RESUMEN
The clinical severity of sickle cell disease (SCD) is strongly influenced by the level of fetal haemoglobin (HbF) persistent in each patient. Three major HbF loci (BCL11A, HBS1L-MYB, and Xmn1-HBG2) have been reported, but a considerable hidden heritability remains. We conducted a genome-wide association study for HbF levels in 1006 Nigerian patients with SCD (HbSS/HbSß0), followed by a replication and meta-analysis exercise in four independent SCD cohorts (3,582 patients). To dissect association signals at the major loci, we performed stepwise conditional and haplotype association analyses and included public functional annotation datasets. Association signals were detected for BCL11A (lead SNP rs6706648, ß = -0.39, P = 4.96 × 10-34) and HBS1L-MYB (lead SNP rs61028892, ß = 0.73, P = 1.18 × 10-9), whereas the variant allele for Xmn1-HBG2 was found to be very rare. In addition, we detected three putative new trait-associated regions. Genetically, dissecting the two major loci BCL11A and HBS1L-MYB, we defined trait-increasing haplotypes (P < 0.0001) containing so far unidentified causal variants. At BCL11A, in addition to a haplotype harbouring the putative functional variant rs1427407-'T', we identified a second haplotype, tagged by the rs7565301-'A' allele, where a yet-to-be-discovered causal DNA variant may reside. Similarly, at HBS1L-MYB, one HbF-increasing haplotype contains the likely functional small indel rs66650371, and a second tagged by rs61028892-'C' is likely to harbour a presently unknown functional allele. Together, variants at BCL11A and HBS1L-MYB SNPs explained 24.1% of the trait variance. Our findings provide a path for further investigation of the causes of variable fetal haemoglobin persistence in sickle cell disease.
Asunto(s)
Anemia de Células Falciformes , Proteínas de Unión al GTP , Estudio de Asociación del Genoma Completo , Haplotipos , Femenino , Humanos , Masculino , Alelos , Anemia de Células Falciformes/genética , Anemia de Células Falciformes/sangre , Predisposición Genética a la Enfermedad , Nigeria , Proteínas Nucleares/genética , Polimorfismo de Nucleótido Simple/genética , Proteínas Represoras/genéticaRESUMEN
BACKGROUND: Anaemia during pregnancy causes adverse outcomes to the woman and the foetus, including anaemic heart failure, prematurity, and intrauterine growth restriction. Iron deficiency anaemia (IDA) is the leading cause of anaemia and oral iron supplementation during pregnancy is widely recommended. However, little focus is directed to dietary intake. This study estimates the contribution of IDA among pregnant women and examines its risk factors (including dietary) in those with moderate or severe IDA in Lagos and Kano states, Nigeria. METHODS: In this cross-sectional study, 11,582 women were screened for anaemia at 20-32 weeks gestation. The 872 who had moderate or severe anaemia (haemoglobin concentration < 10 g/dL) were included in this study. Iron deficiency was defined as serum ferritin level < 30 ng/mL. We described the sociodemographic and obstetric characteristics of the sample and their self-report of consumption of common food items. We conducted bivariate and multivariable logistic regression analysis to identify risk factors associated with IDA. RESULTS: Iron deficiency was observed among 41% (95%CI: 38 - 45) of women with moderate or severe anaemia and the prevalence increased with gestational age. The odds for IDA reduces from aOR: 0.36 (95%CI: 0.13 - 0.98) among pregnant women who consume green leafy vegetables every 2-3 weeks, to 0.26 (95%CI: 0.09 - 0.73) among daily consumers, compared to those who do not eat it. Daily consumption of edible kaolin clay was associated with increased odds of having IDA compared to non-consumption, aOR 9.13 (95%CI: 3.27 - 25.48). Consumption of soybeans three to four times a week was associated with higher odds of IDA compared to non-consumption, aOR: 1.78 (95%CI: 1.12 - 2.82). CONCLUSION: About 4 in 10 women with moderate or severe anaemia during pregnancy had IDA. Our study provides evidence for the protective effect of green leafy vegetables against IDA while self-reported consumption of edible kaolin clay and soybeans appeared to increase the odds of having IDA during pregnancy. Health education on diet during pregnancy needs to be strengthened since this could potentially increase awareness and change behaviours that could reduce IDA among pregnant women with moderate or severe anaemia in Nigeria and other countries.
