Study of Safety and Effect of T-AYU-HM Premium in Sickle Cell Anemia Patient: A Case Study

Sickle cell anaemia is a type of haemoglobin disorder causing morbidity and mortality in many countries. The disease is incurable and therefore requires symptomatic management to improve quality of life. Because the alternative system of medicine can play a significant role in the management of quality of life in sickle cell anaemia, lot many combinations and formulations are attempted across many countries. Unfortunately, very few have reached a preclinical and clinical research level. In the current case study, T-AYU-HM Premium was evaluated as per the standard parameters, and a clinical evaluation considering its effect and safety was performed in this case report of a 24-year-old male with a history of sickle cell disease in hereditary. History was 8 times blood transfusion and 7 times hospitalization in past two year. He was infected with covid-19 and hospitalized, recovered with T-AYU-HM Premium only. Next month because of joint pain, fever, and weakness he visited the daycare clinic. On complete physical and laboratory examination he was started on T-AYU-HM Premium 300mg two tablets twice a day. During 6 months of treatment, he had complained of pain only thrice for which analgesics were prescribed, and no blood transfusion was required. During this 6 month period, there is a remarkable improvement in his haemoglobin, red blood corpuscles, white blood cells, and platelets. There were no untoward complaints from him suggesting that T-AYU-HM Premium exhibited its potential in sustaining the cellular integrity and thereby preventing the lysis of red blood corpuscles. The improvement in laboratory parameters, clinical parameters and established studies indicated that T-AYU-HM Premium is safe and exhibit an observational effect on red blood corpuscles of sickle cell anaemia patient.

Hemoglobin S identification in blood donors: A cross section of prevalence

ABSTRACT Introduction: In Brazil, the sickle cell trait (SCT) has an average prevalence of 4% in the general population and 6-10% among Afro-descendants. Although SCT is highly prevalent, a large segment of the population ignores their status. The Therapeutic Guidelines prohibit the transfusion of SCT red blood cells into patients with hemoglobin disorders or severe acidosis and newborns. Methods: This was a cross-sectional study with data from 37,310 blood donation candidates. The study included only eligible first-time donors qualified to be tested for the presence of hemoglobin S (HbS) at the Fundação Hemominas Juiz de Fora, Brazil. The variables studied were gender, skin color, age, type of donation, place of birth, blood type, result of the solubility test for hemoglobin S (HbST) and hemoglobin electrophoresis (HbEF). Statistical analysis was performed using the Q square test and the Kappa index of agreement for comparing biochemical methods. This project was approved by the National Research Ethics Committee. Results: The analysis of first-time donor data showed that 7166 were considered eligible. A total of 127 of the 7166 donors were carriers of SCT (1.77%). Among the blood donors, 73.23% were from the local area. The HbST and HbEF were found to be 100% in concordance. Sensitivity was not tested in the present study. Conclusions: The HbST is highly specific for identifying the HbS, but sensitivity was not tested in this study. The screening of blood donors for abnormal hemoglobins is useful, helping to detect and counsel heterozygous people. The study seeks to identify the prevalence of SCT in a region of Brazil.

Burden of sickle cell disease in tribal students in Maa-Baadi institutions in southern Rajasthan - A pilot study

Background & objectives: Sickle cell disease (SCD), an inherited disorder of erythrocytes, is highly prevalent in the tribal population of India. The tribal population of India is approximately 100 million and it is necessary to identify the magnitude of this problem. Furthermore, the prevalence of the disease is unknown among the five million tribal people of southern provinces of Rajasthan. In this study, we intended to determine the prevalence and characteristics of sickle cell disorder among the tribal inhabitants of southern Rajasthan. Methods: This cross-sectional study was conducted among the tribal students of the Maa-Baadis and hostels situated in the five tribal sub-plan districts of Rajasthan. Maa-Baadi centres are located in every village, whereas for every four to five villages, one hostel is allocated to accommodate the tribal students. The screening for SCD was done by solubility test and electrophoresis was used for confirmation. Results: A total of 36,752 tribal students were screened from 1,006 Maa-Baadi centres and 243 hostels. The prevalence of SCD among the tribal students was 5.8 per cent. The prevalence of heterozygous and homozygous conditions was 5.61 and 0.17 per cent, respectively. Among the five sub-plan districts, the highest prevalence was observed in Sirohi district (10.5%) followed by Banswara (7.42%), Udaipur (6.53%), Pratapgarh (5.51%) and Dungarpur (1.89%). Among the four major tribes belonging to these districts, the highest prevalence was recorded in Garasia tribes (13.81%). The history of leg ulcers and the mean pulse rate were significantly high in SCD individuals. Interpretation & conclusions: SCD is a significant problem among the tribes of southern Rajasthan, with the highest prevalence among the Garasia tribe. The present study recommends that a structured screening programme targeting the entire tribal population with appropriate counselling as well as providing treatment through the existing health system is the need of the hour.

