Sickle cell disease in childhood. from newborn screening through transition to adult medical care.

Pediatric Clinics of North America

Volumn 60, Issue 6, 2013, Pages 1363-1381

  Quinn, Charles T ^a,b  

^a CINCINNATI CHILDREN'S HOSPITAL MEDICAL CENTER   (United States)

^b UNIVERSITY OF CINCINNATI COLLEGE OF MEDICINE   (United States)

Author keywords

Diagnosis; Genetics; Pathophysiology; Sickle cell disease; Survival; Treatment

Indexed keywords

ANALGESIC AGENT; HEMOGLOBIN; HEMOGLOBIN C; HEMOGLOBIN S; HYDROXYUREA; MENINGOCOCCUS VACCINE; NARCOTIC ANALGESIC AGENT; NONSTEROID ANTIINFLAMMATORY AGENT; PENICILLIN DERIVATIVE; PENICILLIN V; PENICILLIN V POTASSIUM; PLACEBO; PNEUMOCOCCUS VACCINE; PSEUDOEPHEDRINE;

ACUTE CHEST SYNDROME; AGAR GEL ELECTROPHORESIS; ALPHA THALASSEMIA; ANALGESIA; APLASTIC CRISIS; ASTHMA; AVASCULAR NECROSIS; BETA THALASSEMIA; BONE DISEASE; CEREBROVASCULAR ACCIDENT; CHILDHOOD DISEASE; DACTYLITIS; DISEASE SEVERITY; ENDOTHELIAL DYSFUNCTION; ERYTHROCYTE TRANSFUSION; FEVER; GENE MUTATION; GENETIC SCREENING; HAND FOOT SYNDROME; HEMATOPOIETIC STEM CELL TRANSPLANTATION; HEMOGLOBIN SC DISEASE; HEMOGLOBINOPATHY; HEMOLYTIC ANEMIA; HEPATOBILIARY DISEASE; HETEROZYGOSITY; HIGH PERFORMANCE LIQUID CHROMATOGRAPHY; HOMOZYGOSITY; HUMAN; INFANCY; ISOELECTRIC FOCUSING; KIDNEY DISEASE; MORBIDITY; MORTALITY; NEUROLOGIC DISEASE; OSTEOMYELITIS; OXYGEN THERAPY; PARVOVIRUS INFECTION; PATHOPHYSIOLOGY; POLYMERIZATION; POSTOPERATIVE PERIOD; PREOPERATIVE PERIOD; PRIAPISM; PRIMARY PREVENTION; PROGNOSIS; PULMONARY HYPERTENSION; REHYDRATION; REVIEW; RISK FACTOR; SEPSIS; SICKLE CELL ANEMIA; SICKLE CELL BETA THALASSEMIA; SPIROMETRY; SPLEEN DISEASE; SURVIVAL RATE; TRANSIENT ISCHEMIC ATTACK; UROGENITAL TRACT DISEASE;

DIAGNOSIS; GENETICS; PATHOPHYSIOLOGY; SICKLE CELL DISEASE; SURVIVAL; TREATMENT;

ADULT; ANEMIA, SICKLE CELL; CHILD; HUMANS; INFANT, NEWBORN; MORBIDITY; NEONATAL SCREENING;

EID: 84887549095     PISSN: 00313955     EISSN: 15578240     Source Type: Journal    
DOI: 10.1016/j.pcl.2013.09.006     Document Type: Review

References (33)