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Anemia , Deficiencias de Hierro , Embarazo , Femenino , Humanos , Estudios Transversales , Nigeria/epidemiología , Mujeres Embarazadas , Prevalencia , Arcilla , Caolín , Hierro , Anemia/epidemiología , Factores de RiesgoRESUMEN
BACKGROUND: Stroke is a devastating complication of sickle cell anemia (SCA) and can be predicted through abnormally high cerebral blood flow velocity using transcranial Doppler Ultrasonography (TCD). The evidence on the role of alpha-thalassemia and glucose-6-phosphate dehydrogenase (G6PD) deficiency in the development of stroke in children with SCA is conflicting. Thus, this study investigated the association of alpha-thalassemia and G6PD(A- ) variant with abnormal TCD velocities among Nigerian children with SCA. METHODS: One hundred and forty-one children with SCA were recruited: 72 children presented with normal TCD (defined as the time-averaged mean of the maximum velocity: < 170 cm/s) and 69 children with abnormal TCD (TAMMV ≥ 200 cm/s). Alpha-thalassemia (the α-3.7 globin gene deletion) was determined by multiplex gap-PCR, while G6PD polymorphisms (202G > A and 376A > G) were genotyped using restriction fragment length polymorphism-polymerase chain reaction. RESULTS: The frequency of α-thalassemia trait in the children with normal TCD was higher than those with abnormal TCD: 38/72 (52.8%) [α-/ α α: 41.7%, α -/ α -: 11.1%] versus 21/69 (30.4%) [α-/ α α: 27.5%, α -/ α -: 2.9%], and the odds of abnormal TCD were reduced in the presence of the α-thalassemia trait [Odds Ratio: 0.39, 95% confidence interval: 0.20-0.78, p = 0.007]. However, the frequencies of G6PDA- variant in children with abnormal and normal TCD were similar (11.6% vs. 15.3%, p = 0.522). CONCLUSION: Our study reveals the protective role of α-thalassemia against the risk of abnormal TCD in Nigerian children with SCA.
Asunto(s)
Anemia de Células Falciformes/fisiopatología , Deficiencia de Glucosafosfato Deshidrogenasa/complicaciones , Accidente Cerebrovascular/patología , Talasemia alfa/complicaciones , Adolescente , Velocidad del Flujo Sanguíneo , Estudios de Casos y Controles , Circulación Cerebrovascular , Niño , Preescolar , Femenino , Estudios de Seguimiento , Deficiencia de Glucosafosfato Deshidrogenasa/diagnóstico por imagen , Deficiencia de Glucosafosfato Deshidrogenasa/patología , Humanos , Masculino , Nigeria/epidemiología , Pronóstico , Accidente Cerebrovascular/diagnóstico por imagen , Accidente Cerebrovascular/epidemiología , Accidente Cerebrovascular/etiología , Ultrasonografía Doppler Transcraneal , Talasemia alfa/diagnóstico por imagen , Talasemia alfa/patologíaRESUMEN
BACKGROUND/OBJECTIVE: Sickle cell disease (SCD) is a monogenic disease with multiple phenotypic expressions. Previous studies describing SCD clinical phenotypes in Nigeria were localized, with limited data, hence the need to understand how SCD varies across Nigeria. METHOD: The Sickle Pan African Research Consortium (SPARCO) with a hub in Tanzania and collaborative sites in Tanzania, Ghana and Nigeria, is establishing a single patient-consented electronic database with a target of 13,000 SCD patients. In collaboration with the Sickle Cell Support Society of Nigeria, 20 hospitals, with paediatric and adult SCD clinics, are participating in patient recruitment. Demographic and clinical information, collected with uniform case report forms, were entered into Excel spreadsheets and uploaded into Research Electronic Data Capture software by trained data clerks and frequency tables generated. RESULT: Data were available on 3622 patients enrolled in the database, comprising 1889 (52.9%) females and 1434 (39.6%) children ≤15 years. The frequencies of Hb SS, Hb SC and Hb Sß thalassemia in this data set were 97.5%, 2.5% and 0% respectively. Sixty percent, 23.8%, 5.9%, 4.8% and 2.5% have had bone pain crisis, dactylitis, acute chest syndrome, priapism and stroke respectively. The most frequent chronic complications were: leg ulcers (6.5%), avascular necrosis of bone (6.0%), renal (6.3%) and pulmonary hypertension (1.1%). Only 13.2% had been hospitalized while 67.5% had received blood transfusion. CONCLUSION: These data on the spectrum of clinical phenotypes of SCD are useful for planning, improving the management of SCD across Nigeria and provide a foundation for genomic research on SCD.