Impact of hydroxyurea on hospital stay & analgesic utilization in sickle cell anaemia with vaso-occlusive crises

Background & objectives: Hydroxyurea (HU) has been useful in preventing sickle cell vaso-occlusive crises (VOC). A few studies also suggest utility of HU, during acute VOC. Sickle cell anaemia (SCA) is of high prevalence in western districts of Odisha State, India, and VOC is a common presentation, despite being mostly of Arab-Indian haplotype. This study was undertaken to evaluate the impact of HU on hospital stay and analgesic utilization in acute painful VOC of SCA. Methods: Homozygous sickle cell disease (HbSS) patients were categorized as cases who were receiving low-dose HU (10 mg/kg/day) and patients who were not on HU were considered as control. Days of hospital stay, analgesic utilization and visual analogue scale (VAS) score in patients were compared with that of control. Analgesics used to control pain were tramadol hydrochloride, ketorolac and diclofenac. Results: A total of 359 homozygous sickle cell disease (SCD) patients with VOC were studied (187 patients and 172 controls). The patient group had lesser mean days of hospital stay (1.4 days less than controls, P<0.001) and required lesser days of analgesic utilization than controls (1.18 days less than controls, P<0.001). Significant differences were observed between patients and controls concerning VAS score and amount of tramadol hydrochloride, ketorolac and diclofenac utilization (P<0.05). Interpretation & conclusions: In this study, HU was found to have beneficial effects in acute VOC of homozygous SCD, which includes shortening the duration of hospital stay and reducing the net amount of analgesic utilization during hospitalization

Folate levels in children with sickle cell anemia on folic acid supplementation in steady state and crises at a tertiary hospital in Enugu, Nigeria a prospective, comparative study

Folic acid supplementation is an integral aspect of the management of children with sickle cell anaemia (SCA) especially in Africa. In spite of this, there have been concerns about lower folate levels, especially during crisis. AimTo determine red cell folate levels of children with sickle cell anaemia in steady state and during crisis and compare with those with haemoglobin AA genotype. Method This study was prospective, hospital based, and comparative. Fifty children with sickle cell anaemia were recruited during crises and followed up until they met the criteria for attaining steady state. The controls were fifty children matched with those with SCA for age and gender and had haemoglobin AA genotype. Red cell folate estimation was done with the Electrochemiluminescence Immunoassay (ECLIA) method using the automated Roche Cobas e411 equipment. Results The median (IQR) red cell folate level in children during sickle cell crisis was 265.95 (134.50) ng/ml, which was significantly lower than the median (IQR) of 376.30 (206.85) ng/ml obtained during steady state. Most children with SCA (41 out of 50) had significantly higher folate levels during steady state (T=1081, Z-score= -4.660, p < 0.001). Median level of red cell folate was lower during anaemic crisis compared to vaso-occlusive crisis, though not significantly so (N(50), U = 214.00, Z-score= -1.077, p = 0.305). The median red cell folate level of normal controls was 343.55 (92.90) ng/ml, which was significantly lower than the 376.30 (206.85) ng/ml obtained during steady state (N(50), U= 209.00, Z-score= -7.177, p <0.001). Conclusion Median red cell folate levels of the study participants were within normal limits, though most children with SCA had significantly higher levels during steady state compared to crisis. Normal controls had significantly lower red cell folate levels than the children with SCA during steady state

Nuevos aspectos moleculares y fisiopatológicos de la anemia drepanocítica / New molecular and pathophysiological aspects of sickle cell anemia

Introducción: La enfermedad por hemoglobina S es una anemia hemolítica crónica hereditaria cuyas manifestaciones clínicas provienen de la tendencia de esta hemoglobina de polimerizar y deformar los eritrocitos dándoles la típica forma de media luna, platanito, drepanocitos o sickle cell; de aquí el nombre de anemia drepanocítica o sicklemia. Objetivo: Describir los nuevos aspectos moleculares, fisiopatológicos y el diagnóstico de la anemia drepanocítica. Métodos: Se realizó una revisión de la literatura, en inglés y español, a través del sitio web PubMed y el motor de búsqueda Google académico de artículos publicados en los últimos 10 años. Se hizo un análisis y resumen de la bibliografía revisada. Conclusiones: La comprensión de la complejidad y multiplicidad de eventos que conducen a complicaciones graves en la anemia drepanocítica y nuestra incapacidad para predecir el curso clínico en cada caso particular ayudaría en la prevención de estos eventos(AU)

