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

Childhood aplastic anaemia with paroxysmal nocturnal haemoglobinuria clones: A retrospective single-centre study in South Africa

Background: Paroxysmal nocturnal haemoglobinuria (PNH) clones in children are rare but commonly associated with aplastic anaemia (AA) and myelodysplasia.Objective: This study aimed to determine the prevalence of PNH clones in paediatric patients with idiopathic AA, identify differences in clinical and laboratory features and outcomes, and determine the impact of clone size on clinical presentation.Methods: Patients with confirmed idiopathic AA who were tested for PNH between September 2013 and January 2018 at the Inkosi Albert Luthuli Central Hospital, Durban, KwaZulu-Natal, South Africa, were included. PNH clones were detected in neutrophils and monocytes by flow cytometry using fluorescent aerolysin, CD24, CD66b and CD14. Results: Twenty-nine children with AA were identified and 11 were excluded. Ten patients (10/18, 55.6%) had PNH clones ranging from 0.11% to 24%. Compared to the PNH-negative group, these children were older (median: 10 years vs 4 years, p= 0.02) and had significantly lower total white cell counts (median 1.7 × 109/L vs 3.2 × 109/L; p= 0.04). There was no difference in median absolute neutrophil count or haemoglobin concentration. Four patients in each group received immunosuppressive therapy (IST). At six months, all four patients with PNH clones had responded, compared to one in the PNH-negative group. Conclusion: More than half of children with AA had a PNH clone. The size of the clone did not impact clinical severity; however, IST use may positively impact prognosis. We recommend early initiation of IST in patients with AA to avoid delays associated with human leukocyte antigen typing.

Progression of anaemia during antenatal period among South African pregnant women

Background: Anaemia in pregnancy is a known public health problem in South Africa. Maternal, perinatal morbidity and mortality are known to be associated with anaemia in pregnancy. Very little is known from literature with regards to the progression of anaemia during the antenatal period of pregnancy. Objectives: To estimate haemoglobin levels, the prevalence and determinants of anaemia at different gestational ages and to show the trend. Method: A retrospective cohort (follow-up) study was conducted using the antenatal clinic register. Prevalence rates of anaemia (haemoglobin < 11 g/dl) at different gestational ages were measured. Factors associated with anaemia were assessed using chisquare test and stepwise multivariate logistic regression analysis. Results: A total of 801 pregnant women were enrolled at the booking visit and followed-up during their antenatal period. The prevalence of anaemia at the booking visit was 37%. The prevalence of anaemia at 20, 26, 32 and 36 weeks of gestation were 36.6%, 39.6%, 39.8% and 29.2% respectively. Binary logistic regression at the booking visit showed that teenage women were 2.5 times more likely to have anaemia (OR=2.5, p=0.005) than older women. Women who booked during the first trimester were 60% less likely to have anaemia (OR= 0.40, P=0.005) at the booking visit and 62% less likely to be anaemic at 36 weeks of gestation (OR=0.38, p=0.013) compared to those who booked late for antenatal care. Conclusion: Prevalence of anaemia during pregnancy was high. Early booking for antenatal care was a predictor for lower rate of anaemia. Thus, health education strategy should be encouraged for early antenatal booking

Functional iron status of chronic kidney disease patients at the University of Ilorin Teaching Hospital, Ilorin, Nigeria

Background: Functional iron deficiency has been found to be a common cause of poor response to erythropoiesis stimulating agents in anaemic patients with chronic kidney disease (CKD). Objectives: Assess the functional iron status of patients with chronic kidney disease. Methods: This was a hospital based cross sectional study. The study subjects were chronic kidney disease patients with age and sex matched healthy controls. Full blood count, serum ferritin, soluble transferring receptor, C-reactive protein, serum iron and total iron binding capacity were measured in the patients and healthy controls. Data was analyzed with statistical package for the social sciences software version 22.0. And the level of statistical significance was set at p. value < 0.05. Results: The mean ± SD of the age of patient with CKD was 55.0 + 15.4 years, while that of controls was 52.7 + 13.6 years. The mean serum ferritin, serum iron, TIBC and CRP were significantly higher in patients compared with controls (p<0.001, 0.023, <0.001 and 0.001) respectively. Functional iron deficiency was seen in 19.5% of patients with CKD. Conclusion: The predominant form of iron deficiency in our study was functional iron deficiency

