Guideline on the management of acute chest syndrome in sickle cell disease

British Journal of Haematology

Volumn 169, Issue 4, 2015, Pages 492-505

  Howard, Jo ^a   Hart, Nicholas ^b   Roberts Harewood, Marilyn ^c   Cummins, Michelle ^d   Awogbade, Moji ^e   Davis, Bernard ^f  

^a GUY'S AND ST THOMAS' NHS FOUNDATION TRUST   (United Kingdom)

^b ST THOMAS HOSPITAL   (United Kingdom)

^c NORTH MIDDLESEX UNIVERSITY HOSPITAL   (United Kingdom)

^d BRISTOL ROYAL HOSPITAL FOR CHILDREN   (United Kingdom)

^e KING S COLLEGE HOSPITAL   (United Kingdom)

^f WHITTINGTON HOSPITAL   (United Kingdom)

Author keywords

Acute chest syndrome; Respiratory disease; Sickle cell disease

Indexed keywords

AMOXICILLIN PLUS CLAVULANIC ACID; AZITHROMYCIN; BRONCHODILATING AGENT; C REACTIVE PROTEIN; CEFTRIAXONE; CLARITHROMYCIN; CORTICOSTEROID; CREATININE; HYDROXYUREA; INFUSION FLUID; NARCOTIC ANALGESIC AGENT; NITRIC OXIDE; OXYGEN; PENICILLIN V; SECRETORY PHOSPHOLIPASE A2; VANCOMYCIN;

ACUTE CHEST SYNDROME; ACUTE RESPIRATORY FAILURE; ANTIBIOTIC PROPHYLAXIS; ARTICLE; ARTIFICIAL VENTILATION; ASSISTED VENTILATION; ASTHMA; ATELECTASIS; BLOOD CELL COUNT; BLOOD CULTURE; BLOOD GAS ANALYSIS; BLOOD GROUP TYPING; BLOOD TRANSFUSION; BREATHING EXERCISE; CLINICAL FEATURE; DRUG OVERDOSE; FAT EMBOLISM; HIGH RESOLUTION COMPUTER TOMOGRAPHY; HUMAN; HYPERVOLEMIA; HYPOVENTILATION; HYPOXIA; INCENTIVE SPIROMETRY; INFECTION PREVENTION; LUNG ALVEOLUS HYPOVENTILATION; LUNG EMBOLISM; LUNG INFARCTION; LUNG INFECTION; LUNG INFILTRATE; LUNG LAVAGE; LUNG VENTILATION PERFUSION RATIO; MORTALITY; PATHOPHYSIOLOGY; PRIORITY JOURNAL; RESPIRATORY CARE; SICKLE CELL ANEMIA; SPUTUM CULTURE; STEM CELL TRANSPLANTATION; THORAX RADIOGRAPHY; ANEMIA, SICKLE CELL; COMPLICATION; FEMALE; MALE; PATHOLOGY; PRACTICE GUIDELINE;

ACUTE CHEST SYNDROME; ANEMIA, SICKLE CELL; FEMALE; HUMANS; MALE;

EID: 84928407636     PISSN: 00071048     EISSN: 13652141     Source Type: Journal    
DOI: 10.1111/bjh.13348     Document Type: Article

References (80)