Introduction: Hemoglobin S disease is a hereditary chronic hemolytic anemia whose clinical manifestations come from the tendency of this hemoglobin to polymerize and deform erythrocytes, giving the typical crescent, banana, sickle cell or sickle cell shape; hence the name sickle cell anemia or sicklemia. Objective: To describe the new molecular and pathophysiological aspects and the diagnosis of sickle cell anemia. Methods: A literature review was carried out, in English and Spanish, through PubMed website and Google academic search engine for articles published in the last 10 years. An analysis and summary of the revised bibliography was made. Conclusions: Understanding the complexity and multiplicity of events that lead to serious complications in sickle cell anemia and our inability to predict the clinical course in each particular case would help preventing these events(AU)

Homozygous HbS in A Malay Patient: A Rare Variant of Sickle Cell Disease in Malaysia

@#Sickle cell disease in Malay ethnicity is uncommon, with few cases been reported only in Malaysian Indians. Detecting sickle haemoglobin in patients with osteoarticular manifestation is not as simple as those with haemolysis crisis, due to its extremely low incidence in this country. We hereby report a case of a 19-year-old Malay female who presented with a long-standing history of disabling movement of both hip joints, intermittent painful swollen right elbow, and chronic back pain. Imaging investigations revealed features of chronic osteomyelitis and avascular necrosis while blood investigations demonstrated features of mild normochromic normocytic anaemia and extravascular haemolysis. Further blood smear and haemoglobin analysis eventually confirmed the presence of homozygous sickle haemoglobin manifesting as sickle cell anaemia. Our case has highlighted the importance of prompt identification and thorough evaluation of the cause of anaemia in a patient with disabling chronic osteoarticular problem.

Leg Length Discrepancies in Nigerian Children with Sickle Cell Anaemia

Background: Sickle cell anaemia, (SCA) the homozygous and most severe of the sickle cell diseases exerts adverse effects on growth and linear body proportions. In Nigeria, these changes in the lower extremity are scarcely documented. Objectives: To evaluate the differences between the real leg lengths and apparent leg lengths determined by tape measure among children with SCA. Methods:A clinic-based, cross-sectional, comparative study of 140 age and sex matched “SS” and “AA” children. The real leg lengths, anterior superior iliac spine to medial malleolus and to the heel, the apparent leg length, umbilicus to the medial malleolus were measured with a plastic tape and recorded. Leg length discrepancy (LLD), the arithmetric differences between corresponding leg lengths were recorded and classified into nil (0), mild (<2cm), moderate (2-5cm) and severe (>5cm).Results:At the medial malleolus, the subjects had more mild and moderate Real LLD than controls while more controls than subjects had nil Real LLD (p = .033). Both right and left real leg lengths were significantly shorter in the 6-9 year old subjects especially the males, all female subjects, all 10-13 year olds subjects more so the females than their respective counterparts. (All p values < 0.05) Similarly, the right and left leg lengths at the heel were significantly shorter in subjects of the same age and sex groups as above than all their respective counterparts. (P values < 0.05). However, male subjects had only the right real leg length significantly shorter than those of the controls. Real LLD: ASIS-MM was significantly higher in 10-13 year old female subjects, real LLD: ASIS-HEEL in14-18 year old subjects than the controls. The right and left apparent leg length were significantly lower in all 10-13 year old subjects as well as 10-13 year old female subjects than the respective matches. All p values were < 0.05. No significant difference existed in the apparent LLD comparisons

Study of pulmonary hypertension as a complication of sickle cell disease

Background: Sickle cell anaemia is the most common inherited hematological abnormality across human race, particularly prevalent in pockets of central India and pulmonary hypertension (PH) supposedly worsens prognosis. Data from central India is lacking though. Present study aims to study incidence of pulmonary hypertension as a complication in patients of sickle cell disease.Methods: Patients aged more than 12 years diagnosed to have sickle cell anaemia on hemoglobin electrophoresis during the study period (total- 94; 54 SS and 40 AS) were enrolled as cases. Sixty four age/sex matched healthy close relatives of the cases with 慉A� Hb electrophoresis pattern doubled up as controls. Participants were thoroughly assessed with complete history, examination, haematological and biochemical tests, X-ray chest, ECG, pulmonary function tests and 2D-echocardiography and Doppler studies and data analysed.Results: Pulmonary hypertension was observed in a total of five SS cases (5/54, 9.26%). No participant from the AS group or the control group developed pulmonary hypertension. It was more common in females (3/23, 13%) than males (2/26, 7.7%). The mean age of the SS cases with PH (32�15 years) was found to be significantly higher than that of the SS cases without PH (24.2�21 years) (p<0.01).Conclusions: The incidence of PH in sickle cell disease was relatively lower as compared to western countries and was found to affect females and older individuals more. Larger community-based studies are recommended for corroboration.

