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

Acute chest syndrome complicating sickle cell anemia may progress to adult respiratory distress syndrome despite appropriate therapy. Extra-alveolar air leaks may complicate the care of these patients as conventional mechanical ventilation becomes increasingly difficult. We successfully treated a child with sickle cell anemia, acute chest syndrome, adult respiratory distress syndrome, and severe extra-alveolar air leaks using a new combined mode ventilatory approach: pressure control with high-frequency ventilation.
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PMID:Combined pressure control/high frequency ventilation in adult respiratory distress syndrome and sickle cell anemia. 798 30

The efficacy of a long-term transfusion regimen in preventing sickle cell disease complications is unknown. We examined 17 patients before, during, and after transfusion for cerebral vascular accident and vaso-occlusive crisis. Total hospitalization rate, as well as admissions for vaso-occlusive crisis, cases of acute chest syndrome, and bacterial infections decreased while patients were on a transfusion regimen.
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PMID:Effects of a long-term transfusion regimen on sickle cell-related illnesses. 799 63

Management of transfusion therapy in sickle cell disease patients with acute complications is often made difficult because of confusing indications, a variety of methods, disparate goals, and varying needs for maintenance transfusion. In priapism, acute chest syndrome, many major surgical procedures, toxemia of pregnancy, and cerebrovascular accidents, the target hemoglobin A level should be made as close to 100% as possible by mechanized red blood cell exchange. If mechanized exchange is unavailable, manual exchange should be instituted. Hemoglobin A should be maintained at greater than 60% to 70% by periodic simple transfusion until patients are fully recovered. Stroke patients should undergo maintenance transfusions for at least 3 years and perhaps 5 to 12 years. Physicians and patients should be aware of the transfusion-related risks of hepatitis and HIV infection. Alloimmunization and iron overload should be minimized in patients requiring frequent transfusions and chelation therapy should be utilized for iron overload.
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PMID:Transfusion therapy in sickle cell disease patients: methods and acute indications. 812 Apr 39

The etiology of most cases of acute chest syndrome (ACS) in sickle cell disease (SCD) is unknown. Although pulmonary fat embolism (PFE) is frequently found on autopsy, it is rarely considered in the differential diagnosis in pediatric patients. We conducted a study to determine if we could identify PFE in SCD patients with ACS, define the clinical and laboratory course of PFE, and determine if bronchoalveolar lavage is safe and useful in diagnosis of PFE. Twenty-seven SCD patients with ACS were evaluated and compared with 43 control patients. Serial tests (complete blood count, platelet count, nucleated red blood cells [NRBCs], chest x-ray, and oxygen saturations) were compared with steady-state results. Diagnosis of PFE was made by quantitative evaluation of pulmonary macrophages for intracellular fat. No serious complications from bronchoscopy were observed. In the SCD patients with ACS, 12 were PFE+ and 15 were PFE-. The clinical course of the two groups was quite different. All PFE+ patients experienced bone pain and 11 of 12 had chest pain. In contrast, only 6 of 15 of PFE- patients had bone or chest pain. Neurologic symptoms developed in 6 of 12 of the PFE+ group and in none of the PFE- group. Mean hospital days for PFE+ was 13 compared with 7 for PFE-. Laboratory studies in PFE+ showed a significant decrease in hemoglobin (-2 g, P < .05), platelet count (-293,000, P < .001), and an increase in NRBCs/100 white blood cells (+8.3, P < .001) compared with PFE-. These results indicate that when PFE is associated with ACS, it is characterized by a distinct clinical course, and that bronchial lavage is a safe and useful test in diagnosing PFE in patients with ACS.
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PMID:Pulmonary fat embolism: a distinct cause of severe acute chest syndrome in sickle cell anemia. 819 47

Sickle cell disease is enormously variable in its expression and outcome. In addition to this intrinsic variability are the problems of symptomatic selection biasing observations towards the severe end of a wide clinical spectrum and a truly changing natural history as a result of better management. Against this background, there was a need for a description of the disease in a truly representative sample of patients and this objective has been approached in the Jamaican Cohort Study of Sickle Cell Disease. Initiated in 1973, this study is based on all cases of sickle cell disease detected among 100,000 consecutive normal deliveries in Kingston, Jamaica. All affected children as well as age matched normal controls have been followed prospectively and are currently aged 11 to 19 years. The following review is based on lessons learnt from this cohort study. It is not intended to be a comprehensive survey of knowledge of sickle cell disease and does not address major contributions from studies elsewhere. In some ways, therefore, the review may appear unbalanced because of this specific objective. However, a great deal has been learnt about the evolution of the abnormal haematology of sickle cell disease and its relationship to clinical features. The causes of early mortality in sickle cell disease in Jamaica are described and the major complications such as acute splenic sequestration, pneumococcal septicaemia, aplastic crisis, hypersplenism, and acute chest syndrome have been addressed with varying success. Overall survival to the age of 19 years has been 75% and it is planned that the study should continue to define the problems of late adolescence and early adult life.
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PMID:Management of sickle cell disease; lessons from the Jamaican Cohort Study. 824 29

