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chronic pulmonary complications of sickle cell disease

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https://www.readbyqxmd.com/read/29438213/new-no-donor-ncx-1443-therapeutic-effects-on-pulmonary-hypertension-in-the-sad-mouse-model-of-sickle-cell-disease
#1
Shariq Abid, Kanny Kebe, Amal Houssaïni, Françoise Tomberli, Elisabeth Marcos, Emilie Bizard, Marielle Breau, Aurelien Parpaleix, Claire-Marie Tissot, Bernard Maitre, Larissa Lipskaia, Genevieve Derumeaux, Elena Bastia, Armand Mekontso-Dessap, Serge Adnot
Nitric oxide (NO) donors may be useful for treating pulmonary hypertension (PH) complicating sickle cell disease (SCD), as endogenous NO is inactivated by hemoglobin released by intravascular hemolysis. Here, we investigated the effects of the new NO donor NCX1443 on PH in transgenic SAD mice, which exhibit mild SCD without severe hemolytic anemia. In SAD and wild-type (WT) mice, the pulmonary pressure response to acute hypoxia was similar and was abolished by 100 mg/kg NCX1443. The level of PH was also similar in SAD and WT mice exposed to chronic hypoxia (9% O2) alone or with SU5416, and was similarly reduced by daily NCX1443 gavage...
February 8, 2018: Journal of Cardiovascular Pharmacology
https://www.readbyqxmd.com/read/29377071/how-i-manage-red-cell-transfusions-in-patients-with-sickle-cell-disease
#2
David C Rees, Susan Robinson, Jo Howard
Sickle cell disease is one of the commonest serious inherited diseases in the world, and red cell transfusion is still one of the few effective treatments for acute and chronic complications. Transfusion corrects anaemia and dilutes out the number of red cells able to cause vaso-occlusion and vascular damage. Urgent red cell transfusions are used to correct acute anaemia, treat acute chest syndrome and patients with acute neurological symptoms. We use elective transfusions preoperatively for moderate risk surgery, and in some pregnant women...
January 29, 2018: British Journal of Haematology
https://www.readbyqxmd.com/read/29319041/successful-pulmonary-thromboendarterectomy-in-a-patient-with-sickle-cell-disease-and-associated-resolution-of-a-leg-ulcer
#3
Abhinav Agrawal, Rakesh Shah, Matthew D Bacchetta, Arunabh Talwar
Pulmonary hypertension (PH) is a relatively frequent and severe complication of sickle cell disease (SCD). PH associated with SCD is classified as Group 5 PH. The exact pathogenesis of PH in SCD in not known. There are also very limited treatment options available at this time for such patients with Group 5 PH. Patients with SCD are predisposed to a hypercoagulable state and thus can also suffer from chronic thromboembolism. These patients can have associated chronic thromboembolic pulmonary hypertension (CTEPH), thus being classified as Group 4 PH...
January 2018: Lung India: Official Organ of Indian Chest Society
https://www.readbyqxmd.com/read/29279787/acute-chest-syndrome-in-children-with-sickle-cell-disease
#4
REVIEW
Shilpa Jain, Nitya Bakshi, Lakshmanan Krishnamurti
Acute chest syndrome (ACS) is a frequent cause of acute lung disease in children with sickle cell disease (SCD). Patients may present with ACS or may develop this complication during the course of a hospitalization for acute vaso-occlusive crises (VOC). ACS is associated with prolonged hospitalization, increased risk of respiratory failure, and the potential for developing chronic lung disease. ACS in SCD is defined as the presence of fever and/or new respiratory symptoms accompanied by the presence of a new pulmonary infiltrate on chest X-ray...
December 1, 2017: Pediatric Allergy, Immunology, and Pulmonology
https://www.readbyqxmd.com/read/29222288/cardiovascular-complications-in-patients-with-sickle-cell-disease
#5
REVIEW
Mark T Gladwin
Sickle cell disease (SCD) is an autosomal recessive disease in which homozygosity for a single point mutation in the gene encoding the β-globin chain produces hemoglobin S molecules that polymerize within the erythrocyte during deoxygenation; the result is sustained hemolytic anemia and vaso-occlusive events. As patients live to adulthood, the chronic impact of sustained hemolytic anemia and episodic vaso-occlusive episodes leads to progressive end-organ complications. This scenario culminates in the development of 1 or more major cardiovascular complications of SCD for which there are no approved or consensus therapies...