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Anemia de Células Falciformes/complicaciones , Síndrome Torácico Agudo/etiología , Adolescente , Adulto , Anemia/etiología , Anemia de Células Falciformes/epidemiología , Niño , Femenino , Humanos , Úlcera de la Pierna/etiología , Masculino , Nigeria/epidemiología , Dolor/etiología , Accidente Cerebrovascular/etiología , Adulto JovenRESUMEN
Hydroxyurea (HU) is a well-known Hb F-inducing agent with proven clinical and laboratory efficacy for patients with sickle cell disease. However, concerns about its long-term safety and toxicity have limited its prescription by physicians and acceptability by patients. Thus, this study aims to evaluate clinician's barriers to the use of HU in the management of patients with sickle cell disease in Nigeria. An online survey targeted physicians in pediatrics, hematology, medicine, family medicine and general medical practice managing sickle cell disease in Nigeria. The survey was in four sections: demographic, knowledge and experience with HU, and barriers to the use of HU. Ninety-one (73.0%) of 123 contacts completed the survey. Seventy-three percent and 74.0% of the respondents noted that HU reduced transfusion rates and improved overall quality of life (QOL) of patients, respectively. While the majority of the practitioners (55.6%) see between 10-50 patients per month, most (66.7%) write <5 prescriptions for HU per month. Lack of a national guideline for use of HU, especially in children (52.0%), concern for infertility (52.0%), and safety profile of HU in pregnancy and lactation (48.2%), top the factors considered by the respondents as major barriers to the use of HU. Hydroxyurea is grossly under prescribed in Nigeria, despite that the vast majority of physicians who attend patients with sickle cell disease know about its clinical efficacy. Evidence-based clinical practice guidelines could be explored as a way to standardize practices and improve confidence of practitioners to improve physicians' prescription of HU in the management of sickle cell disease.
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Anemia de Células Falciformes/tratamiento farmacológico , Anemia de Células Falciformes/epidemiología , Antidrepanocíticos/uso terapéutico , Hidroxiurea/uso terapéutico , Anemia de Células Falciformes/sangre , Anemia de Células Falciformes/diagnóstico , Antidrepanocíticos/administración & dosificación , Antidrepanocíticos/efectos adversos , Estudios Transversales , Manejo de la Enfermedad , Encuestas de Atención de la Salud , Conocimientos, Actitudes y Práctica en Salud , Humanos , Hidroxiurea/administración & dosificación , Hidroxiurea/efectos adversos , Nigeria , Guías de Práctica Clínica como Asunto , Calidad de Vida , Resultado del TratamientoRESUMEN
CONCLUSIONS: Antigens belonging to the Rh and Kell blood group systems are of major clinical significance because of their immunogenicity and the potential of their consequent antibodies to cause in vivo destruction of exogenous red blood cells (RBCs). Despite the wide-spread use of transfusion, there are sparse data on the prevalence of Rh and Kell system antigens and their ethnic variability in Nigeria. The objective of this study was to determine the prevalence of the five major Rh (D, C, c, E, e) and Kell (K) system antigens in Nigeria with the goal of understanding alloimmunization risk in transfusion recipients and improving transfusion safety through the availability of resources, such as antisera for extended RBC typing and antigen panels for alloantibody detection. A multi-ethnic cohort of 302 healthy Nigerian individuals was created to study RBC antigen prevalence. The antigen status of these individuals for Rh and K antigens was determined using commercially prepared antisera and conventional tube agglutination methods. The prevalence of the Rh antigens in the study cohort was found to be: D (92.7%), C (20.5%), c (97.7%), E (19.5%), and e (97.4%). Dce was the most common Rh phenotype (53.3%). The prevalence of K was 0 percent. For all antigens, there was no association between ethnicity and antigen prevalence. This study is the first to document the prevalence of the major Rh and K antigens in the Nigerian population, using a multi-ethnic cohort. Serologic testing demonstrates a zero prevalence of K antigen, which has never been described. C and E pose the higher risks of alloimmunization, hence showing a need for extended RBC typing and matching in at-risk blood recipients. This study demonstrates that phenotyping for major Rh and K antigens within the Nigerian population can potentially improve transfusion safety and prevent alloimmunization.
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Antígenos de Grupos Sanguíneos/análisis , Tipificación y Pruebas Cruzadas Sanguíneas , Etnicidad , Humanos , Nigeria , PrevalenciaRESUMEN
BACKGROUND: As antiretroviral therapy (ART) programs in sub-Saharan Africa mature, increasing numbers of persons with human immunodeficiency virus (HIV) infection will experience treatment failure, and require second- or third-line ART. Data on second-line failure and development of protease inhibitor (PI) resistance in sub-Saharan Africa are scarce. METHODS: HIV-1-infected adults were included if they received >180 days of PI-based second-line ART. We assessed risk factors for having a detectable viral load (VL, ≥400 cps/mL) using Cox models. If VL was ≥1000 cps/mL, genotyping was performed. RESULTS: Of 227 included participants, 14.6%, 15.2% and 11.1% had VLs ≥400 cps/mL at 12, 24, and 36 months, respectively. Risk factors for a detectable VL were as follows: exposure to nonstandard nonnucleoside reverse-transcriptase inhibitor (NNRTI)-based (hazard ratio, 7.10; 95% confidence interval, 3.40-14.83; P < .001) or PI-based (7.59; 3.02-19.07; P = .001) first-line regimen compared with zidovudine/lamivudine/NNRTI, PI resistance at switch (6.69; 2.49-17.98; P < .001), and suboptimal adherence (3.05; 1.71-5.42; P = .025). Among participants with VLs ≥1000 cps/mL, 22 of 32 (69%) harbored drug resistance mutation(s), and 7 of 32 (22%) harbored PI resistance. CONCLUSIONS: Although VL suppression rates were high, PI resistance was detected in 22% of participants with VLs ≥1000 cps/mL. To ensure long-term ART success, intensified support for adherence, VL and drug resistance testing, and third-line drugs will be necessary.
