Rapidly progressive acute chest syndrome in individuals with sickle cell anemia: a distinct acute chest syndrome phenotype
Open accessAbstract
Current definitions of acute chest syndrome (ACS) in sickle cell anemia (SCA) do not account for rapid progression of respiratory compromise. In this two‐center retrospective cohort study, we tested the hypothesis that in children and adults with ACS and respiratory failure (≤24 hours after onset of respiratory symptoms) have a distinct ACS phenotype associated with multiorgan failure when compared to those with ACS that have a more subacute and protracted course. We identified 173 individuals (97 children <20 years and 76 adults ≥20 years) with SCA and at least one episode of ACS. Only one ACS episode was considered per individual. Rapidly progressive ACS occurred in 21% (n = 16) of adults, but only 2.1% (n = 2) of children. Compared to adults without rapidly progressive ACS, adults with rapidly progressive ACS more frequently developed acute kidney injury (68.8% vs. 3.3%, P < 0.001), hepatic dysfunction (75.0% vs. 15.0%, P < 0.001), altered mental status (43.8% vs. 11.7%, P < 0.001), multiorgan failure (93.8% vs. 10%, P < 0.001), and death (6.3% vs. 0%, P = 0.05). Clinical and laboratory covariates that were evaluable on the first day of respiratory symptoms were evaluated to identify predictors of rapidly progressive ACS. On multivariable analysis, decline in platelet count at presentation was the only predictor of rapidly progressive ACS [odds ratio 4.82 (95% CI 1.20–19.39), P = 0.027]. In conclusion, rapidly progressive ACS is a distinct phenotype that occurs more frequently in adults, is preceded by thrombocytopenia, and is associated with multiorgan failure. Am. J. Hematol. 91:1185–1190, 2016. © 2016 Wiley Periodicals, Inc.
The claims and statistics of this paper haven’t been checked yet.
Run the check — free, no account needed. It reads the open-access text and checks the sources it cites.
Integrity signals
Registry
Cites
Statistics
Claims the paper makes
The claims and statistics of this paper haven’t been checked yet.
Run the check — free, no account needed. It reads the open-access text and checks the sources it cites.
Claims other papers make about this one
Each is checked against this paper’s own text: does it say what the citing paper says it does?
Elevated initial platelet count and dramatic drops during vaso-occlusive crisis have been found to portend a poor prognosis in acute chest syndrome.
“Elevated initial platelet count and dramatic drops during VOC have been found to portend a poor prognosis in ACS (29, 208, 219).”
From Pathophysiology of Sickle Cell Disease (2019) · cites this paper as [29]
The Discussion (page unavailable) associates thrombocytopenia with poor ACS outcomes, and the results identify a decrease in platelet count at presentation as a predictor of rapidly progressive ACS. The paper does not establish that an elevated initial platelet count predicts poor prognosis; its platelet-drop finding concerns presentation with ACS, not specifically drops during vaso-occlusive crisis. (LLM, conf=0.87)
Experimental — accuracy not yet measured
Self-check
Percentages, sums and reported differences re-computed from the paper’s own numbers.
The self-check needs the paper’s full text.
Cites
Harris, P. A., Taylor, R., Thielke, R., Payne, J., Gonzalez, N., & Conde, J. G. (2009) · Journal of Biomedical Informatics · doi:10.1016/j.jbi.2008.08.010 · Cited by 51,384 · see citing works
Acute Kidney Injury Network: report of an initiative to improve outcomes in acute kidney injury
Mehta, R. L., Kellum, J. A., Shah, S. V., Molitoris, B. A., Ronco, C., Warnock, D. G., Levin, A., & the Acute Kidney Injury Network (2007) · Critical Care · doi:10.1186/cc5713 · Cited by 7,046 · see citing works
Mortality in sickle cell disease. Life expectancy and risk factors for early death
Platt, O. S., Brambilla, D. J., Rosse, W. F., Milner, P. F., Castro, O., Steinberg, M. H., & Klug, P. P. (1994) · New England Journal of Medicine · doi:10.1056/nejm199406093302303 · Cited by 3,320 · see citing works
Causes and Outcomes of the Acute Chest Syndrome in Sickle Cell Disease
Vichinsky, E. P., Neumayr, L. D., Earles, A. N., Williams, R., Lennette, E. T., Dean, D., Nickerson, B., Orringer, E., McKie, V., Bellevue, R., et al. (2000) · New England Journal of Medicine · doi:10.1056/nejm200006223422502 · Cited by 1,234 · see citing works
Population Estimates of Sickle Cell Disease in the U.S.
