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

The peak expiratory flow rate (PEFR) was studied in 20 matched pairs of children with homozygous sickle cell disease with either no episodes or six or more episodes of acute chest syndrome. The pairs were carefully matched for height and a highly significant reduction in PEFR was observed in children with multiple episodes of acute chest syndrome. Lateral and anteroposterior chest diameters and chest circumference correlated with PEFR but did not differ between index and control cases. The most likely cause of the reduced PEFR in children with multiple episodes of acute chest syndrome is an accumulating pulmonary fibrosis that decreases lung compliance.
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PMID:Peak expiratory flow rate and the acute chest syndrome in homozygous sickle cell disease. 202 11

Eighteen of 384 patients entered on the Brent sickle cell disease register died between 1974 and 1989, a mortality of one per 128 years of follow up. Two children died from acute splenic sequestration and a third died from fulminant pneumococcal septicaemia: none was taking prophylactic penicillin. Acute chest syndrome was the cause of death in eight young adults and one child. Three deaths occurred after surgery. Cerebrovascular accidents contributed to the cause of death in three cases and there were two sudden unexplained deaths. Ten of the deaths occurred at home or within 24 hours of admission to hospital. Post mortem examinations were made in 14 cases, but the histological appearances of acute chest syndrome were often not recognised. In most cases for whom information was available, the cause of death (chest syndrome, pneumococcal sepsis, postoperative complications) could have been prevented.
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PMID:Patterns of mortality in sickle cell disease in the United Kingdom. 206 23

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

Sickle cell chronic lung disease (SCLD) is a prime contributor to mortality in young adult patients with sickle cell disease, especially those with sickle cell anemia (SS). Both perfusion and diffusion defects have been demonstrated, with generalized pulmonary fibrosis and disabling restrictive lung failure. We report 28 cases (25 SS, 1 S beta(0) thalassemia, 1 S beta(+) thalassemia and 1 SO-Arab) which began during the second decade of life and which ended in death by the fourth decade, after an ordered progression to pulmonary failure and cor pulmonale. Myocardial hypoxia with multifocal fibrosis and segmental infarction occurred in more than one-third of the cases and sudden death was a frequent final event. We define 4 stages of SCLD, based on pulmonary function tests, chest roentgenograms, blood gases, and noninvasive cardiac studies; each stage is 2 or 3 years in length, until death ensues in Stage 4. Case-control analysis showed that the significant risk factors associated with SCLD are 1) the total number of acute chest syndrome events in an individual before the onset of SCLD, (p = 0.0001), 2) sickle cell crisis marked by chest pain (p = 0.03) and 3) aseptic necrosis (p = 0.005). Temporal clustering of acute chest syndrome episodes frequently heralds the onset of SCLD. The pulmonary arterial bed, which has low oxygen tension and low pressure in a slow-flow system, is ideally suited to facilitate the polymerization of sickle hemoglobin, causing endothelial damage and culminating in an obstructive arteriolar vasculopathy. Identification of the significant risk factors predictive of SCLD can lead to early diagnosis of the disease; this is the only hope for effective intervention therapy.
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PMID:Sickle cell chronic lung disease: prior morbidity and the risk of pulmonary failure. 333 82

Reports in the 1970s suggested that acute chest syndrome (ACS) in children with sickle cell disease is usually due to bacterial infection. Studies in adults and more recently in children, however, showed that proved bacterial infection occurs in a minority of these patients and that vascular occlusion is the main pathologic process. In a retrospective study of 32 episodes of ACS in children, a definite bacterial infection was found in 3% (one patient), possible bacterial infection in 11% (four patients), and a possible mycoplasma in 13% (five patients). With the intent to dilute sickle cells, 23 patients received blood transfusion within 24 hours after hospital admission; all showed a dramatic clinical and roentgenographic improvement. Of the nine patients who did not receive a transfusion after hospital admission, the conditions of five patients deteriorated but improved after "late" transfusion; three patients showed slow improvement, and only one patient improved within 48 hours. From this we conclude that vascular occlusion might be the main process in ACS and that early blood transfusion may be valuable in shortening the course and decreasing mortality. The low hemoglobin value at presentation in our patients makes dilution of sickle cells possible by packed red blood cell transfusion rather than exchange transfusion.
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PMID:Beneficial effect of blood transfusion in children with sickle cell chest syndrome. 334 20

