Status of Sickle Cell Disease Among Tharu Population In Banke District of Nepal.

2021 ◽  
Vol 11 (5) ◽  
Author(s):  
Gupta Umesh Prasad ◽  
Bhandari Amrit ◽  
Giri Dhruba ◽  
Adhikari Sushmita ◽  
Paudel Sangita ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Sickle Cell Trait ◽  
Age Groups ◽  
Test Method ◽  
Beta Thalassemia ◽  
Compound Heterozygous ◽  
Cell Disease ◽  
Cross Sectional ◽  
Solubility Test

Sickle cell disease (SCD) is prevalent in malaria-endemic areas because the gene for sickle cell provides its carrier with resistance against malaria. In Nepal, malaria is prevalent in Terai, hence the susceptibility of SCD is high in this region. Being indigenous to the Terai, thousands of people in the Tharu communities of the Banke districting Nepal are believed to have suffered from sickle cell disease. The objective of this study was to find out the status of sickle cell disease among the Tharu population of Banke district, Nepal. A cross-sectional, experimental study was performed among systematically randomly selected 275 samples from 3 Village Development Committee (VDCs). All the samples were first screened for the presence of sickle hemoglobin using the sickle solubility test method in Bheri Zonal Hospital. Then all sickle solubility positive samples were further processed for alkaline hemoglobin electrophoresis by using Interlab GenioS electrophoresis instrument. Out of a total 275 samples, 33 (12.0%) samples were confirmed as sickle solubility test positive. Among which, sickle cell trait was the most common disorder found grossing to 81.8%, followed by homozygous sickle cell disease; (15.2 %). One case (3.0%) of compound heterozygous sickle beta-thalassemia was also found.The Males were found to be more affected than females with ratio of 1.4:1.1. The highest frequency of SCD was found to be in 11-20 age groups comprising about 36.4%. Dangaura Tharu (51.5%) was the most common ethnic group with this disorder. The findings of this study indicate SCD is prevalent among the Tharu population in Banke district of Province-5, Nepal

Sickle Cell Trait: A Benign State?

2016 ◽  
Vol 136 (3) ◽  
pp. 147-151 ◽  
Author(s):  
Taiwo R. Kotila
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Dna Analysis ◽  
Sickle Cell Trait ◽  
Beta Thalassemia ◽  
Compound Heterozygous ◽  
Cell Disease ◽  
Benign Condition ◽  
Red Cell Indices ◽  
Heterozygous Form

Background: Sickle cell trait (SCT) is the heterozygous form of sickle cell disease and expectedly should be a benign state with no complications ascribed to it. There are numerous reports challenging its being a benign condition, though this is controversial. Methods and Results: A review of the results of the accompanying investigations done on some of the patients show that beta thalassemia may be responsible for many of the ascribed symptoms and complications. These patients may therefore have sickle cell beta thalassemia, a compound heterozygous form of sickle cell disease. Conclusion: It is important to screen for beta thalassemia using red cell indices and quantitation of the different hemoglobin fractions before attributing any symptoms to SCT. DNA analysis, though useful in ascertaining the presence of the sickle cell gene, is not sufficient. There is the need to exclude the presence of mutations for beta thalassemia, which often is geographical region-specific.

scholarly journals Acute Sickle Hepatic Crisis after Liver Transplantation in a Patient with Hb SC Disease

2015 ◽  
Vol 2015 ◽  
pp. 1-3 ◽  
Author(s):  
J. H. Gillis ◽  
S. K. Satapathy ◽  
L. Parsa ◽  
P. B. Sylvestre ◽  
N. Dbouk
Keyword(s):  
Liver Transplantation ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Sickle Cell Trait ◽  
Complete Recovery ◽  
Beta Thalassemia ◽  
Low Grade ◽  
Cell Disease ◽  
First Case ◽  
Number Of Patients