Hassell, K. L. (2010) · American Journal of Preventive Medicine · doi:10.1016/j.amepre.2009.12.022 · Cited by 1,214 · see citing works
Castro, O. L., Brambilla, D. J., Thorington, B. D., Reindorf, C. A., Scott, R., GILLETTE, P. N., Vera, J., & Levy, P. S. (1994) · Blood · doi:10.1182/blood.v84.2.643.643 · Cited by 805 · see citing works
Belcher, J. D., Chen, C., Nguyen, J., Milbauer, L., Abdulla, F., Alayash, A. I., Smith, A., Nath, K. A., Hebbel, R. P., & Vercellotti, G. M. (2014) · Blood · doi:10.1182/blood-2013-04-495887 · Cited by 695 · see citing works
The HELLP syndrome: Clinical issues and management. A Review
Haram, K., SVENDSEN, E. K., & Abildgaard, U. (2009) · BMC Pregnancy and Childbirth · doi:10.1186/1471-2393-9-8 · Cited by 632 · see citing works
Castro, O. L., Brambilla, D. J., Thorington, B. D., et al. (1994) · Blood · doi:10.1182/blood.v84.2.643.bloodjournal842643 · Cited by 615 · see citing works
Acute Chest Syndrome in Sickle Cell Disease: Clinical Presentation and Course
Vichinsky, E. P., Styles, L. A., Colangelo, L. H., Wright, E. C., Castro, O., Nickerson, B., & Disease, T. C. S. O. S. C. (1997) · Blood · doi:10.1182/blood.v89.5.1787 · Cited by 573 · cited 2× · see citing works
“ACS is more common in children; however, adults have a more severe clinical course and a mortality rate up to four times that of children [3,4].”
BackgroundDefinitions of the phenotypic manifestations of sickle cell disease
Ballas, S. K., Lieff, S., Benjamin, L. J., Dampier, C., Heeney, M. M., Hoppe, C. C., Johnson, C. S., Rogers, Z. R., Smith‐Whitley, K., Wang, W. C., et al. (2009) · American Journal of Hematology · doi:10.1002/ajh.21550 · Cited by 454 · see citing works
Villagra, J., Shiva, S., Hunter, L. A., Machado, R. F., Gladwin, M. T., & Kato, G. J. (2007) · Blood · doi:10.1182/blood-2006-12-061697 · Cited by 384 · see citing works
Influence of hemolysis on routine clinical chemistry testing
Lippi, G., Luca Salvagno, G., Montagnana, M., Brocco, G., & Cesare Guidi, G. (2006) · Clinical Chemistry and Laboratory Medicine (CCLM) · doi:10.1515/cclm.2006.054 · Cited by 344 · see citing works
MELD and Other Factors Associated with Survival after Liver Transplantation
Menon, K. V., Nyberg, S. L., Harmsen, W. S., DeSouza, N. F., Rosen, C. B. B., Krom, R. A. F., & Wiesner, R. H. (2004) · American Journal of Transplantation · doi:10.1111/j.1600-6143.2004.00433.x · Cited by 329 · see citing works
Extracellular hemin crisis triggers acute chest syndrome in sickle mice
Ghosh, S., Adisa, O. A., Chappa, P., Tan, F., Jackson, K. A., Archer, D. R., & Ofori-Acquah, S. F. (2013) · Journal of Clinical Investigation · doi:10.1172/jci64578 · Cited by 282 · see citing works
Complement activation by heme as a secondary hit for atypical hemolytic uremic syndrome
Frimat, M., Tabarin, F., Dimitrov, J. D., Poitou, C., Halbwachs‐Mecarelli, L., Frémeaux‐Bacchi, V., & Roumenina, L. T. (2013) · Blood · doi:10.1182/blood-2013-03-489245 · Cited by 250 · see citing works
Hassell, K. L., Eckman, J. R., & Lane, P. A. (1994) · The American Journal of Medicine · doi:10.1016/0002-9343(94)90136-8 · Cited by 219 · see citing works
"Acute chest syndrome" in adults with sickle cell anemia. Microbiology, treatment, and prevention
Charache, S. (1979) · Archives of Internal Medicine · doi:10.1001/archinte.139.1.67 · Cited by 200 · see citing works
Mechanisms of hemolysis‐associated platelet activation
Helms, C. C., Marvel, M., Zhao, W., Stahle, M. C., Vest, R., Kato, G. J., Lee, J. S., Christ, G. J., Gladwin, M. T., Hantgan, R. R., et al. (2013) · Journal of Thrombosis and Haemostasis · doi:10.1111/jth.12422 · Cited by 185 · see citing works
Atypical hemolytic uremic syndrome: what is it, how is it diagnosed, and how is it treated?
