eMedicine Specialties > Emergency Medicine > Pediatric

Pediatrics, Sickle Cell Disease: Differential Diagnoses & Workup

Author: Nedra R Dodds, MD, Medical Director, Opulence Aesthetic Medicine
Coauthor(s): Hosseinali Shahidi, MD, MPH, Assistant Professor, Departments of Emergency Medicine and Pediatrics, State University of New York and Health Science Center at Brooklyn
Contributor Information and Disclosures

Updated: Nov 21, 2007

Differential Diagnoses

Altitude Illness - Pulmonary Syndromes
Shock, Hypovolemic
Anemia, Chronic
Shock, Septic
Angioedema
Stroke, Ischemic
Arthritis, Rheumatoid
Withdrawal Syndromes
Cholelithiasis
Pediatrics, Dehydration
Pediatrics, Pneumonia

Other Problems to Be Considered

Sickle cell hemoglobin
Thalassemia

Workup

Laboratory Studies

  • Newborn screening for sickle hemoglobinopathies is mandatory in 43 states. Therefore, most patients presenting to the ED have a known diagnosis.
  • Specific diagnosis is confirmed with hemoglobin electrophoresis performed in specialized reference laboratories.
  • The patient with homozygous sickle cell disease typically has a hemoglobin level of 5-9 g/dL, with a hematocrit level decreased to 17-29%.
  • The total leukocyte count is elevated to 12,000-20,000 cells/mm3 (12-20 X 109/L), with a predominance of neutrophils.
  • The platelet count is increased, and the sedimentation rate is low.
  • The reticulocyte count is usually elevated, but it may vary depending on the extent of baseline hemolysis.
  • Peripheral blood smears demonstrate target cells, elongated cells, and characteristic sickle erythrocytes.
  • Results of hemoglobin solubility testing are positive, but they do not distinguish between sickle cell disease and sickle cell trait.
  • Perform the following laboratory studies in patients younger than 5 years who presents with a temperature >38.5°C:
    • Tests for liver enzyme levels
    • Tests for electrolyte levels
    • Determination of complete blood count (CBC) with differential
    • Determination of the reticulocyte count
    • Urinalysis
    • Blood, urine, and throat cultures
  • During bacterial infection, elevations of the absolute band count to more than 1000/mm3 are common compared to moderate elevations during the painful crisis.
  • Laboratory studies for the patient presenting with a painful crisis should routinely include determination of the CBC and reticulocyte counts for comparison with counts from previous episodes.
  • During the vaso-occlusive crisis, the reticulocyte count may rise to 30%.
  • Perform blood gas analysis if pulmonary symptoms exist.

Imaging Studies

  • In patients with cough or chest pain, prompt chest radiography is warranted to differentiate pneumonia versus acute chest syndrome.
  • If an ill-appearing patient presents with pain in the extremities that differs from his or her usual painful episodes, radiography and/or MRI of the affected extremity is necessary to exclude osteomyelitis.
  • Evaluate any new onset of neurologic changes by performing head CT or brain MRI to exclude a stroke.

Other Tests

  • Use a pulse oximeter on the patient complaining of chest pain to assess hypoxia related to acute chest syndrome.

Procedures

  • Consider lumbar puncture in the pediatric patient with altered mental status, meningeal signs, and fever to exclude meningitis.
  • Also perform lumbar puncture if a subarachnoid hemorrhage is suspected if head CT reveals no evidence of increased intracranial pressure or mass lesion.

More on Pediatrics, Sickle Cell Disease

Overview: Pediatrics, Sickle Cell Disease
Differential Diagnoses & Workup: Pediatrics, Sickle Cell Disease
Treatment & Medication: Pediatrics, Sickle Cell Disease
Follow-up: Pediatrics, Sickle Cell Disease
References
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References

  1. AAP Committee on Genetics. Health supervision for children with sickle cell diseases and their families. American Academy of Pediatrics. Committee on Genetics. Pediatrics. Sep 1996;98(3 Pt 1):467-72. [Medline].

  2. Bachman D, Barkin R, Brennan S. Hematologic and oncologic disorders. In: Pediatric Emergency Medicine: Concepts and Clinical Practice. 2nd ed. 1997:907-11.