Acute sickle hepatic crisis (ASHC) has been observed in approximately 10% of patients with sickle cell disease. It occurs predominantly in patients with homozygous (Hb SS) sickle cell anemia and to a lesser degree in patients with Hb SC disease, sickle cell trait, and Hb S beta thalassemia. Patients commonly present with jaundice, right upper quadrant pain, nausea, low-grade fever, tender hepatomegaly, and mild to moderate elevations in serum AST, ALT, and bilirubin. We describe the case of a patient with a history of hemoglobin SC disease and cirrhosis caused by hepatitis C presenting approximately 1 year after liver transplantation with an ASHC. The diagnosis was confirmed by liver biopsy. Our patient was treated with RBC exchange transfusions, IV hydration, and analgesia and made a complete recovery. Only a limited number of patients with sickle cell disease have received liver transplants, and, to our knowledge, this is the first case of ASHC after transplantation in a patient with Hb SC disease.

scholarly journals Transfusion and alloimmunization in sickle cell disease. The Cooperative Study of Sickle Cell Disease

Blood ◽  
1990 ◽  
Vol 76 (7) ◽  
pp. 1431-1437 ◽  
Author(s):  
WF Rosse ◽  
D Gallagher ◽  
TR Kinney ◽  
O Castro ◽  
H Dosik ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Age Groups ◽  
Beta Thalassemia ◽  
Lewis Antigens ◽  
Age Group ◽  
Cooperative Study ◽  
Cell Disease ◽  
Explicit Function ◽  
Hemoglobin Sc Disease

In 1,814 patients with sickle cell disease who had been transfused, the overall rate of alloimmunization to erythrocyte antigens was 18.6%. The rate of alloimmunization in this group appears to be an explicit function of the number of transfusions received because it increases exponentially with increasing numbers of transfusions. Alloimmunization usually occurred with less than 15 transfusions, although the rate of alloimmunization continued to increase when more transfusions were given. The rate of alloimmunization was less in patients with hemoglobin SC disease and sickle-beta+ thalassemia because these patients had received fewer transfusions. Children less than 10 years old had a slightly lower rate of alloimmunization than patients in other age groups even after correction for the number of transfusions given. Women were more frequently alloimmunized than men; this was largely due to the fact that women received more transfusions than men, but in the age group 16 to 20 years the increase may have been due in part to alloimmunization owing to pregnancy. Forty-five percent of those alloimmunized made antibodies of only one specificity; 17% made four or more antibodies reacting with different antigens. Antibodies to the C and E antigens of the Rh group, the Kell antigen, and the Lewis antigens were most commonly made. These findings may be important in formulating a rational transfusion policy in sickle cell disease.

scholarly journals Psychological Study of the Persons Suffering From Sickle Cell Disease in Raigarh District of Chhattisgarh State

2017 ◽  
Vol 4 (2) ◽  
Author(s):  
Dr. Rajesh Kumar Ajagallay ◽  
Dr. Gaukaran Janghel ◽  
Dr. Vimal Chandra Bhagat ◽  
Viyata Chanda ◽  
Dr. Rakesh Kumar Agrawal ◽  
...  
Keyword(s):  
Mental Health ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Age Groups ◽  
Emotional Stability ◽  
Two Dimensions ◽  
The Body ◽  
Cell Disease ◽  
Cross Sectional ◽  
Blood Disorder

Background: Sickle cell disease is an inherited blood disorder in which the body produces abnormal shaped red blood cells (RBC). The disease affects both biological and psychosocial aspects of patients. Aim: Present study aimed at investigating the different mental health dimensions used by heterozygous and homozygous sickle cell anemic patients. Method: The cross-sectional study design with the total 100 sickle cell anemic adolescents of both the sexes were selected in 10 to 20 year age groups, from various hospitals and health clinics of Chhattisgarh, India. The correlation analysis was used for analyzing the data. Results: Total 100 patients were selected which consisted of 30 homozygous and 70 heterozygous adolescent patients with sickle cell gene. The Emotional Stability was which higher significantly correlated with the intelligence quotients, IQ (r = .387, p< .001) than the other dimensions. Only two dimensions of mental health viz. emotional stability (t- 2.38; p<.018) and self-concept (t- 2.32; p<.001) of sickle cell patients which differed among heterozygous and homozygous patients.