1. van Agtmael, M.A., Cheng, J.D. & Nossent, H.C. (1994) Acute chest syndrome in adult Afro-Carribean patients with sickle cell disease. Analysis of 81 episodes among 53 patients. Archives of Internal Medicine, 154, 557-561.
2. Al Hajeri, A., Serjeant, G.R. & Fedorowicz, Z. (2008) Inhaled nitric oxide for acute chest syndrome in people with sickle cell disease. Cochrane Database Systematic Review, 2008(1), CD006957.
3. Alhashimi, D., Fedorowicz, Z., Alhashimi, F. & Dastgiri, S. (2010) Blood transfusions for treating acute chest syndrome in people with sickle cell disease. Cochrane Database Systematic Review, 2010(1), CD007843.
4. ARDS Definition Task Force, Ranieri, V.M., Rubenfeld, G.D., Thompson, B.T., Ferguson, N.D., Caldwell, E., Fan, E., Camporota, L. & Slutsky, A.S. (2012) Acute respiratory distress syndrome: the Berlin Definition. JAMA, 307, 2526-2533.
5. Atz, A.M. & Wessel, D.L. (1997) Inhaled nitric oxide in sickle cell disease with acute chest syndrome. Anesthesiology, 87, 988-990.
6. Ballas, S.K., Lieff, S., Benjamin, L.J., Dampier, C.D., Heeney, M.M., Hoppe, C., Johnson, C.S., Rogers, Z.R., Smith-Whitley, K., Wand, W.C. & Telen, M.J. (2010) Definitions of the phenotypic manifestations of sickle cell disease. American Journal of Hematology, 85, 6-13.
7. Bellet, P.S., Kalinyak, K.A., Shukla, R., Gelfand, M.J. & Rucknagel, D.L. (1995) Incentive spirometry to prevent acute pulmonary complications in sickle cell diseases. New England Journal of Medicine, 333, 699-703.
8. Bernini, J.C., Rogers, Z.R., Sandler, E.S., Reisch, J.S., Quinn, C.T. & Buchanan, G.R. (1998) Beneficial effect of intravenous dexamethasone in children with mild to moderately severe acute chest syndrome complicating sickle cell disease. Blood, 92, 3082-3089.
9. Bhalla, M., Abboud, M.R., McLoud, T.C., Shepard, J.A., Munden, M.M., Jackson, S.M., Beaty, J.R. & Laver, J.H. (1993) Acute chest syndrome in sickle cell disease: CT evidence of microvascular occlusion. Radiology, 187, 45-49.
10. Boyd, J.H., Macklin, E.A., Strunk, R.C. & DeBaun, M.R. (2006) Asthma is associated with acute chest syndrome and pain in children with sickle cell anemia. Blood, 108, 2923-2927.
11. British Thoracic Society/Scottish Intercollegiate Guidelines Network. (2008) British Guidelines on the Management of Asthma: A National Clinical Guideline. Available at: http://www.brit-thoracic.org.uk/Portals/0/Guidelines/AsthmaGuidelines/sign101%20revised%20June%2009.pdf.
12. Brower, R.G., Ware, L.B., Berthiaume, Y. & Matthay, M.A. (2001) Treatment of ARDS. Chest, 120, 1347-1367.
13. Buchanan, I.D., Woodward, M. & Reed, G.W. (2005) Opioid selection during sickle cell pain crisis and its impact on the development of acute chest syndrome. Pediatric Blood & Cancer, 45, 716-724.
14. Charache, S., Scott, J.C. & Charache, P. (1979) Acute chest syndrome in adults with sickle cell anaemia. Archives of Internal Medicine, 139, 67.
15. Charache, S., Terrin, M.L., Moore, R.D., Dover, G.J., Barton, F.B., Eckert, S.V., McMahon, R.P. & Bonds, D.R. (1995) Effect of hydroxyurea on the frequency of painful crises in sickle cell anemia. Investigators of the Multicenter Study of Hydroxyurea in Sickle Cell Anemia. New England Journal of Medicine, 332, 1317-1322.
16. Davies, S.C., Luce, P.J., Win, A.A., Riordan, J.F. & Brozovic, M. (1984) Acute chest syndrome in sickle cell disease. Lancet, 8367, 36-38.
17. Dean, D., Neumayr, L., Kelly, D.M., Ballas, S.K., Kelman, K., Robertson, S., Iyer, R.V., Ware, R.E., Koshy, M., Rackoff, W., Pegelow, C.H., Waldron, P., Benjamin, L. & Vichinsky, E. (2003) Chlamydia pneumoniae and the acute chest syndrome in patients with sickle cell disease. Journal of Pediatric Hematology/Oncology, 251, 46-55.