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Antirretrovirales/uso terapéutico , Farmacorresistencia Viral , Infecciones por VIH/tratamiento farmacológico , Infecciones por VIH/virología , Inhibidores de la Proteasa del VIH/farmacología , VIH-1/efectos de los fármacos , Adolescente , Adulto , África del Sur del Sahara/epidemiología , Femenino , Infecciones por VIH/epidemiología , VIH-1/aislamiento & purificación , Humanos , Masculino , Persona de Mediana Edad , Mutación , Prevalencia , Estudios Prospectivos , Adulto JovenRESUMEN
BACKGROUND: After the scale-up of antiretroviral therapy (ART) for human immunodeficiency virus (HIV) infection in Africa, increasing numbers of patients have pretreatment drug resistance. METHODS: In a large multicountry cohort of patients starting standard first-line ART in six African countries, pol genotyping was retrospectively performed if viral load (VL) ≥1000 cps/mL. Pretreatment drug resistance was defined as a decreased susceptibility to ≥1 prescribed drug. We assessed the effect of pretreatment drug resistance on all-cause mortality, new AIDS events and switch to second-line ART due to presumed treatment failure, using Cox models. RESULTS: Among 2579 participants for whom a pretreatment genotype was available, 5.5% had pretreatment drug resistance. Pretreatment drug resistance was associated with an increased risk of regimen switch (adjusted hazard ratio [aHR] 3.80; 95% confidence interval [CI], 1.49-9.68; P = .005) but was not associated with mortality (aHR 0.75, 95% CI, .24-2.35; P = .617) or new AIDS events (aHR 1.06, 95% CI, .68-1.64; P = .807). During three years of follow up, 106 (4.1%) participants switched to second-line, of whom 18 (17.0%) switched with VL < 1000 cps/mL, 7 (6.6%) with VL ≥ 1000 cps/mL and no drug resistance mutations (DRMs), 46 (43.4%) with VL ≥ 1000 cps/mL and ≥1 DRMs; no HIV RNA data was available for 32 (30.2%) participants. CONCLUSIONS: Given rising pretreatment HIV drug resistance levels in sub-Saharan Africa, these findings underscore the need for expanded access to second-line ART. VL monitoring can improve the accuracy of failure detection and efficiency of switching practices.
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Fármacos Anti-VIH/uso terapéutico , Farmacorresistencia Viral , Infecciones por VIH/tratamiento farmacológico , Infecciones por VIH/virología , VIH-1/efectos de los fármacos , Carga Viral/efectos de los fármacos , Síndrome de Inmunodeficiencia Adquirida/tratamiento farmacológico , Adolescente , Adulto , África del Sur del Sahara/epidemiología , Fármacos Anti-VIH/farmacología , Terapia Antirretroviral Altamente Activa/normas , Recuento de Linfocito CD4 , Estudios de Cohortes , Farmacorresistencia Viral/genética , Femenino , Estudios de Seguimiento , Genes pol , Genotipo , Infecciones por VIH/epidemiología , Infecciones por VIH/mortalidad , VIH-1/genética , Humanos , Masculino , Mutación , Modelos de Riesgos Proporcionales , Insuficiencia del Tratamiento , Resultado del Tratamiento , Adulto JovenRESUMEN
BACKGROUND: Sickle cell disease impacts the physical, emotional and psychological aspects of life of the affected persons, often times exposing them to disease associated stigma from the society and alters the health related quality of life (HRQoL). This study compared the HRQoL of adolescents with sickle cell disease with their healthy peers, identified socio-demographic and clinical factors impacting HRQoL, and determined the extent and effects of SCD related stigma on quality of life. PROCEDURE: We conducted a cross-sectional survey among 160 adolescents, 80 with SCD and 80 adolescents without SCD. Socio-demographic and clinical data were collected using a pre-tested questionnaire. HRQoL was investigated using the Short Form (SF-36v2) Health Survey. SCD perceived stigma was measured using an adaptation of a perceived stigma questionnaire. RESULTS: Adolescents with SCD have significantly worse HRQoL than their peers in all of the most important dimensions of HRQoL (physical functioning, physical roles limitation, emotional roles limitation, social functioning, bodily pain, vitality and general health perception) except mental health. Recent hospital admission and SCD related complication further lowered HRQoL scores. Over seventy percent of adolescents with SCD have moderate to high level of perception of stigmatisation. Hospitalisation, SCD complication, SCD stigma were inversely, and significantly associated with HRQoL. CONCLUSIONS: Adolescents living with SCD in Nigeria have lower health related quality of life compared to their healthy peers. They also experience stigma that impacts their HRQoL. Complications of SCD and hospital admissions contribute significantly to this impairment.
