients managed for non-burn skin loss were retrieved from the medical records. Demographic details of the patients, the initial diagnosis, final diagnosis, treatment and outcome of treatment was noted. RESULTS: A total of 23 patients were identified, 17 (74%) had idiosyncratic drug reactions. Of this 17, 6 (26%) had Steven Johnson Syndrome, 6 (26%) had Steven Johnson Syndrome/toxic epidermal necrolysis while 5 (22%) presented with toxic epidermal necrolysis. Three of the five patients with toxic epidermal necrolysis died. The age range of patients with idiosyncratic adverse drug reactions was 2-28 years, mean, 10.18+/-1.44 years and male to female ratio of 1:1.83. The body surface area involved ranged from 8 to 78%; mean 26.65+/-6.08%. The agents suspected for the reactions were Co-trimoxazole (41.2%) and combination of Co-trimoxazole, and Fansidar (17.6%). Other conditions seen were two (9%) Staphylococcal Scalded Skin Syndrome, three (13%) had Necrotizing Faciitis, one of whom was HIV positive and died. One (4%) patient presented with pemphigus vulgaris. The presentation and management of the patients was discussed.

Maternal HIV infection and intrauterine growth: a prospective study in Lagos, Nigeria.

BACKGROUND: Low birth weight (LBW) is the most important cause of perinatal mortality and morbidity worldwide and particularly so in developing countries. Maternal HIV (Human Immunodeficiency Virus) infection has been identified as one of the risk factors to the development of low birth weight babies. OBJECTIVE: To evaluate the effect of maternal HIV infection on the birth weight of the newborn at tertiary hospital in West Africa. METHODS: The anthropometry of all HIV seropositive women who delivered in LUTH as well as that of their babies was determined using standard methods. Controls consisted of HIV seronegative women and their babies matched for age and parity with the above subjects. RESULTS: There were a total of 262 subjects of whom 132 (50.4%) were HIV seropositive and 130 (49.6%) were HIV seronegative controls. There were five times more low birth weight (LBW) infants in the HIV seropositive group than in the controls (OR 5.77, CI=2.19-16.80; p=0.000075). The mean maternal body mass index, BMI (p=0.0003), mean maternal weight (p=0.0004) and mean birth weight of newborns (p=0.0002) were significantly lower in the HIV seropositive group than in the controls. Maternal weight and gestational age were significantly associated with low birth weight (OR 15.3, CI=2.6-316.0; p=0.002) and (OR 3.78, CI=1.37-10.9; p=0.007) respectively. CONCLUSION: Maternal HIV infection is strongly associated with low maternal BMI and low birth weight in their offspring.

Haematological abnormalities associated with paediatric HIV/AIDS in Lagos.

INTRODUCTION: In HIV-1-infected children, haematological disturbances include bone marrow abnormalities and peripheral cytopenias. All three major cell lineages can be depressed. METHODS: A cross-sectional study of baseline haematological parameters was undertaken in 68 children with confirmed HIV infection. In all cases, a complete blood count was done and some had CD4+ counts and HIV RNA PCR. The CD4+ count was analysed by the Coulter manual latex particle monoclonal antibody method and HIV RNA PCR by Roche Amplicor Monitor, version 1.5. RESULTS: Anaemia (< 100 g/L) was present in 77.9%, severe (< 60 g/L) in 5.9%, moderate (60-70 g/L) in 32.3% and mild (80-99 g/L) in 39.7%. The mean haemoglobin concentration decreased as disease progressed (p < 0.05); 6% had leucopenia, 17.5% had neutropenia and 2.5% (one case) had thrombocytopenia; also, the four (6%) subjects with leucopenia were in clinical stages B and C. Neutropenia, lymphocytopenia and thrombocytopenia were seen more in clinical stages B and C, though this relationship was not statistically significant. CONCLUSION: Both the erythroid and other cells lines are affected by HIV/AIDS and other associated factors. Anaemia is the most common haematological abnormality. The severity of peripheral cytopenias is related to the disease burden.

