Evaluation of Foetal Haemoglobin Status among Nigerian Patients with Sickle Cell Anaemia Using High Performance Liquid Chromatography.

BACKGROUND: Sickle cell anaemia (SCA, HbSS) is a genetic disorder of haemoglobin with marked variation in clinical manifestation. The aim of this study was to determine the foetal haemoglobin (HbF) status of patients with HbSS, compared with that of individuals with HbAS and HbAA control as well as to establish the relationship between HbF level and age and gender of the participants. METHODS: This was a cross-sectional study in which HbF values of known HbSS patients along with HbAS and HbAA controls were analysed using High Performance Liquid Chromatography. Socio-demographic and other information were obtained with the use of questionnaire. Data was analyzed using SPSS software, version 20.0. Ethical approval was obtained for the study. RESULTS: One hundred and two (102) participants were recruited for the study, comprising 60 patients with HbSS, 22 HbAS and 20 HbAA controls, with mean age of 11.0 years±9.6, 11.7 years ±8.8 and 12.3 years±8.1 respectively. There were 30 (61.2%) males and 30 (56.6%) females for HbSS group, 9 (18.4%) males and 13(24.5%) females for HbAS group and 10(20.4%) males and 10(18.9%) for HbAA group. Mean HbF level among HbSS participants was 8.0 ±6.1% and was significantly higher than that of HbAS (3.0 ±3.4%) and HbAA (2.2 ±4.1%) control (P<0.05). Mean HbF level was higher in children (<18 years) than adults (e"18 years) among HbSS, HbAS and HbAA participants, though not statistically significant (p >0.05). Mean HbF level was also higher among female HbSS, HbAS and HbAA groups compared to corresponding male groups, though only HbSS female group was significant (p = 0.031). CONCLUSION: Patients with HbSS have significantly higher HbF level than individuals with HbAS and HbAA. Foetal haemoglobin level tend to decrease with advancing age and higher in females. Increased HbF level may play a compensatory mechanism in sickling in HbSS, thus the use of agent that increase HbF level may improve clinical outcome.

CONTEXTE: L'anémie falciforme (SCA, HbSS) est un mal de l'hémoglobine avec une variation marquée de la manifestation. Le but de cette étude était de déterminer le fœtus le statut d'hémoglobine (HbF) des patients atteints d'HbSS, par rapport à celle des personnes ayant un contrôle de l'HbAS et de l'HbAA ainsi que établir la relation entre le taux d'HbF et l'âge et le sexe des participants. MÉTHODES: Il s'agissait d'une étude transversale dans laquelle l'HbF valeurs des patients HbSS connus ainsi que l'HbAS et l'HbAA les contrôles ont été analysés à l'aide d'un liquide haute performance Chromatographie. Informations sociodémographiques et autres ont été obtenus à l'aide d'un questionnaire. Les données ont été analysées à l'aide du logiciel SPSS, version 20.0. L'approbation éthique a été obtenue pour l'étude. RÉSULTATS: Cent deux (102) participants ont été recrutés pour l'étude, comprenant 60 patients avec HbSS, 22 HbAS et 20 contrôles HbAA, avec un âge moyen de 11,0 ans ± 9,6, 11,7 ans ± 8,8 et 12,3 ans ± 8,1 respectivement. Il y avait 30 (61,2%) hommes et 30 femmes (56,6%) pour le groupe HbSS, 9 hommes (18,4%) et 13 femmes (24,5%) pour le groupe HbAS et 10 hommes (20,4%) et 10 (18,9%) pour le groupe HbAA. Niveau moyen d'HbF parmi l'HbSS participants était de 8,0 ± 6,1% et était significativement plus élevé que celle du contrôle HbAS (3,0 ± 3,4%) et HbAA (2,2 ± 4,1%) (p <0,05). Le taux moyen d'HbF était plus élevé chez les enfants (<18 ans) que chez les adultes (e "18 ans) chez les participants HbSS, HbAS et HbAA, cependant non statistiquement significatif (p> 0,05). Le taux moyen d'HbF était également plus élevé parmi les groupes féminins HbSS, HbAS et HbAA par rapport aux groupes masculins correspondants, mais seulement au groupe féminin HbSS était significative (p = 0,031). CONCLUSION: Les patients atteints d'HbSS ont des Taux d'HbF que les individus avec HbAS et HbAA. Fœtal le taux d'hémoglobine a tendance à diminuer avec l'âge et plus élevé chez les femmes. L'augmentation du niveau d'HbF peut jouer un rôle compensatoire mécanisme de la faucille dans l'HbSS, donc l'utilisation d'un agent qui l'augmentation du taux d'HbF peut améliorer les résultats cliniques. Mots clés: hémoglobine fœtale, liquide haute performance Chromatographie, drépanocytose, traits drépanocytaires, normaux l'hémoglobine adulte.