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Anemia de Células Falciformes/psicología , Hospitalización/estadística & datos numéricos , Calidad de Vida , Estigma Social , Adolescente , Anemia de Células Falciformes/terapia , Estudios de Casos y Controles , Estudios Transversales , Femenino , Estudios de Seguimiento , Humanos , Esperanza de Vida , Masculino , Nigeria , Percepción , Pronóstico , Estudios Prospectivos , Factores Socioeconómicos , Encuestas y CuestionariosRESUMEN
BACKGROUND: Transcranial Doppler ultrasonography (TCD) measures blood flow velocities in the large cerebral vessels and thus, detects the risk of stroke. This report describes a capacity building program which enabled the use of TCD for detecting stroke risk and also describes the pattern of non-imaging TCD examinations seen in Nigerian children with sickle cell anaemia (SCA). PROCEDURE: Ten university graduates were trained on the use of TCD in a 5-day capacity building workshop after which, the three best candidates were employed to provide a 5-day a week TCD screening service in Lagos. Data from TCD examination collected between March 2011 and September 2013 were analysed and reported. RESULT: Between March 2011 and September 2013, 2,331 children with SCA aged 2-16 years had TCD studies. TCD's findings were classified as normal (standard risk) in 70.4%, conditional in 19% and abnormal (high risk) in 9.3%. The majority of children (76.9%) in the high risk category were aged 2-8 years. TCD study was inadequate for risk categorisation in 1.3% of the patients. CONCLUSION: Effective capacity building of middle level manpower is feasible and can provide a credible TCD screening service to communities with a high demand and a shortage of trained professionals. The pattern of TCD abnormalities seen in Africa are comparable to those obtained in several previous worldwide reports.
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Anemia de Células Falciformes/complicaciones , Creación de Capacidad , Accidente Cerebrovascular/diagnóstico , Ultrasonografía Doppler Transcraneal/métodos , Adolescente , Anemia de Células Falciformes/fisiopatología , Velocidad del Flujo Sanguíneo , Circulación Cerebrovascular , Niño , Preescolar , Femenino , Estudios de Seguimiento , Humanos , Masculino , Nigeria , Pronóstico , Medición de Riesgo , Accidente Cerebrovascular/etiología , Accidente Cerebrovascular/prevención & controlRESUMEN
INTRODUCTION: Postpartum anaemia is often caused by iron deficiency with onset during the antepartum period and can be exacerbated by excessive blood loss at birth. Its prevalence is estimated as 50-80% in low-income and middle-income countries. It poses adverse consequences on the mother and negatively impacts her ability to care for her newborn. Prompt treatment of postpartum anaemia is thus important. Adherence to oral iron is reportedly low in Nigeria due to its side effects and forgetfulness by the mothers. Intravenous iron such as ferric carboxymaltose, given as a single dose, might help overcome adherence issues, but investigation in a high-quality randomised control trial in Nigeria is first required while evaluation of challenges around its implementation is also warranted. OBJECTIVE: To determine the clinical effectiveness, tolerability and safety, of using intravenous ferric carboxymaltose (intervention) vs oral ferrous sulphate (control) for treating moderate to severe iron deficiency anaemia in postpartum women and to evaluate implementation of ferric carboxymaltose in treating postpartum anaemia in Nigeria. METHODS AND ANALYSIS: This study is an open-label randomised controlled trial with a concurrent implementation study. It is a hybrid type 1 effectiveness-implementation design conducted in four states across Northern and Southern Nigeria. A total of 1400 eligible and consenting women with postpartum moderate to severe anaemia (haemoglobin concentration <100 g/L) will be randomised to intravenous ferric carboxymaltose; a single dose at 20 mg/kg to a maximum of 1000 mg infusion administered at enrolment (intervention) or oral ferrous sulphate; 200 mg (65 mg elemental iron) two times per day from enrolment until 6 weeks postpartum (control). The primary outcome, proportion of participants who are anaemic (Hb <110 g/L) at 6 weeks postpartum will be analysed by intention-to-treat. Haemoglobin concentration, full blood count, serum iron, serum ferritin, transferrin saturation and total iron binding capacity will be measured at specific intervals. Implementation outcomes such as acceptability and feasibility of using ferric carboxymaltose for postpartum anaemia treatment in Nigeria will be assessed. ETHICS AND DISSEMINATION: This study is approved by the ethics committee of the teaching hospitals, Ministry of Health of the four states as required, National Health Research Ethics Committee and the drug regulatory agency, National Agency for Food and Drug Administration and Control (NAFDAC). Findings of this research will be presented at conferences and will be published in international peer-reviewed journals and shared with stakeholders within and outside Nigeria. TRIAL REGISTRATION NUMBER: International standard randomised controlled trial number: ISRCTN51426226.