Nester, C., & Thomas, C. P. (2012) · Hematology · doi:10.1182/asheducation.v2012.1.617.3798924 · Cited by 112 · see citing works
Rib infarcts and acute chest syndrome in sickle cell diseases
Rucknagel, D. L., Rucknagel, D. L., Kalkinyak, K. A., & Gelfand, M. J. (1991) · The Lancet · doi:10.1016/0140-6736(91)92525-7 · Cited by 80 · see citing works
Vasculotoxic and Proinflammatory Effects of Plasma Heme: Cell Signaling and Cytoprotective Responses
Belcher, J. D., Nath, K. A., & Vercellotti, G. M. (2013) · ISRN Oxidative Medicine · doi:10.1155/2013/831596 · Cited by 54 · see citing works
Outcomes of Adult Patients With Sickle Cell Disease Admitted to the ICU
Cecchini, J., Lionnet, F., Djibré, M., Parrot, A., Stankovic, K., Girot, R., & Fartoukh, M. S. (2014) · Critical Care Medicine · doi:10.1097/ccm.0000000000000316 · Cited by 50 · see citing works
Anea, C. B., Lyon, M., Lee, I. A., Gonzales, J. N., Adeyemi, A., Falls, G., Kutlar, A., & Brittain, J. E. (2016) · American Journal of Hematology · doi:10.1002/ajh.24224 · Cited by 40 · see citing works
Increased activation of the alternative complement pathway in sickle cell disease
Chudwin, D. S., Korenblit, A. D., Kingzette, M., Artrip, S. G., & Rao, S. (1985) · Clinical Immunology and Immunopathology · doi:10.1016/0090-1229(85)90139-4 · Cited by 39 · see citing works
Vascular occlusion and infarction in sickle cell crisis and the sickle chest syndrome.
Athanasou, N. A., Hatton, C., McGee, J. O., & Weatherall, D. J. (1985) · Journal of Clinical Pathology · doi:10.1136/jcp.38.6.659 · Cited by 30 · see citing works
SICKLEMIA WITH MULTI-ORGAN FAILURE SYNDROME AND THROMBOTIC THROMBOCYTOPENIC PURPURA
Chehal, A. A., Taher, A. T., & Shamseddine, A. I. (2002) · Hemoglobin · doi:10.1081/hem-120016371 · Cited by 29 · see citing works
Thrombotic microangiopathy in sickle cell disease crisis
Shome, D. K., Ramadorai, P., Alajmi, A., Ali, F., & Malik, N. (2012) · Annals of Hematology · doi:10.1007/s00277-012-1647-3 · Cited by 27 · see citing works
Plasma exchange in critically ill patients with sickle cell disease
Boğa, C., Kozanoğlu, İ., Özdoğu, H., & Özyürek, E. (2007) · Transfusion and Apheresis Science · doi:10.1016/j.transci.2006.12.009 · Cited by 27 · see citing works
Multiorgan failure and bone marrow necrosis in three adults with sickle cell‐β+‐thalassemia
Adamski, J., Hanna, C. A., Reddy, V., Litovsky, S. H., Evans, C. A., & Marques, M. B. (2012) · American Journal of Hematology · doi:10.1002/ajh.23117 · Cited by 26 · see citing works
Thrombotic Thrombocytopenic Purpura and Sickle Cell Crisis
Shelat, S. G. (2008) · Clinical and Applied Thrombosis/Hemostasis · doi:10.1177/1076029608323804 · Cited by 24 · see citing works
Lee, H. E., Marder, V. J., Logan, L. J., Friedman, S., & Miller, B. J. (2003) · Annals of Hematology · doi:10.1007/s00277-003-0715-0 · Cited by 23 · see citing works
Vance, L. D., Rodeghier, M., Cohen, R. T., ROSEN, C. L., Kirkham, F. J., Strunk, R. C., & DeBaun, M. R. (2015) · American Journal of Hematology · doi:10.1002/ajh.23959 · Cited by 23 · see citing works
Self-citationGardner, K., & Thein, S. L. (2015) · American Journal of Hematology · doi:10.1002/ajh.24126 · Cited by 19 · see citing works