scholarly journals Pregnancy outcome in women with sickle cell disease/trait in a tertiary care hospital Nagpur, Maharashtra India: a descriptive cross sectional study

2019 ◽  
Vol 8 (10) ◽  
pp. 3943
Author(s):  
Varsha Kose ◽  
Saurabh Kose
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Sickle Cell Trait ◽  
Cross Sectional Study ◽  
Common Complication ◽  
Sectional Study ◽  
Cell Disease ◽  
Cross Sectional ◽  
Disease Trait ◽  
Nicu Admission

Background: Pregnancy in sickle cell women has numerous obstetrical, nonobstetrical and fetal complications. Our objective was to study the pregnancy outcome in women with sickle cell disease/trait.Methods: A descriptive cross sectional study in 2 years study period was conducted. 57 women with sickle cell disease/trait were identified. They were attending the antenatal clinic and were admitted in obstetric ward and followed till 7 days after delivery.Results: Out of total 57 women 49 women (85.96%) were sickle cell trait (AS pattern) and 8 women (14.03%) were sickle cell disease (SS pattern). In women with sickle cell trait Pregnancy induced hypertension was the most common complication. Anaemia and hypothyroidism was the common associated medical problem. 5 babies need NICU admission, 6 babies PBU admission and 33 babies given to mother side just after birth. In women with sickle cell disease severe anaemia, preeclampsia, oligohydramnios and intrauterine growth restriction, lower segment caesarean section for preterm baby was the most common complication. 1 baby required NICU admission, 5 babies require PBU admission and only 2 babies given to mother just after delivery. There were no maternal mortality and neonatal mortality.Conclusions: Maternal morbidity and neonatal morbidity is more in sickle cell disease women. They require early diagnosis, premarital and preconceptional counselling, good multidisciplinary obstetrics and neonatal care and early referral to higher centre.

scholarly journals HbS-Sicilian (δβ)0-Thalassemia: A Rare Variant of Sickle Cell

2017 ◽  
Vol 2017 ◽  
pp. 1-3
Author(s):  
Grace Onimoe ◽  
Genine Smarzo
Keyword(s):  
Sickle Cell Disease ◽  
Rare Variant ◽  
Sickle Cell ◽  
Sickle Cell Trait ◽  
Globin Gene ◽  
Single Amino Acid ◽  
Beta Thalassemia ◽  
Cell Disease ◽  
Chromosome 11 ◽  
Sequencing Studies

Sickle cell disease (SCD) is caused by a mutation in the sixth codon of the β-globin gene on chromosome 11, which leads to a single amino acid substitution (glutamine to valine). Sickle-(δβ)0-thalassemia is a rare variant of sickle cell disease (delta-beta thalassemia occurring in association with sickle hemoglobin, HbS), sparsely reported in literature, and has been associated with symptomatology necessitating careful monitoring and follow-up. We describe a patient who presented with a newborn screen reported as “FS” and a negative family history for sickle cell disease and sickle cell trait. Subsequent gene sequencing studies demonstrated the presence of Sickle-(δβ)0-thalassemia. Clinical course has remained relatively stable for this patient now at 18 months of age without any SCD related symptomatology or complications. As this is a rare variant of SCD with potential complications, it is important to establish diagnosis towards planning comprehensive care.

scholarly journals Increased Prevalence of Alloimmunization in Sickle Cell Disease? Should We Restore Blood Donation in French Guiana?

