RESUMEN
INTRODUCTION/OBJECTIVES: Sickle Cell Anaemia (SCA) is a chronic haemolytic and inflammatory disorder characterized by repeated vaso-occlusive (VOC) and hyperhaemolytic crises (HC). These crises determine the quality of life of SCA patients. This study estimated the plasma L-arginine levels in SCApatients during crises (HC and VOC), and correlated these levels with the markers of inflammation in the patients. METHODS: A cross-sectional study comprising of 120 consenting adults (60 SCA patients and 60 HbAA controls). The SCA were grouped as SCA patients in VOC or SCA patients in HC and HbAA controls. All SCA patients presented at the emergency room or the daycare unit of Haematology Department, University College Hospital, Ibadan, Nigeria. Blood samples were collected and haematological parameters and plasma L-arginine levels were determined. RESULTS: There were 30 SCA patients in VOC, 30 SCA patients in HC, and 60 HbAA controls with age ranging from 18 to 58years. The controls had a higher haematocrit than the SCA patients (HbAA>SCA VOC> SCA HC, p<0.001) in each pair wise analysis. The VOC and the HC groups had higher mean White Blood Cell (WBC), platelet count, Absolute Neutrophil Count (ANC), Absolute Lymphocyte Count (ALC), Absolute Monocyte Count (AMC), and reticulocytes count than the HbAA controls. Similarly, the HC group had higher mean WBC, platelet count,ANC,ALC,AMC, and reticulocytes count, and a lower mean percentage irreversible sickle cell than the VOC group. The mean plasma L-arginine was significantly higher in the HbAA control group than those of SCA patients in crises (HbAA controls>SCAVOC>SCAHC, p < 0.001), and higher in the SCAVOC group than in the HC group. There was no significant correlation between plasma L-arginine levels, reticulocyte count, markers of VOC severity and markers of inflammations. CONCLUSION: The plasma L-arginine levels of SCA patients in VOC and HC were lower than those of the HbAA controls. The degree of reduction was however marked in patients in hyperhaemolytic crisis. There was no significant association between the plasma L-arginine levels and the markers of inflammation in the studied patients. The findings in this study buttress the need for consideration of L-arginine supplementation in patients with SCA, especially during acute hemolytic crises.
INTRODUCTION / OBJECTIFS: La SCA est un mal hémolytique et inflammatoire chronique caractérisé par des crises vaso-occlusives (COV) et hyperhémolytiques (HC) répétées, qui déterminent la qualité de vie des patients atteints de SCA. L'étude a estimé les taux plasmatiques de L-arginine chez les patients atteints de SCA pendant les crises (HC et COV), et a mis en corrélation ces taux avec les marqueurs de l'inflammation chez les patients. MÉTHODES: Une étude transversale comprenant 120 adultes consentants (60 patients SCA et 60 contrôles HbAA) regroupés en patients SCA en COV; Patients SCA à HC (tous les patients SCA présentés à la salle d'urgence ou à l'unité de garde du département d'hématologie, University College Hospital, Ibadan, Nigéria); et contrôles HbAA. Des échantillons de sang ont été prélevés et les paramètres hématologiques et les taux de L-arginine ont été déterminés. RÉSULTATS: Il y avait 30 patients SCA en COV, 30 patients SCA en HC et 60 contrôles HbAA avec un âge allant de 18 à 58 ans. Les témoins avaient un hématocrite plus élevé que les patients SCA (HbAA> SCA VOC> SCAHC, p <0,001) dans chaque analyse par paire. Les groupes VOC et HC avaient des taux moyens de globules blancs, de plaquettes, d'ANC, d'ALC, d'AMC et de réticulocytes plus élevés que les témoins HbAA. De même, le groupe HC avait un nombre moyen de globules blancs, de plaquettes, d'ANC, d'ALC, d'AMC et de réticulocytes plus élevés, et un pourcentage moyen de de drépanocytose irréversible plus faible que le groupe COV. La L-arginine plasmatique moyenne était significativement plus élevée dans le groupe témoin HbAA que chez les patients SCA en crise (contrôles HbAA> SCA VOC> SCA HC, p <0,001), et plus élevée dans le groupe SCA VOC que dans le groupe HC, p <0,001. Il n'y avait pas de corrélation significative entre les taux de L-arginine, le nombre de réticulocytes, les marqueurs de gravité des COV et les marqueurs d'inflammations. CONCLUSION: Les taux plasmatiques de L-arginine chez les patients SCAen COV et HC étaient inférieurs à ceux des contrôles HbAA, et le degré de réduction est cependant marqué chez les patients en crise hyperhémolytique. Il n'y avait pas d'association significative entre les taux plasmatiques de L-arginine et les marqueurs de l'inflammation chez les patients étudiés. Les résultats de cette étude renforcent la nécessité d'envisager une supplémentation en L-arginine chez les patients atteints de SCA, en particulier pendant les crises hémolytiques aiguës. MOTS CLÉS: anémie falciforme, L-arginine, crise vaso-oclussive, crise hyperhémolytique.