DeBaun, M. R. (2009) · American Journal of Hematology · doi:10.1002/ajh.21583 · Cited by 9 · see citing works
Self-citationVlachaki, E., Agapidou, A., Neokleous, N., Adamidou, D., Vetsiou, E., & Boura, P. (2014) · Transfusion and Apheresis Science · doi:10.1016/j.transci.2014.09.001 · Cited by 8 · see citing works
Age‐dependent pathophysiology of acute chest syndrome in children with sickle cell disease
Renella, R. (2015) · American Journal of Hematology · doi:10.1002/ajh.23975 · Cited by 4 · see citing works
Flagged first, then by citations
Cited by
Sorted by citations
Pathophysiology of Sickle Cell Disease
Sundd, P., Gladwin, M. T., & Novelli, E. M. (2019) · Annual Review of Pathology Mechanisms of Disease · doi:10.1146/annurev-pathmechdis-012418-012838 · Cited by 813 · see citing works
Acute Chest Syndrome in Children with Sickle Cell Disease
Jain, S., Bakshi, N., & Krishnamurti, L. (2017) · Pediatric Allergy Immunology and Pulmonology · doi:10.1089/ped.2017.0814 · Cited by 170 · see citing works
Vats, R., Brzóska, T., Bennewitz, M. F., Jimenez, M. A., Pradhan‐Sundd, T., Tütüncüoğlu, E., Jonassaint, J., Gutierrez, E., Watkins, S. C., Shiva, S., et al. (2019) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.201807-1370oc · Cited by 97 · see citing works
Tebbi, C. K. (2022) · Hemato · doi:10.3390/hemato3020024 · Cited by 89 · see citing works
Pecker, L. H., & Lanzkron, S. (2021) · Annals of Internal Medicine · doi:10.7326/aitc202101190 · Cited by 88 · see citing works
Ruhl, A. P., Sadreameli, S. C., Allen, J. L., Bennett, D. P., Campbell, A., Coates, T. D., Diallo, D. A., Field, J. J., Fiorino, E. K., Gladwin, M. T., et al. (2019) · Annals of the American Thoracic Society · doi:10.1513/annalsats.201906-433st · Cited by 57 · see citing works
Self-citationAcute chest syndrome in sickle cell disease
Farooq, S., Omar, M. A., & Salzman, G. A. (2018) · Hospital Practice · doi:10.1080/21548331.2018.1464363 · Cited by 54 · see citing works
Antibiotics for treating acute chest syndrome in people with sickle cell disease
Martí-Carvajal, A. J., Conterno, L. D. O., & Knight‐Madden, J. (2015) · Cochrane Database of Systematic Reviews · doi:10.1002/14651858.cd006110.pub4 · Cited by 42 · see citing works
Farrell, A. T., Panepinto, J. A., Desai, A. A., Kassim, A. A., Lebensburger, J. D., Walters, M. C., Bauer, D. E., Blaylark, R. M., DiMichele, D., Gladwin, M. T., et al. (2019) · Blood Advances · doi:10.1182/bloodadvances.2019000883 · Cited by 40 · see citing works
Self-citationHMOX1 and acute kidney injury in sickle cell anemia
Saraf, S. L., Viner, M., Rischall, A., Raslan, R., Shah, B. N., Zhang, X., Han, J., Gowhari, M., Jain, S., Molokie, R. E., et al. (2018) · Blood · doi:10.1182/blood-2018-05-853929 · Cited by 35 · see citing works
The platelet count and its implications in sickle cell disease patients admitted for intensive care
Shome, D. K., Jaradat, A., Almahozi, A., Sinan, A. S., Ebrahim, A., Alrahim, M., Ebraheem, M. S., Mansoor, E. J., Majed, K. S., & Pasha, S. A. A. (2018) · Indian Journal of Critical Care Medicine · doi:10.4103/ijccm.ijccm_49_18 · Cited by 33 · see citing works
P-selectin–deficient mice to study pathophysiology of sickle cell disease