18. DeBaun, M.R., Gordon, M., McKinstry, R.C., Noetzel, M.J., White, D.A., Sarnaik, S.A., Meier, E.R., Howard, T.H., Majumdar, S., Inusa, B.P.D., Telfer, P.T., Kirby-Allen, M., McCavit, T.L., Kamdem, A., Airewele, G., Woods, G.M., Berman, B., Panepinto, J.A., Fuh, B.R., Kwiatkowski, J.L., King, A.A., Fixler, J.M., Rhodes, M.M., Thompson, A.A., Heiny, M.E., Redding-Lallinger, R.C., Kirkham, F.J., Dixon, N., Gonzalez, C.E., Kalinyak, K.A., Quinn, C.T., Strouse, J.J., Miller, J.P., Lehmann, H., Kraut, M.A., Ball, W.S., Hirtz, D. & Casella, J.F. (2014) Controlled trial of transfusions for silent cerebral infarcts in sickle cell anemia. The New England Journal of Medicine, 371, 699-710.
19. Emre, U., Miller, S.T., Gutierez, M., Steiner, P., Rao, S.P. & Rao, M. (1995) Effects of transfusion in acute chest syndrome of sickle cell disease. Journal of Pediatrics, 127, 901-904.
20. Fartoukh, M., Lefort, Y., Habibi, A., Bachir, D., Galacteros, F., Godeau, B., Maitre, B. & Brochard, L. (2010) Early intermittent noninvasive ventilation for acute chest syndrome in adults with sickle cell disease: a pilot study. Intensive Care Medicine, 36, 1355-1362.
21. Feldman, L., Gross, R., Garon, J., Nallari, A., Kaur, N., Motwani, B., Sukumaran, S., Allen, S. & Westerman, M. (2003) Sickle cell patient presenting with an acute chest syndrome and a negative chest x-ray: potential role of the ventilation and perfusion lung scan. American Journal of Hematology, 74, 214-215.
22. Finkelstein, Y., Schechter, T., Garcia-Bournissen, F., Kirby, M., Nurmohamed, L., Juurlink, D.N., Blanchette, V. & Koren, G. (2007) Is morphine exposure associated with acute chest syndrome in children with vaso-occlusive crisis of sickle cell disease? A 6-year case-crossover study. Clinical Therapeutics, 29, 2738-2743.
23. Fitzgerald, R.K. & Johnson, A. (2001) Pulse oximetry in sickle cell anemia. Critical Care Medicine, 29, 1803-1806.
24. Fitzhugh, C.D., Lauder, N., Jonassaint, J., Telen, M.J., Zhao, X., Wright, E.C., Gilliam, F.R. & De Castro, L.M. (2010) Cardiopulmonary complications leading to premature deaths in adult patients with sickle cell disease. American Journal of Hematology, 85, 36-40.
25. Gaston, M.H., Verter, J.I., Woods, G., Pegelow, C., Kelleher, J., Presbury, G., Zarkowsky, H., Vichinsky, E., Iyer, R., Lobel, J.S., Diamond, S., Holbrook, C.T., Gill, F.M., Ritchey, K. & Falletta, J.M.; for the Prophylactic Penicillin Study Group. (1986) Prophylaxis with oral penicillin in children with sickle cell anemia. A randomized trial. New England Journal of Medicine, 314, 1593-1599.
26. Gelfand, M.J., Shashikant, A.D., Rucknagel, D.L., Kalinyak, K.A. & Paltiel, H.J. (1993) Simultaneous occurrence of rib infarction and pulmonary infiltrates in sickle cell disease patients with acute chest syndrome. Journal of Nuclear Medicine, 34, 614-618.
27. Gillett, D.S., Gunning, K.E., Sawicka, E.H., Bellingham, A.J. & Ware, R.J. (1987) Life threatening acute chest syndrome treated with extracorporeal membrane oxygenation. British Medical Journal (Clinical Research Ed), 294, 81-82.
28. Gladwin, M.T. & Vichinsky, E. (2008) Pulmonary complications in sickle cell disease. New England Journal of Medicine, 358, 2254-2265.
29. Harris, M., Clark, J., Coote, N., Fletcher, P., Harnden, A., McKean, M. & Thomson, A.; British Thoracic Society Standards of Care Committee. (2011) British Thoracic Society guidelines for the management of community acquired pneumonia in children: update 2011. Thorax, 66(Suppl. 2), ii1-ii23.
30. Henderson, J.N., Noetzel, M.J., McKinstry, R.C., White, D.A., Armstrong, M. & DeBaun, M.R. (2003) Reversible posterior leukoencephalopathy syndrome and silent cerebral infarcts are associated with severe acute chest syndrome in children with sickle cell disease. Blood, 101, 415-419.
