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Query: UMLS:C0002871 (anemia)
 52,094 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The Cooperative Study of Sickle Cell Disease reported that dactylitis, severe anemia, and leukocytosis in very young children with sickle cell disease (SCD) increased the risk of later adverse outcomes, including death, stroke, frequent pain, and recurrent acute chest syndrome. This model has not been validated in other cohorts. We evaluated its performance in the Dallas Newborn Cohort, a newborn inception cohort of children with SCD. We studied 168 children (55% male, 97% sickle cell anemia) with a mean follow-up of 7.1 years who provided 1188 patient-years of observation. Of the 23 (13.7%) subjects who experienced adverse events, 2 (1.2%) died, 14 (8.3%) had a stroke, 4 (2.4%) had frequent pain, and 3 (1.8%) had recurrent acute chest syndrome. No relationship existed between early clinical predictors and later adverse outcomes, with the possible exception of leukocyte count. Most subjects who experienced adverse events were predicted to be at low risk for those events. No subject who was predicted to be at high risk actually experienced an adverse outcome. The sensitivity of the model did not rise above 20% until specificity fell below 60%. We suggest that this model not be used as a criterion to initiate early interventions for SCD.
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PMID:Prediction of adverse outcomes in children with sickle cell anemia: a study of the Dallas Newborn Cohort. 1790 76

Described is a case of acute chest syndrome in a sickle-cell patient (hemoglobin SS) who also developed signs and symptoms of thrombotic thrombocytopenic purpura, including thrombocytopenia and hemolysis (anemia, elevated lactate dehydrogenase, presence of schistocytes, dark-colored plasma, and elevations in nucleated red blood cells). The ADAMTS13 activity level was normal. Discussed are the diagnosis and therapeutic management issues and the challenges of differentiating the vasoocclusive and hemolytic complications of sickling red blood cells from the thrombotic microangiopathy of thrombotic thrombocytopenic purpura.
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PMID:Thrombotic thrombocytopenic purpura and sickle cell crisis. 1884 Jun 31

A 10-year-old African American girl with sickle-cell anemia developed headaches and seizures associated with hypertension during hospitalization for a pulmonary abscess. Hypertension developed after multiple transfusions, associated with abnormally high hematocrit and headache. Magnetic resonance imaging was consistent with posterior leukoencephalopathy. Neurologic signs, hypertension, and high hematocrit resolved after erythrocytapheresis. Magnetic resonance imaging, 1 month after the episode, produced normal results. Because reversible posterior leukoencephalopathy syndrome was only described in sickle-cell anemia during severe acute chest syndrome, this report documents that milder illness can be associated with reversible posterior leukoencephalopathy syndrome in sickle-cell anemia, and also highlights subtle signs that may herald serious neurologic events in high-risk patients. Examination of the pathophysiology of reversible posterior leukoencephalopathy syndrome in the context of sickle-cell anemia suggests that patients with sickle-cell anemia and subtle neurologic signs should be treated with high vigilance.
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PMID:Reversible posterior leukoencephalopathy syndrome in sickle-cell anemia. 1930 44

Homozygous, sickle-cell disease (SCD) is responsible for acute complication, especially anaemic crisis and special situation such as acute chest syndrome, stroke and acute priapism. Pregnancy sickle-cell disease presents high risk for the mother and the fetus. In these indications, blood transfusion is the main therapy aiming to reduce anaemia in order to restore hemoglobin's rate or to increase normal Hb proportion. This study aims to assess the short-term efficiency of the red cell transfusion in SCD homozygous form. One hundred and twelve homozygous sickle-cell patients were enrolled in this prospective study: 59 females and 53 males, median age is 21,8 years (extremes: 2 and 45 years). These patients are mostly with very low income. Two groups of patients are included in this study. In the first group, patients present acute anemia crisis caused by infections disease (malaria, bacterial infections). In the second group (20 cases), SCD patients have particularly situations: pregnancy (10 cases); stroke (six cases); cardiac failure (two cases) and priapism (two cases). Transfusion treatment in first group is simple regimen. Transfusion of EC increased median Hb level at 2,9 g/dl (extremes: 1,1 and 4,7). In the second group of patients, 16 cases were transfused by manual partial exchange (1-3) and four patients received simple regimen of transfusion. Median Hb level was 3,1g/dl (extremes: 2,4-4,9 g/dl). HbS percentage reduction was after PTE between -30 and -66,8% (median: -52,6%). According to our diagnostic possibilities (blood serologic test), we have not found any contamination by HIV, HBV and HCV (virus).
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PMID:[Blood transfusion assessment to 112 homozygous sickle-cell disease patients in university hospital of Brazzaville]. 1936 4