Bennewitz, M. F., Tütüncüoğlu, E., Gudapati, S., Brzóska, T., Watkins, S. C., Monga, S. P., Pradhan‐Sundd, T., & Sundd, P. (2020) · Blood Advances · doi:10.1182/bloodadvances.2019000603 · Cited by 31 · see citing works
Health Supervision for Children and Adolescents With Sickle Cell Disease: Clinical Report
Yates, A. M., Aygün, B., Nuss, R., Rogers, Z. R., Hematology/Oncology, S. O., Wetmore, C., Dickens, D., Majumdar, S., Stoffman, J. M., Warwick, A. B., et al. (2024) · PEDIATRICS · doi:10.1542/peds.2024-066842 · Cited by 30 · see citing works
Acute chest syndrome of sickle cell disease: genetics, risk factors, prognosis, and management
Klings, E. S., & Steinberg, M. H. (2022) · Expert Review of Hematology · doi:10.1080/17474086.2022.2041410 · Cited by 29 · see citing works
Acute Chest Syndrome in Sickle Cell Disease
Bhasin, N., & Sarode, R. (2023) · Transfusion Medicine Reviews · doi:10.1016/j.tmrv.2023.150755 · Cited by 27 · see citing works
Recurrent Acute Chest Syndrome in Pediatric Sickle Cell Disease: Clinical Features and Risk Factors
Patterson, G. D., Mashegu, H., Rutherford, J., Seals, S. R., Josey, D., Karlson, C., McNaull, M., May, W., Carroll, C. M., Barr, F. E., et al. (2017) · Journal of Pediatric Hematology/Oncology · doi:10.1097/mph.0000000000001012 · Cited by 26 · see citing works
Louie, J. E., Anderson, C., Fomani, K., Henry, A., Killeen, T. F., Mohandas, N., Yazdanbakhsh, K., Belcher, J. D., Vercellotti, G. M., & Shi, P. A. (2017) · Transfusion · doi:10.1111/trf.14407 · Cited by 26 · see citing works
P‐selectin plays a role in haem‐induced acute lung injury in sickle mice
Ghosh, S., Flage, B., Weidert, F. L., & Ofori-Acquah, S. F. (2019) · British Journal of Haematology · doi:10.1111/bjh.15807 · Cited by 25 · see citing works
Mohammed, A., Podila, P. S. B., Davis, R. L., Ataga, K. I., Hankins, J. S., & Kamaleswaran, R. (2020) · Journal of Medical Internet Research · doi:10.2196/14693 · Cited by 21 · see citing works
Takahashi, T., Okubo, Y., Pereda, M. A., Handa, A., & Miller, S. (2018) · Pediatric Critical Care Medicine · doi:10.1097/pcc.0000000000001643 · Cited by 21 · see citing works
High risk and low prevalence diseases: Acute chest syndrome in sickle cell disease
Koehl, J. L., Koyfman, A., Hayes, B. D., & Long, B. (2022) · The American Journal of Emergency Medicine · doi:10.1016/j.ajem.2022.06.018 · Cited by 19 · see citing works
Sustainability of low maternal mortality in pregnant women with SCD in a low-resource setting
Swarray‐Deen, A., Asare, E. V., Brew, R. A., Ayete-Nyampong, J. B., Dei‐Adomakoh, Y., Olayemi, E., Mensah, E., Osei‐Bonsu, Y., Crabbe, S., Ghunney, W. K., et al. (2022) · Blood Advances · doi:10.1182/bloodadvances.2021005942 · Cited by 18 · see citing works
Self-citationAntibiotics for treating acute chest syndrome in people with sickle cell disease
Martí-Carvajal, A. J., Conterno, L. D. O., & Knight‐Madden, J. (2019) · Cochrane Database of Systematic Reviews · doi:10.1002/14651858.cd006110.pub5 · Cited by 16 · see citing works
Brzóska, T., Vats, R., Bennewitz, M. F., Tütüncüoğlu, E., Watkins, S. C., Ragni, M. V., Neal, M. D., Gladwin, M. T., & Sundd, P. (2020) · JCI Insight · doi:10.1172/jci.insight.139437 · Cited by 15 · see citing works