Asunto(s)
Anemia Ferropénica , Compuestos Férricos , Compuestos Ferrosos , Maltosa , Humanos , Femenino , Compuestos Ferrosos/administración & dosificación , Compuestos Ferrosos/uso terapéutico , Maltosa/análogos & derivados , Maltosa/administración & dosificación , Maltosa/uso terapéutico , Compuestos Férricos/administración & dosificación , Compuestos Férricos/uso terapéutico , Nigeria , Anemia Ferropénica/tratamiento farmacológico , Administración Oral , Administración Intravenosa , Embarazo , Periodo Posparto , Ensayos Clínicos Controlados Aleatorios como Asunto , Trastornos Puerperales/tratamiento farmacológico , Adulto , Hematínicos/administración & dosificación , Hematínicos/uso terapéuticoRESUMEN
BACKGROUND: Timely access to safe blood and blood components is still a challenge in Nigeria. This study aimed to determine blood donation practices, processing and utilization of blood components across government tertiary hospitals (THs) in Nigeria. METHODS: This was a descriptive cross-sectional study done in Nigeria in June-July 2020. Data were analysed with SPSS version 21.0. RESULTS: Data were collected from 50 THs. The majority (68%) of the THs lack facilities for blood component preparation and only 18% and 32% provide cryoprecipitate and platelet concentrate, respectively. Whole blood was most commonly requested (57.04%). All facilities tested blood for HIV, HBV and HCV, but the majority (23 [46%]) employed rapid screening tests alone and nucleic acid testing was not available in any hospitals. The manual method was the most common method of compatibility testing in 90% (45/50) and none of the THs routinely perform extended red cell typing. The average time to process routine, emergency and uncross-matched requests were a mean of 109.58±79.76 min (range 45.00-360.00), 41.62±25.23 (10.00-240.00) and 11.09±4.92 (2.00-20.00), respectively. CONCLUSION: Facilities for blood component preparation were not widely available. Concerned government authorities should provide facilities for blood component preparation.
RESUMEN
BACKGROUND: The prevalence of monoclonal gammopathy of undetermined significance (MGUS), a premalignant plasma-cell disorder has not been determined in our geographic area Nigeria. METHODS: A cross sectional survey was carried on apparently healthy Nigerians selected by multistage sampling technique from the cosmopolitan city of Lagos, Nigeria. Subjects enrolled into the study had 2-step screening for the presence, type and concentration of monoclonal band. Agarose-gel electrophoresis was performed on all serum samples, and any serum sample with a discrete band of monoclonal protein or thought to have a localized band was subjected to Immunofixation. Subjects were also evaluated for Bence jones proteinuria, haematological and biochemical parameters. RESULTS: Four hundred and ten subjects with a mean age of 45.68 ± 10.3 years, a median of 45.00 years and a range of 20 to 80 years were enrolled into the study. MGUS was identified in only one (0.24 percent) of the 410 study subject. This subject was demonstrated to have a double monoclonal gammopathy; IgGλ at 16.9 g/L and IgAκ at 8.5 g/L. None of them including the sole subject with MGUS had a monoclonal urinary light chain. CONCLUSION: Among residents of Lagos, Nigeria, MGUS was found in only 0.24% percent of apparently normal persons with a median age of 45 years. This suggests that MGUS which represents the earliest stage of monoclonal plasma/lymphoid cell proliferation is not a common finding in the relatively young population of Nigeria. Future epidemiologic studies dealing with plasma cell disorders in older people are required to carefully examine the relationship between environmental factors and prevalence of MGUS and its ultimate progression to MM.
