scholarly journals Hematological Indices of Sickle Cell Anaemia Patients with Pulmonary Tuberculosis in Northern Nigeria.

2010 ◽  
Vol 2 (1) ◽  
pp. e2010014 ◽  
Author(s):  
Sagir G. Ahmed ◽  
Audu A. Bukar ◽  
Bashir Jolayemi
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anaemia ◽  
Reticulocyte Count ◽  
Hematological Indices ◽  
Sickle Cells ◽  
The World ◽  
Patient Groups ◽  
Wbc Count ◽  
Haematological Indices ◽  
Age And Sex

Nigeria has the fourth highest prevalence of TB and the highest prevalence of Sickle cell anaemia (SCA) in the world. SCA patients have impaired immunity and are vulnerable to TB. Hence, we studied the haematological indices of SCA patients with TB in Nigeria. A total of 23 SCA patients with TB were studied in parallel with equal number of age and sex matched SCA patients without TB. SCA patients with TB had significantly lower haematocrit, higher level of circulating sickle cells (CSCs) and similar level of reticulocyte count in comparison to patients without TB. SCA patients with TB had significantly higher mean WBC count associated with higher frequency of neutrophilia in comparison to those without TB. Monocytosis and eosinopenia were exclusively found in SCA patients with TB at frequencies of 52% and 65% respectively. Lymphocyte and basophil counts were normal in all patients with and without TB. Mean platelet counts were high in both patient groups but the frequency of thrombocytosis was significantly higher in patients with TB. SCA patients with TB had significantly higher mean ESR than their counterparts without the infection. The findings of this study  revealed that TB in SCA patients was associated with rising level of CSCs, falling level of haematocrit, sub-optimal reticulocytosis, neutrophilia, monocytosis, thrombocytosis, eosinopenia and rising level of ESR. Hence, SCA patients presenting with these haematological indices should be investigated for TB.

scholarly journals Leg Length Discrepancies in Nigerian Children with Sickle Cell Anaemia

2020 ◽  
pp. 70-79
Author(s):  
Eneh Chizoma Ihuarula
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anaemia ◽  
Medial Malleolus ◽  
Leg Length ◽  
P Values ◽  
The Real ◽  
Significant Difference ◽  
The Right ◽  
Age And Sex ◽  
Female Subjects

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 in 14-18 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.

scholarly journals Incidence of Sickle Cell Anaemia among Children Attending Maryam Abacha Women and Children Hospital, Sokoto

2020 ◽  
pp. 66-71
Author(s):  
S. Y. Lema ◽  
J. Suleiman ◽  
J. Ibrahim
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anaemia ◽  
Sub Saharan Africa ◽  
Age Group ◽  
The World ◽  
Genotype Test ◽  
Women And Children ◽  
Sub Saharan ◽  
Male Subjects

Sickle Cell Anaemia is still considered the most common genetic disease worldwide, causing morbidity and mortality in Sub-Saharan Africa, Mediterranean areas, Middle East and India. Nigeria, being the most populous black nation in the world, bears its greatest burden in Sub-Saharan Africa. This study was conducted to determine the incidence of Sickle Cell Anaemia among children attending Maryam Abacha Women and Children Hospital, Sokoto. A total of one hundred (100) blood samples were examined for the disease. Out of the 100 children tested for the disease. (59%) were normal (HbAA), (35%) were carrier (HbAS) and (6%) were Sicklers (HbSS). The result based on gender showed that female has the highest percentage of the disease (5%) against male subjects with only (1%). A child between the age group 6-10 years has the highest rate of sickle cell anaemia (3%) while age group 11-15 years had the lowest rate of the infection. Improved knowledge regarding Sickle cell anaemia disease and its comprehensive care among Nigerian physicians will enhance quality of care for affected childrens and policy for regular genotype test by government and other stakeholders before marriage among Nigerians will help to prevent the disease.

Relationship between foetal haemoglobin and haematological indices in children with sickle cell anaemia from South Western Nigeria

2017 ◽  
Vol 24 (4) ◽  
pp. 195
Author(s):  
MorenikeAgnes Akinlosotu ◽  
SamuelAdemola Adegoke ◽  
SaheedBabajide Oseni ◽  
OluwagbemigaOyewole Adeodu
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anaemia ◽  
Foetal Haemoglobin ◽  
Haematological Indices

scholarly journals Yoda1 and phosphatidylserine exposure in red cells from patients with sickle cell anaemia

2020 ◽  
Vol 10 (1) ◽  
Author(s):  
R. Wadud ◽  
A. Hannemann ◽  
D. C. Rees ◽  
J. N. Brewin ◽  
J. S. Gibson
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anaemia ◽  
Red Cells ◽  
Mechanosensitive Channel ◽  
Sickle Cells ◽  
Molecular Identity ◽  
Calphostin C ◽  
Intracellular Ca ◽  
Pkc Inhibitors ◽  
Low Levels