Razazi, K., Gendreau, S., Cuquemelle, É., Khellaf, M., Guillaud, C., Godeau, B., Melica, G., Moutereau, S., Gomart, C., Fourati, S., et al. (2020) · Journal of Clinical Medicine · doi:10.3390/jcm9113718 · Cited by 13 · see citing works
Noun, P., Ibrahim, A., Hodroj, M. H., Bou‐Fakhredin, R., & Taher, A. T. (2020) · Expert Review of Hematology · doi:10.1080/17474086.2020.1819785 · Cited by 13 · see citing works
Alghamdi, F. A., Al-Kasim, F., Alshhada, F., Ghareeb, E., Azmet, F. R., Almudaibigh, A. H., Baitalmal, L. A., Alnawfal, B. M., & Alluqmani, R. (2024) · Scientific Reports · doi:10.1038/s41598-023-48527-1 · Cited by 11 · see citing works
Inflammatory pathways and anti‐inflammatory therapies in sickle cell disease
Tozatto‐Maio, K., Rós, F. A., Weinlich, R., & Rocha, V. (2024) · HemaSphere · doi:10.1002/hem3.70032 · Cited by 10 · see citing works
Ruiz, M. A., Shah, B. N., Ren, G., Hussain, F. A., Njoku, F. U., Machado, R. F., Gordeuk, V. R., & Saraf, S. L. (2022) · Blood Advances · doi:10.1182/bloodadvances.2022007980 · Cited by 6 · see citing works
The Role of Platelets in Sickle Cell Disease
Brzóska, T., Kato, G. J., & Sundd, P. (2019) · Elsevier eBooks · doi:10.1016/b978-0-12-813456-6.00031-x · Cited by 6 · see citing works
Levy, M., Naudin, J., Geslain, G., Maroni, A., Koehl, B., Bourgeois, F. L., Poncelet, G., Chomton, M., Dehò, A., Julliand, S., et al. (2024) · Annals of Intensive Care · doi:10.1186/s13613-024-01283-5 · Cited by 5 · see citing works
Garnier, M., Hafiani, E. M., Arbelot, C., Blayau, C., Labbé, V., Stankovic, K., Lionnet, F., Bonnet, F. J., Fulgencio, J.-P., Fartoukh, M. S., et al. (2019) · Annals of Intensive Care · doi:10.1186/s13613-019-0583-y · Cited by 5 · see citing works
Triaging acute chest syndrome clinical decision-making using bedside SaO2/FiO2 ratio
Wesevich, A., Woelkers, M., Adegunsoye, A., Wesevich, A. J., Ratain, M. J., & Lapping‐Carr, G. (2025) · Blood Advances · doi:10.1182/bloodadvances.2024015139 · Cited by 4 · see citing works
Clinical and Genetic Predictors of Sickle Cell Nephropathy: A Global Systematic Review
Banda, K., Nguweneza, A., Mnika, K., Nembaware, V., Chagaluka, G., & Wonkam, A. (2025) · OMICS A Journal of Integrative Biology · doi:10.1177/15578100251359276 · Cited by 4 · see citing works
Anemia falciforme e sindrome toracica acuta
Graziani, G., Gencarelli, J., Lama, C., Brocchi, S., Biserna, L., Radice, C., & Marchetti, F. (2020) · Medico e Bambino - Pagine Elettroniche · doi:10.53126/mebxxiiig126 · Cited by 3 · see citing works
Zhang, X., Han, J., Shah, B. N., Saraf, S. L., & Gordeuk, V. R. (2023) · British Journal of Haematology · doi:10.1111/bjh.18898 · Cited by 3 · see citing works
Updates in Pediatric Sickle Cell Lung Disease
Gillespie, M. L., Afolabi‐Brown, O., Machogu, E. M., Willen, S. M., & Kopp, B. T. (2024) · Clinics in Chest Medicine · doi:10.1016/j.ccm.2024.02.022 · Cited by 3 · see citing works
Mohammed, A., Podila, P. S. B., Davis, R. L., Ataga, K. I., Hankins, J. S., & Kamaleswaran, R. (2019) · bioRxiv (Cold Spring Harbor Laboratory) · doi:10.1101/614941 · Cited by 3 · see citing works
Thrombomodulin protects against acute vascular and multiorgan injury in sickle cell disease