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Anemia de Células Falciformes , Arginina , Adolescente , Adulto , Anemia de Células Falciformes/complicaciones , Estudios Transversales , Humanos , Persona de Mediana Edad , Nigeria , Plasma , Calidad de Vida , Índice de Severidad de la Enfermedad , Adulto JovenRESUMEN
BACKGROUND: Haemoglobinopathies are diseases of global importance and the countries with high disease burdens have inaccurate national data on the incidence of the various haemoglobin (Hb) phenotypes. OBJECTIVE: To review the Hb phenotype distribution, the referral identity of patients, and the clinical reasons for phenotyping. METHODS: A retrospective descriptive study conducted at a private diagnostic laboratory in Lagos, South-West, Nigeria. The anonymous results of Hb phenotypes of the patients determined using gel electrophoresis were retrieved from the laboratory information system (LIS). RESULTS: There were 942 patients: 519(55.1%) males and 423(44.9%) females with median ages 30yrs (11months-89yrs) vs 27yrs (9months-89yrs), p=0.0018. The phenotypes were HbAA, 592(63.2%); HbAS(26.4%); HbSS(7.1%); HbAC(2.1%), HbSC(0.7%) and HbCC(0.4%). Of the total, 757(80.4%) were referred by health care providers and 185(19.6%) were selfreferred, and there was no association between gender and referral identity of patients. However, there were more males [353(46.6%)] with clinical reasons for referrals than females [94(12.4%)], p<0.0001, and annual check was the commonest reason for Hb phenotyping. CONCLUSION: Most patients were referred for Hb phenotyping and an annual health check is the commonest reason for phenotype requests. This emphasizes the need for policies to support national Hb phenotype/genotype screening programmes to aid the early detection of sickle cell disease.
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Anemia de Células Falciformes , Hemoglobina Falciforme , Adulto , Femenino , Hemoglobinas/análisis , Humanos , Masculino , Nigeria/epidemiología , Fenotipo , Estudios RetrospectivosRESUMEN
BACKGROUND AND OBJECTIVE: Inflammation is implicated in the pathogenesis of most complications seen in sickle cell anemia (SCA) patients. We aimed to evaluate serum levels of two newly discovered anti-inflammatory cytokines (IL-27 and IL-37), and pro-inflammatory cytokines among Brazilian SCA patients that are not on hydroxyurea therapy (HbSS), compared with hydroxyurea-treated patients (HbSSHU) and healthy controls (HbAA). Furthermore, we demonstrated the effect of IL-27, IL-37, and heme on in vitro secretions of IL-8 in human neutrophils and monocytes. METHODS: A cross-sectional study of 82 consenting SCA (35 HbSS and 47 HbSSHU) patients in steady state and 49 HbAA consenting individuals. Clinical details were obtained from interviews and medical records. Serum levels of IL-27, IL-37, TGF-ß, TNF-α, IL-1ß, IL-6, and IL-8 were quantified by enzyme linked immunosorbent assay (ELISA). Neutrophils and monocytes were isolated from healthy controls, and cultured separately with or without cytokines (IL-27 and IL-37) and heme. Supernatant IL-8 concentration was determined by ELISA. RESULTS: Serum levels of IL-27, IL-37, IL-1ß, IL-6, and IL-8 were significantly elevated in HbSS patients compared to HbAA controls. Serum IL-8 levels were significantly higher in HbSS and HbSSHU patients than in controls. IL-27 and IL-37 were positively correlated in both HbSS and HbSSHU patients. In vitro IL-8 production by IL-27 and IL-37 pre-treated neutrophils and monocytes was significantly inhibited even after heme addition. CONCLUSIONS: Our findings show that IL-27 and IL-37, as well as the pro-inflammatory cytokines, are elevated in HbSS patients compared with controls, suggesting that the secretion of these anti-inflammatory cytokines is driven by the presence of pro-inflammatory cytokines. This role is probably sufficient in preventing further cellular or tissue damage but not potent enough to prevent inflammation. Therefore, IL-27 and IL-37 may be potential immuno-targets for ameliorating complications associated with elevated heme levels seen in SCA and other hemolytic anemias.
