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 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 Clinical events in the first decade in a cohort of infants with sickle cell disease. Cooperative Study of Sickle Cell Disease [see comments]

Blood ◽  
1995 ◽  
Vol 86 (2) ◽  
pp. 776-783 ◽  
Author(s):  
FM Gill ◽  
LA Sleeper ◽  
SJ Weiner ◽  
AK Brown ◽  
R Bellevue ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Clinical Course ◽  
The United States ◽  
Beta Thalassemia ◽  
Acute Chest Syndrome ◽  
Cooperative Study ◽  
Cell Disease ◽  
Splenic Sequestration ◽  
Clinical Events

Within the Cooperative Study of Sickle Cell Disease, 694 infants with confirmed sickle cell disease were enrolled at less than 6 months of age. Information about the nature and frequency of complications was collected prospectively over a 10-year period. Painful crises and acute chest syndrome were the most common sickle cell-related events in homozygous sickle cell anemia (SS), hemoglobin SC disease (SC), and S beta thalassemia patients (overall incidence in SS patients of 32.4 and 24.5 cases per 100 person-years, respectively). Bacteremia occurred most frequently in SS children under 4 years of age and in SC patients less than 2 years of age. The mortality rate was low in this cohort compared with that found in previous reports. Twenty children, all with Hb SS, died (1.1 deaths per 100 person-years among SS patients). Infection, most commonly with Streptococcus pneumoniae and Hemophilus influenzae, caused 11 deaths. Two children died of splenic sequestration, 1 of cerebrovascular accident, and 6 of unclear causes. Two patients underwent cholecystectomies, and 17 underwent splenectomies after one or more splenic sequestration crises. The experience of this cohort should reflect closely the true clinical course of those children with Hb SS and Hb SC disease who are observed in sickle cell centers in the United States.

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

scholarly journals Antiphospholipid Antibodies in Sickle Cell Disease: A Systematic Review and Exploratory Meta-Analysis

2021 ◽  
Vol 27 ◽  
pp. 107602962110029
Author(s):  
Mira Merashli ◽  
Alessia Arcaro ◽  
Maria Graf ◽  
Matilde Caruso ◽  
Paul R. J. Ames ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Antiphospholipid Antibodies ◽  
Meta Analysis ◽  
Age Groups ◽  
Anticardiolipin Antibodies ◽  
Cell Disease ◽  
Pooled Prevalence ◽  
The Relationship

The relationship between antiphospholipid antibodies (aPL) and sickle cell disease (SCD) has never been systematically addressed. Our aim was to evaluate potential links between SCD and aPL in all age groups. EMBASE/PubMed was screened from inception to May 2020 and Peto odds ratios for rare events were calculated. The pooled prevalence (PP) of IgG anticardiolipin antibodies (aCL) was higher in individuals with SCD than in controls (27.9% vs 8.7%, P < 0.0001), that of IgM aCL was similar in the two groups (2.9% vs 2.7%); only individuals with SCD were positive for lupus anticoagulant (LA) (7.7% vs 0%, P < 0.0001). The PP of leg ulcers was similar between aPL positive and negative individuals (44% vs 53%) and between patients in acute crisis and stable patients (5.6% vs 7.3%). Reporting of aPL as a binary outcome and not as a titer precluded further interpretation. The results indicate that a prospective case-control study with serial measurements of a panel of aPL in SCD patients might be warranted, in order to understand further the possible pathogenic role of aPL in SCD.

scholarly journals Hematological and Biochemical Reference Ranges for the Population with Sickle Cell Disease at Steady State in Tanzania

Hemato ◽  
2022 ◽  
Vol 3 (1) ◽  
pp. 82-97
Author(s):  
Anna Daniel Fome ◽  
Raphael Z. Sangeda ◽  
Emmanuel Balandya ◽  
Josephine Mgaya ◽  
Deogratius Soka ◽  
...  
Keyword(s):  
Steady State ◽  
Sickle Cell Disease ◽  
General Population ◽  
Sickle Cell ◽  
Age Groups ◽  
Mean Corpuscular Hemoglobin ◽  
Reference Ranges ◽  
Cell Disease ◽  
Laboratory Reference ◽  
Males And Females

Hematological and biochemical reference values in sickle cell disease (SCD) are crucial for patient management and the evaluation of interventions. This study was conducted at Muhimbili National Hospital (MNH) in Dar es Salaam, Tanzania, to establish laboratory reference ranges among children and adults with SCD at steady state. Patients were grouped into five age groups and according to their sex. Aggregate functions were used to handle repeated measurements within the individual level in each age group. A nonparametric approach was used to smooth the curves, and a parametric approach was used to determine SCD normal ranges. Comparison between males and females and against the general population was documented. Data from 4422 patients collected from 2004–2015 were analyzed. The majority of the patients (35.41%) were children aged between 5–11 years. There were no significant differences (p ≥ 0.05) in mean corpuscular hemoglobin concentration (MCHC), lymphocytes, basophils, and direct bilirubin observed between males and females. Significant differences (p < 0.05) were observed in all selected parameters across age groups except with neutrophils and MCHC in adults, as well as platelets and alkaline phosphatase in infants when the SCD estimates were compared to the general population. The laboratory reference ranges in SCD at steady state were different from those of the general population and varied with sex and age. The established reference ranges for SCD at steady state will be helpful in the management and monitoring of the progress of SCD.

