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Pulmonary Hypertension in Sickle Cell Anemia

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1. Pulmonary hypertension and right ventricular function in Nigerian children with sickle cell anaemia. Full Text available with Trip Pro

Pulmonary hypertension and right ventricular function in Nigerian children with sickle cell anaemia. Pulmonary hypertension (PH), a complication of sickle cell anaemia (SCA), results in considerable morbidity. This study aims to determine the prevalence and associations of echocardiography-suggested PH in children with SCA.We performed a cross-sectional comparative study involving 100 systematically sampled SCA subjects 3-14 y of age in their steady state with matched haemoglobin AA phenotype (...) controls. Clinical, laboratory and echocardiography data (including tricuspid regurgitation velocity [TRV], mean pulmonary arterial pressure [mPAP] and tricuspid annular plane systolic excursion [TAPSE]) were obtained from all patients. Statistical analyses were performed using SPSS version 22 (IBM, Armonk, NY, USA). A p-value <0.05 was considered statistically significant.Of the 100 SCA subjects studied, 22 (22%) had echocardiographic findings suggestive of PH compared with none in the controls

2019 Transactions of the Royal Society of Tropical Medicine & Hygiene

2. Pulmonary hypertension among 5 to 18 year old children with sickle cell anaemia in Nigeria. Full Text available with Trip Pro

Pulmonary hypertension among 5 to 18 year old children with sickle cell anaemia in Nigeria. Pulmonary hypertension (PHT) is a significant cause of mortality in patients with sickle cell disease (SCD). Few studies on PHT in SCD have been carried out in children. This study aimed to estimate the prevalence of PHT in children with sickle cell anaemia (SCA) and determine its clinical and laboratory correlates.In this cross sectional study, evaluation involved obtaining bio-data, history (...) and physical examination findings in 175 SCA subjects with haemoglobin genotype SS aged 5 to 18 years and 175 age and sex matched controls with haemoglobin genotype AA. PHT was determined using peak Tricuspid Regurgitant Velocity (TRV) obtained from echocardiography as a marker. Complete blood count (CBC), lactate dehydrogenase (LDH) assay, reticulocyte count, foetal haemoglobin (HbF) estimation as well as Human Immunodeficiency Virus (HIV) I and II, Hepatitis B Virus (HBV) and Hepatitis C Virus (HCV

2017 PLoS ONE

3. Pulmonary hypertension in Nigerian adults with sickle cell anemia Full Text available with Trip Pro

Pulmonary hypertension in Nigerian adults with sickle cell anemia Sickle cell anemia (SCA) is the commonest hemoglobinopathy and is associated with high morbidity and mortality. Pulmonary hypertension (PH) is reported to play a significant role in this regard. There is very limited literature on PH in SCA in Nigeria.The objectives of this study were to determine the prevalence of Doppler-derived PH in SCA, assess its influence on exercise capacity, and determine the correlates and predictors (...) of measures of estimated pulmonary pressure.A total of 92 SCA subjects had echocardiography and 6-minute self-paced walking exercise. PH was diagnosed by Doppler echocardiography on finding a tricuspid regurgitant velocity (TRV) of ≥2.5 m/s. The pulmonary flow profile was also assessed to estimate mean pulmonary arterial pressure (MPAP).Doppler-derived PH was detected in 23.9% of adults with SCA. The 6-minute walking distance (6MWD) was significantly lower in SCA adults with PH than in those without PH

2017 Vascular health and risk management

4. Neck circumference is independently associated with relative systemic hypertension in young adults with sickle cell anaemia Full Text available with Trip Pro

Neck circumference is independently associated with relative systemic hypertension in young adults with sickle cell anaemia A seemingly interesting observation in patients with sickle cell anaemia (SCA) is that they usually have lower systemic blood pressures (BP) and insulin resistance than persons in the general population in spite of chronic inflammation and vasculopathy. However, relative systemic hypertension (rHTN) has been linked to pulmonary hypertension, increased blood viscosity (...) and renal insufficiency, which could indicate a risk of developing cardiometabolic disorder (CMD) in SCA.We therefore hypothesized that neck circumference (NC) and CMD marker; triglyceride glucose (TyG) index would independently predict rHTN in young adults with SCA in steady state.We compared the anthropometrical, hematological, hemorheological and CMD markers between SCA patients with normal BP < 120/70 mmHg; nHTN, n = 65) and those with rHTN (BP ≥ 120/70 mmHg, n = 32).Our results showed that SCA