31. Howard, J., Malfroy, M., Llewelyn, C., Choo, L., Hodge, R., Johnson, T., Purohit, S., Rees, D.C., Tillyer, L., Walker, I., Fijnvandraat, K., Kirby-Allen, M., Spackman, E., Davies, S.C. & Williamson, L.M. (2013) The Transfusion Alternatives Preoperatively in Sickle Cell Disease (TAPS) study: a randomised, controlled, multicentre clinical trial. Lancet, 381, 930-938.
32. Hsu, L.L., Batts, B.K. & Rau, J.L. (2005) Positive expiratory pressure device acceptance by hospitalized children with sickle cell disease is comparable to incentive spirometry. Respiratory Care, 50, 624-627.
33. Ip, H., Kesse-Adu, R., Howard, J. & Hart, N. (2013) Low flow nocturnal oxygen therapy does not suppress haemoglobin levels or increase painful crises in sickle cell disease. British Journal of Haematology, 161, 455-456.
34. Kaur, N., Motwani, B., Sivasubramaniam, D., Feldman, L., Allen, S., Ferguson, R., Shiomoto, G., Ansell, D., Westerman, M. & Feldman, L. (2004) Potential role of the ventilation and perfusion (V/Q) lung scan in the diagnosis of acute chest syndrome in adults with sickle cell disease. American Journal of Hematology, 77, 407-409.
35. Knight-Madden, J.A. & Hambleton, I.R. (2003) Inhaled bronchodilators for acute chest syndrome in people with sickle cell disease. Cochrane Database Systematic Review, 2003(3), CD003733.
36. Knight-Madden, J.M., Forrester, T.S., Lewis, N.A. & Greenough, A. (2010) The impact of recurrent acute chest syndrome on the lung function of young adults with sickle cell disease. Lung, 188, 499-504.
37. Kopecky, E.A., Jacobson, S., Joshi, P. & Koren, G. (2004) Systemic exposure to morphine and the risk of acute chest syndrome in sickle cell disease. Clinical Pharmacology and Therapeutics, 75, 140-146.
38. Kuo, K.W., Cornell, T.T., Shanley, T.P., Odetola, F.O. & Annich, G.M. (2013) The use of extracorporeal membrane oxygenation in pediatric patients with sickle cell disease. Perfusion, 28, 424-432.
39. Laurie, G.A. (2010) Acute chest syndrome in sickle cell disease. Internal Medicine Journal, 40, 372-376.
40. Leong, C.S. & Stark, P. (1998) Thoracic manifestations of sickle cell disease. Journal of Thoracic Imaging, 13, 128-134.
41. Lim, W.S., Baudouin, S.V., George, R.C., Hill, A.T., Jamieson, C., Le Jeune, I., Macfarlane, J.T., Read, R.C., Roberts, H.J., Levy, M.L., Wani, M., Woodhead, M.A.; Pneumonia Guidelines Committee of the BTS Standards of Care Committee. (2009) BTS guidelines for the management of community acquired pneumonia in adults: update 2009. Thorax, 64(Suppl. 3), iii1-iii55.
42. Lucas, S.B., Mason, D.G., Mason, M. & Weyman, D.; on behalf of NCEPOD. (2008) A Sickle Crisis? A Report of the National Confidential Enquiry in Patient Outcome and Death. Available at: www.ncepod.org.uk/2008report1/Downloads/Sickle_report.pdf.
43. Madani, G., Papadopoulou, A.M., Holloway, B., Robins, A., Davis, J. & Murray, D. (2007) The radiological manifestations of sickle cell disease. Clinical Radiology, 62, 528-538.
44. Maitre, B., Habibi, A., Roudot-Thoraval, F., Bachir, D., Belghiti, D.D., Galacteros, F. & Godeau, B. (2000) Acute chest syndrome in adults with sickle cell disease. Chest, 117, 1386-1392.
45. Martí-Carvajal, A.J., Conterno, L.O. & Knight-Madden, J.M. (2013) Antibiotics for treating acute chest syndrome in people with sickle cell disease. Cochrane Database Systematic Review, 1, CD006110.
46. Miller, S.T., Wright, E., Abboud, M., Berman, B., Files, B., Scher, C.D., Styles, L., Adams, R.J.; STOP Investigators. (2001) Impact of chronic transfusion on incidence of pain and acute chest syndrome during the Stroke Prevention Trial (STOP) in sickle cell anemia. Journal of Pediatrics, 139, 785-789.
47. Needleman, J.P., Benjamin, L.J., Sykes, J.A. & Aldrich, T.K. (2002) Breathing patterns during vaso-occlusive crisis of sickle cell disease. Chest, 122, 43-46.
48. Neumayr, L., Lennette, E., Kelly, D., Earles, A., Embury, S., Groncy, P., Grossi, M., Grover, R., McMahon, L., Swerdlow, P., Waldron, P. & Vichinsky, E. (2003) Mycoplasma disease and acute chest syndrome in sickle cell disease. Pediatrics, 112, 87-95.