AbstractPhosphatidylserine (PS) exposure is increased in red cells from sickle cell anaemia (SCA) patients. Externalised PS is prothrombotic and attractive to phagocytes and activated endothelial cells and thus contributes to the anaemic and ischaemic complications of SCA. The mechanism of PS exposure remains uncertain but it can follow increased intracellular Ca2+ concentration ([Ca2+]i). Normally, [Ca2+]i is maintained at very low levels but in sickle cells, Ca2+ permeability is increased, especially following deoxygenation and sickling, mediated by a pathway sometimes called Psickle. The molecular identity of Psickle is also unclear but recent work has implicated the mechanosensitive channel, PIEZO1. We used Yoda1, an PIEZO1 agonist, to investigate its role in sickle cells. Yoda1 caused an increase in [Ca2+]i and PS exposure, which was inhibited by its antagonist Dooku1 and the PIEZO1 inhibitor GsMTx4, consistent with functional PIEZO1. However, PS exposure did not necessitate an increase in [Ca2+]i. Two PKC inhibitors were also tested, chelerytherine chloride and calphostin C. Both reduced PS exposure whilst chelerytherine chloride also reduced Yoda1-induced increases in [Ca2+]i. Findings are therefore consistent with the presence of PIEZO1 in sickle cells, able to mediate Ca2+ entry but that PKC was also involved in both Ca2+ entry and PS exposure.

scholarly journals Prevalence and Predictors of Elevated Pulmonary Artery Pressure in Nigerian Children with Sickle Cell Anaemia

2020 ◽  
pp. 48-59
Author(s):  
Usman Muhammad Sani ◽  
Usman Muhammad Waziri ◽  
Nma M. Jiya ◽  
Hamidu Ahmed ◽  
Khadijat Omeneke Isezuo ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Artery ◽  
Pulmonary Artery Pressure ◽  
Sickle Cell ◽  
Sickle Cell Anaemia ◽  
Reticulocyte Count ◽  
Systolic Pressure ◽  
Full Blood Count ◽  
High Systolic Blood Pressure ◽  
Artery Pressure

Background: Sickle Cell Anaemia (SCA) is the most common inherited disorder in Nigeria. Pulmonary Hypertension (PH) is a known complication of SCA that commences from childhood and progresses as they grow older. Aim: To determine the prevalence and predictors of elevated pulmonary artery pressure (PAP) in children with SCA. Study Design: This is an analytical cross-sectional study. Methods: Three hundred subjects with SCA in steady state and 300 HbAA controls, aged 6 months – 15 years, were enrolled into the study. Children with SCA were recruited by simple random sampling, from the Paediatric Sickle Cell Clinic, Usmanu Danfodiyo University Teaching Hospital, Sokoto. All the children had clinical evaluation, echocardiography, full blood count and reticulocyte count. Pulmonary artery systolic pressure (PASP) and mean pulmonary artery pressure (MPAP) were determined by measurement of tricuspid regurgitant velocity (TRV) and mean gradient (MG) respectively.  Results: Seventy-two children with SCA (24.0%) had elevated PAP (defined as TRV ≥ 2.5 m /s) while only 9 (3.0%) had pulmonary hypertension (defined as MPAP ≥ 25 mmHg). Older age (O.R = 1.03, p < 0.001), high systolic blood pressure (O.R = 1.2, p = 0.01), and high reticulocyte count (O.R = 1.33, p = 0.04) were weakly associated with increased likelihood of elevated PAP. Normal pulse oxygen saturation (SPO2 ≥ 95%, O.R = 0.4, p = 0.002) and normal heart sounds on auscultation (O.R = 0.2, p = 0.04) were associated with a reduced likelihood of elevated PAP. Presence of left parasternal heave (O.R = 8.4, p = 0.001) significantly predicted elevated PAP in children with SCA. Conclusion: PH occurs as a complication in children with SCA and certain clinical and laboratory parameters do predict this complication in children.

scholarly journals Case Report on Sickle Cell Anaemia (SS Pattern)

2021 ◽  
pp. 271-275
Author(s):  
Mayur Waghaye ◽  
Sheetal Sakharkar ◽  
Samrudhi Gujar ◽  
Swapna Morey ◽  
Archana Dhengare ◽  
...  
Keyword(s):  
Hemolytic Anemia ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Sickle Cell Anaemia ◽  
Bodily Pain ◽  
Sickle Hemoglobin ◽  
Hemoglobin Gene ◽  
History Of ◽  
Wbc Count ◽  
Rbc Count

Sickle cell anemia is a kind of hemolytic anemia that is passed down in families. It is a kind of hemolytic anemia caused by inheriting the sickle hemoglobin gene. Africans, as well as individuals from the Middle East, the Mediterranean region, and India's aboriginal tribes, have a lower level of the sickle hemoglobin (HbS) gene. A kind of anemia that affects both children and adults is sickle cell anemia. Clinical Finding: Since 5 days, A 25-year-old man has been experiencing generalized bodily pain and anxiety. Examining the Problem: ALT (SGPT)- 97 U/L, AST (SGOT)- 56 U/L, total bilirubin – 5.4 mg percent, bilirubin conjugated – 1.7 mg percent, bilirubin unconjugated – 3.7 mg percent, total    RBC count – 3.71 million/cu mm, total WBC count – 22100 cu mm, total platelets count – 6.46 lack/cu. Ultrasonography: Heterogeneous spleen. Therapeutic Intervention: Inj. Piptaz 4.5 gm TDS, inj. Levoflox 500 mg, tab. Hydroxyurea 500 mg, tab. Neurobion forte, inj. Pan 40 mg, inj. Tramadol 100 mg. Outcome: The client's condition has improved as a result of the treatment. He has no longer generalized bodily aches, and his anxiety levels have decreased. Conclusion: A 25-year-old man was admitted to Acharya Vinoba Bhave Hospital's Medicine ward with a history of sickle cell anaemia and complaints of nonspecific body aches and anxiousness. His condition improved after he received proper therapy.