Glutathione and Bilirubin Concentrations as Markers of Oxidative Stress Measured among Sickle Cell Anaemia Subjects Attending University of Calabar Teaching Hospital, Calabar Nigeria

Aims: This study aimed at examining the oxidative stress level of sickle cell anaemia subjects using glutathione and bilirubin levels as markers as well as the red cell parameters. Study Design: Case-control study. Place and Duration of Study: University of Calabar Teaching Hospital, Calabar-Nigeria, between August 2018 and July 2019. Methodology: Subjects comprised 45 SCA patients (27 females, 18 males; age range 10-45 years) attending clinic at University of Calabar Teaching Hospital Calabar, Nigeria and equal number of age and sex-matched control subjects with Hb AA. Blood samples were collected and analyzed by standard methods. The red cell parameters were analysed by automation using FY-Smart-1 auto haematology analyzer. Bilirubin assay was performed using the colorimetric method, while glutathione was performed by enzyme-linked immunosorbent assay technique. Results: The red blood cell count, haemoglobin concentration, and haematocrit values of SCA subjects were significantly lower (p=0.001) compared to values from control subjects, while the red cell indices and red cell distribution width values were increased in SCA subjects (p=0.001). The Total bilirubin, conjugated bilirubin and unconjugated bilirubin were significantly increased (p=0.001) among SCA subjects, while the glutathione concentration values were reduced (p=0.001) when compared to values obtained from control subjects. Conclusion: SCA subjects have marked red cell size variation, lymphocytosis and thrombocytosis. The haemolytic events that occur in sickle cell anaemia results in glutathione depletion.

L- Arginine and Nitric Oxide Levels among Children with Sickle Cell Disease in a Steady State in Federal Teaching Hospital Gombe, Northeastern Nigeria

Sickle cell disease is a global public health problem. L-arginine is an amino acid that helps in improving blood in the arteries of the heart and improved symptoms of clogged arteries, chest pain or angina and coronary arteries disease. Nitric oxide is a powerful neurotransmitter that helps blood vessels relax and improve circulation. The l-arginine and nitric oxide levels of sickle cell disease (SCD) subjects with steady stages were also significantly low. The objective of this study was to evaluate L-arginine and Nitric oxide levels in children with sickle cell disease at steady state for 8 weeks. This study included children with a confirmed HbSS electrophoretic pattern aged 1-14 years presented to the sickle cell clinic unit of Federal Teaching Hospital Gombe. The L-arginine and nitric oxide levels were significantly higher post supplementation compared to baseline levels (p = 0.002 and 0.000 respectively). It is recommended that L-arginine supplementation be included in the management of patients with sickle cell disease. L-arginine supplement should be made available in the paediatric emergency unit, clinic and pharmacy department as given to patients with sickle cell disease to prevent the adverse effects during the crisis and potentially reduce the length of stay in the hospital.

Crescimento e puberdade em uma coorte prospectiva de pacientes com anemia falciforme: avaliação em dez anos / Growth and puberty in a prospective cohort of patients with sickle-cell anaemia: an assessment over ten years

INTRODUCTION: Hereditary haemoglobinopathies are the most common group of monogenic hereditary diseases in the world. Erythrocytes in sickle form, cellular expression of polymerization of deoxygenated HbS, cause intermittent vascular obstruction, leading to tissue ischaemia and consequent chronic damage in organs and endocrine glands. OBJECTIVE: The evaluation of the growth pattern and pubertal development of a group of patients with sickle-cell anaemia from childhood to adulthood. METHODS: Thirty patients withsickle-cell anaemia between the ages of 10 and 23 years were evaluated in a prospective longitudinal study at three points in time (Te1: 2005; Te2: 2010 and Te3: 2015) and compared with controls. Anthropometric, pubertal and hormonal evaluations were carried out. Age- and gender-specific Z-scores for weight, height and body mass index were calculated according to the reference growth standards. RESULTS: Thirty patients with sickle-cell anaemia (mean age= 13.93 years) were evaluated at Te1 and 26 patients (mean age = 25.08 years) at Te3. The sickle-cell anaemia group lower showed Z-scores for weight (p = 0.0002), height (p= 0.0184) and body mass index (p = 0.0011) than the control group at Te1. At Te3, there was no difference in height, but weight (p = < 0.0001) and body mass index (p= < 0.0001) were lower in the sickle-cell anaemia group. Men showed greater weight commitment than women at the three study times (Te1: p= 0.0340, Te2: p= 0.0426 and Te3: p= 0.0387) and lower body mass index in Te3 (p= 0.0155) in the sickle-cell anaemia group. There was a significant increase in weight when comparing Te1 with Te3 (p= 0.0009) and in height when comparing Te1 with Te2 (p= 0.0292) and with Te3 (p= 0.0003) in the sickle-cell anaemia group. There was a significant increase in weight when comparing Te1 and Te3 (p= 0.0009) and in height when comparing Te1 and Te2 (p= 0.0292) and Te3 (p= 0.0003) in the sickle-cell anaemia group. At Te1, 14 cases and 2 controls were prepubertal. Bone age was delayed in 12 patients. Age at menarche was delayed and lower in the sickle-cell anaemia group (mean = 15 years). Five patients had gestated, but no patient had experienced fatherhood. At Te1, TSH levels were higher (p= 0.0080) and T3 levels were lower (p= 0.0020) in the sickle-cell anaemia group. At Te3, LH and FSH levels were higher in men with sickle-cell anaemia (p= 0.0014; p; 0.0002). IGF-I levels were lower in cases both at Te1 (p= 0.0002) and at Te3 (p= 0.0032). CONCLUSION: Patients with sickle-cell anaemia showed growth impairment and pubertal delay compared with healthy controls. However, albeit belatedly, they reached normal sexual maturation and height in adulthood. Women with sickle-cell anaemia showed no fertility problems. The findings highlight the need to investigate the intention of paternity and fertility among men with sickle-cell anaemia.