RESUMEN
Pregnancy in women with sickle cell disease (SCD) is fraught with complications, some of which are life-threatening. Managing pregnancy in these women can be challenging, especially with poor resources, which is often the case in low-income countries. In Nigeria, for instance, up to 90% of patients pay out of pocket for medical care due to the poorly developed health insurance system, and this worsens the morbidity and mortality associated with this condition. We describe a pragmatic approach to routinely managing pregnant women with SCD in the antenatal period, showing the feasibility of effective management of these high-risk pregnancies in limited-resource settings. We also present the case of a pregnant Nigerian woman with SCD who has intrauterine growth restriction (IUGR) and acute chest syndrome (ACS), conditions that are life-threatening for the fetus and the mother, respectively, and require prompt intervention. We highlight how we successfully managed this woman in a cost-effective manner by employing relatively inexpensive tests for diagnosis and treating her effectively with oxygen, appropriate antibiotics and manual exchange blood transfusion for the ACS, and finger pulse oximeters to monitor oxygen saturation. We explore pathophysiological concepts to IUGR in women with SCD and briefly discuss the appropriate mode of delivery, including the options for pain relief in labor.
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Síndrome Torácico Agudo , Anemia de Células Falciformes , Complicaciones Hematológicas del Embarazo , Femenino , Humanos , Embarazo , Anemia de Células Falciformes/complicaciones , Anemia de Células Falciformes/terapia , Complicaciones Hematológicas del Embarazo/diagnóstico , Complicaciones Hematológicas del Embarazo/terapia , Retardo del Crecimiento Fetal/terapiaRESUMEN
BACKGROUND: Coagulation abnormality is a significant complication and cause of mortality in children with uncorrected congenital heart defects (CHD). The aim of this study was to determine the prevalence of coagulation abnormalities and the associated factors in children with uncorrected CHD. METHOD: A cross sectional study conducted to determine the prevalence of coagulation abnormalities among 70 children with uncorrected CHD aged six months to 17 years and 70 age and sex matched apparently healthy controls. Coagulation abnormalities was determined using complete blood count, prothrombin time, activated partial thromboplastin time and D-dimer assay. RESULTS: The prevalence of coagulation abnormalities among children with CHD and controls was 37.1% and 7.1% respectively. Children with Cyanotic CHD had a significantly higher prevalence of coagulation abnormalities compared to children with Acyanotic CHD (57.1% versus 17.1%). Haematocrit and oxygen saturation levels were significantly associated with coagulation abnormalities. CONCLUSION: This study affirms that coagulation abnormalities are frequent in children with uncorrected CHD. Oxygen saturation and haematocrit are risk factors of coagulation abnormalities. Routine coagulation screen is recommended especially in children with cyanotic congenital heart defects to improve their quality of life and reduce morbidity and mortality while awaiting definitive surgeries.
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Trastornos de la Coagulación Sanguínea , Cardiopatías Congénitas , Trastornos de la Coagulación Sanguínea/complicaciones , Niño , Estudios Transversales , Cianosis/complicaciones , Países en Desarrollo , Cardiopatías Congénitas/cirugía , Hospitales de Enseñanza , Humanos , Lactante , Calidad de VidaRESUMEN
BACKGROUND: Anaemia in pregnancy is highly prevalent in African countries. High-dose oral iron is the current recommended treatment for pregnancy-related iron deficiency anaemia (IDA) in Nigeria and other African countries. This oral regimen is often poorly tolerated and has several side effects. Parenteral iron preparations are now available for the treatment of IDA in pregnancy but not widely used in Africa. The IVON trial is investigating the comparative effectiveness and safety of intravenous ferric carboxymaltose versus oral ferrous sulphate standard-of-care for pregnancy-related IDA in Nigeria. We will also measure the implementation outcomes of acceptability, feasibility, fidelity, and cost-effectiveness for intravenous ferric carboxymaltose. METHODS: This is an open-label randomised controlled trial with a hybrid type 1 effectiveness-implementation design, conducted at 10 health facilities in Kano (Northern) and Lagos (Southern) states in Nigeria. A total of 1056 pregnant women at 20-32 weeks' gestational age with moderate or severe anaemia (Hb < 10g/dl) will be randomised 1:1 into two groups. The interventional treatment is one 1000-mg dose of intravenous ferric carboxymaltose at enrolment; the control treatment is thrice daily oral ferrous sulphate (195 mg elemental iron daily), from enrolment till 6 weeks postpartum. Primary outcome measures are (1) the prevalence of maternal anaemia at 36 weeks and (2) infant preterm birth (<37 weeks' gestation) and will be analysed by intention-to-treat. Maternal full blood count and iron panel will be assayed at 4 weeks post-enrolment, 36 weeks' gestation, delivery, and 6 weeks postpartum. Implementation outcomes of acceptability, feasibility, fidelity, and cost will be assessed with structured questionnaires, key informant interviews, and focus group discussions. DISCUSSION: The IVON trial could provide both effectiveness and implementation evidence to guide policy for integration and uptake of intravenous iron for treating anaemia in pregnancy in Nigeria and similar resource-limited, high-burden settings. If found effective, further studies exploring different intravenous iron doses are planned. TRIAL REGISTRATION: ISRCTN registry ISRCTN63484804 . Registered on 10 December 2020 Clinicaltrials.gov NCT04976179 . Registered on 26 July 2021 The current protocol version is version 2.1 (080/080/2021).
