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

Fifty-two episodes of fever, chest pain, increased leukocytosis, and pulmonary infiltrate ("acute chest syndrome") were studied in 28 adults with sickle cell anemia. Possible bacterial pathogens were identified in sputum cultures from less than half of the episodes; no pneumococci were found, and Staphylocococcus aureus was the only bacterium associated with a longer illness than that seen when only normal flora were recovered. Much disease diagnosed as "pneumonia" in adults with sickle cell anemia is probably pulmonary infarction. Many of these patients will recover with no more than modest supportive care; if antibiotics are used they should be directed against S aureus (and possibly Hemophilus species). Pneumococcal polysaccharide vaccine has great potential for preventing life-threatening infection in children with sickle cell anemia, but may not change the incidence or severity of the acute chest syndrome in adults.
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PMID:"Acute chest syndrome" in adults with sickle cell anemia. Microbiology, treatment, and prevention. 3 55

The injury-vasospasm hypothesis of IHD was discussed in relation to coronary artery autoregulation and the anoxic-feedback mechanism. Observations in the recent literature, not usually attributed to spasm, were examined in light of this phenomenon. This includes reperfusion models of experimental AMI, the association of AMI with myocarditis, and findings in AMI and SCD as necrotic microlesions, prodromata, and epicardial arterial plaque rupture and hemorrhage. The disparity between the severity of coronary disease and the occurrence of the various types of IHD suggest that atherosclerosis itself does not precipitate attacks of chest pain. It was emphasized that plaque rupture due to spasm might help induce CAT. With exercise, the possible importance of the autoregulatory system was explored in the prevention and induction of AMI and SCD, and the improvement of AP. The role of spasm in IHD should be defined.
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PMID:The injury-vasospasm hypothesis of ischemic heart disease, revisited. 33 91

The experience with 45 patients with lung abscess over a three-year period at the University College Hospital (UCH), Ibadan, is presented. This study confirms the rarity of this disease among Nigerian children and its prevalence in young adults in the third and fourth decades of life. The most common presenting symptoms were purulent cough, chest pain, fever, and life-threatening hemoptysis which was the sole indication for emergency operation in 14 out of 16 patients who were treated surgically. The predominance of these abscesses in the right lung, especially in the superior segment of the lower lobe, supports the fact that aspiration of infected material, following depressed level of consciousness, esophageal obstruction, foreign bodies, and oral sepsis form the major causative factors in patients with lung abscess. The frequent association of sickle cell disease, bronchiectasis, hypertension, and pulmonary aspergilloma contribute significantly to the morbidity and mortality attendant to this disease in our environment. Twenty-nine patients were treated medically with five deaths and 16 patients were treated surgically with six deaths. The high operative mortality (37.5 percent) in this series was due to the extreme emergency conditions under which these patients were operated.
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PMID:Lung abscess: a review of three-years' experience at the University College Hospital, Ibadan. 42 74

The symptoms of thromboembolism in sickle cell anemia patients with acute chest syndromes are difficult to differentiate from the similar symptoms of painful thoracic crises and infectious pulmonary episodes. Furthermore, the chronic pulmonary abnormalities in sickle cell disease frequently contribute to the confusing results of noninvasive diagnostic procedures usually employed in evaluating pulmonary thromboembolism. In this study the chronic pulmonary status of asymptomatic sickle cell patients was defined, and this information was used in the evaluation of patients with acute chest syndromes suggestive of pulmonary thromembolism. Sixteen asymptomatic sickle cell patients were prospectively studied by chest roentgenography, spirometry, arterial gas analyses, and radioisotopic lung scans. There was an appreciable degree of preexisting chronic restrictive lung disease with mild to moderate arterial hypoxemia and abnormal lung scans in more than half of the patients. These prospective baseline data were incorporated into the diagnostic evaluation of four of these patients who later developed an acute chest syndrome suggestive of pulmonary thromboembolism. Determination of the cause of the chest pain was greatly faciliated by the existence of the baseline pulmonary data. In another patient persistence of abnormal studies following a presumed thromboembolic episode aided diagnostic evaluation when another episode of chest pain occurred. The findings indicate that comprehensive pulmonary studies in sickle cell patients while in an asymptomatic state will provide baseline data which aid the evaluation of possible pulmonary thromboembolism in acute chest syndromes without resorting to high-risk invasive studies.
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PMID:The diagnosis of pulmonary thromboembolism in sickle cell disease. 54 37

Acute illness characterized by fever, cough, chest pain and pulmonary infiltrates on chest film is characteristic of patients witb sickle cell anemia and with sickle-C disease. The underlying hemoglobinopathy is usually recognized in the former, but because patients with sickle-C disease as a rule have less severe anemia and fewer, less severe crises their abnormal hemoglobin may not be detected until adolescence or adulthood. Acute pulmonary illness in such patients may therefore present a diagnostic dilemma for the unwary. Two cases are presented to highlight this point. Pulmonary angiographic findings in patients with sickle cell states might add information about the pathogenesis of this disorder since vascular occlusion appears to play a major role. Pulmonary angiograms in the two patients we describe documented the presence of localized abnormalities of perfusion. In one, the presence of filling defects in medium-sized arteries suggests intravascular thromboembolism.
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PMID:Hemoglobin S-C disease presenting as acute pneumonitis with pulmonary angiographic findings in two patients. 112 91

