scholarly journals Outcomes of febrile events in pediatric patients with sickle cell anemia

2018 ◽  
Vol 65 (11) ◽  
pp. e27379 ◽  
Author(s):  
Krishnaveni Sirigaddi ◽  
Inmaculada Aban ◽  
Amelia Jantz ◽  
Brandi M. Pernell ◽  
Lee M. Hilliard ◽  
...  
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Pediatric Patients ◽  
Febrile Events

Population Pharmacokinetic and Exposure–Response Analyses of Prasugrel in Pediatric Patients with Sickle Cell Anemia

2017 ◽  
Vol 57 (2) ◽  
pp. 243-254 ◽  
Author(s):  
Brian A. Moser ◽  
Elizabeth S. LaBell ◽  
Emmanuel Chigutsa ◽  
Joseph A. Jakubowski ◽  
David S. Small
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Pediatric Patients ◽  
Population Pharmacokinetic ◽  
Exposure Response

scholarly journals Evaluation of caries-associated virulence of biofilms from Candida albicans isolated from saliva of pediatric patients with sickle-cell anemia

2014 ◽  
Vol 22 (6) ◽  
pp. 484-489 ◽  
Author(s):  
Fernanda Lourenção BRIGHENTI ◽  
Amanda Coelho MEDEIROS ◽  
Bruno Mello MATOS ◽  
Zulene Eveline Abreu RIBEIRO ◽  
Cristiane Yumi KOGA-ITO
Keyword(s):  
Candida Albicans ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Pediatric Patients

scholarly journals Evaluating risk factors for chronic kidney disease in pediatric patients with sickle cell anemia

2017 ◽  
Vol 32 (9) ◽  
pp. 1565-1573 ◽  
Author(s):  
Jeffrey D. Lebensburger ◽  
Gary R. Cutter ◽  
Thomas H. Howard ◽  
Paul Muntner ◽  
Daniel I. Feig
Keyword(s):  
Risk Factors ◽  
Chronic Kidney Disease ◽  
Kidney Disease ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Pediatric Patients

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.

Hydroxyurea Shows Clinical Benefit without Alteration of Fetal Hemoglobin, MCV, or Hemoglobin in Unselected Adult and Pediatric Patients with Sickle Cell Anemia

Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 2187-2187
Author(s):  
Crawford John Strunk ◽  
Biree Andemariam ◽  
Fredericka Sey ◽  
Fatimah Farooq ◽  
Rebekah Urbonya ◽  
...  
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Multicenter Study ◽  
Pediatric Patients ◽  
Laboratory Data ◽  
Fetal Hemoglobin ◽  
Adult Patients ◽  
Clinical Severity ◽  
Laboratory Studies ◽  
Laboratory Values

Abstract Introduction Sickle cell anemia is the most common single gene defect in the United States, affecting approximately 100,000 individuals (Hassel Am J Prev Med 2010). It is characterized by chronic hemolysis, unpredictable vaso-occlusive episodes (VOEs), and chronic organ damage leading to early death in patients affected by the disorder. Hydroxyurea, a small molecule chemotherapeutic agent, has been used to treat patients with severe sickle cell disease since 1984 (Brawley Ann Intern Med 2008). Two randomized controlled trials, the Multicenter Study of Hydroxyurea (Charache N Engl J Med 1995) in adults and the Baby HUG trial (Wang Lancet 2011) in children, showed that hydroxyurea reduced the number of VOEs and hospital admissions, while simultaneously increasing hemoglobin and fetal hemoglobin in patients with sickle cell anemia. The goal of this study was to determine the clinical effectiveness of hydroxyurea in reducing the number VOEs and hospitalizations in unselected patients with sickle cell anemia. Methods The CASIRE group is an international multi-institutional collaborative group evaluating the clinical severity of patients with sickle cell anemia through a validated questionnaire, chart review and laboratory studies. Patients were enrolled on the CASIRE study after informed consent and assent was obtained from either the parent or patient when appropriate. The study was approved at each participating institution's IRB. A questionnaire was answered by the parents and/or patient, and baseline and current laboratory studies were collected. Patients were stratified into those who were not on hydroxyurea, and those who were currently on hydroxyurea. Number of VOEs, admissions, baseline and current fetal hemoglobin, and change in hemoglobin and MCV were compared. Results There were 349 patients in this study (134 on hydroxyurea). Baseline laboratory data are reported in table 1. Hemoglobin level and MCV were not statistically different in patients prior to and after taking hydroxyurea (table 2). Fetal hemoglobin in adults increased 2.7 times baseline, whereas in children it was unchanged. All patients on hydroxyurea had a reduction of VOEs, ED visits and admissions compared to prior to hydroxyurea (see table 3). Table 1. Baseline laboratory data Baseline data Patients on Hydroxyurea Patients not on Hydroxyurea Pediatric Adult Pediatric Adult N 78 56 140 75 Age 10 26.9 8.6 28.3 Hemoglobin (g/dL) 8.7 9.7 9.39 9.4 MCV (fL) 91 91.5 79 86 Fetal Hemoglobin (%) 15.1 12.4 9.6 5 Table 2. Clinical data for patients on HU Patients on Hydroxyurea Pediatric (78) Adult (56) Dose of HU (mg/kg) 23.8 20.5 # doses missed/wk 1 1.55 Fetal Hemoglobin on HU (%) 14.5 13.8 D MCV from baseline (fL) +5.4 +0.1 D Hgb from baseline (g/dL) +0.23 +0.4 Table 3. Number of pain episodes in patients on HU. Prior to HU In last year on HU 2 tailed paired t test Pediatric patients (N = 78) # pain episodes/year 25 12.9 0.62 # requiring ED/year 2.66 1 0.93 # requiring admission/year 4.28 1.79 0.017 Adult patients (N = 56) # pain episodes/year 36.7 28.6 0.021 # requiring ED/year 5.7 2.4 0 # requiring admission/year 6.6 3.15 0.117 Conclusion The Multicenter Study of Hydroxyurea and the BABY HUG study showed that hydroxyurea is efficacious for patients with sickle cell anemia. No previous study has evaluated the effectiveness of hydroxyurea in clinical practice. Our study suggests that, although baseline and current laboratory values are similar in patients prior to versus after taking hydroxyurea, there was a clear reduction in the number of VOEs and admissions, similar to the Baby HUG and MSH studies. These results suggest that the reduction of VOEs could be the product of a generalized decrease in overall inflammation and hemolysis or increased nitric oxide production rather than an increase in fetal hemoglobin by itself. Reasons for the similarity in laboratory values could include the length of time patients have been on hydroxyurea or that hydroxyurea was not escalated to maximum tolerated dose. Another reason may be the degree of compliance of patients in a clinical setting. We noted that 1/3 of our pediatric and ½ of our adult patients missed at least 1 dose of hydroxyurea per week suggesting that even partial compliance with hydroxyurea may prove beneficial clinically. This study demonstrates that hydroxyurea is effective in reducing the number of VOEs and admissions for unselected patients with sickle cell anemia. Disclosures No relevant conflicts of interest to declare.