December 8, 2017: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/28919009/distinct-patterns-of-response-to-transfusion-therapy-for-different-chronic-complications-of-sickle-cell-disease-a-useful-insight
#6
Dimitris A Tsitsikas, Funmilayo Orebayo, Alexandra Agapidou, Roger J Amos
Two main sub-phenotypes have been described in sickle cell disease: one with higher baseline haemoglobin and a higher rate of painful crises and one with lower baseline haemoglobin, increased markers of haemolysis and a higher incidence of pulmonary hypertension, priapism and leg ulcers. We compared the patterns of response to regular automated red cell exchange transfusion over a five-year period of 21 patients with recurrent painful crises from the first group and 3 patients with pulmonary hypertension and 5 with recurrent severe stuttering priapism form the second and found them to be distinctly different...
August 18, 2017: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/28672087/interventions-for-chronic-kidney-disease-in-people-with-sickle-cell-disease
#7
REVIEW
Noemi Ba Roy, Patricia M Fortin, Katherine R Bull, Carolyn Doree, Marialena Trivella, Sally Hopewell, Lise J Estcourt
BACKGROUND: Sickle cell disease (SCD) is one of the commonest severe monogenic disorders in the world, due to the inheritance of two abnormal haemoglobin (beta-globin) genes. SCD can cause severe pain, significant end-organ damage, pulmonary complications, and premature death. Kidney disease is a frequent and potentially severe complication in people with SCD.Chronic kidney disease is defined as abnormalities of kidney structure or function, present for more than three months. Sickle cell nephropathy refers to the spectrum of kidney complications in SCD...
July 3, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28248201/intravascular-hemolysis-and-the-pathophysiology-of-sickle-cell-disease
#8
REVIEW
Gregory J Kato, Martin H Steinberg, Mark T Gladwin
Hemolysis is a fundamental feature of sickle cell anemia that contributes to its pathophysiology and phenotypic variability. Decompartmentalized hemoglobin, arginase 1, asymmetric dimethylarginine, and adenine nucleotides are all products of hemolysis that promote vasomotor dysfunction, proliferative vasculopathy, and a multitude of clinical complications of pulmonary and systemic vasculopathy, including pulmonary hypertension, leg ulcers, priapism, chronic kidney disease, and large-artery ischemic stroke. Nitric oxide (NO) is inactivated by cell-free hemoglobin in a dioxygenation reaction that also oxidizes hemoglobin to methemoglobin, a non-oxygen-binding form of hemoglobin that readily loses heme...
March 1, 2017: Journal of Clinical Investigation
https://www.readbyqxmd.com/read/28094851/blood-transfusion-for-preventing-primary-and-secondary-stroke-in-people-with-sickle-cell-disease
#9
REVIEW
Lise J Estcourt, Patricia M Fortin, Sally Hopewell, Marialena Trivella, Winfred C Wang
BACKROUND: Sickle cell disease is one of the commonest severe monogenic disorders in the world, due to the inheritance of two abnormal haemoglobin (beta globin) genes. Sickle cell disease can cause severe pain, significant end-organ damage, pulmonary complications, and premature death. Stroke affects around 10% of children with sickle cell anaemia (HbSS). Chronic blood transfusions may reduce the risk of vaso-occlusion and stroke by diluting the proportion of sickled cells in the circulation...
January 17, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/27642150/-pulmonary-complications-of-sickle-cell-disease-in-children
#10
T Pincez, L Calamy, Z Germont, A Lemoine, A-A Lopes, A Massiot, J Tencer, C Thivent, A Hadchouel
Acute and chronic pulmonary complications are frequent in sickle cell disease (SCD), with different spectrum and characteristics in children and adults. Chronic hypoxia is frequent and plays a role in several respiratory complications in SCD. Furthermore, hypoxia has been associated with a higher risk of cerebral ischemia. Despite differing oxygen affinity between hemoglobin A and S, standard pulse oximetry was shown to be accurate in diagnosing hypoxia in SCD patients. Whereas acute hypoxia management is similar to non-SCD patients, chronic hypoxia treatment is mainly based on a transfusion program rather than long-term oxygen therapy...
