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Radiologic Manifestations of Sickle Cell Hemoglobinopathy FREE

Jack Reynolds, MD
[+] Author Affiliations

Reprint requests to 5323 Harry Hines Blvd, Dallas, TX 75235 (Dr Reynolds).

Edited by Z. Danilevicius, MD, Senior Editor.


JAMA. 1977;238(3):247-250. doi:10.1001/jama.1977.03280030053026
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SICKLE CELL hemoglobinopathy is capable of producing distinctive radiological signs in many organ systems. In the homozygous state (SS), it is a relatively common disorder, being present at birth in one of 625 black Americans.1 Its victims suffer a debilitating, frequently painful illness. Symptomatic forms of this hemoglobinopathy also occur when a single S gene is inherited in combination with the gene for another abnormal hemoglobin such as β thalassemia (S Thal) or hemoglobin C (SC).2 The clinical course and the frequency and magnitude of roentgenographic signs in patients with the mixed forms of sickle cell disease vary greatly among persons with these genotypes. In some, the condition is almost indistinguishable from SS, whereas others exhibit few if any of the stigmas of their hereditary disorder. While there are some notable exceptions, symptoms and roentgenographic findings are usually absent in persons with sickle cell trait (AS), since the

REFERENCES

Motulsky AG:  Frequency of sickling disorders in U.S. blacks . N Engl J Med 288:31-33, 1973;.
Reynolds J, Pritchard JA, Ludders D, et al:  Roentgenographic and clinical appraisal of sickle cell beta-thalassemia disease . Am J Roentgenol Radium Ther Nucl Med 118:378-400, 1973;.
Konotey-Ahula FD:  The sickle cell diseases: Clinical manifestations including the "sickle crisis." Arch Intern Med 133:611-619, 1974;.
Reynolds J: The Roentgenological Features of Sickle Cell Disease and Related Hemoglobinopathies . Springfield, Ill, Charles C Thomas Publisher, 1965;.
Weinberg AG, Currarino G:  Sickle cell dactylitis . Am J Clin Pathol 58:518-523, 1972;.
Reynolds J:  A re-evaluation of the "fish vertebra" sign in sickle cell hemoglobinopathy . Am J Roentgenol Radium Ther Nucl Med 97:693-707, 1966;.
Wigh R, Thompson HJ Jr:  Cortical fissuring in osteomyelitis complicating sickle cell anemia . Radiology 55:553-556, 1950;.
Lutzker L, Alavi A:  Bone and marrow imaging in sickle cell disease: Diagnosis of infarction . Semin Nucl Med 6:83-93, 1976;.
Majd M, Frankel RS:  Radionuclide imaging in skeletal inflammatory and ischemic disease in children . Am J Roentgenol Radium Ther Nucl Med 126:832-841, 1976;.
Bromberg PA:  Pulmonary aspects of sickle cell disease . Arch Intern Med 133:652-657, 1974;.
Barrett-Connor E:  Pneumonia and pulmonary infarction in sickle cell anemia . JAMA 224:997-1000, 1973;.
Uzsoy NK:  Cardiovascular findings in patients with sickle cell anemia . Am J Cardiol 13:320-328, 1964;.
Buckalew VM, Someren A:  Renal manifestations of sickle cell disease . Arch Intern Med 133:660-669, 1974;.
Margolies SI, Minkin SD:  Sickle cell disease: The roentgenographic manifestations of urinary tract abnormalities in adults . Am J Roentgenol Radium Ther Nucl Med 107:702-710, 1969;.
Marquis JR, Khazem B:  Sickle-cell disease: Renal roentgenographic changes in children . Radiology 98:47-52, 1971;.
Khademi M, Marquis JR:  Renal angiography in sickle-cell disease . Radiology 107:41-46, 1973;.
Khademi M:  Angiographic measurement of renal compartments . Radiology 113:51-58, 1974;.
Eckert DE, Jonutis AJ, Davidson AJ:  The incidence and manifestations of urographic papillary abnormalities in patients with S hemoglobinopathies . Radiology 113:59-63, 1974;.
Phillips JC, Gerald BE:  The incidence of cholelithiasis in sickle cell disease . Am J Roentgenol Radium Ther Nucl Med 113:27-28, 1971;.

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Motulsky AG:  Frequency of sickling disorders in U.S. blacks . N Engl J Med 288:31-33, 1973;.
Reynolds J, Pritchard JA, Ludders D, et al:  Roentgenographic and clinical appraisal of sickle cell beta-thalassemia disease . Am J Roentgenol Radium Ther Nucl Med 118:378-400, 1973;.
Konotey-Ahula FD:  The sickle cell diseases: Clinical manifestations including the "sickle crisis." Arch Intern Med 133:611-619, 1974;.
Reynolds J: The Roentgenological Features of Sickle Cell Disease and Related Hemoglobinopathies . Springfield, Ill, Charles C Thomas Publisher, 1965;.
Weinberg AG, Currarino G:  Sickle cell dactylitis . Am J Clin Pathol 58:518-523, 1972;.
Reynolds J:  A re-evaluation of the "fish vertebra" sign in sickle cell hemoglobinopathy . Am J Roentgenol Radium Ther Nucl Med 97:693-707, 1966;.
Wigh R, Thompson HJ Jr:  Cortical fissuring in osteomyelitis complicating sickle cell anemia . Radiology 55:553-556, 1950;.
Lutzker L, Alavi A:  Bone and marrow imaging in sickle cell disease: Diagnosis of infarction . Semin Nucl Med 6:83-93, 1976;.
Majd M, Frankel RS:  Radionuclide imaging in skeletal inflammatory and ischemic disease in children . Am J Roentgenol Radium Ther Nucl Med 126:832-841, 1976;.
Bromberg PA:  Pulmonary aspects of sickle cell disease . Arch Intern Med 133:652-657, 1974;.
Barrett-Connor E:  Pneumonia and pulmonary infarction in sickle cell anemia . JAMA 224:997-1000, 1973;.
Uzsoy NK:  Cardiovascular findings in patients with sickle cell anemia . Am J Cardiol 13:320-328, 1964;.
Buckalew VM, Someren A:  Renal manifestations of sickle cell disease . Arch Intern Med 133:660-669, 1974;.
Margolies SI, Minkin SD:  Sickle cell disease: The roentgenographic manifestations of urinary tract abnormalities in adults . Am J Roentgenol Radium Ther Nucl Med 107:702-710, 1969;.
Marquis JR, Khazem B:  Sickle-cell disease: Renal roentgenographic changes in children . Radiology 98:47-52, 1971;.
Khademi M, Marquis JR:  Renal angiography in sickle-cell disease . Radiology 107:41-46, 1973;.
Khademi M:  Angiographic measurement of renal compartments . Radiology 113:51-58, 1974;.
Eckert DE, Jonutis AJ, Davidson AJ:  The incidence and manifestations of urographic papillary abnormalities in patients with S hemoglobinopathies . Radiology 113:59-63, 1974;.
Phillips JC, Gerald BE:  The incidence of cholelithiasis in sickle cell disease . Am J Roentgenol Radium Ther Nucl Med 113:27-28, 1971;.
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