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Anemia de Células Falciformes/metabolismo , Interleucina-1/metabolismo , Interleucina-8/metabolismo , Interleucinas/metabolismo , Monocitos/metabolismo , Neutrófilos/metabolismo , Adulto , Células Cultivadas , Estudios Transversales , Citocinas/metabolismo , Femenino , Humanos , Inflamación/metabolismo , Masculino , Adulto JovenRESUMEN
Introduction: Hypereosinophilic syndrome (HES) is a rare disorder. It is defined as eosinophilia of greater than1.5x109 /L persisting for at least 6 months or death before 6 months without an identifiable cause and with eosinophil-mediated organ dysfunction. We present a rare case of hypereosinophilic syndrome with severe hypokalaemia in a Nigerian female patient. Case Presentation: A 43year old food vendor referred to the Haematology Department, University College Hospital, Ibadan on account of a 6-week history of cough productive of mucoid, brownish, foul smelling sputum with associated breathlessness, high grade intermittent fever, and intense pruritus. She had accompanying non-projectile,non-bloody vomiting of recently ingested meals. There was absolute eosinophilia of 83x109/L and bone marrow cytology revealed marked eosinophilia with blasts of less than 5%. She also had asymptomatic severe hypokalaemia (1.9mmol/l) likely due to vomiting and reduced dietary intake. The aetiology of the hypereosinophilia could not be ascertained.She was admitted and commenced on intranasal oxygen, Tabs Loratidine, intravenous hydration.The severe hypokalaemia was corrected with IV KCL over 48hours followed with the administration of slow K tablets 600mg tds. She also had tabs Hydroxyurea for cytoreduction and Allopurinol to prevent hyperuricaemia. She improved with the above line of management. Conclusion: This appears to be the first reported case of HES with asymptomatic severe hypokalaemia in the literature. Being a rare disorder it could easily have been missed without a review of the peripheral blood film and marrow aspirate. This finding suggests a possible relationship between hypereosinophilia and hypokalemia which needs to be explored.
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Síndrome Hipereosinofílico/diagnóstico , Hipopotasemia/diagnóstico , Neumonía/diagnóstico , Adulto , Alopurinol/uso terapéutico , Antibacterianos/uso terapéutico , Antipruriginosos/uso terapéutico , Enfermedades Asintomáticas , Tos/etiología , Disnea/etiología , Disnea/terapia , Inhibidores Enzimáticos/uso terapéutico , Femenino , Fluidoterapia , Humanos , Hidroxiurea/uso terapéutico , Síndrome Hipereosinofílico/complicaciones , Síndrome Hipereosinofílico/terapia , Hiperuricemia/prevención & control , Hipopotasemia/complicaciones , Hipopotasemia/terapia , Loratadina/uso terapéutico , Nigeria , Terapia por Inhalación de Oxígeno , Neumonía/complicaciones , Neumonía/tratamiento farmacológico , Cloruro de Potasio/uso terapéutico , Índice de Severidad de la Enfermedad , Vómitos/etiologíaRESUMEN
BACKGROUND: Sickle cell disease (SCD) is a major public health problem in Nigeria. Carrier prevalence is about 25% and it affects about 2 - 3% of the Nigerian population. The disease runs a chronic course, characterized by recurrent ill-health, progressive organ damage and shortened life-span. There is a need for SCD control through public education and other preventive measures. OBJECTIVE: This study aimed at assessing the level of knowledge regarding SCD among a cross-section of new tertiary graduates in Nigeria, as well as factors influencing their knowledge. It also describes their attitudes and patterns of control practices engaged by the respondents regarding SCD. METHOD: This was an analytic, cross-sectional study among 370 new tertiary graduates (youth corps members) in Benin City, Nigeria. Bio-data, data on knowledge, their attitude and control practices of sickle cell disease were obtained using a structured questionnaire. Association between the mean level of knowledge and other variables such as age, gender, course of study, etc were tested using one way analysis of variance. RESULTS: Most of the study participants were aged 22 - 29 years. A large proportion (63.5%) of the respondents was females. Only 17.8% of the respondents had a good knowledge of SCD despite high level of awareness (98.4%). Those who studied courses related to medical sciences had significantly higher mean knowledge score. About 94.6% of the respondents knew their SCD carrier status and 80.8% were willing to avoid carrier marriages. Only 38.1% will accept prenatal diagnosis/selective abortion if locally available. CONCLUSION: Most participants demonstrated moderate level of public health knowledge regarding SCD in Nigeria. Considering the relative lack of prenatal diagnostic services, low acceptability of selective abortion among the respondents, sub-optimal care of affected persons and poor access to haemopoeitic stem cell transplantation (HSCT) in our environment, stronger efforts should be directed and sustained at primary prevention through public education and screening regarding SCD.