scholarly journals Longitudinal analysis of cardiac abnormalities in pediatric patients with sickle cell anemia and effect of hydroxyurea therapy

2021 ◽  
Author(s):  
Arushi Dhar ◽  
Tung Ming Leung ◽  
Abena Appiah-Kubi ◽  
Dorota Gruber ◽  
Banu Aygun ◽  
...  
Keyword(s):  
Young Adults ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Cardiac Remodeling ◽  
Left Ventricular ◽  
Ventricular Dilation ◽  
Left Ventricular Dilation ◽  
Children And Young Adults ◽  
Hydroxyurea Therapy ◽  
Reverse Cardiac Remodeling

Cardiac abnormalities such as left ventricular hypertrophy, dilation and pulmonary hypertension in sickle cell anemia, have been previously described. Hydroxyurea, a disease modifying therapy for sickle cell anemia, has been used for several decades. Longitudinal assessment of echocardiographic abnormalities in children and young adults with sickle cell anemia on hydroxyurea therapy is lacking. In this retrospective study, we aim to determine the prevalence of echocardiographic abnormalities in children and young adults with sickle cell anemia and to examine the effects of hydroxyurea on reverse cardiac remodeling. We reviewed the records of patients with sickle cell anemia who underwent routine cardiac screening at Cohen Children's Medical Center between 2010 and 2017, followed by retrospective longitudinal analysis of echocardiograms performed on patients receiving treatment with hydroxyurea. Data on a total of 100 patients with sickle cell anemia were analyzed; 60 (60%) were on hydroxyurea. Twenty-five (41.6%) of the patients on hydroxyurea had been treated for less than 1 year; these patients had a significantly greater prevalence of left ventricular dilation compared to those who had been on treatment for more than 1 year. Serial echocardiograms were then analyzed on patients receiving hydroxyurea. Left ventricular dilation and hypertrophy improved significantly with hydroxyurea treatment. Additionally, the left ventricular volume and mass correlated negatively with duration of treatment with hydroxyurea. Our study provides evidence that prolonged hydroxyurea therapy may lead to reverse cardiac remodeling. Future studies should attempt to follow this patient cohort for a longer duration.

scholarly journals Reduced fitness and abnormal cardiopulmonary responses to maximal exercise testing in children and young adults with sickle cell anemia

2015 ◽  
Vol 3 (4) ◽  
pp. e12338 ◽  
Author(s):  
Robert I. Liem ◽  
Madhuri Reddy ◽  
Stephanie A. Pelligra ◽  
Adrienne P. Savant ◽  
Bo Fernhall ◽  
...  
Keyword(s):  
Young Adults ◽  
Sickle Cell ◽  
Exercise Testing ◽  
Sickle Cell Anemia ◽  
Maximal Exercise ◽  
Children And Young Adults

scholarly journals Left ventricular hypertrophy in children, adolescents and young adults with sickle cell anemia

2015 ◽  
Vol 37 (5) ◽  
pp. 324-328 ◽  
Author(s):  
Gustavo Baptista de Almeida Faro ◽  
Osvaldo Alves Menezes-Neto ◽  
Geodete Santos Batista ◽  
Antônio Pereira Silva-Neto ◽  
Rosana Cipolotti
Keyword(s):  
Young Adults ◽  
Left Ventricular Hypertrophy ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Ventricular Hypertrophy ◽  
Left Ventricular ◽  
Adolescents And Young Adults

scholarly journals Children and Young Adults Admitted to a NYC Children's Hospital Had a Similar Rate of Severe COVID-19 Coagulopathy As That Reported in Older Adults

Blood ◽  
2020 ◽  
Vol 136 (Supplement 1) ◽  
pp. 27-28
Author(s):  
William Beau Mitchell ◽  
Jennifer G. Davila ◽  
Janine Keenan ◽  
Jenai Jackson ◽  
Adit Tal ◽  
...  
Keyword(s):  
Older Adults ◽  
Young Adults ◽  
Venous Thromboembolism ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Ventilatory Support ◽  
Children's Hospital ◽  
Children’S Hospital ◽  
Children And Young Adults ◽  
D Dimer