Polymorphonuclear leukocyte (PMN) aggregation and chemotaxis were studied in 27 patients with sickle cell disease (SCD). Pain-free patients with SCD had a significantly impaired aggregation response to stimulation with n-formylmethionyl-leucyl-phenylalanine (FMLP) with or without cytochalasin B (CB), compared with normal volunteers (p less than 0.001). Patients with SCD in vaso-occlusive crisis had PMN aggregation induced by FMLP with or without CB that was significantly increased compared with the cohort of pain-free SCD patients (p less than 0.001). PMN from pain-free patients had normal chemotaxis, whereas patients in vaso-occlusive crisis had a significant impairment in PMN chemotaxis. PMN chemotaxis was inversely related to the PMN aggregation response to FMLP with CB (r = -0.75). Thus, the PMN from pain-free patients with SCD appears to have normal or decreased "stickiness" and to develop increased stickiness during vaso-occlusive crisis. The mechanisms responsible for these changes need further elucidation. Alterations in PMN function may be responsible, in part, for the increased risk of infection noted in individuals with SCD and may play a role in the development of the acute chest syndrome.
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PMID:Vaso-occlusive crisis-associated neutrophil dysfunction in patients with sickle-cell disease. 366 21

We have examined our experience of sickle cell disease in the London Borough of Haringey over the past 20 years. There are currently (1986) 145 patients on the Haringey Sickle Cell Register and when comparison is made with other centres, admission for painful crises in Hb SS disease is more frequent than in Jamaica, but the acute chest syndrome appears to be less common in the United Kingdom than in Jamaica. Splenomegaly is less frequent in Hb SC patients in this country and there is also a lower incidence of leg ulceration in both Hb SS and Hb SC disease in the United Kingdom than in Jamaica. The incidence of alloimmunization among transfused sickle cell disease individuals in Haringey is 21%, the most frequently encountered antibodies being anti-E and anti-Kell. All pregnant patients with sickle cell disease were managed with regular blood transfusion throughout pregnancy starting at 12 weeks of gestation. There were no fetal or maternal mortalities, although three babies born were 'small for dates' despite uneventful antenatal courses and adequate Hb A levels being achieved in the mother. Examination of the effect of seasonal variation on the incidence of hospital admissions for painful crises reveals no significant clustering of cases in a particular month or season of the year.
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PMID:Sickle cell disease in North London. 366 11

A child with sickle cell disease presented acute chest syndrome, acute cholestasis and a neurological syndrome. Bilirubin, LDH, alkaline phosphatase, urea and creatinines were markedly increased. Circulating immune complexes were slightly elevated. The patient improved dramatically after a plasma exchange.
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PMID:Plasma exchange for acute cholestasis in homozygous sickle cell disease. 372 21

Early symptoms were observed in a representative sample of 166 children with sickle cell-hemoglobin C disease diagnosed at birth. Symptoms were uncommon in the first year of life; in approximately 50% specific symptoms had developed by 5 years, but 22% remained without specific symptoms to 10 years. The age at presentation was significantly earlier in patients with low hemoglobin F levels, but was not influenced by heterozygous alpha-thalassemia-2. Painful crisis was the initial manifestation in 77% of the children; other symptoms included dactylitis (14%) and pneumococcal septicemia and acute splenic sequestration (4% each). The commonest nonspecific symptom was acute chest syndrome. The relatively mild early clinical course of sickle cell-hemoglobin C disease indicates that neonatal diagnosis does not have the same urgency as for homozygous sickle cell disease.
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PMID:Clinical presentation of sickle cell-hemoglobin C disease. 376 Oct 71

A 6-year-old girl with sickle cell disease was admitted to the hospital with the diagnosis of the acute chest syndrome. The laboratory findings and the radionuclear lung scan supported a diagnosis of pulmonary infarction rather than pneumonia. She improved with intravenous fluids, oxygen, penicillin, and theophylline. The most likely explanation for the rapid resolution of the clinical syndrome, the chest x-ray, and lung scan abnormalities is that masses of sickled cells caused transient pulmonary vascular occlusion leading to perfusion defects and ischemia, and that the sickled cell thrombi were dislodged before the infarction occurred. To our knowledge, this phenomenon has not been described as a cause of the acute chest syndrome in sickle cell disease in children.
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PMID:Acute reversible pulmonary ischemia. A cause of the acute chest syndrome in sickle cell disease. 406 60


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