INTRODUÇÃO: Hemoglobinopatias hereditárias são o grupo das doenças hereditárias monogênicas mais frequentes no mundo. Os eritrócitos na forma de foice, expressão celular da polimerização da Hemoglobina S desoxigenada, causam obstrução vascular intermitente, levando à isquemia tecidual e consequente dano crônico em órgãos e glândulas endócrinas. OBJETIVO: Avaliar o padrão de crescimento e desenvolvimento puberal de um grupo de portadores de anemia falciforme desde a infância até a vida adulta. MÉTODO: Trinta pacientes com anemia falciforme entre 10 e 23 anos foram avaliados de forma longitudinal prospectiva em três tempos (Te1: 2005, Te2: 2010 e Te3: 2015) comparativamente a controles. Foram realizadas avaliações antropométrica, puberal e hormonal. Z-escores de peso, estatura e índice de massa corpórea para idade e sexo foram calculados através da comparação com padrões de referência. RESULTADOS: Em Te1, foram avaliados 30 pacientes com média de idade de 13,93 anos; em Te3, 26 pacientes com média de 25,08 anos. Os controles tiveram média de idade e proporção de sexo similares ao grupo anemia falciforme . Em Te1, o grupo anemia falciforme apresentou Z-escores de peso (p: 0,0002); estatura (p: 0,0184) e IMC (p: 0,0011) menores que o grupo controle. Em Te3, não houve diferença quanto à estatura, mas peso (p: < 0,0001) e índice de massa corpórea (p: < 0,0001) foram menores no grupo anemia falciforme. Os homens apresentaram maior comprometimento ponderal em relação às mulheres nos três tempos (Te1 p: 0,0340, Te2 p: 0,0426 e Te3 p: 0,0387) e menor índice de massa corpórea em Te3 (p: 0,0155). No grupo anemia falciforme houve aumento significativo de peso quando comparados Te1 e Te3 (p: 0,0009) e da estatura quando comparado Te1 ao Te2 (p: 0,0292) e ao Te3 (p: 0,0003). Em Te1, 14 casos e 2 controles foram considerados impúberes. Idade óssea foi atrasada em 12 pacientes. Idade da menarca foi maior no grupo anemia falciforme (média = 15 anos). Cinco pacientes já haviam gestado, porém nenhum paciente havia experimentado a paternidade. Em Te1, níveis de TSH foram maiores (p: 0.0080) e de T3 menores (p: 0.0020) no grupo anemia falciforme. Em Te3, os níveis de LH e FSH foram maiores nos homens com anemia falciforme (p: 0.0014; p; 0.0002). Níveis de IGF-1 foram menores nos casos em Te1 (p: 0.0002) e Te3 (p: 0.0032). CONCLUSÃO: Pacientes com anemia falciforme apresentaram comprometimento de crescimento e atraso puberal quando comparados a controles. Todavia, ainda que tardiamente, atingem maturação sexual normal. Além disso, alcançaram estatura normal na idade adulta, diferentemente do que ocorreu com peso e índice de massa corpórea. As mulheres com anemia falciforme não relataram dificuldade em relação à fertilidade.