1. Gaston M.H., Verter J.I., Woods G., et al. Prophylaxis with oral penicillin in children with sickle cell anemia. NEngl J Med 1986, 314(25):1593-1599.
2. Falletta J.M., Woods G.M., Verter J.I., et al. Discontinuing penicillin prophylaxis in children with sickle cell anemia. Prophylactic Penicillin Study II. JPediatr 1995, 127(5):685-690.
3. Lane P.A., O'Connell J.L., Lear J.L., et al. Functional asplenia in hemoglobin SC disease. Blood 1995, 85(8):2238-2244.
4. Quinn C.T., Rogers Z.R., McCavit T.L., et al. Improved survival of children and adolescents with sickle cell disease. Blood 2010, 115(17):3447-3452.
5. Telfer P., Coen P., Chakravorty S., et al. Clinical outcomes in children with sickle cell disease living in England: a neonatal cohort in East London. Haematologica 2007, 92(7):905-912.
6. McCavit T.L., Quinn C.T., Techasaensiri C., et al. Increase in invasive Streptococcus pneumoniae infections in children with sickle cell disease since pneumococcal conjugate vaccine licensure. JPediatr 2011, 158(3):505-507.
7. McCavit T.L., Xuan L., Zhang S., et al. Hospitalization for invasive pneumococcal disease in a national sample of children with sickle cell disease before and after PCV7 licensure. Pediatr Blood Cancer 2012, 58(6):945-949.
8. Wilimas J.A., Flynn P.M., Harris S., et al. Arandomized study of outpatient treatment with ceftriaxone for selected febrile children with sickle cell disease. NEngl J Med 1993, 329(7):472-476.
9. Cauff B.E., Quinn C.T. Transient parvovirus-associated hypoplasia of multiple peripheral blood cell lines in children with chronic hemolytic anemia. Pediatr Blood Cancer 2008, 50(4):861-864.
10. Berger E., Saunders N., Wang L., et al. Sickle cell disease in children: differentiating osteomyelitis from vaso-occlusive crisis. Arch Pediatr Adolesc Med 2009, 163(3):251-255.
11. Quinn C.T., Buchanan G.R. The acute chest syndrome of sickle cell disease. JPediatr 1999, 135(4):416-422.
12. Bernini J.C., Rogers Z.R., Sandler E.S., et al. Beneficial effect of intravenous dexamethasone in children with mild to moderately severe acute chest syndrome complicating sickle cell disease. Blood 1998, 92(9):3082-3089.
13. Quinn C.T., Stuart M.J., Kesler K., et al. Tapered oral dexamethasone for the acute chest syndrome of sickle cell disease. Br J Haematol 2011, 155(2):263-267.
14. Field J.J., DeBaun M.R. Asthma and sickle cell disease: two distinct diseases or part of the same process?. Hematology Am Soc Hematol Educ Program 2009, 2009(1):45-53.
15. Gladwin M.T., Sachdev V., Jison M.L., et al. Pulmonary hypertension as a risk factor for death in patients with sickle cell disease. NEngl J Med 2004, 350(9):886-895.
16. Lee M.T., Small T., Khan M.A., et al. Doppler-defined pulmonary hypertension and the risk of death in children with sickle cell disease followed for a mean of three years. Br J Haematol 2009, 146(4):437-441.
17. Adams R.J., McKie V.C., Hsu L., et al. Prevention of a first stroke by transfusions in children with sickle cell anemia and abnormal results on transcranial Doppler ultrasonography. NEngl J Med 1998, 339(1):5-11.
18. Adams R.J., Brambilla D., Optimizing Primary Stroke Prevention in Sickle Cell Anemia (STOP 2) Trial Investigators Discontinuing prophylactic transfusions used to prevent stroke in sickle cell disease. NEngl J Med 2005, 353(26):2769-2778.
19. McCavit T.L., Xuan L., Zhang S., et al. National trends in incidence rates of hospitalization for stroke in children with sickle cell disease. Pediatr Blood Cancer 2013, 60(5):823-827.
20. Bernaudin F., Verlhac S., Arnaud C., et al. Impact of early transcranial Doppler screening and intensive therapy on cerebral vasculopathy outcome in a newborn sickle cell anemia cohort. Blood 2011, 117(4):1130-1140.
21. DeBaun M.R., Sarnaik S.A., Rodeghier M.J., et al. Associated risk factors for silent cerebral infarcts in sickle cell anemia: low baseline hemoglobin, sex, and relative high systolic blood pressure. Blood 2012, 119(16):3684-3690.
22. Quinn C.T., McKinstry R.C., Dowling M.M., et al. Acute silent cerebral ischemic events in children with sickle cell anemia. JAMA Neurol 2013, 70(1):58-65.
23. Dowling M.M., Quinn C.T., Plumb P., et al. Acute silent cerebral ischemia and infarction during acute anemia in children with and without sickle cell disease. Blood 2012, 120:3891-3897.
24. Rogers Z.R. Priapism in sickle cell disease. Hematol Oncol Clin North Am 2005, 19:917-928.
25. Vichinsky E.P., Haberkern C.M., Neumayr L., et al. Acomparison of conservative and aggressive transfusion regimens in the perioperative management of sickle cell disease. NEngl J Med 1995, 333(4):206-213.
26. Howard J., Malfroy M., Llewelyn C., et al. The Transfusion Alternatives Preoperatively in Sickle Cell Disease (TAPS) study: a randomised, controlled, multicentre clinical trial. Lancet 2013, 381(9870):930-938.
27. Fu T., Corrigan N.J., Quinn C.T., et al. Minor elective surgical procedures using general anesthesia in children with sickle cell anemia without pre-operative blood transfusion. Pediatr Blood Cancer 2005, 45(1):43-47.
28. Voskaridou E., Christoulas D., Bilalis A., et al. The effect of prolonged administration of hydroxyurea on morbidity and mortality in adult patients with sickle cell syndromes: results of a 17-year, single-center trial (LaSHS). Blood 2010, 115(12):2354-2363.
29. Lopes de Castro Lobo C., Pinto J.F., Nascimento E.M., et al. The effect of hydroxcarbamide therapy on survival of children with sickle cell disease. Br J Haematol 2013, 161(6):852-860.
30. Wang W.C., Ware R.E., Miller S.T., et al. Hydroxycarbamide in very young children with sickle-cell anaemia: a multicentre, randomised, controlled trial (BABY HUG). Lancet 2011, 377(9778):1663-1672.
31. Ware R.E., Helms R.W., SWiTCH Investigators Stroke with transfusions changing to hydroxyurea (SWiTCH). Blood 2012, 119(17):3925-3932.
32. Miller S.T., Sleeper L.A., Pegelow C.H., et al. Prediction of adverse outcomes in children with sickle cell disease. NEngl J Med 2000, 342(2):83-89.
33. Quinn C.T., Lee N.J., Shull E.P., et al. Prediction of adverse outcomes in children with sickle cell anemia: a study of the Dallas Newborn Cohort. Blood 2008, 111(2):544-548.