The coagulopathy associated with COVID-19 has not been previously described in children and young adults. We reviewed the clinical and laboratory characteristics of children and young adults admitted for COVID-19 to an urban Children's Hospital in New York City, focusing on coagulation and venous thromboembolism. Clinical and laboratory data were analyzed from 54 patients aged 2 months to 30 years treated by the Pediatric Hematology service at a single Children's Hospital between January 1 and May 31, 2020. Information was obtained from hospital records with IRB approval. There was a moderate male predominance, with 32 (59%) males and 22 (41%) females. There were 28 (52%) patients younger than 18 years and 26 (48%) patients 18 - 30 years old. 26% of patients identified as Black, and 57% as Hispanic/Latino, similar to the community demographics in the Bronx in the 2019 census. Obesity was the most prevalent comorbid condition, with 19 (35%) patients having BMI of 30 or higher. There were also 12 (22%) patients with sickle cell anemia. There were 28 (52%) patients in this cohort with severe and critical illness, as based on established criteria, and 25 (46%) patients required increased ventilatory support. This was defined by the need for > 5L nasal cannula, high-flow nasal cannula, non-rebreather, or intubation. 11 patients (20%) had documented venous thromboembolism (VTE). Four patients died of COVID-19 complications at ages 2 months, 11, 14 and 18 years old. The VTE rate was similar in those patients under 18 years of age (5 of 28, 18%) and those 18 - 30 years of age (6 of 26, 23%). Most (94%) patients had a D-Dimer > 0.5 (upper limit of normal) at admission and 57% developed peak D-Dimer > 5 ug/mL during their admission. Elevated D-dimer > 5 was a risk factor for VTE with 3 of 23 (13%) and 7 of 17 (41%) patients developing VTE with D-dimer < 5 and > 5, respectively (OR 4.7, p=0.042). Patients requiring increased ventilatory support had a 36% rate of VTE as compared to 1 of 28 (4%) of those without (OR 15.2, p=0.003). Six of 24 patients on prophylactic anticoagulation developed VTE. One patient developed a pulmonary embolism 10 days post discharge from the hospital. No patients on anti-Xa-based low molecular weight heparin prophylaxis developed VTE. None of 12 patients with sickle cell anemia developed VTE, had peak D-Dimer > 5 ug/mL or required increased ventilatory support. Hospitalized children and young adults with COVID-19 in our cohort developed a coagulopathy similar to that of older adults, characterized by elevated D-Dimer and high rate of VTE. This is in contrast to the published pediatric series out of China and Singapore that described mild illness and did not comment on VTE rates. Presence of elevated D-dimer or need for increased ventilatory support were significant risk factors for thrombosis. Patients with sickle cell anemia had a lower risk of VTE and less severe illness. Anti-Xa monitored thromboprophylaxis may aid in preventing or ameliorating the COVID-19 coagulopathy in children and young adults. Institutional anticoagulation guidelines were developed based on these observations. Disclosures Davila: Spire Learning: Speakers Bureau; ATHN: Other: Grant Funding. O'Brien:Bristol Myers Squibb: Consultancy, Membership on an entity's Board of Directors or advisory committees.

scholarly journals Enuresis Is a Common and Persistent Problem Among Children and Young Adults with Sickle Cell Anemia

Urology ◽  
2008 ◽  
Vol 72 (1) ◽  
pp. 81-84 ◽  
Author(s):  
Joshua J. Field ◽  
Paul F. Austin ◽  
Ping An ◽  
Yan Yan ◽  
Michael R. DeBaun
Keyword(s):  
Young Adults ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Persistent Problem ◽  
Children And Young Adults

scholarly journals Hydroxyurea for treatment of severe sickle cell anemia: a pediatric clinical trial

Blood ◽  
1996 ◽  
Vol 88 (6) ◽  
pp. 1960-1964 ◽  
Author(s):  
A Ferster ◽  
C Vermylen ◽  
G Cornu ◽  
M Buyse ◽  
F Corazza ◽  
...  
Keyword(s):  
Young Adults ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Clinical Course ◽  
Initial Dosage ◽  
Fetal Hemoglobin ◽  
Complete Disappearance ◽  
Long Term Effects ◽  
Biologic Effects ◽  
Children And Young Adults

Hydroxyurea (HU) enhances the synthesis of fetal hemoglobin (HbF) and can improve the clinical course of some adult patients with sickle cell anemia (SCA). In a randomized trial, we studied the biologic effects and the clinical benefit of HU in children and young adults with severe SCA. Twenty-five patients (median age, 9 years) were randomized to receive either HU (at the initial dosage of 20 mg/kg/d) or a placebo for 6 months and were then switched to the other arm for the next 6 months. Among the 22 evaluable patients (median age, 8 years), significant increases in HbF and mean corpuscular volume occurred during the HU treatment period. The white blood cell and reticulocytes counts decreased significantly, but these changes were not clinically relevant. Sixteen of 22 patients (73%) experienced a complete disappearance of events requiring hospitalization. The number of days of hospitalization as well as the number of hospitalizations for patients on HU, as compared with those for the patients receiving placebo, were significantly reduced. We conclude that treatment with HU in children and young adults is feasible, well-tolerated, and improves the clinical course of SCA. The long-term effects of HU require further investigation.

Effect of Body Size, Ponderosity, and Blood Pressure on Left Ventricular Growth in Children and Young Adults in the Bogalusa Heart Study

Circulation ◽  
1995 ◽  
Vol 91 (9) ◽  
pp. 2400-2406 ◽  
Author(s):  
Elaine M. Urbina ◽  
Samuel S. Gidding ◽  
Weihang Bao ◽  
Arthur S. Pickoff ◽  
Kaliope Berdusis ◽  
...  
Keyword(s):  
Blood Pressure ◽  
Young Adults ◽  
Body Size ◽  
Left Ventricular ◽  
Bogalusa Heart Study ◽  
Heart Study ◽  
Children And Young Adults

scholarly journals Methemoglobin measure in adult patients with sickle-cell anemia: influence of hydroxyurea therapy

2014 ◽  
Vol 50 (3) ◽  
Author(s):  
Marilia Rocha Laurentino ◽  
Teresa Maria de Jesus Ponte Carvalho ◽  
Talyta Ellen de Jesus dos Santos ◽  
Maritza Cavalcante Barbosa ◽  
Thayna Nogueira dos Santos ◽  
...  
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Adult Patients ◽  
Hydroxyurea Therapy
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