Chlamydia pneumoniae has now been associated with pneumonia, bronchitis, pharyngitis, acute chest syndrome of sickle cell disease, and asthma. Because of the difficulty of primary isolation and tissue-culture adaptation of this organism, we used a previously developed polymerase chain reaction-enzyme immunoassay (PCR-EIA) to screen 132 culture-negative bronchoalveolar lavage (BAL) specimens from 108 immunocompromised patients (34% of whom were positive for human immunodeficiency virus) and 7 healthy volunteers. Thirteen specimens (9.8%) from 12 immunocompromised patients (11.1%) gave a positive result; one patient had two positive specimens obtained 3 days apart. No healthy volunteer had a PCR-EIA-positive BAL specimen. Twelve (11.1%) of the immunocompromised patients also had diagnostic levels of antibody. Four patients had positive results in both PCR-EIA and serological tests. Thus 20 (18.5%) of the 108 patients had laboratory evidence of C. pneumoniae infection. These data indicate that diagnosis of acute infection with C. pneumoniae can be established more rapidly and reliably by PCR-EIA than by culture or serology, particularly among immunocompromised patients, in whom serological changes in response to infection are relatively undependable. With an infection rate of 11.1% according to PCR-EIA, C. pneumoniae should be considered in the evaluation and treatment of pneumonia in immunocompromised patients.
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PMID:Detection of Chlamydia pneumoniae by polymerase chain reaction-enzyme immunoassay in an immunocompromised population. 826 55

A patient suffering from sickle cell anaemia who presented with the acute chest syndrome is described. The syndrome is reported to be one of the commonest causes of death in adults suffering from sickle cell disease. As it is difficult at presentation to distinguish the syndrome from infection, it is usual to give treatment appropriate for both. Early exchange transfusion is thought to limit further damage from sickling and offers the best hope of recovery.
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PMID:Acute chest syndrome in sickle cell anaemia. 829 44

Fifteen S/S children with severe SCD were transplanted with marrow from HLA identical siblings. All developed frequent (> 3/y) vaso-occlusive crises (VOC) associated with recurrent acute chest syndrome episodes (n = 10), osteitis (n = 3), osteonecrosis (n = 3), strokes (n = 3) or frequent massive deglobulisation (n = 2). Two children undergone splenectomy, two were chelated and two had an erythroid allo-immunization. Ethnic origins were from various countries in Africa (n = 11), North-Africa (n = 3) or West Indies (n = 1). At BMT, they were 2y 3m to 14y 9m old (mean: 8y 7m). Donors were AS (n = 11) or AA (n = 4). At first, various conditioning regimens were used consisting of busulfan (BU) plus Cyclophosphamide (CY) at different doses: CY:200 mg/kg (n = 13) or 260 mg/kg (n = 2); BU: 14 mg/kg (n = 1), 16 mg/kg (n = 9), > 16 mg/kg (n = 5); one patient received also TLI and the last two anti-thymoglobulin (ATG): 20 mg/kg. GVHD prophylaxis was CSA alone (n = 4) or CSA plus short-term MTX (n = 11). Median follow-up is 28 months (5 m to 53 m). All patients had an engraftment (d12 to d32) with a stable total chimerism in 10/14 patients. In the 4 others, partial chimerism was observed: one patient had a early and progressive rejection of his graft but is doing very well (35 m follow-up) without any manifestation of SCD, with a high stable 22% Hb F level.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Treatment of severe forms of sickle cell anemia with bone marrow allograft: French experience (15 cases). SFGM]. 833 53

In 44 episodes of acute chest syndrome of sickle cell disease occurring in 37 children, simple clinical severity score, duration of hospital stay, transfusion data, and alveolar-arterial oxygen gradient were analyzed as indicators of severity of disease. The alveolar-arterial oxygen gradient, measured during breathing of room air, proved, on multivariate analysis, to be the strongest predictor of both clinical severity and the need for blood transfusion.
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PMID:Alveolar-arterial oxygen gradient in acute chest syndrome of sickle cell disease. 834 26

Fourteen S/S children with severe SCD were transplanted with marrow from HLA identical siblings. All developed frequent (> 4/y) vasoocclusive crises (VOC) and recurrent acute chest syndrome episodes (n:10), osteitis (n:3), osteonecrosis (n:3), strokes (n:3) or frequent massive deglobulisation (n:2). Two children undergone splenectomy, 2 were chelated and 2 had erythroid allo-immunization. Ethnic origins were from various countries in Africa (n:10), North-Africa (n:3) or West Indies (n:1). At BMT, they were 2y 3m to 14y 9m old (mean:8y 7m). Donors were AS (n:11) or AA (n:3). At first, various conditioning regimens were used consisting of busulfan (BU) plus Cyclophosphamide (CY) at different doses: CY:200 mg/kg (n:12) or 260 mg/kg (n:2); BU:14 mg/kg (n:1), 16 mg/kg (n:9), > 16 mg/kg (n:4); 1 patient received also TLI and one other antithymoglobulin (ATG): 20 mg/kg. GVHD prophylaxis was CSA alone (n:4) or CSA plus short-term MTX (n:10). Median follow-up was 23 months (8 m. to 48 m.). All patients had an engraftment (d13 to d32) with a stable total chimerism in 10/14 patients who are cured. In the 4 others, partial chimerism was observed: one patient had a early and progressive rejection of his graft but is doing very well (28 m. follow-up) without any manifestation of SCD, with a high stable 22% Hb F level. One patient developed an aplastic anaemia 15 m after BMT: a second BMT was achieved 21 m after the first one with engraftment and total chimerism. Two patients have a relatively stable partial chimerism with still undergoing CSA therapy (11 m. and 23 m. follow-up).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Bone marrow transplantation (BMT) in 14 children with severe sickle cell disease (SCD): the French experience. GEGMO. 837 51


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