Ultrasonographic measurements of the hepatobiliary axis of children with sickle cell anaemia in steady state

Sickle cell anaemia is associated with structural manifestations in the hepatobiliary axis but these manifestations have not been sufficiently examined in Nigerian or African children. This study wasconducted to evaluate the results of ultrasono-graphic measurements of the hepatobiliary axis of children with sickle cell anaemia in a Nigerian teaching hospital. One hundred and twenty children with Hb SS in steady state aged between 12 months and 15 years and sixty children with Hb AA matched by age and sex (as controls) were consecutively recruited from April through November 2002. Past and present medical histories were obtained and physical examination and abdominal ultrasonography were performed. The mean liver span; the common hepatic duct and common bile duct diameters; the longitudinal and transverse dimensions (but not the wall thickness) of the gallbladder were significantly higher in test subjects than controls (p 0.05). Multiple regression showed that age correlated significantly with all the studied ultrasound scan (USS) measurements (p 0.05). Recurrent episodes of abdominal pain correlated with gallbladder wall thickness (p

Serum magnesium, phosphate and calcium in Nigerian children with sickle cell disease.

BACKGROUND: Biochemical abnormalities have been associated with sickle cell disease. Studies on phosphorus and magnesium in sickle cell disease have been conflicting. There is paucity of information on the role of these ions in the pathogenesis and management of sickle cell disease. This study was set out to determine the serum levels of magnesium, phosphorus and calcium in Nigerian children with sickle cell disease. STUDY DESIGN: A case-control study carried out on children with HbSS genotype (cases) and age-matched controls with HbAA. Serum magnesium, calcium, phosphorus and albumin were measured using colorimetric methods. RESULTS: Eighty-six subjects and forty-five controls were studied. The mean serum magnesium was 0.99mmol/L (0.02) and 0.98mmol/L (0.02) in the cases and controls respectively. The difference was not significant. Mean serum calcium was significantly lower in the cases [2.1mmol/L (0.3)] compared with the controls [2.3mmol/ L (0.15)]; p<0.01. Serum phosphorus was significantly higher in the cases than in the controls [2.2mmol/L (0.7) versus (1.5mmol/L (0.6); p<0.001]. There was no statistical difference in the albumin binding of calcium in both groups. A positive correlation existed between serum phosphorus and magnesium and also between serum calcium and magnesium in the cases group but no correlation between these parameters and age was found. CONCLUSION: Children with sickle cell anaemia in this study had normo-magnesaemia, hyperphosphataemia and hypocalcaemia. Further studies on changes in intracellular concentrations of these ions in children with sickle cell disease are required. Such findings could be useful in designing better management in individuals with this abnormality.

Changes in erythrocytes following supplementation with alpha-tocopherol in children suffering from sickle cell anaemia.

INTRODUCTION: Low plasma vitamin E level in children with sickle cell anaemia has been related to elevated level of irreversibly sickled cells which has been implicated in the pathogenesis of painful crisis. In adult patients supplementation with alpha-tocopherol has been shown to reduce irreversibly sickled cell count. In-addition, vitamin E supplementation increases resting forearm blood flow and reduces forearm vascular resistance in adult sickle cell anaemia sufferers. AIM: The aim of the study was to examine the effects of supplementation with alpha-tocopherol on arterial blood pressure, some haematological parameters and osmotic fragility in children suffering from sickle cell anaemia. METHODOLOGY: The effects of supplementation with alpha-tocopherol (100 mg. per day for 6 weeks) on packed cell volume (PCV) haemoglobin concentration ([Hb]), mean corpuscular haemoglobin concentration (MCHC), per cent foetal haemoglobin (% HbF), per cent irreversibly sickled cells (%ISC), erythrocyte osmotic fragility and blood pressure have been assessed in ten (10) children suffering from sickle cell anaemia. Their ages ranged from 4.0 to 10.0 years. RESULTS: Supplementation with alpha-tocopherol significantly increased packed cell volume (p<0.001), haemoglobin concentration (p<0.01) and per cent foetal haemoglobin (p<0.001), but significantly reduced mean corpuscular haemoglobin concentration (p<0.01) and per cent irreversibly sickled cells (p<0.001). alpha-Tocopherol also increased the resistance of the cells to lysis. Supplementation with alpha-tocopherol had little or no effect on arterial blood pressure. CONCLUSION: This study shows that short-term supplementation with alpha-tocopherol may be beneficial to the paediatric sickle cell sufferer.