Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002895 (sickle cell disease)
 11,747 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Although microvascular occlusion has been considered a basis for pathophysiology of the myocardium during the crisis of sickle cell anemia, the status of the left ventricle in uncertain. To determine if left ventricular performance is affected by crisis, 11 patients were evaluated noninvasively by the systolic time interval method on the first day of crisis and serially until recovery. There were no significant differences in the time intervals over this period. In addition, since the serum CPK-MB isoenzyme was not elevated during crisis and evidence of acute injury was not present on ECG, myocardial necrosis appeared unlikely. Four patients on subsequent admission exhibited systolic time interval values similar to the earlier crisis. To determine if there were chronic changes in cardiac function, subjects with sickle cell hemoglobin were studied between crises. Those under 23 years of age were not dissimilar from a group of normals and a group of patients with chronic blood loss anemia A significant abnormality of the PEP/LVET ratio was observed in subjects over 23 years of age. Similar observations were made on echocardiography, with subjects over the age of 23 demonstrating an abnormal ejection fraction compared to the younger group, despite enhanced end-diastolic diameter. Thus, it is suggested that the chronic hemolytic process in subjects with sickle cell anemia may effect cumulative myocardial alterations, resulting in chronic cardiac malfunction in the apparent absence of acute ischemia during crises.
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PMID:Left ventricular performance during and after sickle cell crisis. 43 33

Hypoxemia is common in SCD and likely exacerbates SCD vasculopathy. Pulse oximeter correlation with arterial oxygen tension in patients with SCD may at times be poor and arterial blood gas confirmation is required in hypoxic patients. Supplemental oxygen should be administered for the correction of hypoxemia, which if untreated creates a risk of multi-organ failure. Transfusion and hydroxyurea can improve oxygen delivery to tissues and organs. The role of supplemental oxygen therapy in preventing or reversing SCD vasculopathy is controversial. Nitric oxide therapy for VOC pain has not fulfilled promise to date. On the other hand, lung distension (CPAP, incentive spirometry, PEP therapy) are promising treatments requiring further study.
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PMID:Hypoxemia in sickle cell disease: significance and management. 2446 42