2021 ◽  
Vol 8 ◽  
Author(s):  
Salomé Conrath ◽  
Vincent Vantilcke ◽  
Mickael Parisot ◽  
Françoise Maire ◽  
Pierre Selles ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Blood Cell ◽  
Sickle Cell ◽  
Red Blood Cell ◽  
French Guiana ◽  
Blood Donation ◽  
Cross Sectional Study ◽  
Compound Heterozygous ◽  
Cell Disease ◽  
Cross Sectional

Patients with sickle cell disease often undergo frequent blood transfusions. This increases their exposure to red blood cell alloantigens of donor units, thus making it more likely that they produce alloantibodies. This cross-sectional study aimed to describe the prevalence of allo-immunization in patients with sickle cell disease who were monitored at Cayenne Hospital in 2016. Of the 451 patients recruited during the study period, 238 (52.8%) were female. There were 262 (58.1%) homozygous sickle cell and 151 (33.5%) compound heterozygous sickle cell patients. The median age of the participants was 23.09 years (range, 0.5–68). We noted different red blood cell extended phenotypes: -in the Duffy system, the Fya- Fyb–profile was found in 299 patients (66%);—for the Kidd system, the most represented profile was Jka+ Jkb-, with 213 patients (47%). The Jka antigen was present in 355 patients;—in the MNS system, the S-s+ profile was found in 297 patients (66%);—the Lea antigen of the Lewis system was absent in 319 patients. The most frequent Rh phenotype in our patients was D+ C- E- c+ e+ K-, representing 51% of the patients. A total of 6,834 transfused packed red blood cell units were recorded. Sixty-eight patients (23%; 95% confidence interval, 20–25%) had detectable RBC alloantibodies. In multivariate logistic regression, only the mean number of single transfusions was statistically higher for the alloimmunized patients (p &lt; 0.04). Thirteen (19%) of the patients with alloimmunization developed a delayed hemolytic transfusion reaction, thus representing 4.4% of the total number of transfused patients. Whether differences between donors from France vs. recipients from French Guiana could explain this high prevalence of alloimmunization to be examined. In conclusion, careful transfusion strategies for patients with RBC alloantibodies should allow further reduction of the rate of alloimmunization.

scholarly journals Study of Prevalance of Nephropathy among Sickle Cell Disease Patients in Waghodia Region, Vadodara, Gujarat

2021 ◽  
pp. 607-613
Author(s):  
Charmi C. Thakkar ◽  
Inampudi Sailaja
Keyword(s):  
Renal Failure ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Sickle Cell Trait ◽  
Mean Value ◽  
Cell Disease ◽  
Protein Loss ◽  
Cross Sectional ◽  
Blood Disorder ◽  
The Mean

Introduction: Sickle-cell disease (or drepanocytosis) is a life-long blood disorder Characterized by red blood cells that assume an abnormal, rigid, sickle shape. Sickle cell disease (SCD) has several complications, including chronic renal failure, manifesting with hypertension (high blood pressure) proteinuria (protein loss in the urine), hematuria (redblood cells in urine) and worsening anaemia. Progression to end-stage renal failure confers a poor prognosis. Objective: The objective of the study was to determine the Prevalence of Nephropathy among sickle cell disease patients. Materials and Methods: This cross sectional study includes a total 150 participants who suffering from sickle cell anemia and attending our Institute. Renal function test and Urine examination of all participants was done. Estimated Glomerular Filtration Rate (eGFR) calculated using the Cockroft Gault formula. Comparison of results was done between Sickle cell trait and Sickle cell disease Group. Results: The mean age of the SCA patients were 25.54±10 years. Maximum participants are found to be from age group 25-30 yr(n=35) followed by 20-25 yr(n=30). Of the 150 SCA patients, 89 (59.33%), and 61 (40.66%) were males and females, respectively. The Mean value of S.Creatinine of SCT group is 0.73±0.46 mg/dl and SCD is 1.0±0.35 mg/dl, while the Mean value of eGFR is 134.19±87.21 ml/min and 124.20 ±58.25 ml/min in SCT and SCD Group respectively. Conclusions: From our study we conclude that the Derangement of Kidney function in sickle cell disease is frequent in our setting especially among young adult. It concerns SCD as well as SCT patients. Albuminuria is more frequent in homozygote patients and its prevalence increase with age. Age ≥ 25 years is associated with high risk of CKD in SCA group and albuminuria in SCD.