A internalização do cuidado pelo escolar frente à doença falciforme

Este estudo teve por objetivos descrever os instrumentos e signos mediadores da internalização do cuidado pelo escolar frente à doença falciforme; analisar a internalização docuidado pelo escolar frente à doença falciforme nos variados contextos sociais; discutir ainternalização do cuidado pelo escolar com doença falciforme e suas implicações para a saúde. Buscou-se apoio na teoria do desenvolvimento de Vigotski e o conceito de cuidado de Collière, como referenciais teóricos. Trata-se de um estudo qualitativo, com 15 escolares de 6a 12 anos, de um hospital de Vitoria - ES. Um formulário para caracterização dos participantes e uma entrevista semi-estruturada foram utilizados. A análise foi temática. Osresultados apontaram para os cuidados relacionados a hidratação e eliminação; o brincar;prevenção e manejo da crise falcêmica; alimentos e vestuário. Os escolares referiram ingerir variados tipos de líquidos, sendo a água e o suco os mais citados e a sua ingestão estava relacionada à prevenção da crise falcêmica. O atendimento às necessidades de eliminação da criança no contexto escolar mostrou-se prejudicada pela rigidez das normas estabelecidas pelas instituições. Quanto às brincadeiras e atividades físicas, não houve diferença entre o escolar com doença falciforme e as brincadeiras relatadas foram predominantemente ativas. A dor foi um signo mediador da internalização e os auxiliou na identificação de determinadas brincadeiras, como fator desencadeante da crise falcêmica. A interação se estabeleceu entre o escolar e outras crianças ou animais, principalmente no contexto domiciliar e escolar. Os medicamentos citados pelos escolares foram a hidroxiureia e ácido fólico, bem como os analgésicos e antitérmicos associados à manutenção e reparação de sua saúde...

This study had the objective of describing the mediating instruments and signs in the careinternalization by the school child in coping with Sickle Cell disease; To analyse theinternalization of care by the school child in coping with Sickle Cell disease in the severaldifferent social contexts; To discuss the internalization of care of Sickle Cell disease by theschool child, and its implications for the health. The development theory of Vigotski, as wellas the concept of care of Collière, as theoretical references. This is a qualitative study donewith 15 school children from 6 to 12 years old at a hospital of Vitoria - ES. A form for thecharacterization of the participants and a semi-structured interview were used. The analysiswas thematic. The results pointed to care related to hydration and elimination; playing;prevention and management of the Sickle Cell crisis; feeding and clothing. The schoolchildren refered ingesting several different types of liquides, being water and juice the mostcited, and the ingestion was related to the prevention of the Sickle Cell crisis. Meeting theneeds of the child for elimination was impaired in the school context because of the strictrules established by the institutions. In relation to the playing and physical activity there wasno difference between the school child with Sickle Cell disease and the other children, and theplaying was reported as predominantly active. Pain was a mediating sign of theinternalization, and it has helped them to identify certain ways of playing as triggering factorsof the Sickle Cell crisis. There was interaction established between the school child and otherchildren or animals, mainly in the home and school contexts. The drugs reported by theschool children were hydroxyurea and folic acid, as well as analgesics, antipyretic associatedto the maintenance and repare of their health...

Este estudio tuvo como objetivo describir los instrumentos y signos mediadores deinternalización de la atención por el niño en la enfermedad de células falciformes; analizar lainternalización de la atención por el niño en la enfermedad de células falciformes endiferentes contextos sociales; discutir la interiorización de la atención para el niño con laenfermedad de células falciformes y sus consecuencias para la salud. Se buscó seguir la teoríadel desarrollo de Vygotsky y el concepto de atención Collière como referencias teóricas. Estees un estudio cualitativo con 15 niños de 6 a 12, en un hospital de Vitoria - ES. Se utilizó unformulario para caracterizar los participantes y entrevista semi-estructurada. El análisis fuetemático. Los resultados apuntan a la atención relacionada con la hidratación y la eliminación,el juego, la prevención y gestión de crisis de células falciformes, la comida y la ropa. Losniños ingieren varios tipos de líquido, como agua y jugo. Estos eran los más citados y suconsumo se relaciona con la prevención de crisis de células falciformes. La atención a lasnecesidades de eliminación del niño en el contexto escolar resultó ser obstaculizado por larigidez de las reglas establecidas por las instituciones. En cuanto a juegos y actividadesfísicas, se encontró que no hay diferencia entre el niño con la enfermedad de célulasfalciformes y la población infantil en general, se dedican predominantemente a juegos activos.El dolor fue un signo mediador de internalización y les ayudó a identificar ciertos juegoscomo el factor desencadenante de la crisis de células falciformes. La interacción se establecióentre el niño y otros niños o animales sobre todo en el entorno de la casa y de la escuela. Losmedicamentos citados por los niños fueron hidroxiurea y ácido fólico, así como analgésicos yantipiréticos asociados con el mantenimiento y la reparación de su salud...

Association of BCL11A genetic variant (rs11886868) with severity in β-thalassaemia major & sickle cell anaemia.