Pattern of blood donation and characteristics of blood donors in Enugu, Southeast Nigeria.

BACKGROUND: Despite increasing need for blood donation in medical practice, little is known about pattern of blood donation and characteristics of blood donors in some African populations like Nigeria. This information is necessary in designing strategies and policies for improving blood transfusion services in Africa. AIM: The aim of this study is to determine the pattern of blood donation and characteristics of blood donors in Enugu, Southeast, Nigeria. MATERIALS AND METHODS: A cross-sectional study of 3377 blood donors at a tertiary hospital in Enugu, Nigeria from May 1, 2016 to April 30, 2017. Information sought included donors' sociodemographic characteristics; type of blood donor: Voluntary nonremunerated donor (VNRD), family replacement donor (FRD), or paid donor (PD); willingness to become VNRD in the future after counseling and education (at point of entry to blood bank) on its benefits to clinical practice; hemoglobin (Hb) level; and transfusion transmissible infections (TTIs). RESULTS: Mean age of blood donors was 28.8 ± 8.5 years, majority were male (3011, 89.2%) and students (1289, 32.8%). FRDs were the highest in number (1998, 59.2%), followed by PDs (746, 22.1%) and finally VNRDs (633, 18.7%). Of the 3377 persons that came for blood donation, 2537 (75.1%) were found eligible to donate while 840 (24.9%) were deferred on account of low Hb (602/3377, 17.8%) or positive infectious screening test (238/3377, 7.0%). The odds of a male donor being a VNRD were about one and half times that of a female donor (582/3011 [19.3%] vs. 51/366 [13.9%]; odds ratio: 1.48; 95% confidence interval: 1.09-2.02; P = 0.01). After counseling of FRDs and PDs, majority (54.3%) were willing to become VNRDs in the future. Donors' age >30 years, being of male sex, having tertiary level of education, and being employed were strongly associated with willingness to become VNRDs in the future (P < 0.001). CONCLUSION: VNRD made up <20% of the total number of donors in Enugu, Nigeria. There is a need for improvement in public enlightenment on the need for VNRDs and employment opportunities of the populace to improve voluntary blood donation.

Challenges in the management of bleeding disorders in Nigeria.

BACKGROUND: Bleeding disorders (BDs) are characterized by abnormal bleeding for which effective management requires a combination of skill, workforce, diagnostic facilities, and adequate therapeutic options. OBJECTIVES: The objectives of this study were to determine the capacity of Nigerian hematologists to handle BDs and to assess availability of required infrastructure, equipment, and treatment options. MATERIALS AND METHODS: This descriptive study was conducted during the 2016 scientific conference of the Nigerian Society for Hemetology and Blood Transfusion. A structured questionnaire was distributed to hematologists in attendance. Data were analyzed with SPSS version 21. RESULTS: A total of 55 (76.4%) hematologists from 27 centers responded. The most frequently carried out tests to assess bleeding were hemoglobin or packed cell volume (100%), full blood count (96.3%), and prothrombin time/international normalized ratio and activated partial thromboplastin time (77%). Many centers did not have a coagulometer (47.8%) or cold centrifuge (43.4%) and none had thromboelastography or rotational thromboelastometry. Fresh whole blood was the most accessible (88.5%) and up to one-third of the centers did not have access to component therapy. Only 39.1% centers had factor concentrates available. CONCLUSION: Facilities required for diagnosing and treating BD are significantly deficient in most centers in Nigeria. Funding to provide facility and training is required to improve on this inadequacy.

Rates and determinants of peripartum and puerperal anemia in Enugu, Nigeria.