2018 Clinical hypertension

5. Pulmonary Hypertension in Sickle Cell Anemia

Pulmonary Hypertension in Sickle Cell Anemia Pulmonary Hypertension in Sickle Cell Anemia Toggle navigation Brain Head & Neck Chest Endocrine Abdomen Musculoskeletal Skin Infectious Disease Hematology & Oncology Cohorts Diagnostics Emergency Findings Procedures Prevention & Management Pharmacy Resuscitation Trauma Emergency Procedures Ultrasound Cardiovascular Emergencies Lung Emergencies Infectious Disease Pediatrics Neurologic Emergencies Skin Exposure Miscellaneous Abuse Cancer (...) Administration 4 Pulmonary Hypertension in Sickle Cell Anemia Pulmonary Hypertension in Sickle Cell Anemia Aka: Pulmonary Hypertension in Sickle Cell Anemia , Sickle Cell Anemia Related Pulmonary Hypertension From Related Chapters II. Epidemiology : Up to 30% of adults III. Symptoms IV. Signs Increased V. Imaging Right ventricular enlargement Tricuspid regiurgitation VI. Prognosis Increases mortality risk 10 fold VII. References Images: Related links to external sites (from Bing) These images are a random

2018 FP Notebook

6. Phosphodiesterase Type-5 Inhibitor Therapy in Sickle Cell People With Pulmonary Hypertension

: National Heart, Lung, and Blood Institute (NHLBI) Information provided by (Responsible Party): National Institutes of Health Clinical Center (CC) ( National Heart, Lung, and Blood Institute (NHLBI) ) Study Details Study Description Go to Brief Summary: Background: Sickle cell disease (SCD) is a common inherited blood disorder. Many people with SCD are at risk to get pulmonary hypertension (PH). PH means that the blood pressure in the blood vessels to the lungs is high, and is a serious disease (...) Posted: January 23, 2019 Last Verified: January 15, 2019 Layout table for additional information Studies a U.S. FDA-regulated Drug Product: No Studies a U.S. FDA-regulated Device Product: No Keywords provided by National Institutes of Health Clinical Center (CC) ( National Heart, Lung, and Blood Institute (NHLBI) ): Outcomes Assessment Sickle Cell Disease Additional relevant MeSH terms: Layout table for MeSH terms Hypertension Hypertension, Pulmonary Anemia, Sickle Cell Vascular Diseases

2018 Clinical Trials

7. Diagnosis, Risk Stratification, and Management of Pulmonary Hypertension of Sickle Cell Disease: An Official ATS Clinical Practice Guideline

distance; ANA = anti-nuclear antibody; CXR = chest X-ray; EKG = electrocardiogram; LFTs = liver function tests; mPAP = mean pulmonary artery pressure; NT-pro-BNP = N-terminal pro–brain natriuretic peptide; PAWP = pulmonary artery wedge pressure; PH = pulmonary hypertension; PVR = pulmonary vascular resistance; SCD = sickle cell disease; TRV = tricuspid regurgitant jet velocity. 1 Note: The use of the term screening refers to mortality risk assessment. Echocardiography should be performed while patients (...) Castro, Erika B. Rosenzweig, Namita Sood, Lewis Hsu, KevinC.Wilson,MarilynJ.Telen,LauraM.DeCastro,LakshmananKrishnamurti,MartinH.Steinberg,DavidB.Badesch, and Mark T. Gladwin; on behalf of the ATS Ad Hoc Committee on Pulmonary Hypertension of Sickle Cell Disease THISOFFICIALCLINICALPRACTICEGUIDELINE OF THEAMERICANTHORACICSOCIETY WAS APPROVED BY THEATSBOARD OFDIRECTORS,NOVEMBER2013.THESE GUIDELINES WERE ALSO ENDORSED BY THE AMERICAN COLLEGE OF CHEST PHYSICIANS,OCTOBER 2013, AND BY THE PULMONARY

2014 American Thoracic Society

9. Study to Evaluate Efficacy and Safety of Oral Treprostinil in Subjects With Pulmonary Hypertension (PH) Associated With Sickle Cell Disease (SCD)

to Share IPD: No Layout table for additional information Studies a U.S. FDA-regulated Drug Product: Yes Studies a U.S. FDA-regulated Device Product: No Keywords provided by United Therapeutics: Oral treprostinil 6-Minute Walk Distance PH sickle cell Additional relevant MeSH terms: Layout table for MeSH terms Hypertension Hypertension, Pulmonary Anemia, Sickle Cell Vascular Diseases Cardiovascular Diseases Lung Diseases Respiratory Tract Diseases Anemia, Hemolytic, Congenital Anemia, Hemolytic Anemia (...) Study to Evaluate Efficacy and Safety of Oral Treprostinil in Subjects With Pulmonary Hypertension (PH) Associated With Sickle Cell Disease (SCD) Study to Evaluate Efficacy and Safety of Oral Treprostinil in Subjects With Pulmonary Hypertension (PH) Associated With Sickle Cell Disease (SCD) - Full Text View - ClinicalTrials.gov Hide glossary Glossary Study record managers: refer to the if submitting registration or results information. Search for terms x × Study Record Detail Saved Studies Save

