The McConnell Sign is Seen in Patients With Acute Chest Syndrome

2018 ◽  
Vol 37 (10) ◽  
pp. 2433-2437 ◽  
Author(s):  
John Bates McCutcheon ◽  
Pascha Schaffer ◽  
Matthew Lyon ◽  
Richard Gordon
Keyword(s):  
Acute Chest Syndrome ◽  
Mcconnell Sign

scholarly journals ECMO Therapy in Acute Chest Syndrome for Patients with Sickle Cell Disease: a Case Report and Literature Review

2021 ◽  
Author(s):  
Soi Avgeridou ◽  
Ilija Djordjevic ◽  
Anton Sabashnikov ◽  
Kaveh Eghbalzadeh ◽  
Laura Suhr ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Scientific Data ◽  
Acute Chest Syndrome ◽  
Data Sets ◽  
Limited Data ◽  
Cell Disease ◽  
Life Saving ◽  
Institutional Experience ◽  
Ecmo Therapy

AbstractExtracorporeal membrane oxygenation (ECMO) plays an important role as a life-saving tool for patients with therapy-refractory cardio-respiratory failure. Especially, for rare and infrequent indications, scientific data is scarce. The conducted paper focuses primarily on our institutional experience with a 19-year-old patient suffering an acute chest syndrome, a pathognomonic pulmonary condition presented by patients with sickle cell disease. After implementation of awake ECMO therapy, the patient was successfully weaned off support and discharged home 22 days after initiation of the extracorporeal circulation. In addition to limited data and current literature, further and larger data sets are necessary to determine the outcome after ECMO therapy for this rare indication.

scholarly journals Streptococcus pneumoniae and Its Virulence Factors H2O2 and Pneumolysin Are Potent Mediators of the Acute Chest Syndrome in Sickle Cell Disease

Toxins ◽  
2021 ◽  
Vol 13 (2) ◽  
pp. 157
Author(s):  
Joyce Gonzales ◽  
Trinad Chakraborty ◽  
Maritza Romero ◽  
Mobarak Abu Mraheil ◽  
Abdullah Kutlar ◽  
...  
Keyword(s):  
Streptococcus Pneumoniae ◽  
Sickle Cell Disease ◽  
Virulence Factors ◽  
Sickle Cell ◽  
Biological Activities ◽  
Acute Chest Syndrome ◽  
Antibiotics Resistance ◽  
Cell Disease ◽  
Antibiotic Drug ◽  
Tract Infections

Sickle cell disease (SCD) is one of the most common autosomal recessive disorders in the world. Due to functional asplenia, a dysfunctional antibody response, antibiotic drug resistance and poor response to immunization, SCD patients have impaired immunity. A leading cause of hospitalization and death in SCD patients is the acute chest syndrome (ACS). This complication is especially manifested upon infection of SCD patients with Streptococcus pneumoniae (Spn)—a facultative anaerobic Gram-positive bacterium that causes lower respiratory tract infections. Spn has developed increased rates of antibiotics resistance and is particularly virulent in SCD patients. The primary defense against Spn is the generation of reactive oxygen species (ROS) during the oxidative burst of neutrophils and macrophages. Paradoxically, Spn itself produces high levels of the ROS hydrogen peroxide (H2O2) as a virulence strategy. Apart from H2O2, Spn also secretes another virulence factor, i.e., the pore-forming exotoxin pneumolysin (PLY), a potent mediator of lung injury in patients with pneumonia in general and particularly in those with SCD. PLY is released early on in infection either by autolysis or bacterial lysis following the treatment with antibiotics and has a broad range of biological activities. This review will discuss recent findings on the role of pneumococci in ACS pathogenesis and on strategies to counteract the devastating effects of its virulence factors on the lungs in SCD patients.

Anti-IH Related Hemolytic Reaction in a Sickle Cell Patient and Further Management While Avoiding Transfusion

2020 ◽  
Vol 154 (Supplement_1) ◽  
pp. S165-S166
Author(s):  
N Yurtsever ◽  
K Wilson-Sandberg ◽  
J Dikeman ◽  
J Louie ◽  
K Fomani
Keyword(s):  
Sickle Cell ◽  
Clinical Symptoms ◽  
Acute Chest Syndrome ◽  
Reference Laboratory ◽  
Cold Temperatures ◽  
Donor Cells ◽  
Thermal Amplitude ◽  
Local Reference ◽  
Hemolytic Transfusion Reaction ◽  
Group B

Abstract Introduction/Objective H antigen is a precursor for A and B antigens and is mostly converted except for the O blood group, which has the highest amount of H antigen. I is present on all adult RBCs. Anti-IH is usually an IgM antibody active at cold temperatures, and rarely demonstrates a wide thermal amplitude and can cause a significant hemolytic transfusion reaction. Methods Data was collected from patient information and transfusion management systems. Results 38 year old female with sickle cell disease presented to the emergency room with dizziness, tachypnea and Hgb: 5.9 g/dl Hct:19.1%. Upon further review, patient chart showed that she had received an emergency RBC exchange transfusion 21 days prior to this admission for acute chest syndrome. She was B positive. The units for RBC exchange consisted of 5 group B and 1 group O units and an additional 1 group O unit later. All RBC units were matched for her phenotype; Rh, K, Duffy & Kidd, except anti-S which was ruled out. At the time of discharge, Hgb was 9.3 g/dl and Hct was 27.7%. The drop in Hgb between discharge and the present admission prompted a suspicion for delayed hemolytic reaction/hyperhemolysis. The sample sent to the local Reference Laboratory came back as follows: DAT/Coombs Positive, DAT C3 positive; positive for cold auto-anti-IH antibody. A thermal amplitude test indicated that the anti-IH was reactive at 30 C and therefore had the potential to be of clinical significance. Her Hgb continued to drop and 3 days later Hb=3.7 g/dl with instructions not to transfuse unless clinically emergent. With treatment of IVIG and steroids, reducing further blood draws and monitoring the patient for clinical symptoms only, her Hgb/Hct started to rise and the patient was discharged 4 days later with Hgb: 6.4 g/dl, and no symptoms of anemia. Conclusion Our case study is important in two ways: Firstly, it raises awareness of the severity of a cold autoantibody, i.e. anti-IH, with a wide thermal amplitude. Specifically, in this case, our attempt to provide phenotypically similar RBCs resulted in the destruction of all the type O donor cells as well as some of the B donor cells. Secondly, even with Hgb counts as low as 3.7, treating the patient and not the number proved to be better clinical practice. In conclusion, a good monitoring protocol for sickle cell patients is required to transfuse less and avoid serious complications.