BACKGROUND: Anemia in the peripartum or postpartum period could pose a significant risk for maternal morbidity and mortality during and after delivery. AIMS: To determine the rates of anemia at term and in the puerperium and describe the determinants of puerperal anemia among a cohort of women with both normal and cesarean deliveries, following uncomplicated term singleton pregnancies in Enugu, Nigeria. METHODS: A prospective longitudinal study involving women with uncomplicated singleton pregnancies who were recruited at term at two tertiary maternity centers and were followed up with the determination of hemoglobin and ferritin concentrations till 6 weeks after delivery. Data were analyzed with descriptive and inferential statistics at 95% level of confidence using the Statistical Package for Social Sciences computer software version 20.0 for Windows (IBM Corporation, Armonk, NY, USA). RESULTS: A total of 202 women were studied. The mean hemoglobin levels at term, 48 h, and 6 weeks postpartum were 11.1 ± 0.9 g/dL, 10.5 ± 0.8 g/dL, and 11.2 ± 1.0 g/dL, respectively. The proportions of women with anemia at term, 48 h, and at 6 weeks postpartum were 46.0%, 72.8%, and 47.5%, respectively. Forty-eight hours postdelivery, 17.3% had anemia with low serum ferritin compared to 7.4% by 6 weeks postdelivery. Anemia at term (adjusted odds ratio [aOR] 2.02; 95% confidence interval [CI] 1.01, 4.05), anemia at 48 h postdelivery (aOR 6.17; 95% CI 3.30, 11.6), and low ferritin at 48 h postdelivery (aOR 3.11; 95% CI 1.51, 5.09) all increased the likelihood of anemia at 6-week postpartum. CONCLUSIONS: A high proportion of low-risk pregnant women in the study centers could go through delivery with undetected anemia and this would predispose to high rates of postpartum anemia. Screening of low-risk women at term and in the immediate postdelivery periods may be necessary to improve detection of such cases.

Frequency and clinical impact of ETV6/RUNX1, AF4-MLL, and BCR/ABL fusion genes on features of acute lymphoblastic leukemia at presentation.

BACKGROUND: Variations in disease presentation and outcome of leukemia treatment has been associated with the presence of certain mutant genes. Three major translocations (ETV6-RUNX1, BCR-ABL, and AF4-MLL) in acute lymphoblastic leukemia (ALL) have been shown to affect treatment outcome. This study is aimed at assessing the relationship between these translocations and the presence of other indicators of disease severity (white cell count, hemoglobin concentration, platelet count, and hematocrit) in ALL. PATIENTS AND METHODS: Forty chemotherapy naïve patients aged between 9 months and 54 years had their marrow samples analyzed for the prevalent mutations. Their clinical and laboratory details on presentation were also obtained. RESULTS: Abnormal genes detected were BCR/ABL1 major transcript in 5 (12.5%), ETV6/RUNX1 in 2 (5.0%), MLL/AF4 none and none of the patients had more than one fusion gene. There was no relationship between the presence of these fusion genes and the clinical and laboratory features of ALL. An association exists between the fusion genes and ethnic origin of the patients (P = 0.005). There is no significant association between the abnormal fusion genes detected and some laboratory features of prognostic importance, which include total white blood cell count (P = 0.416) and FAB subtype (P = 0.576). CONCLUSION: Presence of fusion the genes BCR/ABL1, ETV6/RUNX1, and MLL/AF4 does not have any impact on the clinical and laboratory features of ALL at presentation.

Prevalence and associations of symptomatic renal papillary necrosis in sickle cell anemia patients in South-Eastern Nigeria.

AIM: To assess the prevalence and associations of symptomatic renal papillary necrosis (RPN) in sickle cell anemia patients. PATIENTS AND METHODS: The case notes of homozygous hemoglobin (Hb) S patients diagnosed with RPN were retrospectively assessed. Diagnosis was based on microscopic hematuria and positive ultrasound findings. Their steady state diastolic blood pressure, Hb, leukocyte count, platelet count, serum direct bilirubin, and aspartate transaminase, were obtained by automated analyzers. These were evaluated for any relationship with the occurrence of RPN. RESULTS: Two hundred and twenty patients were assessed aged 6-55 years with a median age of 24 years. The prevalence of symptomatic RPN was found to be 2.3%. RPN was positively associated with the female gender (Chi-square P value 0.001), but not with any other clinical or laboratory variable. However, other predictors of disease severity were positively associated with RPN such as age, diastolic blood pressure 0.180 (P = 0.016), serum aspartate transaminase, serum bilirubin 0.145 (0.027), Hb, and leukocyte count - 0.155 (P = 0.003). CONCLUSION: The prevalence of symptomatic RPN is low in this group of homozygous S patients and occurs more commonly in females. Improvement in care for these patients will reduce these chronic complications.