2017 Clinical Trials

10. Prostacyclin-analog therapy in sickle cell pulmonary hypertension. Full Text available with Trip Pro

-Oncology, Department of Medicine, University of Pittsburgh School of Medicine, PA, USA katogj@upmc.edu. eng ZIA HL006014 HL NHLBI NIH HHS United States Letter Research Support, N.I.H., Intramural 2017 01 25 Italy Haematologica 0417435 0390-6078 0 Antihypertensive Agents DCR9Z582X0 Epoprostenol IM Adolescent Adult Anemia, Sickle Cell complications diagnosis Antihypertensive Agents therapeutic use Blood Pressure drug effects Echocardiography Epoprostenol analogs & derivatives therapeutic use Female (...) Prostacyclin-analog therapy in sickle cell pulmonary hypertension. 28126964 2018 09 10 2018 11 13 1592-8721 102 5 2017 05 Haematologica Haematologica Prostacyclin-analog therapy in sickle cell pulmonary hypertension. e163-e165 10.3324/haematol.2015.131227 Weir Nargues A NA Cardiovascular and Pulmonary Branch, National Institutes of Health, Bethesda, MD. Saiyed Rehan R Hematology Branch, National Heart, Lung and Blood Institute, National Institutes of Health, Bethesda, MD. Alam Shoaib S

2017 Haematologica

11. Updated consensus statement on the diagnosis and treatment of pediatric pulmonary hypertension Full Text available with Trip Pro

S8 online), or cardiomyopathy with elevated left ventricular end-diastolic filling pressures. AVT, acute vasoreactivity testing; ASD, atrial septal defect; CHD, congenital heart disease; iNO, inhaled nitric oxide; PAH, pulmonary arterial hypertension; PDA, patent ductus arteriosus; PH, pulmonary hypertension; PHVD, pulmonary hypertensive vascular disease; pre-OP, preoperatively; PVR, pulmonary vascular resistance; PVRi, pulmonary vascular resistance index; Qp, pulmonary blood flow; Qs, systemic (...) ; CXR, chest X-ray; ECG, electrocardiogram; FC, functional class; HIV, human immunodeficiency virus; HPAH, heritable pulmonary arterial hypertension; HR, high resolution; IPAH, idiopathic pulmonary arterial hypertension; LFT, liver function test; MRI, magnetic resonance imaging; PCH, pulmonary capillary hemangiomatosis; PH, pulmonary hypertension; PHVD, pulmonary hypertensive vascular disease; PVOD, pulmonary veno-occlusive disease; VQ, ventilation/perfusion; WHO, World Health Organization. Modified

2019 International Society for Heart and Lung Transplantation

12. Pulmonary hypertension in chronic hemolytic anemias: Pathophysiology and treatment. (Abstract)

Pulmonary hypertension in chronic hemolytic anemias: Pathophysiology and treatment. Pulmonary hypertension has emerged as a major cause of morbidity and mortality in patients with hemoglobinopathies and chronic hemolytic anemias. These hematological diseases include - but are not limited to - sickle cell disease (SCD), thalassemia, paroxysmal nocturnal hematuria, and hereditary spherocytosis. Although most studies have been based on the use of echocardiography as a screening tool for pulmonary (...) hypertension as opposed to the gold standard of right heart catheterization for definitive diagnosis, the association between chronic hemolytic anemia and pulmonary hypertension is evident. Studies have shown that patients with SCD and a tricuspid regurgitant velocity (TRV) ≥ 2.5 m/sec are at increased risk of pulmonary hypertension and are at increased mortality risk. Additional markers of risk of pulmonary hypertension and increased mortality include a pro-BNP >160 pg/mL combined with a 6-min walk