October 2016: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/27582423/arterial-stiffness-impairment-in-sickle-cell-disease-associated-with-chronic-vascular-complications-the-multinational-african-cadre-study
#11
Brigitte Ranque, Aymeric Menet, Pierre Boutouyrie, Ibrahima Bara Diop, Samuel Kingue, Mamadou Diarra, Roland N'Guetta, Dapa Diallo, Saliou Diop, Ibrahima Diagne, Ibrahima Sanogo, Aissata Tolo, David Chelo, Guillaume Wamba, Jean Paul Gonzalez, Cochise Abough'elie, Cheick Oumar Diakite, Youssouf Traore, Gaëlle Legueun, Indou Deme-Ly, Blaise Felix Faye, Moussa Seck, Boidy Kouakou, Ismael Kamara, Sylvain Le Jeune, Xavier Jouven
BACKGROUND: Although a blood genetic disease, sickle cell disease (SCD) leads to a chronic vasculopathy with multiple organ involvement. We assessed arterial stiffness in SCD patients and looked for associations between arterial stiffness and SCD-related vascular complications. METHODS: The CADRE (Coeur Artères et Drepanocytose, ie, Heart Arteries and Sickle Cell Disease) study prospectively recruited pediatric and adult SCD patients and healthy controls in Cameroon, Ivory Coast, Gabon, Mali, and Senegal...
September 27, 2016: Circulation
https://www.readbyqxmd.com/read/27353687/cardiovascular-complications-and-risk-of-death-in-sickle-cell-disease
#12
REVIEW
Mark T Gladwin
In sickle-cell disease, a point mutation in the β-globin chain causes haemoglobin to polymerise within erythrocytes during deoxygenation, altering red blood cell rheology and causing haemolysis. Improvements in health infrastructure, preventive care, and clinical treatments have reduced the morbidity and mortality of sickle-cell disease in developed countries. However, as these patients live longer, the chronic effects of sustained haemolytic anaemia and episodic vaso-occlusive events drive the development of end-organ complications...
June 18, 2016: Lancet
https://www.readbyqxmd.com/read/27348613/delayed-hemolytic-transfusion-reaction-in-adult-sickle-cell-disease-presentations-outcomes-and-treatments-of-99-referral-center-episodes
#13
Anoosha Habibi, Armand Mekontso-Dessap, Constance Guillaud, Marc Michel, Keyvan Razazi, Mehdi Khellaf, Btissam Chami, Dora Bachir, Claire Rieux, Giovanna Melica, Bertrand Godeau, Frédéric Galacteros, Pablo Bartolucci, France Pirenne
Delayed hemolytic transfusion reaction (DHTR) is one of the most feared complications of sickle-cell disease (SCD). We retrospectively analyzed the clinical and biological features, treatments and outcomes of 99 DHTRs occurring in 69 referral center patients over 12 years. The first clinical signs appeared a median of 9.4 [IQR, 3-22] days after the triggering transfusion (TT). The most frequent DHTR-related clinical manifestation was dark urine/hemoglobinuria (94%). Most patients (89%) had a painful vaso-occlusive crisis and 50% developed a secondary acute chest syndrome (ACS)...
October 2016: American Journal of Hematology
https://www.readbyqxmd.com/read/27198469/regular-long-term-red-blood-cell-transfusions-for-managing-chronic-chest-complications-in-sickle-cell-disease
#14
REVIEW
Lise J Estcourt, Patricia M Fortin, Sally Hopewell, Marialena Trivella, Ian R Hambleton, Gavin Cho
BACKGROUND: Sickle cell disease is a genetic haemoglobin disorder, which can cause severe pain, significant end-organ damage, pulmonary complications, and premature death. Sickle cell disease is one of the most common severe monogenic disorders in the world, due to the inheritance of two abnormal haemoglobin (beta globin) genes. The two most common chronic chest complications due to sickle cell disease are pulmonary hypertension and chronic sickle lung disease. These complications can lead to morbidity (such as reduced exercise tolerance) and increased mortality...
May 20, 2016: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/27099528/the-role-of-the-arginine-metabolome-in-pain-implications-for-sickle-cell-disease
#15
REVIEW
Nitya Bakshi, Claudia R Morris
Sickle cell disease (SCD) is the most common hemoglobinopathy in the US, affecting approximately 100,000 individuals in the US and millions worldwide. Pain is the hallmark of SCD, and a subset of patients experience pain virtually all of the time. Of interest, the arginine metabolome is associated with several pain mechanisms highlighted in this review. Since SCD is an arginine deficiency syndrome, the contribution of the arginine metabolome to acute and chronic pain in SCD is a topic in need of further attention...
2016: Journal of Pain Research
https://www.readbyqxmd.com/read/27049871/endothelin-receptor-antagonists-in-sickle-cell-disease-a-promising-new-therapeutic-approach
#16
REVIEW
Brandon M Fox, Malgorzata Kasztan
Sickle cell disease (SCD) is a genetic hematologic disorder that is characterized by a variety of potentially life threatening acute and chronic complications. Currently, hydroxyurea is the only clinically approved pharmacological therapy for the treatment of SCD, and the continued prevalence of severe disease complications underscores the desperate need for the development of new therapeutic agents. Central features of the sickle cell disease milieu, including hypoxia, oxidative stress, and thrombosis, are established enhancers of endothelin-1 (ET-1) synthesis...