Background & objectives: The amount of foetal haemoglobin that persists in adulthood affects the clinical severity of haemoglobinopathies including β-thalassaemia major and sickle cell anaemia (SCA). The present study was undertaken to analyse β-thalassaemia as well as SCA patients for the single nucleotide polymorphism (SNP), rs11886868 (T/C) in BCL11A gene and to evaluate the association between this polymorphism and severity of β-thalassaemia major and SCA. Methods: A total of 620 samples (420 β-thalassaemia major and 200 SCA cases) were analysed before blood transfusion using basic screening tests like complete blood analysis and osmotic fragility and further confirmed by high performance liquid chromatography (HPLC), amplification refractory mutation system-polymerase chain reaction (ARMS-PCR) and reverse dot blot techniques. All patients were transfusion dependent. Patients with β-thalassaemia and SCA were classified into mild, moderate, severe according to the severity score based on Hb levels, age of onset, age at which patients received their first blood transfusion, the degree of growth retardation and splenectomy. β-thalassaemia as well as SCA patients were analysed for the SNP, rs11886868 (T/C) in BCL11A gene and association between this polymorphism and severity of β-thalassaemia major as well as SCA was evaluated. Results: There was a significant difference in genotypic and allelic frequencies of BCL11A gene polymorphism between mild and moderate and mild and severe cases in both the groups. A significant (P<0.001) difference was observed in the mean HbF levels between the three genotypes in different severity groups. HbF levels were found to be high in CC genotype bearing individuals followed by TC and TT in β-thalassaemia major as well as SCA. Interpretation & conclusions: This study confirms that the T/C variant (rs11886868) of the BCL11A gene causing downregulation of BCL11A gene expression in adult erythroid precursors results in the induction of HbF and ameliorates the severity of β-thalassaemia as well as SCA.

Complicated Sickle Cell Anemia: A Challenging Case.

Sickle cell anaemia may lead to serious problems during anaesthesia and surgery, due to the nature of the disease and the various Multi-Organ affections and complications that can ensue, SCD patients are at high risk, thus making anaesthesia stressful and challenging even in a state of the art settings. This is a case study of a Saudi patient with complicated SCA who was brought in for an urgent surgery after a fracture of the right neck of the femur following a Road Traffic Accident (RTA). The evidence base oriented approach was taken to decide and stratify patient cardiac condition for prediction of the risk and its management, beside his end stage kidney diseases and his dependency on regular hemodialysis. At the same time the effect of sickle cell anaemia itself and the problem of his raised pulmonary hypertension. This case study emphasizes the proper preoperative assessment and evaluation of SCD patients in addition to the collaboration with other medical specialists, including a hematologist, physician, cardiologist, anesthesiologist, and an orthopaedic surgeon in multi-disciplinary approach.

The Interactive Roles of Some Toxic Metals, Micronutrients, Antioxidant Vitamin and Sex Hormones in Nigerians with Sickle Cell Disease.

Aims: To define the involvement of sex hormones-estradiol, testosterone, toxic metals and micronutrients in individuals with sickle cell disease (SCD). Study Design: A case control study. Place and Duration of Study: Departments of Chemical Pathology and Haematology, College of Medicine, University of Ibadan, Ibadan, Haematology Outpatient Clinic, Ring Road State Hospital, Oyo State, Nigeria between Dec 2007 and Jul 2008. Methodology: One hundred and twenty participants, consisting of 68 with Haemoglobin S (SCD) and 52 with Haemoglobin A aged 17-43 years were recruited for the study. Five ml blood samples were obtained from each participant. Anthropometry and reproductive history were obtained by standard methods. Serum estradiol and testosterone concentrations were estimated using enzyme linked-immunoassay methods, Zinc, Copper, Selenium, Lead and Cadmium by atomic absorption spectrophotometry and vitamin E by High Performance Liquid Chromatography. Results: The mean Zinc, Copper, Vitamin E concentrations in SCD were significantly lower, while those of lead and Cadmium were significantly higher when compared with the corresponding control values (P <.001). The mean testosterone in male and estradiol in female (SCD) were significantly lower than the corresponding control values (P <.03). The mean age at menarche in SCD was significantly higher than the value in control participants. In female SCD, the serum estradiol was positively correlated with height and negatively correlated with lead in the control (P =.05). The incidence of priapism in male SCD was significantly higher than the value in male control subjects (P <.007). In both male and female SCD, the mean body mass index and body weight were significantly lower than the corresponding values in the control group (P <.006). Conclusion: A significant number of Nigerians with SCD had priapism and the observed oxidative stress in SCD probably due to hypogonadism, may be amenable to micronutrient supplementation.

Neutrophil to Lymphocyte Ratio in Sickle Cell Anaemia Patients with Nephropathy.

Background: Sickle cell anaemia (SCA) is associated with a chronic inflammatory component; blood neutrophil to lymphocyte ratio (NLR) has been described as a marker of organ dysfunction and clinical outcome in diseases associated with systemic inflammation. Objective: To evaluate NLR in Nigerian SCA patients with nephropathy. Methods: Seventy-nine (79) SCA patients in steady state and 50 aged-matched controls were prospectively enrolled for this study. Full blood count and dip-stick macro-albuminuria were done for each participant and data was analyzed using descriptive and inferential statistics. The level of statistical significance was defined as p ≤ 0.05. Results: The NLR was significantly higher in cases compared with controls (1.49±0.76 vs. 1.20±0.34, P=0.01) and the ANC was significantly higher in those with NLR ≥ 3.0 compared with those with NLR <3.0 (12.22±5.26x109/L vs. 6.10±2.96x109/L, respectively, P<0.001). Between cases with and without macro-albuminuria and controls, the means of NLR was significantly different (P=0.024). Macro-albuminuria was present in 16 (21.9%) of cases (all of which had NLR <3.0), this was not significantly correlated with NLR (r=-0.99, P=0.71). Conclusion: No significant relationship was observed between NLR and SCA nephropathy.