Important clinical and laboratory correlates of glomerular filtration rate in sickle cell anemia.

BACKGROUND: Renal impairment is routinely assessed using the estimated glomerular filtration rate (eGFR) and it may be helpful to obtain certain clinical or laboratory markers, which show relationship with glomerular filtration rate (GFR) in sickle cell disease (SCD). AIM: To assess the relationship between important clinical and laboratory parameters in SCD, and the eGFR. PATIENTS AND METHODS: Steady state clinical and laboratory data were obtained from 228 homozygous SCD patients seen over a 7-year period. The GFR was estimated using (isotope dilution mass spectrometry) traceable modification of diet in renal disease (MDRD) and Cockroft-Gault methods. The correlation coefficient and independent t-test were done to assess the level of significance between the eGFR_MDRD and the known indicators of disease severity. RESULTS: The serum alkaline phosphatase (ALP) and serum direct bilirubin levels both showed significant inverse relationship with eGFR_MDRD P = 0.012 and P = 0.24, respectively. The patients' age, Hb, leukocyte count, platelet count, serum direct bilirubin and aspartate transaminase did not show a significant correlation. The eGFR_MDRD was more discriminatory revealing that 20.3% of the patients had suboptimal GFR. Proteinuria in steady state was observed in 20.3% of the patients. CONCLUSION: High serum direct bilirubin and ALP are associated with a deteriorating eGFR_MDRD. Other clinical and laboratory indicators of disease severity in SCD do not show the relationship with the GFR. MDRD_GFR estimation seemed to be a more appropriate method of estimating GFR in SCD.

Avascular necrosis in sickle cell (homozygous S) patients: predictive clinical and laboratory indices.

BACKGROUND: Pathogenetic mechanism as well as laboratory and clinical correlates of osteonecrosis in sickle cell have not been fully investigated. The aim of this study is to investigate the predictive value of the steady state white cell and platelet count as well as the frequency of bone pain crisis per annum to detect sickle cell patients who will eventually develop avascular necrosis (AVN). PATIENTS AND METHODS: A 5 year retrospective analysis of 122 homozygous S (HbSS) patients, aged 6-49 years (mean age 24.7 ± 7 years), out of which 16 patients (13.1%) had developed AVN within the years under review. RESULTS: The prevalence of AVN in sickle cell patients was determined to be 13.1 per 1000. The steady state white cell count, platelet count, frequency of bone pain crisis and hematocrit, was compared in patients that develop AVN and those who had not over the period. Only the steady state platelet count was found to differ significantly ( P = 0.011) between these two patient groups and to correlate positively (Pearson correlation coefficient = -0.251) with development of AVN. The hematocrit, white cell count, and frequency of bone pain crisis were found neither to differ significantly nor correlate with the development of AVN. CONCLUSION: In conclusion, patients with a raised steady state platelet count may have a higher tendency to develop AVN and may require closer orthopedic review and prophylactic intervention.

Multiple myeloma in Nigeria: an insight to the clinical, laboratory features, and outcomes.

AIM: In developing African nations, late presentation and occurrence of complications adversely affects survival. This study aims at identifying initial clinical and basic laboratory features of multiple myeloma (MM), which will aid the physician to entertain a high index of suspicion and therefore target his investigations in order to prevent late presentation and avert complications. MATERIALS AND METHODS: A retrospective analysis of 32 patients diagnosed and managed in Nigeria, West Africa was done. Information on the clinical, laboratory, and radiological data as well as response to treatment was obtained at presentation, 3, 6, 12, and 24 months and analyzed. RESULTS: The median age at diagnosis was 62 years, 17 (53.1%) males and 15 (46.9%) females. The median duration of follow-up was 24 weeks (range, 2-288 weeks). The average percentage of bone marrow plasmacytosis at diagnosis was 38%. Clinical features at presentation were anemia (71.9%) and bone pains (78.1%), while pathological fractures were found in 69%, and nephropathy in 13.8%. The longest duration of survival of 288 and 252 weeks were recorded in patients on melphalan and prednisolone with or without thalidomide. CONCLUSION: Presence of bone pain and anemia in elderly patients should alert the clinician to investigate along the lines of MM. Majority of patients have osteolytic lesions on X-ray and pathological fractures, and benefit from melphalan based combinations in situations where facilities for transplant are not available.

