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.

The orientation of sickle hemoglobin fibers within fascicles

1988 ◽  
Vol 46 ◽  
pp. 400-401
Author(s):  
Christopher A. Miller ◽  
Bridget Carragher ◽  
William A. McDade ◽  
Robert Josephs
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Relative Orientation ◽  
Unit Cell ◽  
Red Cell ◽  
High Ph ◽  
Sickle Hemoglobin ◽  
Polar Crystal ◽  
Helical Configuration

Highly ordered bundles of deoxyhemoglobin S (HbS) fibers, termed fascicles, are intermediates in the high pH crystallization pathway of HbS. These fibers consist of 7 Wishner-Love double strands in a helical configuration. Since each double strand has a polarity, the odd number of double strands in the fiber imparts a net polarity to the structure. HbS crystals have a unit cell containing two double strands, one of each polarity, resulting in a net polarity of zero. Therefore a rearrangement of the double strands must occur to form a non-polar crystal from the polar fibers. To determine the role of fascicles as an intermediate in the crystallization pathway it is important to understand the relative orientation of fibers within fascicles. Furthermore, an understanding of fascicle structure may have implications for the design of potential sickling inhibitors, since it is bundles of fibers which cause the red cell distortion responsible for the vaso-occlusive complications characteristic of sickle cell anemia.

scholarly journals KNOWLEDGE AND ATTITUDE TOWARDS PREMARITAL GENOTYPE SCREENING AMONG WOMEN OF CHILD-BEARING AGE IN KUMO AKKO LOCAL GOVERNMENT AREA OF GOMBE STATE

2020 ◽  
Vol 1 (1) ◽  
pp. 10-19
Author(s):  
O. Agofure ◽  
M. A. Danzaria
Keyword(s):  
Family History ◽  
Local Government ◽  
Sickle Cell ◽  
Sickle Cell Anaemia ◽  
Health Workers ◽  
Local Government Area ◽  
Child Bearing ◽  
History Of ◽  
Knowledge And Attitude ◽  
Genotype Screening

The prevalence of sickle cell anaemia has been shown to be on the increase in Nigeria. Therefore, preventive measures such as premarital genotype screening have been proposed as one of the ways of reducing the scourge of the disease. Therefore, this study was designed to assess the knowledge and attitude of women of child bearing age in Kuma Akko Local Government Area of Gombe State towards premarital genotype screening. The study adopted a descriptive cross-sectional design. A total of 296 respondents were sampled through simple random sampling technique and the collected data were analyzed using Statistical Package for the Social Sciences (SPSS) version 20.0. Majority of the respondents 200(71.40%) were between the ages of 15-34 years old and 64(22.90%) of the respondents affirmed that they had a family history of sickle cell disease. In addition, 58.60% of the respondents demonstrated poor knowledge of premarital genotype screening while 57.10% exhibited negative perception towards premarital genotype screening and 63.20% exhibited poor attitude towards premarital genotype screening. There was a significant relationship between respondents with family history of sickle cell anaemia and their knowledge of premarital genotype screening. The study therefore recommends that efforts should be intensified by the governments and health workers in primary, secondary and tertiary level of healthcare deliveries, to raise awareness on the importance of premarital genotype screening in order to reduce the prevalence of Sickle cell anaemia in the locality. Agofure, O. | Department of Public and Community Health, Novena University, Ogume, Delta State, Nigeria.

Hydroxyurea corrects the dysregulated L-selectin expression and increased H2O2 production of polymorphonuclear neutrophils from patients with sickle cell anemia

Blood ◽  
2002 ◽  
Vol 99 (7) ◽  
pp. 2297-2303 ◽  
Author(s):  
Malika Benkerrou ◽  
Charlotte Delarche ◽  
Lamia Brahimi ◽  
Michèle Fay ◽  
Etienne Vilmer ◽  
...  
Keyword(s):  
Adhesion Molecules ◽  
Sickle Cell ◽  
Adhesion Molecule ◽  
Sickle Cell Anemia ◽  
Plasma Levels ◽  
Polymorphonuclear Neutrophil ◽  
H2o2 Production ◽  
Soluble Adhesion Molecules ◽  
Hydroxyurea Treatment ◽  
History Of

Impaired polymorphonuclear neutrophil (PMN) functions during sickle cell anemia (SCA) may have a pathogenic role in the onset of vasoocclusive events. We used flow cytometry to study, in whole blood, the adhesion molecule expression and respiratory burst of PMNs from children with SCA. Three different clinical groups were studied: (1) patients with no history of vasoocclusive events (n = 15); (2) patients with a history of vasoocclusive events (n = 17); and (3) patients receiving hydroxyurea therapy for severe vasoocclusive events (n = 9). Unstimulated PMNs showed decreased L selectin expression and increased H2O2 production whatever the severity of the disease, reflecting PMN activation. This could contribute to endothelial activation reflected by abnormal plasma levels of soluble adhesion molecules (soluble intercellular adhesion molecule-1, sE selectin, and sL selectin). After stimulation with bacterial N-formyl peptides (N-formyl-methionyl-leucyl-phenylalanine [fMLP]), PMNs from untreated patients with a history of vasoocclusive events showed dysregulated L selectin shedding and increased H2O2 production. Furthermore, in these patients, tumor necrosis factor priming followed by fMLP stimulation induced an H2O2 production significantly higher than in the other patient groups and controls. These impairments could immobilize PMNs on the endothelium, thereby inducing reduced blood flow and fostering microvascular occlusion and vascular damage. In contrast, children treated with hydroxyurea showed near-normal basal and poststimulation H2O2 production as well as normal L selectin shedding after stimulation but no change in plasma levels of soluble adhesion molecules. To our knowledge, this is the first report showing major qualitative changes of PMN abnormalities upon hydroxyurea treatment in SCA patients. This strongly suggests that PMNs are a primary target of this drug.