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Anemia Ferropénica , Anemia , Deficiencias de Hierro , Nacimiento Prematuro , Anemia/diagnóstico , Anemia/tratamiento farmacológico , Anemia Ferropénica/diagnóstico , Anemia Ferropénica/tratamiento farmacológico , Femenino , Compuestos Férricos/efectos adversos , Humanos , Recién Nacido , Hierro , Nigeria/epidemiología , Embarazo , Mujeres Embarazadas , Ensayos Clínicos Controlados Aleatorios como AsuntoRESUMEN
Understanding the interplay of genetic factors with haemoglobin expression and pathological processes in sickle cell disease is important for pharmacological and gene-therapeutic interventions. In our nascent study cohort of Nigerian patients, we found that three major disease-modifying factors, HbF levels, α-thalassaemia deletion and BCL11A genotype, had expected beneficial haematological effects. A key BCL11A variant, while improving HbF levels (5.7%-9.0%), also led to a small, but significant decrease in HbA2. We conclude that in general, interventions boosting HbF are likely to reduce HbA2 in patients' erythroid cells and that such therapeutic strategies might benefit from a parallel stimulation of HbA2 through independent mechanisms.
RESUMEN
BACKGROUND: The human immunodeficiency virus infection remains a devastating disease of public health importance. OBJECTIVES: To assess the association between oral health and quality of life and the factors affecting the oral health related quality of life among HIV positive patients in Nigeria. METHODS: This was a cross sectional study of HIV positive patients attending two HIV outpatient clinics in Nigeria. Impact of oral health on quality of life was assessed using the OHIP-14. Oral health status was assessed by the DMFT and Simplified OHI indices. Level of significance was set at p< 0.05. RESULTS: Three hundred and fifty-two patients were seen, 64.2% being females. Prevalence of impact was 8.5%; and the mean OHIP scores was 8.05±9.54. Highest impact was "painful aching" 67(19.1%) with the domain of physical pain scoring the highest mean impact of 2.32. Most patients (88.6%) were on HAART. Following logistic regression, after controlling for potential confounders, independent factors associated with poor OHRQoL were perceived need for dental treatment, HAART use, and higher DMFT (p<0.05). CONCLUSION: The domain of physical pain had the highest impact, while perceived need for dental treatment, HAART use and higher caries index were contributory to poor OHRQoL.
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Infecciones por VIH/complicaciones , Infecciones por VIH/psicología , Salud Bucal/estadística & datos numéricos , Calidad de Vida , Adulto , Anciano , Instituciones de Atención Ambulatoria , Estudios Transversales , Femenino , Infecciones por VIH/tratamiento farmacológico , Infecciones por VIH/epidemiología , Humanos , Masculino , Persona de Mediana Edad , Nigeria/epidemiología , DolorRESUMEN
BACKGROUND: HIV is a chronic inflammatory state with the production of many acute-phase-reactant proteins. Some of these proteins have procoagulant activities that predispose HIV-infected patients to thrombosis. OBJECTIVES: The aim of the study was to evaluate the effects of HIV infection on the serum levels of C4b-binding protein (C4BP) and protein S as markers of predisposition to thrombosis in HIV-infected adults. METHODS: The study population comprised of 61 HIV-infected adults on antiretroviral treatment (ART) who had achieved virological suppression, 58 HIV-infected adults not yet on ART and 59 HIV-negative healthy controls. The serum levels of free protein S, C4BP and the euglobulin clot lysis time (ECLT) were determined. RESULTS: The mean plasma-free protein S level of HIV-infected patients on ART (86.9% ± 25.8%) was significantly higher than that of treatment-naïve HIV-infected patients (75.7% ± 27.3%) (p = 0.005). Conversely, there was no statistically significant difference between the protein S levels of the HIV-infected subjects on ART (86.9% ± 25.8%) and those of the controls (94.9% ± 7.9%) (p = 0.119). The mean C4BP was significantly higher in the treatment-naïve HIV-infected subjects (36.7 ± 1.7â ng/dL) than that in those on ART (30.7 ± 2.6â ng/dL) and that in the controls (22.4 ± 2.4â ng/dL) (p < 0.0001). Protein S deficiency was more prevalent among the subjects with elevated C4BP (p = 0.023). The mean ECLT was significantly more prolonged in the treatment-naïve HIV-infected subjects (241.9 ± 34.7â s) than controls (189.5 ± 40.7â s) (p < 0.0001). CONCLUSION: HIV infection causes elevated levels of C4BP and diminishes the serum levels of free protein S. We infer that the risk of thrombosis (as measured by these biomarkers) decreases with the use of antiretroviral drugs.