Silent infarcts have been reported most commonly in school-aged children with homozygous sickle cell disease (SCD-SS) and are associated with neurocognitive deficits. However, the prevalence of silent infarcts in younger children with SCD-SS is not well defined. In this retrospective study, brain magnetic resonance imaging and angiography (MRI/A) studies performed before 6 years of age in a cohort of children with SCD-SS were analysed and the prevalence of abnormalities was calculated. Clinical and laboratory parameters were compared between the groups with and without silent infarcts. Sixty-eight of 96 children in the cohort had brain MRI/A performed prior to age 6 years. Of the 65 who were neurologically asymptomatic, 18 (27.7%, 95% CI 17.3-40.2%) had silent infarcts (mean age 3.7 +/- 1.1 years, range 1.3-5.9 years). Factors associated with silent infarcts included cerebral vessel stensosis by magnetic resonance angiography, lower rates of vaso-occlusive pain and acute chest syndrome and lower haemoglobin levels. The prevalence of silent infarcts in young children with SCD-SS is similar to that of older children and anaemia and severe vasculopathy may be risk factors.
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PMID:Silent infarcts in young children with sickle cell disease. 1950 Jan 5

Low steady state haemoglobin oxygen saturation in patients with sickle cell anaemia has been associated with the degree of anaemia and haemolysis. How much pulmonary dysfunction contributes to low saturation is not clear. In a prospective study of children and adolescents with sickle cell disease aged 3-20 years at steady state and matched controls, 52% of 391 patients versus 24% of 63 controls had steady state oxygen saturation <99% (P < 0.0001), 9% of patients versus no controls had saturation <95% (P = 0.008) and 8% of patients versus no controls had exercise-induced reduction in saturation > or =3%. Decreasing haemoglobin concentration (P < or = 0.001) and increasing haemolysis (P < or = 0.003) but not pulmonary function tests were independent predictors of both lower steady-state saturation and exercise-induced reduction in saturation. Neither history of stroke nor history of acute chest syndrome was significantly associated with lower steady-state oxygen saturation or exercise-induced reduction in saturation. Tricuspid regurgitation velocity was higher in patients with lower steady state haemoglobin oxygen saturation (P = 0.003) and with greater decline in oxygen saturation during the six-minute walk (P = 0.022). In conclusion, lower haemoglobin oxygen saturation is independently associated with increasing degrees of anaemia and haemolysis but not pulmonary function abnormalities among children and adolescents with sickle cell disease.
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PMID:Prospective evaluation of haemoglobin oxygen saturation at rest and after exercise in paediatric sickle cell disease patients. 1969 21