2018 Respiratory medicine

13. Guidelines on autopsy practice: Autopsy in sickle cell disease and persons with sickle trait

are not clear. [Level of evidence: GPP.] 14 Examples of cause of death opinions/statements 1a. Acute cardio-respiratory failure 1b. Acute chest syndrome following painful crisis 1c. Sickle cell disease 1a. Anaemia 1b. Hepato-splenic sequestration 1c. Sickle cell disease 1a. Severe sepsis 1b. Pneumococcal bacteraemia 1c. Sickle cell disease 1a. Cardiopulmonary failure/cor pulmonale 1b. Chronic sickle pulmonary arteriopathy 1c. Sickle cell disease 1a. Subarachnoid haemorrhage 1b. Chronic sickle cerebral (...) 2000;342: 1855 1865. 11 De Castro LM, Jonassaint JC, Graham FL, Ashley-Koch A, Telen MJ. Pulmonary hypertension associated with sickle cell disease: clinical and laboratory endpoints and disease outcomes. Am J Haematol 2008;83:19 25. 12 Gladwin MT. Cardiovascular complications and risk of death in sickle cell disease. Lancet 2016;387:2565 2574. 13 Inusa B, Casale M, Booth C, Lucas SB. Subarachnoid haemorrhage and cerebral vasculopathy in a child with sickle cell anaemia. BMJ Case Rep 2014; doi:10.1136

2017 Royal College of Pathologists

14. Associations of α-thalassemia and BCL11A with stroke in Nigerian, United States, and United Kingdom sickle cell anemia cohorts Full Text available with Trip Pro

associations with clinical and laboratory variables than the individual genetic components in the University of Ibadan cohort (n=249). We then replicated our findings in SCA cohorts from the University of Illinois at Chicago (UIC)(n=260) and Walk-Treatment of Pulmonary Hypertension and Sickle cell disease with Sildenafil Therapy (Walk-PHaSST)(n=387). High-risk was associated with higher reticulocytes (15.0% vs. 7.8%, P=0.08) and stroke history (6% vs. 1%, P=0.02) than standard risk patients (...) Associations of α-thalassemia and BCL11A with stroke in Nigerian, United States, and United Kingdom sickle cell anemia cohorts Alpha-thalassemia and the BCL11A rs1427407 T allele are commonly observed in sickle cell anemia (SCA) patients and are associated with reduced hemolysis and higher hemoglobin F levels, respectively. We investigated whether a high-risk genetic profile, defined as SCA patients who did not inherit either α-thalassemia or the BCL11A rs1427407 T allele, had stronger

2017 Blood advances

15. Thrombospondin-1 Gene Polymorphism is Associated with Estimated Pulmonary Artery Pressure in Patients with Sickle Cell Anemia Full Text available with Trip Pro

of Pittsburgh, Pittsburgh, Pennsylvania. eng K23 HL112848 HL NHLBI NIH HHS United States R01 HL121386 HL NHLBI NIH HHS United States R01 HL133864 HL NHLBI NIH HHS United States R01 MD009162 MD NIMHD NIH HHS United States Clinical Trial Letter Research Support, N.I.H., Extramural 2017 02 03 United States Am J Hematol 7610369 0361-8609 0 Thrombospondin 1 0 thrombospondin-1, human IM Anemia, Sickle Cell genetics physiopathology Blood Pressure Female Humans Hypertension, Pulmonary genetics physiopathology Male (...) Thrombospondin-1 Gene Polymorphism is Associated with Estimated Pulmonary Artery Pressure in Patients with Sickle Cell Anemia 28033687 2017 11 29 2019 01 09 1096-8652 92 3 2017 03 American journal of hematology Am. J. Hematol. Thrombospondin-1 gene polymorphism is associated with estimated pulmonary artery pressure in patients with sickle cell anemia. E31-E34 10.1002/ajh.24635 Jacob Seethal A SA 0000-0003-2906-6102 Division of Hematology/Oncology, Children's Hospital of Pittsburgh, Pittsburgh

2017 American journal of hematology

16. Newborn screening for sickle cell disease

manifests only after birth, when the prenatally dominant Extract of final report S18-01 Version 1.0 Newborn screening for sickle cell disease (SCD) 25 July 2019 Institute for Quality and Efficiency in Health Care (IQWiG) - 2 - HbF is increasingly replaced by HbS [10]. From about the 3 rd month of life, the concentration of HbS is high enough to potentially cause symptoms. Haemolytic anaemia, high blood viscosity, and vaso-occlusion result in reduced oxygen supply to the tissues. Chronic damage to almost (...) leads to chronic haemolytic anaemia [7]. In addition, sickle cells are less flexible, which increases the viscosity of blood and results in recurrent and often painful vascular occlusion. The severity of SCD and the onset of symptoms and complications vary [1, 6, 8, 9]. Most of the haemoglobin formed in a foetus is foetal haemoglobin F (HbF), which does not consist of 2 a-globins and 2 ß-globins like HbA, but of 2 a-globins and 2 ?-globins; therefore, due to a mutation in the gene for ß-globin, SCD