scholarly journals To Give or Not to Give: RhD Immunoglobulin for an RHD*39 Pregnant Woman with Sickle Cell Disease

2021 ◽  
pp. 1-5
Author(s):  
Justin E. Juskewitch ◽  
Craig D. Tauscher ◽  
Sheila K. Moldenhauer ◽  
Jennifer E. Schieber ◽  
Eapen K. Jacob ◽  
...  
Keyword(s):  
Pregnant Woman ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Pregnancy Termination ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Childbearing Age ◽  
Procedural Complications ◽  
History Of ◽  
Chronic Hemolysis

Introduction: Patients with sickle cell disease (SCD) have repeated episodes of red blood cell (RBC) sickling and microvascular occlusion that manifest as pain crises, acute chest syndrome, and chronic hemolysis. These clinical sequelae usually increase during pregnancy. Given the racial distribution of SCD, patients with SCD are also more likely to have rarer RBC antigen genotypes than RBC donor populations. We present the management and clinical outcome of a 21-year-old pregnant woman with SCD and an RHD*39 (RhD[S103P], G-negative) variant. Case Presentation: Ms. S is B positive with a reported history of anti-D, anti-C, and anti-E alloantibodies (anti-G testing unknown). Genetic testing revealed both an RHD*39 and homozygous partial RHCE*ceVS.02 genotype. Absorption/elution testing confirmed the presence of anti-G, anti-C, and anti-E alloantibodies but could not definitively determine the presence/absence of an anti-D alloantibody. Ms. S desired to undergo elective pregnancy termination and the need for postprocedural RhD immunoglobulin (RhIG) was posed. Given that only the G antigen site is changed in an RHD*39 genotype and the potential risk of RhIG triggering a hyperhemolytic episode in an SCD patient, RhIG was not administered. There were no procedural complications. Follow-up testing at 10 weeks showed no increase in RBC alloantibody strength. Discussion/Conclusion: Ms. S represents a rare RHD*39 and partial RHCE*ceVS.02 genotype which did not further alloimmunize in the absence of RhIG administration. Her case also highlights the importance of routine anti-G alloantibody testing in women of childbearing age with apparent anti-D and anti-C alloantibodies.

Acute chest syndrome in children with sickle cell disease

1995 ◽  
Vol 62 (2) ◽  
pp. 201-205 ◽  
Author(s):  
H. A. Srair ◽  
J. A. Owa ◽  
H. A. Aman ◽  
M. A. Madan
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Acute Chest Syndrome ◽  
Cell Disease

scholarly journals Cholecystectomy in sickle cell disease patients: Is there more acute chest syndrome after laparoscopy? A case controlled study

2008 ◽  
Vol 6 (3) ◽  
pp. 220-223 ◽  
Author(s):  
B. Diarra ◽  
J. Roudié ◽  
A. Coulibaly ◽  
F. Ehua Somian ◽  
J.-B. Kanga-Miessan ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Controlled Study ◽  
Acute Chest Syndrome ◽  
Cell Disease

scholarly journals Pulmonary Thromboembolism in a Child with Sickle Cell Hemoglobin D Disease in the Setting of Acute Chest Syndrome

2013 ◽  
Vol 2013 ◽  
pp. 1-3 ◽  
Author(s):  
Hazel Villanueva ◽  
Sandeepkumar Kuril ◽  
Jennifer Krajewski ◽  
Aziza Sedrak
Keyword(s):  
Pulmonary Artery ◽  
Sickle Cell ◽  
Pulmonary Thromboembolism ◽  
Acute Chest Syndrome ◽  
Standard Management ◽  
Pulmonary Angiogram ◽  
Right Pulmonary Artery ◽  
Ct Pulmonary Angiogram ◽  
Complete Thrombosis ◽  
The Right

Introduction. Sickle cell hemoglobin D disease (HbSD) is a rare variant of sickle cell disease (SCD). Incidence of pulmonary thromboembolism (PE) and deep venous thrombosis (DVT) in children with HbSD is unknown. PE and DVT are known complications of SCD in adults but have not been reported in the literature in children with HbSD.Case Report. We are reporting a case of a 12-year-old boy with HbSD with acute chest syndrome (ACS) complicated by complete thrombosis of the branch of the right pulmonary artery and multiple small pulmonary artery emboli seen on computed tomography (CT) pulmonary angiogram and thrombosis of the right brachial vein seen on Doppler ultrasound. Our patient responded to treatment with anticoagulant therapy.Conclusion. There are no cases reported in children with HbSD disease presenting as ACS with pulmonary thromboembolism. We suggest that PE should be suspected in patients presenting with ACS who do not show improvement with standard management. CT pulmonary angiogram should be utilized for early diagnosis and appropriate management as there is no current protocol for management of PE/DVT in pediatric patients with SCD.

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