scholarly journals Risk Factors and Complications Among Pediatric Patients With Sickle Cell Anemia: A Single Tertiary Center Retrospective Study

Cureus ◽  
2021 ◽  
Author(s):  
Fatma Alzahrani ◽  
Anas M Fallatah ◽  
Fatimah M Al-Haddad ◽  
Shahad T Khayyat ◽  
Wasayf M AlMehmadi ◽  
...  
Keyword(s):  
Risk Factors ◽  
Retrospective Study ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Pediatric Patients ◽  
Tertiary Center

STUDIES ON CONGENITAL HEMOLYTIC SYNDROMES

PEDIATRICS ◽  
1959 ◽  
Vol 23 (3) ◽  
pp. 462-475
Author(s):  
Marion E. Erlandson ◽  
Irving Schulman ◽  
Carl H. Smith
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Pediatric Patients ◽  
Clinical Manifestations ◽  
Adult Patients ◽  
Present Series ◽  
Risk Of Infection ◽  
Laboratory Examination ◽  
Careful Observation ◽  
Compensation Index

Four adult patients were studied in whom classic congenital spherocytic anemia was evident clinically and by laboratory examination. Anemia, as determined by total volume of erythrocytes, was marked although concentrations of hemoglobin were only slightly low. Rates of destruction and production of erythrocytes were markedly accelerated. Six pediatric patients with minimal clinical manifestations but definite laboratory evidence of congenital spherocytosis were evaluated. Anemia, in terms of total volume of erythrocytes, was present in all patients despite normal concentrations of hemoglobin in some patients. A potential for future severe clinical manifestations was shown to be present in five of six children by the demonstration of marked hemolytic defects equivalent to the hemolytic defects present in adult patients with classic congenital spherocytic anemia. In the present series of patients with congenital spherocytosis in whom concentrations of hemoglobin were normal or slightly low, values for the total volume of erythrocytes and the compensation index were similar to values in patients with intermediate (homozygous) thalassemia and in many patients with sickle cell anemia. Abnormally low values for total volume of erythrocytes and compensation index were present in four patients in whom the rate of destruction of erythrocytes was less than six times normal. Rates of production of erythrocytes were less than six times normal in the majority of patients studied. This is contrary to a previous concept of the capacity for erythropoietic compensation in such patients. These values imply that some factor(s) which has not as yet been identified regulates the rate of erythropoiesis in these patients. Studies of four family groups of patients with congenital spherocytosis demonstrated the absence of any specific familial pattern of manifestations of the disease. It has been suggested that management of children with minimal symptoms resulting from the presence of this disease consists of careful observation, splenectomy being deferred until late in childhood to avoid a maximal risk of infection after splenectomy. It has also been suggested that splenectomy be performed before the age of 10 years in order to avoid the complication of gallstones, and that splenectomy be performed at an even earlier age if persistent severe symptoms are present. The existence of a truly mild hemolytic defect was demonstrated in only 1 of the 10 patients studied. It would seem that splenectomy is not indicated in this unusual patient.

scholarly journals Genetic mapping and exome sequencing identify 2 mutations associated with stroke protection in pediatric patients with sickle cell anemia

Blood ◽  
2013 ◽  
Vol 121 (16) ◽  
pp. 3237-3245 ◽  
Author(s):  
Jonathan M. Flanagan ◽  
Vivien Sheehan ◽  
Heidi Linder ◽  
Thad A. Howard ◽  
Yong-Dong Wang ◽  
...  
Keyword(s):  
Genetic Mapping ◽  
Association Study ◽  
Exome Sequencing ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Genetic Variants ◽  
Pediatric Patients ◽  
Genetic Component ◽  
Key Points

Key Points The complication of stroke is common in patients with SCA, and there is a genetic component. We have performed a large-association study to identify 2 genetic variants that protect patients with SCA from stroke.

Effect of chronic transfusion therapy on progression of neurovascular pathology in pediatric patients with sickle cell anemia

2011 ◽  
Vol 47 (2) ◽  
pp. 125-128 ◽  
Author(s):  
Sarah Bishop ◽  
M. Gisele Matheus ◽  
Miguel R. Abboud ◽  
Ian D. Cane ◽  
Robert J. Adams ◽  
...  
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Pediatric Patients ◽  
Transfusion Therapy
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