Haemogram pattern at diagnosis of malignant disorders and variations post-chemotherapy.

A total of eighty patients with various malignancies seen between September 2008 and April 2009 at the University of Nigeria Teaching Hospital, (UNTH) Ituku-Ozalla Enugu Nigeria had their haemogram values done at Days 0 and 12 of the first cycle of their various chemotherapeutic regimens. They were adult patients who had been diagnosed of various malignancies, consisting of Breast cancer 36 patients (45%), Non-Hodgkin's iymphoma 8 (10%), Hodgkin's lymphoma 13 (16.25%), Colorectal carcinoma 6 (7.5%), Multiple myeloma 7 (8.75%), Cervical carcinoma 1 (1.25%) and other malignancies 9 (11.25%). Haematological indices evaluated include: packed cell volume, haemoglobin concentration; total white blood cell count, platelet count and erythrocyte sedimentation rate. The socio demographic data of the patients were assessed from a questionnaire. There were 27 males (33.75%) and 53 females (66.25%). The age of the patients ranged from 18-80 years with a median of 45 years. Haematological parameters which were found to be significantly reduced include: haemoglobin concentration, packed cell volume and total white cell count.

Absolute neutrophil values in malignant patients on cytotoxic chemotherapy.

A total of eighty patients with various malignancies seen between September 2008 and April 2009 at the University of Nigeria Teaching Hospital (UNTH) Ituku Ozalla, Enugu, Nigeria, had their absolute neutrophil counts, done at Days 0 and 12 of the first cycle of their various chemotherapeutic regimens. They were adult patients who had been diagnosed of various malignancies, consisting of Breast cancer 36 (45%), Non-Hodgkin's lymphoma 8 (10%), Hodgkin's lymphoma 13 (16.25%), Colorectal carcinoma 6 (7.5%), Multiple myeloma 7 (8.75%), Cervical carcinoma 1 (1.25%) and other malignancies 9 (11.25%), Manual counting of absolute neutrophil count was done using Turks solution and improved Neubauer counting chamber and Galen 2000 Olympus microscope. The socio demographic data of the patients were assessed from a questionnaire. There were 27 males (33.75%) and 53 females (66.25%). Their ages ranged from 18 - 80 years with a median of 45 years. The mean absolute neutrophil count of the respondents pre-and post chemotherapy was 3.7 +/- 2.1 x 10(9)/L and 2.5 +/- 1.6 x 10(9)/L respectively. There were significant differences in both the absolute neutrophil count (p=0.00) compared to the pre-chemotherapy values. Chemotherapeutic combinations containing cyclophosphamide and Adriamycin were observed to cause significant reduction in absolute neutrophil.

Implant associated surgical site infection in orthopaedics: a regional hospital experience.

BACKGROUND: Post operative surgical site infection following implant surgery is a major problem in orthopedic surgical practice. Infection occurring after internal fixation of a fracture is a devastating complication and may be difficult to treat. The frequency of occurrence of surgical site infection has decreased with improvements in aseptic technique. The objectives of the study are to determine the incidence of surgical site infection following orthopaedic related implant surgeries and to indentify the predisposing factors. METHODS: The study was a prospective study conducted at the National orthopedic hospital, Enugu. Wound surveillance was carried out for the 97 patients included in this study for a period of 6 months postoperatively. The diagnosis of surgical site infection was in accordance with the CDC's guideline for prevention of surgical site infection published in 1999. RESULTS: The study included 61 males and 36 females giving a ratio of 1.7:1. The study population was aged 7 to 83 years with a mean age of 38.7 +/- 18.3 years. The infection rate was found to be 9.3% with staphylococcus aureus as the most common causative organism in 55.6% of cases. Two of the nine infected cases required implant removal. Significant factor was a theatre population of more than 6 persons. CONCLUSION: Surgical site infection following implant surgery is relatively common in our environment with staphylococcus aureus as the major causative organism. Increased theatre populations increase the risk of implant associated surgical site infection.