A link between cigarette smoking and "acute chest syndrome" in sickle cell anemia is suggested. Acute chest syndrome in the patient with sickle cell anemia is characterized by fever, leukocytosis, cough, chest pain, and pulmonary infiltrates in the chest radiograph. This article describes the results of a study of 69 adolescent and young adult sickle cell anemia patients. Twenty-nine of these patients were smokers, three were former smokers, and 37 were nonsmokers. Patients completed respiratory questionnaires that focused on smoking habits and included a history of chest syndrome. Information obtained was confirmed by review of clinical records. The chi-square test demonstrated a strong relationship between cigarette smoking and chest syndrome in sickle cell anemia. All 29 smokers had a history of chest syndrome, but only 24 of 37 nonsmokers had such a history. Although the exact mechanism of the relationship between smoking and the development of acute chest syndrome remains speculative, cigarette smoking joins infection, hypoxia, acidosis, infarction, dehydration, and analgesics as a causative factor in adolescent and adult patients with sickle cell anemia. Behavioral modification of the smoking habit in patients with sickle cell anemia may decrease the frequency of acute chest syndrome and sequelae of sickle cell lung disease.
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PMID:Smoking is a factor in causing acute chest syndrome in sickle cell anemia. 157 2

In the absence of evidence for pneumonia or pulmonary embolus, primary pulmonary infarction has been assumed to be the cause of the syndrome of chest pain, fever, and pulmonary infiltrate on chest X-ray that commonly complicates sickle cell anaemia. To find out whether the syndrome might be due to rib infarction, 99mTc-diphosphonate bone scans were done. In the eleven episodes thus investigated (10 patients) the scans showed segmental areas of increased radionuclide uptake in ribs, indicative of bone infarction. A possible sequence of events is that the rib infarcts are primary and cause bone pain, followed by soft tissue reaction, pleuritis, and splinting. The resultant hypoventilation leads to atelectasis and subsequent development of the radiographic changes of the acute chest syndrome. Prevention of hypoventilation and treatment of bone pain are important therapeutic goals.
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PMID:Rib infarcts and acute chest syndrome in sickle cell diseases. 167 22

This article describes a 28-yr-old black man with sickle cell anemia who presented with severe chest pain secondary to acute infarction of the body of the sternum, hypoventilation, and hypoxemia with no evidence of acute chest syndrome. A bone scan performed 5 days after admission revealed increased uptake in the sternum, suggesting sternal infarction. Repeat bone scan performed 2 mo later demonstrated normal concentration in the sternum.
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PMID:Severe hypoxemia secondary to acute sternal infarction in sickle cell anemia. 186 89

The possibility that myocardial ischemia may be associated with chest pain during painful crises was evaluated prospectively in 20 patients (11 women and nine men) with sickle cell disease (19 SS, 1 S beta + thalassemia). Sixteen of 20 (80%) had abnormal ECGs, 7 (35%) had transient ST-T wave changes, and 3 (15%) had persistent ST-T wave changes, both consistent with ischemia; 6 (30%) had nonspecific ST-T changes, and 4 (20%) had normal tracings. Serum enzymes (CK, SGOT, LDH) were abnormal in 16 of 19 (84%); 1 had CK-MB detected, (5%) and 1 had LDH1 to LDH2 reversal. All 10 Tc-99m pyrophosphate scans performed were negative; 4 of 6 (66%) thallium-201 scans had focal defects, and 5 of 8 (63%) radionuclide angiograms (MUGAs) had focal wall motion abnormalities. Three of 8 (38%) MUGAs showed cardiac dilation, diffuse hypokinesis, and reduced ejection fractions. Thus, myocardial damage may be a potentially serious complication of patients with sickle cell anemia who present with chest pain during painful crises. Studies are indicated to define the significance and pathophysiology of these observations.
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PMID:Sickle cell anemia: does myocardial ischemia occur during crisis? 203 80

Vaso-occlusive crisis is the most common cause of morbidity in patients with sickle cell anemia (SCA). Those patients are frequently admitted with chest signs and symptoms suggesting an infective process. The term acute chest syndrome (ACS) is used to describe those chest episodes in SCA patients, and it is postulated that a primary bone vaso-occlusive crisis may be the cause of the acute chest syndrome in SCA patients. In this study we report 52 episodes of ACS in a group of 22 children with SCA. Chest pain, fever, and leukocytosis were a constant clinical finding. The hematological, radiological, and bacteriological studies are reported. There is a constant and significant fall in hemoglobin levels from 88 +/- 10 g/L (8.8 +/- 1.0 g/dl) to 68 +/- 15 g/L (6.8 +/- 5 g/dl). Unilateral or bilateral pulmonary basal infiltrations were found in 50 episodes. Pleural effusion was noticed in 60 episodes, and it was bilateral in three. No significant bacteriological findings were present.
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PMID:Acute chest syndrome in children with sickle cell anemia. 239 72


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