scholarly journals The role of WNK in modulation of KCl cotransport activity in red cells from normal individuals and patients with sickle cell anaemia

2019 ◽  
Vol 471 (11-12) ◽  
pp. 1539-1549
Author(s):  
David C.-Y. Lu ◽  
Anke Hannemann ◽  
Rasiqh Wadud ◽  
David C. Rees ◽  
John N. Brewin ◽  
...  
Keyword(s):  
Oxygen Tension ◽  
Sickle Cell ◽  
Sickle Cell Anaemia ◽  
Kinase Inhibitors ◽  
Red Cells ◽  
Red Cell ◽  
Sickle Cells ◽  
Kcl Cotransport ◽  
Pre Treatment

AbstractAbnormal activity of red cell KCl cotransport (KCC) is involved in pathogenesis of sickle cell anaemia (SCA). KCC-mediated solute loss causes shrinkage, concentrates HbS, and promotes HbS polymerisation. Red cell KCC also responds to various stimuli including pH, volume, urea, and oxygen tension, and regulation involves protein phosphorylation. The main aim of this study was to investigate the role of the WNK/SPAK/OSR1 pathway in sickle cells. The pan WNK inhibitor WNK463 stimulated KCC with an EC50 of 10.9 ± 1.1 nM and 7.9 ± 1.2 nM in sickle and normal red cells, respectively. SPAK/OSR1 inhibitors had little effect. The action of WNK463 was not additive with other kinase inhibitors (staurosporine and N-ethylmaleimide). Its effects were largely abrogated by pre-treatment with the phosphatase inhibitor calyculin A. WNK463 also reduced the effects of physiological KCC stimuli (pH, volume, urea) and abolished any response of KCC to changes in oxygen tension. Finally, although protein kinases have been implicated in regulation of phosphatidylserine exposure, WNK463 had no effect. Findings indicate a predominant role for WNKs in control of KCC in sickle cells but an apparent absence of downstream involvement of SPAK/OSR1. A more complete understanding of the mechanisms will inform pathogenesis whilst manipulation of WNK activity represents a potential therapeutic approach.

Predictors of fetal hemoglobin response in children with sickle cell anemia receiving hydroxyurea therapy

Blood ◽  
2002 ◽  
Vol 99 (1) ◽  
pp. 10-14 ◽  
Author(s):  
Russell E. Ware ◽  
Barry Eggleston ◽  
Rupa Redding-Lallinger ◽  
Winfred C. Wang ◽  
Kim Smith-Whitley ◽  
...  
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Reticulocyte Count ◽  
Fetal Hemoglobin ◽  
Hemoglobin Concentration ◽  
School Aged Children ◽  
Baseline Values ◽  
Wbc Count ◽  
Hydroxyurea Therapy ◽  
Baseline Hemoglobin

In the phase I/II pediatric hydroxyurea safety trial (HUG-KIDS), school-aged children with sickle cell anemia receiving hydroxyurea at the maximally tolerated dose (MTD) had variable increases in the percentage of fetal hemoglobin (%HbF). To identify predictors of the HbF response to hydroxyurea therapy, baseline clinical and laboratory values (age, sex, hemoglobin concentration, %HbF, reticulocytes, white blood cell [WBC], platelets, and serum chemistries), as well as treatment variables (number of toxicities, noncompliance, MTD dose, and MTD blood counts) were analyzed in 53 HUG-KIDS children who achieved MTD. Baseline %HbF values (P = .001), baseline hemoglobin concentration (P = .01), MTD dose (P = .02), and compliance (P = .02) were significantly associated with a higher %HbF at MTD; in contrast, age, sex, number of toxicities, and other baseline hematologic parameters were not. After adjusting for variations in baseline %HbF, the baseline reticulocyte count (P = .05) and baseline WBC count (P = .05) were also significantly associated with a higher %HbF at MTD. Hydroxyurea-induced increases in the hemoglobin concentration and mean corpuscular volume (both higher absolute values at MTD and larger positive changes from baseline values), as well as hydroxyurea-induced decreases in reticulocytes and WBC count, were significantly associated with a higher %HbF at MTD. These data suggest that selected baseline laboratory parameters, a higher MTD dose with attention to compliance, and greater therapy-related changes in blood counts may predict the HbF response to hydroxyurea therapy for children with sickle cell anemia. The HbF response to hydroxyurea is variable and complex, however, and even children with low baseline %HbF values can develop substantial increases in %HbF at MTD.

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