  3. Berman B. Sickle cell anemia. In: Manual of Emergency Pediatrics. 4th ed. 1992:61-4.

  4. Dampier C, Setty BN, Eggleston B, et al. Vaso-occlusion in children with sickle cell disease: clinical characteristics and biologic correlates. J Pediatr Hematol Oncol. Dec 2004;26(12):785-90. [Medline].

  5. Girot R, Begue P. [Sickle cell disease in childhood in 2004]. Bull Acad Natl Med. 2004;188(3):491-505; discussion 505-6. [Medline].

  6. Gulbis B, Haberman D, Dufour D, et al. Hydroxyurea for sickle cell disease in children and for prevention of cerebrovascular events. The Belgian experience. Blood. Dec 16 2004;[Medline].

  7. Martin P, Pearson H. The hemoglobinopathies and thalassemias. In: Principles and Practice of Pediatrics. 2nd ed. 1994:1660-1.

  8. Nordness ME, Lynn J, Zacharisen MC, et al. Asthma is a risk factor for acute chest syndrome and cerebral vascular accidents in children with sickle cell disease. Clin Mol Allergy. Jan 21 2005;3(1):2. [Medline].

  9. Serjeant GR, Serjeant BE, Thomas PW, et al. Human parvovirus infection in homozygous sickle cell disease. Lancet. May 15 1993;341(8855):1237-40. [Medline].

  10. Vichinsky EP, Styles LA, Colangelo LH, et al. Acute chest syndrome in sickle cell disease: clinical presentation and course. Cooperative Study of Sickle Cell Disease. Blood. Mar 1 1997;89(5):1787-92. [Medline].

  11. Wethers DL. Sickle cell disease in childhood: Part II. Diagnosis and treatment of major complications and recent advances in treatment. Am Fam Physician. Sep 15 2000;62(6):1309-14. [Medline].

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Further Reading

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Keywords

sickle cell anemia, sickle disease, sickle hemoglobinopathy syndromes, hemolytic anemia, aplastic anemia crisis, hemoglobin synthesis, sickle cells, homozygous sickle cell disease

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Contributor Information and Disclosures

Author

Nedra R Dodds, MD, Medical Director, Opulence Aesthetic Medicine
Nedra R Dodds, MD is a member of the following medical societies: American Academy of Anti-Aging Medicine, American Academy of Cosmetic Surgery, American College of Emergency Physicians, American Medical Association, National Medical Association, and Society for Academic Emergency Medicine
Disclosure: Nothing to disclose.

Coauthor(s)

Hosseinali Shahidi, MD, MPH, Assistant Professor, Departments of Emergency Medicine and Pediatrics, State University of New York and Health Science Center at Brooklyn
Hosseinali Shahidi, MD, MPH is a member of the following medical societies: American Academy of Pediatrics, American College of Emergency Physicians, and American Public Health Association
Disclosure: Nothing to disclose.

Medical Editor

Garry Wilkes, MBBS, FACEM, Director of Emergency Medicine, Bunbury Health Service, Western Australia Country Health Service; Adjunct Associate Professor, School of Exercise, Biomedical and Health Sciences, Faculty of Computing, Health and Science, Edith Cowan University; Medical Director, St John Ambulance Service
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine.com, Inc
Disclosure: Nothing to disclose.

Managing Editor

Grace M Young, MD, Associate Professor, Department of Pediatrics, University of Maryland Medical Center
Grace M Young, MD is a member of the following medical societies: American Academy of Pediatrics and American College of Emergency Physicians
Disclosure: Nothing to disclose.

CME Editor

John Halamka, MD, Chief Information Officer, CareGroup Healthcare System, Assistant Professor of Medicine, Department of Emergency Medicine, Beth Israel Deaconess Medical Center; Assistant Professor of Medicine, Harvard Medical School
John Halamka, MD is a member of the following medical societies: American Academy of Emergency Medicine and Society for Academic Emergency Medicine
Disclosure: Nothing to disclose.

Chief Editor

Richard G Bachur, MD, Assistant Professor of Pediatrics, Harvard Medical School; Associate Chief and Fellowship Director, Attending Physician, Division of Emergency Medicine, Children's Hospital of Boston
Richard G Bachur, MD is a member of the following medical societies: American Academy of Pediatrics, Society for Academic Emergency Medicine, and Society for Pediatric Research
Disclosure: Nothing to disclose.

 
 
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