Although cardiopulmonary disease is associated with decreased functional capacity among adults with sickle cell disease (SCD), its impact on functional capacity in children with SCD is unknown. We evaluated 6-min walk (6MW) distance in 77 children and young adults with SCD undergoing screening for cardiopulmonary disease. Of 30 subjects who also underwent cardiopulmonary exercise testing, we found evidence for decreased exercise capacity in a significant proportion. Exercise capacity was related to baseline degree of anemia and was significantly lower in subjects with a history of recurrent acute chest syndrome. We found that 6MW distance adjusted for weight and body surface area was shorter in subjects with restrictive lung disease but that only 6MW adjusted for weight remained significantly shorter when we controlled for baseline hemoglobin. Exercise capacity was not significantly different in subjects with and without cardiopulmonary disease. We conclude that restrictive lung disease is associated with shorter 6MW distances in children and young adults with SCD, but that variables associated with decreased exercise capacity, other than anemia, remain unclear. Our study underscores the importance of further delineating the direct pathophysiologic processes that contribute to decreased exercise capacity observed among individuals with SCD and cardiopulmonary disease.
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PMID:Functional capacity in children and young adults with sickle cell disease undergoing evaluation for cardiopulmonary disease. 1970 33

The substitution of one amino acid in the hemoglobin molecule results in sickle hemoglobin. As a result, RBCs sickle in low oxygen states causing occlusion of blood vessels, increased viscosity, and inflammation. These RBCs are prematurely removed from the circulation, resulting in a chronic hemolytic anemia. With newborn screening and early treatment, the death rate among children with SCD has declined. In addition, a variety of treatments are being introduced to help manage the various manifestations of disease. Transfusion, simple or exchange, is a mainstay of therapy, since it reduces the amount of Hgb S in circulation and suppresses erythropoiesis. Transfusion is indicated for symptomatic anemia and specifically to prevent stroke (first or recurrent), during acute stroke, and for acute chest syndrome. Unfortunately, transfusion carries risks for infectious disease transmission, as well as immunologic and inflammatory sequelae. For patients with SCD who may be chronically transfused, iron overload occurs frequently. In addition, due to differences in RBC antigens between donors and recipients, these patients are at increased risk for development of RBC alloantibodies, which can complicate further transfusion. It is, therefore, important to prevent alloimmunization by transfusing leukoreduced RBCs that match the patient for the C, E, and K1 antigens. Human progenitor cell (from bone marrow, peripheral blood stem cells, or umbilical blood) transplant can cure the disease, and is used for patients with severe disease for whom conventional therapy may not be effective.
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PMID:Sickle cell disease: a review. 1992 23

In sickle cell disease transfusions improve blood flow by reducing the proportion of red cells capable of forming sickle hemoglobin polymer. This limits hemolysis and the endothelial damage that result from high proportions of sickle polymer-containing red cells. Additionally, transfusions are used to increase blood oxygen carrying capacity in sickle cell patients with severe chronic anemia or with severe anemic episodes. Transfusion is well-defined as prophylaxis (stroke) and as therapy (acute chest syndrome and stroke) for major complications of sickle cell disease and has been instituted, based on less conclusive data, for a range of additional complications, such as priapism, vaso-occlusive crises, leg ulcers, pulmonary hypertension, and during complicated pregnancies. The major and unavoidable complication of transfusions in sickle cell disease is iron overload. This paper provides an overview of normal iron metabolism, iron overload in transfused patients with sickle cell disease, patterns of end organ damage, diagnosis, treatment, and prevention of iron overload.
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PMID:Iron overload in sickle cell disease. 2049 Mar 52

Acute chest syndrome describes new respiratory symptoms and findings, often severe and progressive, in a child with sickle cell disease and a new pulmonary infiltrate. It may be community-acquired or arise in children hospitalized for pain or other complications. Recognized etiologies include infection, most commonly with atypical bacteria, and pulmonary fat embolism (PFE); the cause is often obscure and may be multifactorial. Initiation of therapy should be based on clinical findings. Management includes macrolide antibiotics, supplemental oxygen, modest hydration and often simple transfusion. Partial exchange transfusion should be reserved for children with only mild anemia (Hb > 9 g/dL) but deteriorating respiratory status. Therapy with corticosteroids may be of value; safety, efficacy and optimal dosing strategy need prospective appraisal in a clinical trial. On recovery, treatment with hydroxyurea should be discussed to reduce the likelihood of recurrent episodes.
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PMID:How I treat acute chest syndrome in children with sickle cell disease. 2140 23


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