Ren, G., Fraidenburg, D. R., Setty, S., Chen, J., Gonzales, J., Ruiz, M. A., Ivy, Z. K., Eskandari, N., Minshall, R. D., Lash, J. P., et al. (2025) · JCI Insight · doi:10.1172/jci.insight.193884 · Cited by 2 · see citing works
CD39 polymorphism enables lung thrombosis in sickle cell disease
Brzóska, T., Kaminski, T. W., Katoch, O., Menchikova, E. V., Alagbe, A. E., Tashbook, S. E., Tofovic, S. P., Jonassaint, J., Croix, C. M. S., Watkins, S. C., et al. (2026) · Nature Communications · doi:10.1038/s41467-026-68396-2 · Cited by 2 · see citing works
Pulmonary Complications of Sickle Cell Disease
Cohen, R. T. (2023) · American Academy of PediatricsItasca, IL eBooks · doi:10.1542/9781610026536-51 · Cited by 2 · see citing works
Lipid and hemolysis parameters predicting acute chest syndrome in adulthood with sickle cell disease
Feugray, G., Grall, M., Dumesnil, C., Brunel, V., Benhamou, Y., Quillard‐Muraine, M., & Billoir, P. (2024) · Lipids in Health and Disease · doi:10.1186/s12944-024-02135-8 · Cited by 3 · see citing works
Kidney Injuries in Sickle Cell Disease
Ghosh, S. (2022) · IntechOpen eBooks · doi:10.5772/intechopen.102839 · Cited by 1 · see citing works
Physician perspectives about the diagnosis and management of acute chest syndrome
Bhasin, N., LeBlanc, D. M., Yates, S. G., Eichbaum, Q. G., Pham, A., Sharma, D., Zhang, L., Vichinsky, E. P., & Sarode, R. (2024) · Transfusion · doi:10.1111/trf.18034 · Cited by 1 · see citing works
Moura, M. J., Costa, É., Canelas, M. A., Maia, R., & Ramalheira, S. (2025) · Acta Médica Portuguesa · doi:10.20344/amp.23392 · Cited by 1 · see citing works
Acute Chest Syndrome in Children with Sickle Cell Disease: A Narrative Review
Ramirez, V., & Mercier‐Ross, J. (2026) · Children · doi:10.3390/children13050670 · Cited by 1 · see citing works
Acute Chest Syndrome Masquerading As Sepsis: A Rare Presentation of Sickle Cell Anemia Complication
Mohan, B., Kavya, N. P., Choudhury, A., & Bairwa, M. C. (2022) · Cureus · doi:10.7759/cureus.30208 · Cited by 1 · see citing works
Disorders of red cells and platelets
MBBS, F. B. J. B. (2021) · doi:10.1002/9781119820307.ch8 · Cited by 1 · see citing works
Johnkennedy, N., JOSEPH, N. C., NKIRUKA, O.-A., Chidozie, N. J., Joy, A. C. A., & Okechukwu-Ezike, N. C. (2020) · Innovare Journal of Medical Sciences · doi:10.22159/ijms.2020.v8i2.37003
Inmigracion: el desafio de la convivencia
Terán, D. B. (2001) · Tiempo de paz
Morrone, K. A., Andreca, M., Silver, E. J., Xiang, A., Strumph, K. L., Manwani, D. G., Rinke, M. L., Kurian, J., Orentlicher, R. J., & Liszewski, M. C. (2023) · Pediatric Pulmonology · doi:10.1002/ppul.26634
Crisis in the Chest: Acute Chest Syndrome as a Sequela of Tricuspid Valve Endocarditis
Patel, N. N., Ebubechukwu, U. J., Saith, S. E., Ugoala, O. S., Kahn, A. B., McFarlane, S. I., & John, S. (2024) · Cureus · doi:10.7759/cureus.61061
Let’s Not Keep Acute Chest Syndrome Under an Umbrella
Klings, E. S., Cohen, R. T., & Ruhl, A. P. (2025) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.202503-0639ed
Anwar, T., Mukherjee, S., Prakash, S., Sahu, A., Mishra, D., John, B., & Mitra, J. K. (2025) · Asian Journal of Transfusion Science · doi:10.4103/ajts.ajts_183_24
Platt, M. O., Lee, H. S., & Hatoum, L. (2025) · Physiology in health and disease · doi:10.1007/978-3-031-99609-2_11 · Cited by 1 · see citing works