August 15, 2016: Life Sciences
https://www.readbyqxmd.com/read/26836905/chronic-pulmonary-complications-of-sickle-cell-disease
#17
REVIEW
Alem Mehari, Elizabeth S Klings
Sickle cell disease (SCD), the most common genetic hemolytic anemia worldwide, affects 250,000 births annually. In the United States, SCD affects approximately 100,000 individuals, most of African descent. Hemoglobin S (HbS) results from a glutamate-to-valine mutation of the sixth codon of the β-hemoglobin allele; the homozygous genotype (HbSS) is associated with the most prevalent and severe form of the disease. Other SCD genotypes include HbSC, composed of one HbS allele and one HbC (glutamate-to-lysine mutation) allele; and HbS-β-thalassemia(0) or HbS-β-thalassemia(+), composed of one HbS allele and one β-thalassemia allele with absent or reduced β-chain production, respectively...
May 2016: Chest
https://www.readbyqxmd.com/read/26765484/soluble-fas-fasl-ratio-as-a-marker-of-vasculopathy-in-children-and-adolescents-with-sickle-cell-disease
#18
Amira A Adly, Eman A Ismail, Nevine G Andrawes, Mai M Mahmoud, Rasha Eladawy
OBJECTIVES: Sickle cell disease (SCD) is characterized by chronic inflammation due to ischemic tissue damage, accentuated during acute complications. Fas and its ligand (FasL) are members of tumor necrosis factor receptor superfamily and a major pathway for induction of apoptosis. Fas/FasL interactions may be related to augmentation of inflammatory response. We assessed the levels of sFas and sFasL in 35 children and adolescents with SCD compared with 35 healthy controls in relation to hemolysis, iron overload, sickle vasculopathy including kidney disease...
March 2016: Cytokine
https://www.readbyqxmd.com/read/26348869/successful-matched-sibling-donor-marrow-transplantation-following-reduced-intensity-conditioning-in-children-with-hemoglobinopathies
#19
Allison A King, Naynesh Kamani, Nancy Bunin, Indira Sahdev, Joel Brochstein, Robert J Hayashi, Michael Grimley, Allistair Abraham, Jacqueline Dioguardi, Ka Wah Chan, Dorothea Douglas, Roberta Adams, Martin Andreansky, Eric Anderson, Andrew Gilman, Sonali Chaudhury, Lolie Yu, Jignesh Dalal, Gregory Hale, Geoff Cuvelier, Akshat Jain, Jennifer Krajewski, Alfred Gillio, Kimberly A Kasow, David Delgado, Eric Hanson, Lisa Murray, Shalini Shenoy
Fifty-two children with symptomatic sickle cell disease sickle cell disease (SCD) (N = 43) or transfusion-dependent thalassemia (N = 9) received matched sibling donor marrow (46), marrow and cord product (5), or cord blood (1) allografts following reduced intensity conditioning (RIC) with alemtuzumab, fludarabine, and melphalan between March 2003 and May 2014*. The Kaplan-Meier probabilities of overall and event-free survival at a median of 3.42 (range, 0.75-11.83) years were 94.2% and 92.3% for the group, 93% and 90...
December 2015: American Journal of Hematology
https://www.readbyqxmd.com/read/26286849/genetic-diminution-of-circulating-prothrombin-ameliorates-multiorgan-pathologies-in-sickle-cell-disease-mice
#20
Paritha I Arumugam, Eric S Mullins, Shiva Kumar Shanmukhappa, Brett P Monia, Anastacia Loberg, Maureen A Shaw, Tilat Rizvi, Janaka Wansapura, Jay L Degen, Punam Malik
Sickle cell disease (SCD) results in vascular occlusions, chronic hemolytic anemia, and cumulative organ damage. A conspicuous feature of SCD is chronic inflammation and coagulation system activation. Thrombin (factor IIa [FIIa]) is both a central protease in hemostasis and a key modifier of inflammatory processes. To explore the hypothesis that reduced prothrombin (factor II [FII]) levels in SCD will limit vaso-occlusion, vasculopathy, and inflammation, we used 2 strategies to suppress FII in SCD mice. Weekly administration of FII antisense oligonucleotide "gapmer" to Berkeley SCD mice to selectively reduce circulating FII levels to ∼10% of normal for 15 weeks significantly diminished early mortality...
October 8, 2015: Blood
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