Protein C and antithrombin levels in patients with sickle cell anemia in Ahmadu Bello University Teaching Hospital Zaria, Nigeria.

BACKGROUND: Alterations in the components of hemostasis, namely platelet function, the procoagulant, anticoagulant, and the fibrinolytic systems, are observed in sickle cell anemia (SCA) and are in favor of a procoagulant phenotype. Therefore, study of protein C and antithrombin (AT) levels in patients with SCA in steady state may be used in the treatment and/or prevention of SCA-related thrombotic complications. We studied the changes of these naturally occurring anticoagulants in patients with SCA attending the sickle cell clinic in Ahmadu Bello University Teaching Hospital, Zaria. METHODS: We conducted a case-control study involving 50 SCA (HbSS) patients in the steady state as cases and 25 healthy volunteers with normal hemoglobin (HbAA) as controls. Protein C and AT levels were estimated by semi-automation using Diagnostica Stago hematology coagulation analyzer. Frequencies, proportions, and independent t test were performed using SPSS version 20. RESULTS: The mean ages of both the patients and controls were 23.80 ± 7.46 and 24.28 ± 3.48 years, respectively, and study participants comprised 40 (53.0%) women between the ages of 15-50 years and 15-34 years (P = 0.76). The mean values of protein C and AT levels in patients with SCA in the steady state and the control group were 60.26 ± 20.58% versus 81.30 ± 19.74%, 95% CI 11.13-30.96, and 42.11 ± 5.01% versus 61.88 ± 11.27%, 95% CI 16.03-23.51 with P values (P < 0.001), respectively. CONCLUSIONS: This study showed that there was a significant decrease in the levels of protein C and AT between the SCA patients in the steady state and the controls. We recommend baseline investigations of these naturally occurring anticoagulants in patients with SCA, especially in those with frequent vaso-occlusive crises. This will give us an insight into the additional pathophysiologic mechanism in SCA-related thrombotic complications for better patient management and outcome.

Variations in the ß-globin genes of sickle cell anaemia patients in Zaria, Northwestern, Nigeria.

CONTEXT: Sickle Cell Anaemia (SCA) is a genetic disorder with a life-long disability, which is of public health importance. The diversity in its clinico-pathologic and laboratory presentations may be due to the interplay between additional genetic differences and environmental factors. The genetic factors may be within the ß-globin gene itself, the ß-globin gene cluster or elsewhere in the genome. AIM: To characterize the ß-globin gene for variations associated with the Sickle Cell mutation. SETTINGS AND DESIGN: A cross-sectional descriptive study involving 51 adult SCA patients attending Sickle Cell Clinic of Haematology Department Ahmadu Bello University (ABUTH) Zaria, Kaduna State, Nigeria. METHODS AND MATERIAL: The buccal swab specimens were collected and ß-globin gene DNA sequencing was done. The sequences obtained were compared with a Genbank Reference ß-globin gene (NC_000011.9) using Basic Local Alignment Search Tool (BLAST), and variations noted. Data generated were analyzed using SPSS Version 20.0. STATISTICAL ANALYSIS USED: Data generated was summarized by using charts, means±2SD, and 95% confidence intervals. RESULTS: There were 40 (78.43%) females and 11 (21.57%) males. The mean age of the participants was 25.35 ± 7.67 years, 95% CI (23.20, 27.51). The classic sickle cell mutation A T was present in all participants. The mean number of ß-Globin gene variations was 8.61±11.30, 95% CI (5.43, 11.78). The number of Substitutions were 122 (27.79%), insertions 184 (41.91%), and deletions 133 (30.30%). These occurred in various combinations. The mean number of substitutions, insertions, and deletions were 2.39±3.23, 3.61±7.66, and 2.60±2.46 with 95% CIs of (1.48, 3.30), (1.45, 5.76), and (1.92, 3.30) respectively. CONCLUSIONS: There are ß-globin gene variations in SCA patients in Zaria, and locally relevant genetic database of the SCA population will be the cornerstone in understanding genotype-phenotype interactions in this disorder.

Assessment of biochemical liver function tests in relation to age among steady state sickle cell anemia patients.