49. NICE. (2010) Venous Thromboembolism: Reducing The Risk: Reducing the Risk of Venous Thromboembolism (deep vein thrombosis and pulmonary embolism) in Patients Admitted to Hospital. NICE Clinical Guideline 92. National Institute for Health and Care Excellence, London. Available at: www.nice.org.uk/guidance/CG92.
50. Noto, H. (1999) Haemoglobin SC disease presenting as acute chest syndrome with ventilation-perfusion mismatches. Respiration, 66, 73-77.
51. Ohene-Frempong, K., Weiner, S.J., Sleeper, L.A., Miller, S.T., Embury, S., Moohr, J.W., Wethers, D.L., Pegelow, C.H. & Gill, F.M. (1998) Cerebrovascular accidents in sickle cell disease: rates and risk factors. Blood, 91, 288-294.
52. Ortiz, F.O., Aldrich, T.K., Nagel, R.L. & Benjamin, L.J. (1999) Accuracy of pulse oximetry in sickle cell disease. American Journal of Respiratory and Critical Care Medicine, 159, 447-451.
53. Padman, R. & Henry, M. (2004) The use of bilevel positive airway pressure for the treatment of acute chest syndrome of sickle cell disease. Delaware Medical Journal, 76, 199-203.
54. Pelidis, M.A., Kato, G.J., Resar, L.M., Dover, G.J., Nichols, D.G., Walker, L.K. & Casella, J.F. (1997) Successful treatment of life-threatening acute chest syndrome of sickle cell disease with venovenous extracorporeal membrane oxygenation. Journal of Pediatric Hematology/Oncology, 19, 459-461.
55. Platt, O.S., Brambilla, D.J., Rosse, W.F., Milner, P.F., Castro, O., Steinberg, M.H. & Klug, P.P. (1994) Mortality in sickle cell disease: life expectancy and risk factors for early death. New England Journal of Medicine, 330, 1639-1644.
56. Powars, D., Weidman, J.A., Odom-Maryon, T., Niland, J.C. & Johnson, C. (1988) Sickle cell chronic lung disease: prior morbidity and the risk of pulmonary failure. Medicine, 67, 66-76.
57. Quinn, C.T., Stuart, M.J., Kesler, K., Ataga, K.I., Wang, W.C., Styles, L., Smith-Whitley, K., Wun, T., Raj, A., Hsu, L.L., Krishnan, S., Kuypers, F.A., Setty, Y., Rhee, S., Key, N.S. & Buchanan, G.R.; Investigators of the Comprehensive Sickle Cell Centers. (2011) Tapered oral dexamethasone for the acute chest syndrome of sickle cell disease. British Journal of Haematology, 155, 263-267.
58. Rees, D.C., Olujohungbe, A.D., Parker, N.E., Stephens, A.D., Telfer, P., Wright, J.; British Committee for Standards in Haematology General Haematology Task Force by the Sickle Cell Working Party. (2003) Guidelines for the management of the acute painful crisis in sickle cell disease. British Journal of Haematology, 120, 744-752.
59. Rucknagel, D.L., Kalinyak, K.L. & Gelfand, M.J. (1991) Rib infarcts and acute chest syndrome in sickle cell diseases. Lancet, 337, 831-833.
60. Sickle Cell Society. (2008) Standards for the Clinical Care of Adults with Sickle Cell Disease in the UK. Available at: www.sct.screening.nhs.uk/standardsandguidelines.
61. Sobota, A., Graham, D.A., Heeney, M.M. & Neufeld, E.J. (2010) Corticosteroids for acute chest syndrome in children with sickle cell disease: variation in use and association with length of stay and readmission. American Journal of Hematology, 85, 24-28.
62. Strouse, J.J., Takemoto, C.M., Keefer, J.R., Kato, G.J. & Casella, J.F. (2008) Corticosteroids and increased risk of readmission after acute chest syndrome in children with sickle cell disease. Pediatric Blood & Cancer, 50, 1006-1012.
63. Stuart, M.J. & Setty, B.N.Y. (1999) Sickle cell acute chest syndrome: pathogenesis and rationale for treatment. Blood, 94, 1555-1560.
64. Styles, L.A., Schalkwijk, C.G., Aarsman, A.J., Vichinsky, E.P., Lubin, B.H. & Kuypers, F.A. (1996) Phospholipase A2 levels in acute chest syndrome of sickle cell disease. Blood, 87, 2573-2578.
