Case Report on Sickle Cell Anaemia (SS Pattern)

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.

Predicting risk factors for thromboembolic complications in patients with sickle cell anaemia – lessons learned for prophylaxis

Objective To assess the clinical and laboratory predictors of venous thromboembolism (VTE) in patients with sickle cell anaemia (SCA) and its relationship to morbidity and mortality. Methods This retrospective case–control study analysed data from patients with SCA that experienced VTE compared with matched control patients with SCA but no VTE (2:1 ratio). Results A total of 102 patients with SCA were enrolled (68 cases with VTE and 34 controls). Amongst the 68 cases (median age, 29.5 years), 26 (38.2%) presented with isolated pulmonary embolism (PE). A higher prevalence of splenectomy (73.5% versus 35.3%) was observed in the cases compared with the controls. A significantly higher prevalence of central venous catheter (CVC) insertion (42.6% versus 8.8%) was observed in the cases compared with the controls. High white blood cell counts, serum lactic dehydrogenase (LDH), bilirubin and C-reactive protein (CRP) and low haemoglobin (Hb) and HbF were significant risk factors for VTE. Forty-two cases (61.8%) developed acute chest syndrome, 10 (14.7%) had a stroke and seven (10.3%) died. Conclusions VTE in patients with SCA has a high impact on morbidity and mortality. PE was the leading presentation of VTE, with CVC insertion, high LDH, bilirubin, CRP and white blood cell counts along with low Hb and HbF constituting other significant risk factors.

Clinical depression in children and adolescents with sickle cell anaemia: influencing factors in a resource-limited setting

Background Sickle cell anaemia (SCA) is the commonest monogenic haematologic disorder resulting from the inheritance of homozygous mutant haemoglobin genes from both parents. Some factors have been identified as important in explaining the variability in depression in sickle cell anaemia (SCA). Information on this is limited in a resource-limited setting like Nigeria. This study aims to determine factors which influence depression in children and adolescents with sickle cell anaemia in a resource-limited setting. Methods Systematic random sampling technique was used in this cross-sectional study to select children and adolescents aged 7–17 years at the weekly sickle cell clinic of the University of Nigeria Teaching hospital (UNTH) Enugu, Nigeria. Pretested, structured questionnaire was used to collect sociodemographic and disease severity data while depression was assessed using the Children’s Depression Inventory. Results Age and educational level had significant positive linear relationships with depression (r = 0.253, p = 0.02; r = 0.225, p = 0.04 respectively) while gender (χ2 = 0.531, p = 0.466), socioeconomic status (χ2 = 0.451, p = 0.798) and disease severity (χ2 = 0.422, p = 0.810) had no relationship with depression in children and adolescents with SCA. Conclusion Depression in children and adolescents with SCA increased with increasing age and educational level. Psychological evaluation should be integrated into routine assessment of children with SCA during their follow up visits as they get older and progress in class.

Ovarian reserve in nigerian women with sickle cell anaemia: a cross- sectional study

Introduction Sickle cell disease is the most common monogenetic disorder worldwide. There have been reports of endocrine dysfunction and gonadal failure among affected individuals, especially in males. The findings on ovarian reserve and failure in women with sickle anaemia have been inconsistent. Aim and objective The aim of this study was to determine and compare the ovarian reserve of Nigerian women with and without sickle cell anaemia attending a University Teaching Hospital. Study Design This cross-sectional study was carried out at the Adult Sickle Cell Clinic and the Community Health Clinic of the Lagos University Teaching Hospital. Methodology A total of 166 participants who met the selection criteria, were recruited for the study. The study population consisted of two groups of women matched for age: 83 women with HbSS and 83 women with HbAA. The haemoglobin phenotype of each participant was determined on alkaline electrophoresis (pH 8.4) before analysis. Serum Anti-Mullerian Hormone (AMH) was determined using Enzyme-linked immunosorbent assay (ELISA) method (Calbiotech Inc. USA, Catalog no AM448T). Results The mean ± SD of serum AMH level in women with HbSS was 3.64 ± 0.65 ng/mL and was lower than that of women with HbAA 7.35 ±1.19 ng/mL (p < 0.001). Serum AMH negatively correlated with age in both study groups (HbAA and HbSS). Also, a significant negative correlation was found between serum AMH and BMI in women with HbAA. Conclusion The study showed diminished ovarian reserve in women with HbSS when compared to age-matched women with HbAA.

Isolation and characterisation of quercitrin as a potent anti-sickle cell anaemia agent from Alchornea cordifolia.

Alchornea cordifolia MÜll. Arg. (commonly known as Christmas Bush) has been used traditionally in Africa to treat sickle cell anaemia (a recessive disease, arising from the S haemoglobin [Hb] allele) but the active compounds are yet to be characterised. Herein we describe the use of sequential fractionation coupled with in vitro anti-sickling assays to purify the active component. Sickling was induced in HbSS genotype blood samples using sodium metabisulphite (Na2S2O5) or incubation in 100 % N2. Methanol extracts of A. cordifolia leaves and its sub-fractions showed >70 % suppression of HbSS erythrocyte sickling. Purified compound demonstrated 87.2 ± 2.39 % significant anti-sickling activity and 93.1 ± 2.69 % erythrocyte sickling-inhibition at 0.4 mg/mL. Nuclear magnetic resonance (NMR) spectra and high-resolution mass spectroscopy identified it as quercitrin (quercetin 3-rhamnoside). Purified quercitrin also inhibited the polymerisation of isolated HbS and stabilized sickle erythrocytes membranes. Metabolomic comparisons of blood samples using flow-infusion electrospray-high resolution mass spectrometry indicated that quercitrin could convert HbSS erythrocyte metabolomes to be similar to HbAA. Sickling was associated with changes in anti-oxidants, anaerobic bioenergy, and arachidonic acid metabolism, all of which were reversed by quercitrin. The findings described could inform efforts directed to the development of an anti-sickling drug or quality control assessments of A. cordifolia preparations.