Levy, M., Naudin, J., Geslain, G., Maroni, A., Koehl, B., Bourgeois, F. L., Poncelet, G., Chomton, M., Dehò, A., Julliand, S., et al. (2024) · Figshare · doi:10.6084/m9.figshare.25572517
Emerging concepts in sickle cell disease acute chest syndrome
Gillespie, M. L., King, T., Cohen, R. T., Willen, S. M., Koya, A. I., Ruhl, A. P., & Kopp, B. T. (2026) · Blood Red Cells & Iron · doi:10.1016/j.brci.2026.100060
Genetic Contribution to Asthma Informs Acute Chest Syndrome Pathophysiology and Risk Stratification
Aouhel, S. E., Bellegarde, V., Faria, S. D. S., St-Laurent, T., Lecluze, E., D'Orengiani, A.-L. P. H. D., Galactéros, F., Bartolucci, P., Legault, M., Lettre, G., et al. (2026) · American Journal of Hematology · doi:10.1002/ajh.70262
Supportive care, prevention, and emerging therapies for acute chest syndrome in sickle cell disease
Rice, R. R., & Willen, S. M. (2026) · Expert Review of Hematology · doi:10.1080/17474086.2026.2702549
Shaybah, M. E., Alsehli, O., Al-Harthi, A., Bajuaifer, N., Bafaqih, M., Alzhrani, M., Alasmari, A., Kashgari, A., Alhazmi, O., & Munshi, A. S. (2026) · Medicina · doi:10.3390/medicina62091731
- Authors
- Chaturvedi, S., Ghafuri, D. L., Glassberg, J., Kassim, A. A., Rodeghier, M., & DeBaun, M. R.
- Affiliations
- Vanderbilt University (US)
- Amsterdam UMC Location University of Amsterdam (NL)
- Mount Sinai Hospital (US)
- Rodeghier Consultants Chicago Illinois
- Vanderbilt University Medical Center (US)
- Corresponding author
- DeBaun, Michael R.
- ORCID
- Venue
- American Journal of Hematology
- Publisher
- Wiley
- Published
- 2016-10-03
- Volume
- 91
- Issue
- 12
- Pages
- 1185-1190
- ISSN
- 0361-8609 (ISSN-L)
- 1096-8652
- OpenAlex
- W2511476568
- Cited by
- 60
- References
- 36
- Citations by year
- 2026: 6 · 2025: 7 · 2024: 9 · 2023: 4 · 2022: 7 · 2021: 2 · 2020: 7 · 2019: 8 · 2018: 5 · 2017: 3 · 2015: 1
- FWCI
- 3.87
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- American Journal of Hematology (publishedVersion)
- PubMed (publishedVersion)
- Funders
- Burroughs Wellcome Foundation
- American Society of Hematology
- NCATS/NIH (UL1 TR000445)
- Topics
- Health Sciences › Medicine › Genetics › Hemoglobinopathies and Related Disorders
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Methemoglobinemia and Tumor Lysis Syndrome
- Health Sciences › Medicine › Infectious Diseases › COVID-19 Clinical Research Studies
- Keywords
- acute chest syndrome, sickle cell disease, phenotypes, respiratory failure, multiple organ failure, acute kidney injury, liver dysfunction, thrombocytopenia, platelet count decline, mortality, children and adults
- MeSH terms
- Adolescent, Adult, Anemia, Sickle Cell, Child, Child, Preschool, Female, Humans, Male, Multiple Organ Failure, Phenotype, Respiratory Insufficiency, Retrospective Studies, Time Factors, Cohort Studies, Disease Progression, Young Adult, Acute Chest Syndrome
About these checks
Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.
Statistics and arithmetic are recomputed, not judged.
Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.
What counts as a claim ›How we measure confidence ›How we check this ›
Are you an author, or do you see a problem with this page? Request a review