65. Styles, L.A., Aarsman, A.J., Vichinsky, E.P. & Kuypers, F.A. (2000) Secretory phospholipase A2 predicts impending acute chest syndrome in sickle cell disease. Blood, 96, 3276-3278.
66. Styles, L.A., Abboud, M., Larkin, S., Lo, M. & Kuypers, F.A. (2007) Transfuion prevents acute chest syndrome predicted by elevated secretory phospholipase A2. British Journal of Haematology, 136, 343-344.
67. Styles, L., Wager, C.G., Labotka, R.J., Smith-Whitley, K., Thompson, A.A., Lane, P.A., McMahon, L.E., Miller, R., Roseff, S.D., Iyer, R.V., Hsu, L.L., Castro, O.L., Ataga, K.I., Onyekwere, O., Okam, M., Bellevue, R., Miller, S.T.; Sickle Cell Disease Clinical Research Network (SCDCRN). (2012) Refining the value of secretory phospholipase A2 as a predictor of acute chest syndrome in sickle cell disease: results of a feasibility study (PROACTIVE). British Journal of Haematology, 157, 627-636.
68. Sullivan, K.J., Goodwin, S.R., Evangelist, J., Moore, R.D. & Mehta, P. (1999) Nitric oxide successfully used to treat acute chest syndrome of sickle cell disease in a young adolescent. Critical Care Medicine, 27, 2563-2568.
69. Swerdlow, P.S. (2006) Red cell exchange in sickle cell disease. Hematology/the Education Program of the American Society of Hematology, 48-53.
70. Sylvester, K.P., Patey, R.A., Milligan, P., Rafferty, G.F., Broughton, S., Rees, D., Thein, S.L. & Greenough, A. (2006) Impact of acute chest syndrome on lung function of children with sickle cell disease. Journal of Pediatrics, 149, 17-22.
71. van Tuijn, C.F., Nur, E., van Beers, E.J., Zaaijer, H.L. & Biemond, B.J. (2010) Acute chest syndrome in sickle cell disease due to the new influenza A (H1N1) virus infection. American Journal of Hematology, 85, 303-304.
72. Turner, J.M., Kaplan, J.B., Cohen, H.W. & Billett, H.H. (2009) Exchange versus simple transfusion for acute chest syndrome in sickle cell anemia adults. Transfusion, 49, 863-868.
73. Velasquez, M.P., Mariscalco, M.M., Goldstein, S.L. & Airewele, G.E. (2009) Erythrocytapheresis in children with sickle cell disease and acute chest syndrome. Pediatric Blood & Cancer, 53, 1060-1063.
74. Vichinsky, E.P. (2001) Current issues with blood transfusion in sickle cell disease. Seminars in Hematology, 38, 14-22.
75. Vichinsky, E., Williams, R., Das, M., Earles, A.N., Lewis, N., Adler, A. & McQuitty, J. (1994) Pulmonary fat embolism: a distinct cause of severe acute chest syndrome in sickle cell anemia. Blood, 83, 3107-3112.
76. Vichinsky, E.P., Styles, L.A., Colangleo, L.H., Wright, E.C., Castro, O. & Nickerson, B. (1997) Acute chest syndrome in sickle cell disease: clinical presentation and course. Blood, 89, 1787-1792.
77. Vichinsky, E.P., Neumayr, L.D., Earles, A.N., Williams, R., Lennette, E.T., Dean, D., Nickerson, B., Orringer, E., McKie, V., Bellevue, R., Daeschner, C. & Manci, E.A. (2000) Causes and outcomes of the acute chest syndrome in sickle cell disease. The New England Journal of Medicine, 342, 1855-1865.
78. Wang, W.C., Ware, R.E., Miller, S.T., Iyer, R.V., Casella, J.F., Minniti, C.P., Rana, S., Thornburg, C.D., Rogers, Z.R., Kalpatthi, R.V., Barredo, J.C., Brown, R.C., Sarnaik, S.A., Howard, T.H., Wynn, L.W., Kutler, A., Armstrong, F.D., Files, B.A., Goldsmith, J.C., Waclawiw, M.A., Huang, X. & Thompson, B.W.; BABY HUG investigators. (2011) Hydroxycarbamide in very young children with sickle-cell anaemia: a multicentre, randomised, controlled trial (BABY HUG). Lancet, 377, 1663-1672.
79. Wratney, A.T., Gentile, M.A., Hamel, D.S. & Cheifetz, I.M. (2004) Successful treatment of acute chest syndrome with high-frequency oscillatory ventilation in pediatric patients. Respiratory Care, 49, 263-269.
80. Yildizdaş, D., Yapicioğlu, H., Yilmaz, H.L. & Sertdemir, Y. (2004) Correlation of simultaneously obtained capillary, venous, and arterial blood gases of patients in a paediatric intensive care unit. Archives of Disease in Childhood, 89, 176-180.