BACKGROUND AND OBJECTIVE: Multiorgan failure including liver dysfunction is a common finding in sickle cell anemia (SCA) patients, the cause of which is multifactorial with advancing age said to be a major determinant. There is a paucity of data on liver function among SCA patients in relation to age in northern Nigerian hospitals, including Ahmadu Bello University Teaching Hospital (ABUTH), Zaria. This study was to assess the biochemical liver function tests (LFTs) as they relate to age among SCA patients in steady state, with a view to improving the overall monitoring of these patients. SUBJECTS AND METHODS: This study was carried out in ABUTH, Zaria, Northern Nigeria. LFTs were carried out in 100 SCA and 100 apparently healthy participants (controls). The SCA group was made up of fifty adults and fifty children diagnosed of SCA, whereas the control group was made up of fifty adults and fifty children who were apparently healthy and had hemoglobin AA. Paired two-tailed Student's t-test for matched samples and Pearson's linear correlation statistical methods were employed for the data analysis using Microsoft Office Excel 2007. A P ≤ 0.05 was considered as statistically significant. RESULTS: The serum concentrations of total bilirubin (TB), alanine transaminase (ALT), aspartate transaminase (AST), alkaline phosphatase (ALP), and AST/ALT ratio were significantly higher in SCA patients compared to the controls (P = 0.001, P = 0.001, P = 0.05, P = 0.05 and P = 0.001, respectively). Serum total protein (TP) and ALB were significantly lower (P = 0.01 and P < 0.05, respectively) in SCA patients compared with the controls. The levels of TB, ALT, AST, ALP, and AST/ALT were significantly lower in SCA adults compared to SCA children, whereas TP and ALB were higher in SCA adults compared to the SCA children. There were significant negative correlations between age and each of TB, ALT, AST, ALP, and AST/ALT, and significant positive correlations between age and each of TP and ALB in SCA patients. CONCLUSION: There are mild LFTs derangements in SCA patients even in steady state with the extent of the abnormalities decreasing with advancing age of the patients.

Autosplenectomy of sickle cell disease in zaria, Nigeria: an ultrasonographic assessment.

OBJECTIVES: During infancy and early childhood, the spleen commonly enlarges in patients with sickle cell anemia (SCA), and it thereafter undergoes progressive atrophy due to repeated episodes of vaso-occlusion and infarction, leading to autosplenectomy in adult life. However, this may not always be the case as some studies have reported splenomegaly persisting into adult life. This study aims to determine and review the prevalence of autosplenectomy by abdominal ultrasonography in sickle cell anemic patients in Zaria, Nigeria. METHODS: An ex-post-facto cross study of 74 subjects was carried out between May to July in 2010. Hematological parameters were determined by an analyzer while B mode Ultrasonography was used to determine the craniocaudal length of the spleen, if visualized. RESULTS: The mean age of the sickle cell subjects was 23.2 ±5.3 years, while that of the controls was 22.7±12.4 years. Of the 74 sickle cell subjects, 55.4% were females; while of the 20 controls, 50% were females. Forty one subjects (55.4%) had autosplenectomy and a significant difference existed in the mean splenic size compared with the control (p<0.0001). Only 3 (4.05%) subjects had splenomegaly, while 23 (31%) had a shrunken spleen. CONCLUSION: Anatomical autosplenectomy is not an uncommon finding in SCA patients. This may be related to inadequate clinical care due to the lack of good health education, ignorance, poverty, and poor standard of care, as well as the lack of newer therapeutic agents.

Neutropenia and human immunodeficiency virus-1 infection: analysis of 43 cases.

BACKGROUND: Neutropenia has been reported in patients with early asymptomatic HIV infection as well as in those with more advance HIV-related immunodeficiency. As with other peripheral cytopenias in the setting of HIV infection, multiple aetiologies may be present either singly or in combination. The study aims to determine the prevalence of Neutropenia and the association with the level of deterioration of CD4+ T lymphocyte subset in antiretroviral naïve HIV-1 infected patients. METHOD: Four hundred consecutive HIV-1 infected patients undergoing investigations for pre treatment and staging were recruited over a one year period, at the HIV subspecialty clinic of Ahmadu Bello University Teaching Hospital, Zaria. All the patients were confirmed HIV-1 infected, repeatedly reactive by ELIZA. White cell count was determined by standard manual method and CD4+ T cell enumeration by Dynal (Oslo Norway) manual method. RESULTS: Of the 400 patients studied, the prevalence of neutropenia was 17.5%; 4.5% and 13% amongst the asymptomatic and symptomatic group respectively. There is a signification positive correlation between absolute neutrophil count and CD4 + T lymphocyte count p<0.05. CONCLUSION: This study demonstrates that Neutropenia is relatively common in HIV infected patients and is associated with the severity of the immunological deterioration.

Pure red cell Aplasia in Zaria. A 10- year review.

Pure red cell Aplasia (PRCA) is a transfusion dependent condition characterised by anaemia, reticulocytopaenia and a paucity of erythroid precursors in the bone marrow. Records of patients with PRCA seen in Ahmadu Bello University Teaching Hospitals between 1991 and 2001 were reviewed and the outcome of their management was assessed. Fifty per cent of the ten patients had high dose methyl Prednisolone (HDMP) therapy. Seven patients recovered, 2 died and one was lost to follow up. All patients had red cell concentrate transfusion.