2019 Institute for Quality and Efficiency in Healthcare (IQWiG)

17. Crizanlizumab, Voxelotor, and L-Glutamine for Sickle Cell Disease: Effectiveness and Value

significant acute complications such as acute chest syndrome, serious infections, stroke, renal necrosis, and priapism. 4 Chronic complications can emerge across multiple organs and include delayed puberty, avascular necrosis, skin ulcers, chronic pain, neurocognitive impairment, chronic kidney injury, pulmonary hypertension, cardiovascular disease, and can result in early mortality. 4 Resultant health care costs are high, with the total health system economic burden of SCD estimated at $2.98 billion per (...) of this report, which is solely the work of the ICER team and its affiliated researchers. For a complete list of stakeholders from whom we requested input, please visit: https://icer-review.org/material/sickle-cell-disease-stakeholder-list/ Expert Reviewers Ashley Valentine, MRes Co-Founder of Sick Cells Consultant for Emmaus Life Sciences, CRISPR Therapeutics, and Community Patient Advisory Board for Global Blood Therapeutics. Sick Cells receives sponsorship and advocacy grants from various industry

2020 California Technology Assessment Forum

18. Crizanlizumab, Voxelotor, and L-Glutamine for Sickle Cell Disease: Effectiveness and Value

significant acute complications such as acute chest syndrome, serious infections, stroke, renal necrosis, and priapism. 4 Chronic complications can emerge across multiple organs and include delayed puberty, avascular necrosis, skin ulcers, chronic pain, neurocognitive impairment, chronic kidney injury, pulmonary hypertension, cardiovascular disease, and can result in early mortality. 4 Resultant health care costs are high, with the total health system economic burden of SCD estimated at $2.98 billion per (...) of this report, which is solely the work of the ICER team and its affiliated researchers. For a complete list of stakeholders from whom we requested input, please visit: https://icer-review.org/material/sickle-cell-disease-stakeholder-list/ Expert Reviewers Ashley Valentine, MRes Co-Founder of Sick Cells Consultant for Emmaus Life Sciences, CRISPR Therapeutics, and Community Patient Advisory Board for Global Blood Therapeutics. Sick Cells receives sponsorship and advocacy grants from various industry

2020 California Technology Assessment Forum

19. Differences of microparticle patterns between sickle cell anemia and hemoglobin SC patients. Full Text available with Trip Pro

Differences of microparticle patterns between sickle cell anemia and hemoglobin SC patients. Sickle cell anemia (SCA) and hemoglobin SC (HbSC) disease are the two most common forms of sickle cell disease (SCD), a frequent hemoglobinopathy which exhibits a highly variable clinical course. Although high levels of microparticles (MPs) have been consistently reported in SCA and evidence of their harmful impact on the SCA complication occurrences have been provided, no data on MP pattern in HbSC (...) not detect any association between blood MP concentrations and the occurrence of painful vaso-occlusive crises, acute chest syndrome and pulmonary hypertension in both patient groups. We also demonstrated for the first time, that whatever the considered genotype, RBC-derived MPs exhibited higher externalized phosphatidylserine level and were larger than PLT-derived MPs.

2017 PLoS ONE

20. Clinical Practice Guideline for Screening and Management of High Blood Pressure in Children and Adolescents

. Thaker , Elaine M. Urbina , SUBCOMMITTEE ON SCREENING AND MANAGEMENT OF HIGH BLOOD PRESSURE IN CHILDREN This article has corrections. Please see: Abstract These pediatric hypertension guidelines are an update to the 2004 “Fourth Report on the Diagnosis, Evaluation, and Treatment of High Blood Pressure in Children and Adolescents.” Significant changes in these guidelines include (1) the replacement of the term “prehypertension” with the term “elevated blood pressure,” (2) new normative pediatric blood (...) of high blood pressure (BP) in children is largely derived from data from the NHANES and typically is based on a single BP measurement session. These surveys, conducted since 1988, indicate that there has been an increase in the prevalence of childhood high BP, including both HTN and elevated BP. , High BP is consistently greater in boys (15%–19%) than in girls (7%–12%). The prevalence of high BP is higher among Hispanic and non-Hispanic African American children compared with non-Hispanic white

2017 American Academy of Pediatrics

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