Definitions of the phenotypic manifestations of sickle cell disease

SK Ballas, S Lieff, LJ Benjamin… - American journal of …, 2010 - Wiley Online Library
Sickle cell disease (SCD) is a pleiotropic genetic disorder of hemoglobin that has profound
multiorgan effects. The low prevalence of SCD (∼ 100,000/US) has limited progress in …

Endothelial pathomechanisms in acute lung injury

NA Maniatis, A Kotanidou, JD Catravas… - Vascular pharmacology, 2008 - Elsevier
Acute lung injury (ALI) and its most severe extreme the acute respiratory distress syndrome
(ARDS) refer to increased-permeability pulmonary edema caused by a variety of pulmonary …

Outcome of sickle cell anemia: a 4-decade observational study of 1056 patients

DR Powars, LS Chan, A Hiti, E Ramicone, C Johnson - Medicine, 2005 - journals.lww.com
Based on a prospective cohort study of 1056 patients with sickle cell anemia (Hb SS)
initiated in 1959, we investigated the influence of calendar era, age, sex, and prior medical …

Extracellular hemin crisis triggers acute chest syndrome in sickle mice

S Ghosh, OA Adisa, P Chappa, F Tan… - The Journal of …, 2013 - Am Soc Clin Investig
The prevention and treatment of acute chest syndrome (ACS) is a major clinical concern in
sickle cell disease (SCD). However, the mechanism underlying the pathogenesis of ACS …

[PDF][PDF] Guideline on the management of acute chest syndrome in sickle cell disease.

J Howard, N Hart… - British journal of …, 2015 - sichelzellkrankheit.de
1Department of Haematology, Guy's and St Thomas' NHS Foundation Trust, 2Lane Fox
Respiratory Unit, St Thomas' Hospital, Guy's and St Thomas' NHS Foundation Trust …

[PDF][PDF] Guidelines on red cell transfusion in sickle cell disease. Part I: principles and laboratory aspects.

BA Davis, S Allard, A Qureshi, JB Porter… - British journal of …, 2017 - stgeorges.nhs.uk
1Whittington Health, 2Barts Health NHS Trust & NHS Blood and Transplant, London,
3Oxford University Hospitals NHS Foundation Trust, Oxford, 4University College London …

Pulmonary hypertension and cor pulmonale during severe acute chest syndrome in sickle cell disease

A Mekontso Dessap, R Leon, A Habibi… - American journal of …, 2008 - atsjournals.org
Rationale: Steady-state mild pulmonary hypertension is a risk factor for death in adults with
sickle cell disease. Acute pulmonary hypertension has been reported during exercise and …

Asthma in children with sickle cell disease and its association with acute chest syndrome

JM Knight-Madden, TS Forrester, NA Lewis… - Thorax, 2005 - thorax.bmj.com
Background: Pulmonary complications are a major cause of morbidity and mortality in sickle
cell disease (SCD). The relationship of asthma with SCD and acute chest syndrome (ACS) …

Nitric oxide-dependent generation of reactive species in sickle cell disease: actin tyrosine nitration induces defective cytoskeletal polymerization

M Aslan, TM Ryan, TM Townes, L Coward… - Journal of Biological …, 2003 - ASBMB
The intermittent vascular occlusion occurring in sickle cell disease (SCD) leads to ischemia-
reperfusion injury and activation of inflammatory processes including enhanced production …

Respiratory management of acute chest syndrome in children with sickle cell disease

B Ahmed, M Arigliani, A Gupta - European Respiratory …, 2024 - publications.ersnet.org
Acute chest syndrome (ACS) is a leading cause of respiratory distress and hospitalisation in
children with sickle cell disease (SCD). The aetiology is multifactorial and includes fat …