Vitamin A Status of Steady State Sickle Cell Anaemia Patients Compared to Normal Control in Maiduguri North Eastern Nigeria.

Sickle cell anaemia is an inherited disorder of haemoglobin characterized by sickled red blood cells and increased destruction of these cells. Antioxidants protect cells from the damaging effects of free radicals. The aim of this study was to determine antioxidant vitamin A in steady state sickle cell anaemia patients and that of controls in Maiduguri, Borno state North-Eastern Nigeria. The study was carried out at UMTH Maiduguri. Sixty sickle cell anaemia patients were compared with sixty controls, aged ranged 1 year 3 months to 33 years of age, using HPLC for vitamin A status. The mean vitamin A in sickle cell patients according to age ranged between 0.047±0.002 to 0.053±0.002 mg/ml, while that of controls is 0.053± 0.001 to 0.091±0.001 mg/ml. The maximum mean serum vitamin A (0.053±0.001 mg/ml), in SCA was found in the business and children groups while the minimum vitamin A (0.039±0.001 mg/ml) was recorded in the un-employed SCA patients. The study showed the antioxidant vitamin A was found to be lower in the SCA subjects than in normal control in all age groups. Level of education also plays a role in the level of antioxidant vitamins in the blood.

Hepcidin and Erythropoietin Level in Sickle Cell Disease.

Background: Sickle cell anaemia is an inherited disorder in which the red blood cells become rigid and sticky, and change from being disc-shaped to being crescent-shaped. The change in shape is as a result of an abnormal form of haemoglobin which leads to damage of some organs. Aim: The study was carried out to determine the levels of hepcidin and erythropoietin in sickle cell anemia. Materials and Methods: Thirty confirmed sickle cell patients in steady state (HbSS-SS) and thirty persons with normal hemoglobin (HbAA) between the ages of 15 to 30 years were selected in this study. Results: The results obtained showed that the levels of hepcidinin sickle cell anaemia patients were significantly elevated when compared with normal haemoglobin (HbAA) (P<0.05). However, the levels of erythropoietin were significantly decreased in sickle cell anaemia stable state when compared to HbAA subjects (P<0.05). Conclusion: This could probably imply that the elevation in hepcidin affect level of erythropoietin negatively. Therefore, it is possible that an antihepcidin could be developed as a therapeutic tool in sickle cell anaemia.

Indices of Insulin Sensitivity and Oral Disposition Index in Adult Nigerians with Sickle Cell Anaemia: A Pilot Study.

Aim: To determine indices of insulin sensitivity and oral disposition index (DIo) derived from 30 min of glucose challenge in adults with sickle cell anaemia; a group in whom there is chronic inflammation. Study Design: Case-controlled study. Place of Study: Department of Chemical Pathology and Department of Haematology, University of Ibadan/University College Hospital, Ibadan, Nigeria. Methodology: Twenty five (25) adults with sickle cell anaemia (SCA) in steady state and 25 age, sex and body mass index (BMI) matched healthy individuals with HbAA genotype were recruited into this study. After an overnight fast of about 10 hr, 5 ml of venous blood was obtained from each participant for the determination of plasma glucose and serum insulin. Thereafter, each subject underwent a 75-g oral glucosetolerance test and at 30min, 5ml of venous blood was obtained for the determination of plasma glucose and serum insulin. Serum insulin was determined using ELISA while the plasma glucose was estimated using glucose oxidase method and indices of insulin sensitivity and β-cell function were calculated appropriately. Differences between variables with Gaussian distribution were determined using independent Student’s t-test while Mann-Whitney U was used for the non-Gaussian variables. P-values less than 0.05 were considered significant. Results: The mean fasting plasma glucose (FPG) was within the normal limit but was significantly lower in subjects with SCA compared with controls. All other indices of insulin sensitivity (insulinogenic index, fasting insulin resistance index, modified Matsuda index of insulin sensitivity and insulin secretion/insulin resistance index) and oral disposition index (DIo) were similar in both groups. Conclusion: It could be concluded from this study that SCA subjects have a similar insulin sensitivity status with HbAA subjects. This suggests that SCA subjects might not be more predisposed to the development of type 2 diabetes mellitus than those with HbAA despite the chronic inflammation associated with the former.