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.

Correlation of Iron Levels with Asthma and Lung Function in Pediatric Patients with Sickle Cell Anemia

Blood ◽  
2020 ◽  
Vol 136 (Supplement 1) ◽  
pp. 12-12
Author(s):  
Yusra D Shaikh ◽  
Nataly Apollonsky ◽  
Bruce Bernstein
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Serum Iron ◽  
Pediatric Patients ◽  
Pulmonary Complications ◽  
Acute Chest Syndrome ◽  
Iron Level ◽  
Obstructive Sleep ◽  
History Of ◽  
Hb S

Introduction:Significant morbidity and mortality in patients with sickle cell disease (SCD) is attributed to the pulmonary sequalae of the disease. Patients with SCD often suffer airway hyper-reactivity, acute chest syndrome (ACS), chronic lung disease, pulmonary hypertension (PHTN), and obstructive sleep apnea (OSA). Recent literature has provided evidence supporting the strong association between asthma and airway hyper-reactivity in SCD. One of the factors linked to chronic inflammation and asthma is iron status. The present study examined whether iron levels are associated with pulmonary complications in pediatric patients with SCD. Method:Through retrospective review of electronic medical records (EMR) we evaluated patients with diagnosis of asthma and SCD. All patients with available PFT (3/21/2013-3/11/2020) and iron studies were included in the analysis. Chi square and ANOVA tests were used to explore relationships of respiratory conditions with lab data and relevant medical history. Results:The analysis reviewed information of 100 patients with SCD -- 56 males and 44 females The sample population had the following genotypes: 63% Hemoglobin (Hb) SS, 23% Hb SC, 2% Hb S Beta Zero Thalassemia, and 12% Hb S Beta Thalassemia. 38% of these patients were receiving treatment via hydroxyurea. The results generated found that patients with a large airway obstruction (LAO) had a marginally statistically significantly higher serum iron level than those with no LAO (p=0.067.) Patients with homozygous Hb S disease were four times as likely to have a history of ACS (p=0.004) than those without and were marginally significantly more likely to be SS and SB0Thal (p=0.052). Patients with history of ACS had a significantly higher mean iron saturation and lower total iron binding capacity (TIBC.) Patients with PHTN had significantly higher serum iron levels (p=0.029). Conclusion:Our findings reveal that while iron might play a more significant role in the development of PHTN and ACS in patients with SCD, the role in asthma is borderline in our sample. These findings, although of borderline statistical significance p=0.067, are clinically noteworthy. These results may open a new window for therapy targeted at maintaining iron in normal physiologic ranges to decrease pulmonary complications in patients with sickle cell anemia. Further studies with larger samples are necessary to clarify the meaning of our marginally significant findings. Disclosures No relevant conflicts of interest to declare.

Transcranial Doppler in Sickle Cell Anemia Adult Patients: Brazilian Experience.

Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 3736-3736
Author(s):  
Gisele S. Silva ◽  
Maria S. Figueiredo ◽  
Perla Vicari ◽  
Airton R. Massaro ◽  
Adauto Castelo Filho ◽  
...  
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Transcranial Doppler ◽  
Stroke Risk ◽  
Arterial Disease ◽  
Neurological Complications ◽  
Adult Patients ◽  
History Of ◽  
The Mean

Abstract Sickle cell anemia (SCA) may cause a variety of neurological complications, including stroke and headaches. Stroke occurs in up to 9% of children with SCA, and transcranial Doppler (TCD) studies have demonstrated that increased velocities are related to higher stroke risk. Throbbing headache occurs in SCA but its cause, frequency, and relationship to TCD velocities have received little attention. On the other hand, there are few TCD studies in adult patients. Our aims were: 1) to describe the main features of TCD in adult SCA patients, and 2) to investigate if there were correlation between TCD features and presence of headache. TCD was performed in 56 adult SCA patients (≥ 16 years old) and in 56 healthy individuals (HI), matched by age and race. There were 6 patients with a remote history of stroke but none were on chronic transfusion. The SCA group was submitted to a neurological evaluation and specifically asked about the occurrence of headache and its characteristics. The highest flow velocity (maxFV) recorded for each artery was considered the most representative. We analyzed the frequency of FV asymmetry (side-to-side difference > 20%) and focal FV changes. The mean maxFV was significantly higher in patients (117.7 ± 21.6 cm/s) than in HI (72.45 ± 11.48 cm/s) (p<0.005). Only one patient had maxFV higher than 170 cm/s. The frequencies of asymmetry and of focal FV changes were significantly higher in SCA. Forty-one patients (73.2%) reported having headaches. Twenty-eight patients (50%) had severe (= 5 for pain intensity at a 1–10 scale) and frequent headaches (at least once a month). This group of patients presented TCD velocities significantly higher than patients without or with milder headaches (p=0.035). In conclusion, TCD maxFV was significantly higher in adult patients with SCA than HI, however, only one patient was considered at risk of stroke according to TCD criteria described in children. FV asymmetry and focal FV changes may be markers for arterial disease in adult SCA patients, and need to be further confirmed by neuroimaging and clinical follow up studies. The patients with severe headaches presented TCD velocities significantly higher than patients without or with milder headaches, but this finding needs to be confirmed by more and larger studies.

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