Age-related prescription medication utilization for the management of sickle cell disease among Texas Medicaid patients

2021 ◽  
Vol 17 (4) ◽  
pp. 301-310
Author(s):  
Nidhi Shukla, MS, MBA ◽  
Jamie C. Barner, PhD, FAACP, FAPhA ◽  
Kenneth A. Lawson, PhD, FAPhA ◽  
Karen L. Rascati, PhD
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Age Groups ◽  
Dual Therapy ◽  
Cell Disease ◽  
Opioid Use ◽  
Medication Utilization ◽  
Age Related ◽  
Nonopioid Analgesics ◽  
Chi Square Analysis

Introduction: Sickle cell disease (SCD) is associated with recurrent complications and healthcare burden. Although SCD management guidelines differ based on age groups, little is known regarding actual utilization of preventative (hydroxyurea) and palliative therapies (opioid and nonopioid analgesics) to manage complications. This study assessed whether there were age-related differences in SCD index therapy type and SCD-related medication utilization.Design and patients: Texas Medicaid prescription claims from September 1, 2011 to August 31, 2016 were retrospectively analyzed for SCD patients aged 2-63 years who received one or more SCD-related medications (hydroxyurea, opioid, or nonopioid analgesics).Outcome measures: The primary outcomes were SCD index drug type and medication utilization: hydroxyurea adherence, and days’ supply of opioid, and nonopioid analgesics. Chi-square, analysis of variance, and Kruskal–Wallis tests were used.Results: Index therapy percentages for included patients (N = 2,339) were the following: opioids (45.7 percent), nonopioids (36.6 percent), dual therapy-opioids and nonopioids (11.2 percent), and hydroxyurea (6.5 percent), and they differed by age-groups (χ2 = 243.0, p 0.0001). Hydroxyurea as index therapy was higher among children (2-12:9.1 percent) compared to adults (26-40:3.7 percent; 41-63:2.9 percent). Opioids as index therapy were higher among adults (18-25:48.0 percent; 26-40:54.9 percent; 41-63:65.2 percent) compared to children (2-12:36.6 percent). Mean hydroxyurea adherence was higher (p 0.0001) for younger ages, and opioid days’ supply was higher for older ages.Conclusions: Texas Medicaid SCD patients had low hydroxyurea utilization and adherence across all age groups. Interventions to increase the use of hydroxyurea and newer preventative therapies could result in better management of SCDrelated complications and reduce the frequency of pain crises, which may reduce the need for opioid use.

Perspective From the American Academy of Pediatrics

PEDIATRICS ◽  
1989 ◽  
Vol 83 (5) ◽  
pp. 913-914
Author(s):  
Neil A. Holtzman
Keyword(s):  
Sickle Cell Disease ◽  
American Academy ◽  
Sickle Cell ◽  
Second Phase ◽  
Pilot Program ◽  
Cooperative Study ◽  
Cell Disease ◽  
Pilot Studies ◽  
American Academy Of Pediatrics

Ten years ago, the initial report of the Committee on Genetics of the American Academy of Pediatrics was published, drawing heavily on the landmark report published 2 years earlier by the National Academy of Sciences. That report stated that new mass screening tests should not be implemented without pilot studies or facilities for follow-up. I would like to deal with the efficacy of screening as determined by pilot studies, the effectiveness of routine screening, and the importance of follow-up. The Cooperative Study of Sickle Cell Disease was essentially the first phase of a large pilot program that systematically demonstrated that sepsis, meningitis, and acute splenic sequestration occurred in young infants with sickle cell disease. Previous reports suggested, and the cooperative study corroborated, that without prior diagnosis many of these infants would die. The randomized trial of oral penicillin prophylaxis was the second phase of the pilot program, demonstrating that early treatment significantly reduced mortality. Thus, the results of this pilot program, conducted with support of the National Institutes of Health, documented that newborn screening for sickle cell disease satisfied a major criterion for screening: An intervention capable of reducing mortality would be efficacious if applied before the usual age of clinical diagnosis. The pilot program was conducted under ideal conditions. The findings are not enough to conclude that screening for sickle cell disease and other hemoglobinopathies will always be effective when performed routinely.

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