scholarly journals Transfusion and alloimmunization in sickle cell disease. The Cooperative Study of Sickle Cell Disease

Blood ◽  
1990 ◽  
Vol 76 (7) ◽  
pp. 1431-1437 ◽  
Author(s):  
WF Rosse ◽  
D Gallagher ◽  
TR Kinney ◽  
O Castro ◽  
H Dosik ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Age Groups ◽  
Beta Thalassemia ◽  
Lewis Antigens ◽  
Age Group ◽  
Cooperative Study ◽  
Cell Disease ◽  
Explicit Function ◽  
Hemoglobin Sc Disease

Abstract In 1,814 patients with sickle cell disease who had been transfused, the overall rate of alloimmunization to erythrocyte antigens was 18.6%. The rate of alloimmunization in this group appears to be an explicit function of the number of transfusions received because it increases exponentially with increasing numbers of transfusions. Alloimmunization usually occurred with less than 15 transfusions, although the rate of alloimmunization continued to increase when more transfusions were given. The rate of alloimmunization was less in patients with hemoglobin SC disease and sickle-beta+ thalassemia because these patients had received fewer transfusions. Children less than 10 years old had a slightly lower rate of alloimmunization than patients in other age groups even after correction for the number of transfusions given. Women were more frequently alloimmunized than men; this was largely due to the fact that women received more transfusions than men, but in the age group 16 to 20 years the increase may have been due in part to alloimmunization owing to pregnancy. Forty-five percent of those alloimmunized made antibodies of only one specificity; 17% made four or more antibodies reacting with different antigens. Antibodies to the C and E antigens of the Rh group, the Kell antigen, and the Lewis antigens were most commonly made. These findings may be important in formulating a rational transfusion policy in sickle cell disease.

scholarly journals Sickle cell disease and the adherence to guidelines for the use of blood transfusions in Duhok, Kurdistan, Iraq

2021 ◽  
Vol 25 (1) ◽  
pp. 486-492
Author(s):  
Fahad Jameel ◽  
Adnan Sadeeq
Keyword(s):  
Sickle Cell Disease ◽  
Blood Transfusion ◽  
Sickle Cell ◽  
Cross Sectional Study ◽  
Beta Thalassemia ◽  
Blood Transfusions ◽  
Cell Disease ◽  
Cross Sectional ◽  
Medical Visits ◽  
Transfusion Guidelines

Background and objective: One of the important health problems in Duhok city, Iraqi Kurdistan Region, is sickle cell disease. Blood transfusion remains a significant therapeutic intervention in patients with sickle cell disease that reduces complications related to vaso-occlusions. This study aimed to assess compliance to guidelines for the use of blood transfusionsin Duhok, Kurdistan, Iraq. Methods: This is a cross-sectional study that included 135 patients with sickle cell disease registered at Jeen center of pediatric hematological diseases in Duhok, Kurdistan, Iraq. Between April 1st and July 31st, 2019, 205 medical visits of sickle cell disease were registered. Every patient was evaluated to record the clinical setting and explanations behind visiting and indications for transfusion. Results: Of 135 patients,65.9% had sickle cell anemia (HbSS disease), 33.3% had sickle beta thalassemia, and one patient (0.7%) had Sickle/D disease. A total of 205 medical visits of sickle cell disease were registered with 84 blood transfusion decisions. The most common indicated guideline reasons for transfusion were symptomatic anemia and acute hemolytic crisis with a drop of hemoglobin >2 g/dl below steady state hemoglobin and severe painful crisis only accounted for 38.1%. Conclusion: In this study of patients with sickle cell disease, most blood transfusions were not indicated according to the transfusion guidelines (British Committee for Standards in Haematology – BCSH, 2017). Keywords: Transfusion guidelines for sickle cell disease (BCSH 2017); Sickle cell disease; Iraq.

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