Plasma lipid levels in relation to disease severity in sickle cell anaemia in Abakaliki, Southeast Nigeria

Dyslipidaemia has been implicated in the pathophysiology of sickle cell disease (SCD) complications; hence its role requires further elucidation. Objectives: To investigate the relationship between disease severity and plasma lipid levels of patients with sickle cell anaemia. Methods: A cross-sectional study design was used for the survey. A total of 50 patients with sickle cell anaemia and 50 controls without SCD were recruited for the study. The clinical data and plasma lipid levels of lipids and haemoglobin parameters were analysed. Results: The majority of the participants were aged 18-25 years. Total plasma cholesterol and HDL-C were significantly lower in individuals with SCA compared with the controls (3.3±1.2 vs 4.2±1.2; p<0.001) and (1.3±0.5 vs 1.5±0.4; p = 0.038) respectively. Most patients with SCA had moderate disease severity (24; 48%). There was no statistically significant difference in the plasma levels of total cholesterol and HDL-C across the disease severity groups of SCA (p = 0.694 and 0.262). There was also no significant correlation between total cholesterol, HDL-C, and markers ofhaemolysis, haemoglobin F, and haemoglobin S levels. Conclusion: SCA is characterised by lower mean plasma TC and HDL than controls. However, no relationship was found between TC, HDL levels and SCD disease severity, markers of haemolysis, HbF and HbS levels. Further studies are required to ascertain the implications of plasma lipid levels in SCD

Oral Health Status of Children with Thalassaemia in Melaka: A Pilot Study

ABSTRACT@#Thalassemia is an inherited disorder that involves abnormal haemoglobin formation. This cross-sectional study with universal sampling was conducted from May 2018 to January 2019 to evaluate the oral health status of children with thalassaemia. The sample consisted of 54 transfusion-dependent patients between the ages of 3–17 years old, who were registered at the paediatric day-care centre of Hospital Melaka. Oral health status was evaluated which included soft tissue examination, recording of dental caries, malocclusion and oral hygiene status of the children, while their parents answered a pre-validated self-administered questionnaire on sociodemographic and oral health practices and attitudes. The mean deft (decayed, exfoliated and filled teeth) among the 3–5 years old was 6.71±6.07. The mean DMFT (decayed, missing and filled teeth) was 3.00±3.94 among the 13–17 years old and 1.12±1.42 among the 6–12 years old. Most of the 3–5 years old children exhibited good oral hygiene (71.4%). Dentofacial findings among the 3–5 years old were increased overjet (35.7%) and open bite (28.6%) predominantly. Fractured teeth (69.2%), increased overjet (46.2%) and discolouration (23.1%) were the common dentofacial findings among the 6–12 years old while increased overjet (71.4%), soft tissue lesions (57.1%) and posterior crossbite (28.6%) were seen among the 13–17 years old. In this cohort of children with thalassaemia, the prevalence of dental caries was high in preschool children and older children.

Haematological Parameters Associated with Malaria and Its Controls

Aims: This research aimed to evaluate the haematological parameters associated with malaria and its controls.Materials and Methods: A convenient cross-sectional technique was used for the study for which the sample size was determined by using the formula; n= Z² (P) (1-P) / (A) ². The haematological profile was performed using the Sysmex 2000i automated blood cell counter machine.Results and Discussion:The erythrocyte profiles (RBC, HB, HCT, RDW-SD and RDW-CV) are highly affected by malaria, whereas MCH, MCHC, and MCV did not show significant variations between the positive malaria cases and negative malaria cases. Means of haemoglobin concentrations, RBC count and HCT values for cases with positive malaria were significantly lower than negative malaria cases and controls for all the age groups and sexes. Conclusion:The study showed that there were haematological profiles between the positive and negative malaria cases and this can be used in conjunction with clinical and microscopic parameters to heighten the suspicion of malaria as well as prompt initiation of therapy for diagnosing malaria

Diabetic Ketoacidosis in Two Nigerian Adolescents with Homozygous Sickle Cell Anaemia.

In the tropics where the prevalence of sickle cell anaemia (SCA) is high, reports of concurrence of sickle cell anaemia and diabetes mellitus are rare with diabetic ketoacidosis (DKA), being rarer. In this case report, we present the cases of two Nigerian adolescents (one male and one female) with homozygous SCA who presented in DKA. Sickle cell anaemia was diagnosed eight and nine months respectively prior to their presentation with DKA. There was no history of previous multiple blood transfusions. Neither of the two cases had positive family history of diabetes mellitus. The diagnosis of DKA in each case was based on the presence of hyperglycaemia, ketonuria and acidosis. The families of these two patients were of low socio-economic status. Conclusion: Although concurrent homozygous sickle cell anaemia and diabetic ketoacidosis is rare, it does occur in Nigerian children and adolescents.

Prevalence and spectrum of hemoglobinopathies in tertiary care centre in a rural area of Madhya Pradesh.

Background: Haemoglobinopathies like thalassaemia and sickle cell anaemia etc are increasing due to unawareness of rural population. This study indicates type of haemoglobinopathies amongst the patients of a rural based tertiary care hospital in one year and nine months. Methods: Five hundred ten patients were studied during last one year and nine month for all suspected cases of haemolytic anaemia based on Complete Blood Count, Red cell indices and Peripheral blood smear examination. Sickling test, test for Hb F and haemoglobin electrophoresis with quantification of bands are done in all these cases Results: Out of all 510 cases of anaemia 461 cases (90.39%) were confirmed to nonhaemolytic anaemia whereas 49 cases (9.60%) had shown abnormal haemoglobin bands on electrophoresis. Out of these 49 cases 29 (59.18%) were Males and 20 (40.81%) were females. Most common Haemoglobinopathy observed was Sickle cell  Thalassaemia 23 (4.50%) followed by  Thalassaemia Trait 9 (1.76%), Sickle Cell trait 7 (1.37%).  Thalassaemia Major 5 (0.98%) & Sickle Cell Disease 5 (0.98%) have equal prevalence. The onset of disease was most prominent in Neonatal to pediatric age group including early adolescent (0-18 years) followed by reproductive age group (19- 45 years). Few cases of old age (46+ years) were detected. Conclusion: Study provides data on the spectrum & pattern of Haemoglobinopathies in a rural tertiary care centre. Screening of all anemic patients should be done for Haemoglobinopathy and proper Genetic counseling must be given to all cases to prevent incidence of cases in future generation.

Compound Heterozygous Sickle and Thalassemia Trait: A Case Report.

Sickle cell disease is a type of hemoglobinopathy, which is fairly common in certain parts of the world. We would like to report an interesting case of a child who was labeled as sickle cell anemia but subsequently turned out to be a case of compound heterozygous sickle cell and thalassemia trait.