Aplasies médullaires de l'enfant au CHU de Brazzaville

Introduction : L'Aplasie Médullaire (AM) est une insuffisance quantitative de l'hématopoïèse responsable d'une pancytopénie avec une moelle osseuse pauvre. Elle peut être constitutionnelle ou acquise (toxique, infectieuse, idiopathique). Si le traitement est bien codifié dans les pays riches avec l'amélioration du pronostic à long terme depuis plusieurs années, cette maladie demeure hautement mortelle dans les pays en voie de développement.Objectif : Etudier les caractéristiques cliniques, thérapeutiques et évolutives de l'AM chez l'enfant au CHU de Brazzaville.Matériels et méthodes : Il s'est agi d'une cohorte historique, réalisée sur la base des dossiers d'enfants hospitalisés pour une AM sur une période de 15 ans (2001- 2015). La classification de CAMITTA avait été utilisée.Résultats : 22 dossiers avaient été retenus. La moyenne d'âge était de 12,16 ans. Le sex-ratio garçon/fille était de 0,8. Les AM étaient toutes idiopathiques. Le syndrome anémique était le principal motif d'admission. Le taux moyen d'hémoglobine était de 4,3 g/dL, celui des polynucléaires neutrophiles de 0,340 Giga/l et celui des plaquettes de 13,2 Giga/l. Seize patients sur 22 (72,72%) souffraient de la forme sévère. Dans 90,90% des cas le traitement était symptomatique (concentrés érythrocytaires et plaquettaires, antibiothérapie) parfois associé à la corticothérapie ; 2 patients/22 (9,10%) ont reçu de la ciclosporine. Le taux de mortalité était de 94,73% après un suivi moyen de 9 semaines.Conclusion : L'AM de l'enfant au CHU de Brazzaville est essentiellement idiopathique et de pronostic redoutable

Crises aigues de deglobulisation chez le drepanocytaire homozygote : bilan etiologique et pronostique dans le service d'hematologie au CHU de Brazzaville

Contexte : La drepanocytose dans sa forme homozygote touche environ 1 a 2de la population congolaise. Les crises drepanocytaires; parmi lesquelles les crises aigues anemiques (crises de deglobulisation); emaillent la vie permanente de ces patients. Peu d'etudes completes ont permis d'evaluer les etiologies de ces complications aigues. Le but vise par ce travail est de contribuer a l'amelioration de la prise en charge des drepanocytaires. Objectifs : Recenser differentes etiologies des crises de deglobulisation ; enumerer les differentes entites des crises ; apprecier a travers l'evolution le pronostic et enfin evaluer a court terme l'evolution de ces crises. Patients et methodes : il s'est agi d'une enquete essentiellement retrospective qui a permis d'inclure 205 patients hospitalises au terme d'une periode qui s'etale entre le 1er juillet 2002 et le 30 juin 2007. Cette enquete qui a collige 142 dossiers (malades repondant a des criteres rigoureux) a permis de retenir 55 patients de sexe masculin et 87 patients de sexe feminin; soit un ratio de 0;6. L'age moyen de ces patients a ete de 23 ans (avec des extremes de 3 ans a 49 ans). Resultats : L'enquete clinique et paraclinique a permis de retrouver les types de crises suivants : 98;6des crises aigues hyperhemolytiques; 0;7des crises de sequestration splenique et enfin 0;7des crises aigues aplasiques. Les principales etiologies qui ont pu etre recensees au cours de cette enquete sont : le paludisme dans 30;3des cas et les infections bacteriennes dans 62;7. Dans 22;5des cas; le germe responsable de ces infections n'etait pas isole du fait de l'insuffisance du plateau technique. Dans 5;6des cas; le tableau de crise aigue de deglobulisation avait ete retrouve chez des femmes enceintes. Le niveau socio-economique a ete le principal facteur favorisant. L'evolution clinique des malades a ete favorable dans la majorite des cas. Conclusion : Les crises aigues de deglobulisation restent des urgences chez le sujet drepanocytaire homozygote a cause du risque important de morbidite et de mortalite. Ces crises semblent survenir chez les patients drepanocytaires appartenant a la couche des patients demunis. Le paludisme et les infections bacteriennes semblent representer les principales etiologies. La prevention de ces infections permettra certainement de reduire l'incidence; voire la frequence de ce type de complications