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Query: UMLS:C0002895 (sickle cell disease)
 11,747 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Patients with sickle cell disease often develop acute chest syndrome (ACS). Signs of ACS include chest pain, fever, prostration, and pulmonary opacities. Pneumonia and infarction have been implicated in the pathogenesis of this syndrome. Infarction as a result of microvascular occlusion and pneumonia are not easily differentiated with chest radiography or ventilation-perfusion scintigraphy. The authors evaluated the ability of thin section (3-mm) chest computed tomography (CT) to help diagnose microvascular occlusion in ACS and thus help differentiate two of its most likely causes. CT scans of the chest of 10 patients with moderate to severe ACS were retrospectively reviewed by two observers, who listed the number of bronchopulmonary segments showing consolidation; areas of ground-glass attenuation due to early hemorrhagic edema; and paucity or absence of small vessels, arterioles, and venules. In all patients, the degree of hypoxia was out of proportion to the extent of consolidation evident at chest radiography. The CT scans showed microvascular occlusion and areas of ground-glass attenuation in nine patients. Infection was ruled out in eight patients. High-resolution CT may play an important role in the initial evaluation and timely selection of an appropriate treatment regimen aimed at improving tissue perfusion, thus forestalling irreversible organ damage and chronic pulmonary arterial hypertension in patients with sickle cell disease.
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PMID:Acute chest syndrome in sickle cell disease: CT evidence of microvascular occlusion. 845 35

All 302 children treated at the rheumatology clinic of a children's hospital in Santo Domingo between September 1985 and September 1986 were included in a prospective study of the causes of joint pain in children. The 137 girls and 165 boys were grouped in five categories according to the underlying condition. The largest group, reactive arthritic conditions, affected 78 patients (25.9%). 72 of the 78 had rheumatic fever. The second largest category, hematological processes, affected 75 patients (22.6%); 40 of the 75 patients had sickle cell disease, 25 had hemophilia or other conditions, and 8 had neoplasias. 63 patients (20.8%) had infectious processes, including 33 with septic arthritis, 17 with abscesses and cellulitis, 7 with arthritis and osteomyelitis, and 6 with osteomyelitis. Prostration and pain on movement were more pronounced in patients with septic arthritis. 42 children (12.6%) had collagen vascular disease. 32 had juvenile rheumatoid arthritis, 4 had lupus, 3 vasculitis, 2 dermatomyositis, and 1 each had scleroderma and erythum nodosum. 25 patients (8.2%) had a miscellaneous array of other conditions. Sickle cell disease or neoplasia were most likely when anemia was also present. Extreme prostration and signs of inflammation suggested infectious processes. The small joints were primarily affected in juvenile rheumatoid arthritis and sickle cell disease.
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PMID:[Causes of joint pain in children]. 1229 May 50